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Sample records for bilateral perisylvian syndrome

  1. Bilateral perisylvian syndrome not related to malformations: report of two cases.

    PubMed

    Grasel, R P; Carvalho Neto, A; Bruck, I; Antoniuk, S A

    1996-06-01

    In this case report we present the neuroimaging findings and clinical features of two patients with a bilateral perisylvian syndrome not related to malformations, but probably to ischemic etiology. Evaluations including history, general and neurologic examinations, electroencephalograms, and imaging data were reviewed as recent literature about the subject. PMID:8984989

  2. Anterior corpus callosotomy combined with anterior temporal resection with amygdalohippocampectomy: outcome in a patient with congenital bilateral perisylvian syndrome.

    PubMed

    Junming, Zhu; Yuanyuan, Zhao; Fang, Feng; Weiming, Fu; Ryan, Hays; Jianmin, Zhang; Li, Feng; Xiao, Jin; Shuda, Chen

    2014-01-01

    Congenital bilateral perisylvian syndrome (CBPS) is characterized by epilepsy, cognitive deficits, pseudobulbar palsy and diplegia of the facial, pharyngeal and masticatory muscles. Epilepsy has been described in nearly 90% of affected patients. The epilepsy is usually severe and pharmacoresistant in about 55 percent of CBPS patients. Until now, only 12 cases of surgical treatment on CBPS have been reported; the surgical treatment is usually corpus callosotomy. In this paper, we describe a previously unreported combination of anterior corpus callosotomy plus anterior temporal lobectomy with amygdalohippocampectomy for a patient with CBPS, resulting in a satisfactory clinical outcome. Based on this case, we suggest that palliative focal resective surgery combined with anterior corpus callosotomy should be considered when a predominance of the epileptiform discharges suggests focal onset in patients with CBPS. Meanwhile, the clinical decision to adopt this combination surgery must be based on a thorough pre-surgical evaluation, and should take into account the clinical, radiological, and EEG features. PMID:24535795

  3. Connectivity between perisylvian and bilateral basal temporal cortices.

    PubMed

    Koubeissi, Mohamad Z; Lesser, Ronald P; Sinai, Alon; Gaillard, William D; Franaszczuk, Piotr J; Crone, Nathan E

    2012-04-01

    Language processing requires the orchestrated action of different neuronal populations, and some studies suggest that the role of the basal temporal (BT) cortex in language processing is bilaterally distributed. Our aim was to demonstrate connectivity between perisylvian cortex and both BT areas. We recorded corticocortical evoked potentials (CCEPs) in 8 patients with subdural electrodes implanted for surgical evaluation of intractable epilepsy. Four patients had subdural grids over dominant perisylvian and BT areas, and 4 had electrode strips over both BT areas and left posterior superior temporal gyrus (LPSTG). After electrocortical mapping, patients with grids had 1-Hz stimulation of language areas. Patients with strips did not undergo mapping but had 1-Hz stimulation of the LPSTG. Posterior language area stimulation elicited CCEPs in ipsilateral BT cortex in 3/4 patients with left hemispheric grids. CCEPs were recorded in bilateral BT cortices in 3/4 patients with strips upon stimulation of the LPSTG, and in the LPSTG in the fourth patient upon stimulation of either BT area. This is the first in vivo demonstration of connectivity between LPSTG and both BT cortices. The role of BT cortex in language processing may be bilaterally distributed and related to linking visual information with phonological representations stored in the LPSTG. PMID:21715651

  4. Connectivity between Perisylvian and Bilateral Basal Temporal Cortices

    PubMed Central

    Lesser, Ronald P.; Sinai, Alon; Gaillard, William D.; Franaszczuk, Piotr J.; Crone, Nathan E.

    2012-01-01

    Language processing requires the orchestrated action of different neuronal populations, and some studies suggest that the role of the basal temporal (BT) cortex in language processing is bilaterally distributed. Our aim was to demonstrate connectivity between perisylvian cortex and both BT areas. We recorded corticocortical evoked potentials (CCEPs) in 8 patients with subdural electrodes implanted for surgical evaluation of intractable epilepsy. Four patients had subdural grids over dominant perisylvian and BT areas, and 4 had electrode strips over both BT areas and left posterior superior temporal gyrus (LPSTG). After electrocortical mapping, patients with grids had 1-Hz stimulation of language areas. Patients with strips did not undergo mapping but had 1-Hz stimulation of the LPSTG. Posterior language area stimulation elicited CCEPs in ipsilateral BT cortex in 3/4 patients with left hemispheric grids. CCEPs were recorded in bilateral BT cortices in 3/4 patients with strips upon stimulation of the LPSTG, and in the LPSTG in the fourth patient upon stimulation of either BT area. This is the first in vivo demonstration of connectivity between LPSTG and both BT cortices. The role of BT cortex in language processing may be bilaterally distributed and related to linking visual information with phonological representations stored in the LPSTG. PMID:21715651

  5. A locus for bilateral perisylvian polymicrogyria maps to Xq28.

    PubMed

    Villard, Laurent; Nguyen, Karine; Cardoso, Carlos; Martin, Christa Lese; Weiss, Ann M; Sifry-Platt, Mara; Grix, Arthur W; Graham, John M; Winter, Robin M; Leventer, Richard J; Dobyns, William B

    2002-04-01

    Polymicrogyria (PMG) is one of a large group of human cortical malformations that collectively account for a significant percentage of patients with epilepsy, congenital neurological deficits, and intellectual disability. PMG is characterized by an excess of small gyri and abnormal cortical lamination. The most common distribution is bilateral, symmetrical, and maximal, in the region surrounding the sylvian fissures, and is known as "bilateral perisylvian polymicrogyria" (BPP). Most cases are sporadic, although several families have been observed with multiple affected members, usually following an X-linked inheritance pattern. Here we report the first genetic locus for BPP mapped by linkage analysis in five families. Linkage places the critical region for BPP at Xq28 (LOD score 3.08 in Xq28, distal to DXS8103 by multipoint analysis). We suggest that this region contains a gene that is necessary for correct neuronal organization and that the identification of this gene will both enhance our understanding of normal cortical development and accelerate the identification of other genes responsible for PMG. PMID:11822025

  6. Congenital unilateral perisylvian syndrome: radiological basis and clinical correlations.

    PubMed Central

    Sébire, G; Husson, B; Dusser, A; Navelet, Y; Tardieu, M; Landrieu, P

    1996-01-01

    DESIGN--Advances in neuroimaging have allowed correlations between radiological patterns and clinical features of brain malformations. This paper reports clinical, prognosis, and electroencephalographic features of six children with a previously unrecognised neuroimaging picture of unilateral widening and verticalisation of the sylvian fossa associated with an abnormal ipsilateral perisylvian cortex. RESULTS--All children had reduced hemisphere size and thalamostriatal hypoplasia ipsilateral to the cleft and hemiplegia. Cognitive development was mostly impaired. Epilepsy occurred in two patients and was mainly characterised by partial seizures. Studies with EEG showed hemispheric slowing of background activity homolateral to the perisylvian dysplasia. Occurrence of the malformation among their siblings was not found. CONCLUSION--Similar brain malformations occasionally reported in older patients confirm the clinical picture, sporadic occurrence, and prognosis found, allowing the validation of a unilateral perisylvian syndrome. Images PMID:8676160

  7. Bilateral Subclavian Steal Syndrome

    PubMed Central

    Amini, Reza; Gornik, Heather L.; Gilbert, Leslie; Whitelaw, Sue; Shishehbor, Mehdi

    2011-01-01

    Bilateral subclavian steal syndrome is a rare condition. It is usually due to reversal of vertebral blood flow in the setting of bilateral proximal subclavian or left subclavian plus innominate artery severe stenosis or occlusion. This finding may cause cerebral ischemia related to upper extremities exercise. We report a case of bilateral subclavian steal secondary to total occlusion of the innominate and left subclavian arteries in a patient who presented with cardiomyopathy and flow reversal in the right carotid and bilateral vertebral arteries. PMID:24804108

  8. Anatomical predictors of aphasia recovery: a tractography study of bilateral perisylvian language networks.

    PubMed

    Forkel, Stephanie J; Thiebaut de Schotten, Michel; Dell'Acqua, Flavio; Kalra, Lalit; Murphy, Declan G M; Williams, Steven C R; Catani, Marco

    2014-07-01

    Stroke-induced aphasia is associated with adverse effects on quality of life and the ability to return to work. For patients and clinicians the possibility of relying on valid predictors of recovery is an important asset in the clinical management of stroke-related impairment. Age, level of education, type and severity of initial symptoms are established predictors of recovery. However, anatomical predictors are still poorly understood. In this prospective longitudinal study, we intended to assess anatomical predictors of recovery derived from diffusion tractography of the perisylvian language networks. Our study focused on the arcuate fasciculus, a language pathway composed of three segments connecting Wernicke's to Broca's region (i.e. long segment), Wernicke's to Geschwind's region (i.e. posterior segment) and Broca's to Geschwind's region (i.e. anterior segment). In our study we were particularly interested in understanding how lateralization of the arcuate fasciculus impacts on severity of symptoms and their recovery. Sixteen patients (10 males; mean age 60 ± 17 years, range 28-87 years) underwent post stroke language assessment with the Revised Western Aphasia Battery and neuroimaging scanning within a fortnight from symptoms onset. Language assessment was repeated at 6 months. Backward elimination analysis identified a subset of predictor variables (age, sex, lesion size) to be introduced to further regression analyses. A hierarchical regression was conducted with the longitudinal aphasia severity as the dependent variable. The first model included the subset of variables as previously defined. The second model additionally introduced the left and right arcuate fasciculus (separate analysis for each segment). Lesion size was identified as the only independent predictor of longitudinal aphasia severity in the left hemisphere [beta = -0.630, t(-3.129), P = 0.011]. For the right hemisphere, age [beta = -0.678, t(-3.087), P = 0.010] and volume of the long segment of the arcuate fasciculus [beta = 0.730, t(2.732), P = 0.020] were predictors of longitudinal aphasia severity. Adding the volume of the right long segment to the first-level model increased the overall predictive power of the model from 28% to 57% [F(1,11) = 7.46, P = 0.02]. These findings suggest that different predictors of recovery are at play in the left and right hemisphere. The right hemisphere language network seems to be important in aphasia recovery after left hemispheric stroke. PMID:24951631

  9. Ischemic bilateral opercular syndrome.

    PubMed

    Milanlioglu, Aysel; Ayd?n, Mehmet Nuri; Gökgül, Alper; Hamamc?, Mehmet; Erkuzu, Mehmet Atilla; Tombul, Temel

    2013-01-01

    Opercular syndrome, also known as Foix-Chavany-Marie syndrome, is a paralysis of the facial, pharyngeal, masticatory, tongue, laryngeal, and brachial muscles. It is a rare cortical form of pseudobulbar palsies caused by vascular insults to bilateral operculum. Its clinical presentations include anarthria, weakness of voluntary muscles involving face, tongue, pharynx, larynx, and masticatory muscles. However, autonomic reflexes and emotional activities of these structures are preserved. In the present case, an 81-year-old male presented with acute onset of anarthria with difficulties in chewing, speaking, and swallowing that was diagnosed with opercular syndrome. PMID:23476665

  10. Ischemic Bilateral Opercular Syndrome

    PubMed Central

    Milanlioglu, Aysel; Aydın, Mehmet Nuri; Gökgül, Alper; Hamamcı, Mehmet; Erkuzu, Mehmet Atilla; Tombul, Temel

    2013-01-01

    Opercular syndrome, also known as Foix-Chavany-Marie syndrome, is a paralysis of the facial, pharyngeal, masticatory, tongue, laryngeal, and brachial muscles. It is a rare cortical form of pseudobulbar palsies caused by vascular insults to bilateral operculum. Its clinical presentations include anarthria, weakness of voluntary muscles involving face, tongue, pharynx, larynx, and masticatory muscles. However, autonomic reflexes and emotional activities of these structures are preserved. In the present case, an 81-year-old male presented with acute onset of anarthria with difficulties in chewing, speaking, and swallowing that was diagnosed with opercular syndrome. PMID:23476665

  11. Bilateral Nevus Comedonicus Syndrome

    PubMed Central

    Ito, Takamichi; Mitamura, Yasutaka; Tsuji, Yayoi; Harada, Kayo; Urabe, Kazunori

    2013-01-01

    Nevus comedonicus is an uncommon skin abnormality characterized by an aggregation of dilated follicular orifices filled with keratinous material. Nevus comedonicus is occasionally complicated with other conditions including cataracts, skeletal defects, central nervous system abnormalities or other extra-cutaneous diseases (nevus comedonicus syndrome). Although most cases of nevus comedonicus occur unilaterally on the face, neck and chest, the lesions occasionally show a bilateral distribution (bilateral nevus comedonicus). We report here an unusual case of bilaterally disseminated nevus comedonicus with various systemic complications. A 62-year-old Japanese man presented with a 50-year history of numerous keratotic papules and comedo-like lesions, which gradually worsened with time. Physical examination revealed that the papules were skin-colored and 1–4 mm in diameter. Some papules had dark-black keratinous materials on their surface, giving them a comedo-like appearance. The lesions were located predominantly on the face, head, neck and trunk with symmetric distribution, following Blaschko’s lines. The patient’s past medical history was noteworthy; he had undergone surgical treatments for thyroid cancer, pneumothorax and schwannoma in the cauda equina. He also suffered from scoliosis, cervical spondylosis and atrial fibrillation. Histopathologic examination revealed dilated and invaginated follicular structures filled with lamellar keratin, compatible with nevus comedonicus. Our patient’s case did not fit with any previously reported diseases, and we thought a diagnosis of “bilateral nevus comedonicus syndrome” was the most appropriate for our patient’s condition. He was treated with topical retinoic acid and activated vitamin D3 ointment for 3 months each, but the lesions remained unchanged. PMID:24031154

  12. Congenital polymicrogyria including the perisylvian region in early childhood.

    PubMed

    Takano, Tomoyuki; Matsuwake, Kumiko; Yoshioka, Seiichirou; Takeuchi, Yoshihiro

    2010-03-01

    Six pediatric cases including four infants with congenital polymicrogyria including the perisylvian region are presented herein. Their clinical features were analyzed and compared with patients suffering from congenital bilateral perisylvian syndrome (CBPS). Two specific abnormalities were diagnosed as accompanying disorders in two cases, namely Kabuki syndrome and Peters' anomaly. In the other four cases, the pathogenetic etiology was not elucidated. Subtle symptoms, such as choking and drooling became detectable in one case each, and expressive language development was delayed in two patients. A developmental delay became apparent in five cases during the follow-up period, and epilepsy was observed in one patient with onset at 12 years of age. Our results indicate that the presence of perisylvian polymicrogyria may not always result in the development of oropharyngoglossal dysfunction or dysarthria, although most patients tend to gradually show the onset of developmental disorders. To support cognitive and psychosocial development, an early integrated approach, including not only conventional speech and language therapy, but also various communication methods is essential for patients with congenital polymicrogyria including the perisylvian region. PMID:20201970

  13. Lemierre syndrome causing bilateral cavernous sinus thrombosis.

    PubMed

    Miller, Brooke; Khalifa, Yousuf; Feldon, Steven E; Friedman, Deborah I

    2012-12-01

    Lemierre syndrome is an uncommon septic thrombophlebitis of the veins of the head and the neck usually occurring after a severe oropharyngeal infection. Although subsequent septic emboli most commonly affect distant sites, such as the lungs and joints, the authors present a case of Lemierre syndrome causing bilateral cavernous sinus syndrome. PMID:22868639

  14. [Bilateral acute depigmentation of the iris syndrome].

    PubMed

    Portmann, A; Gueudry, J; Siahmed, K; Muraine, M

    2011-05-01

    Bilateral acute depigmentation of the iris syndrome (BADI syndrome) is a new clinical entity. Young females from 20 to 45 years of age are most commonly affected. It is characterized by bilateral nontransilluminating depigmentation of the iris stroma. During the acute phase, this clinical entity also combines with red painful eye, pigmentation of the trabecular meshwork, anterior chamber flare, circulating pigment, and pigmented deposit on the endothelium cornea. At the acute stage, the symptoms are controlled with topical corticosteroid treatment. The prognosis is good. We report a 41-year-old woman presenting with BADI syndrome. PMID:21531477

  15. Bilateral peroneal compartment syndrome after horse riding.

    PubMed

    Naidu, Krishant S; Chin, Terence; Harris, Christain; Talbot, Simon

    2009-09-01

    A healthy 20-year-old woman developed acute ischemia of the lateral compartment of both calves shortly after a 30-minute horse ride. On one side, she developed compartment syndrome with resultant complete myonecrosis of the compartment, whereas on the other side, there was spontaneous resolution. To our knowledge, this is the first report of bilateral lateral compartment ischemia after horse riding. Atraumatic compartment syndrome is a rare entity and is often missed at initial presentation. We discuss aspects of her management together with a review of the literature. Late fasciotomy and exploration may be beneficial in decompressing the deep peroneal nerve in peroneal compartment syndrome. Awareness of atraumatic compartment syndrome is important in any case of limb pain and swelling. PMID:19683135

  16. Bilateral ovarian fibroma associated with Gorlin syndrome

    PubMed Central

    Aram, Shahnaz; Moghaddam, Noushin Afshar

    2009-01-01

    Gorlin syndrome (GS), also known as nevoid basal cell carcinoma syndrome (NBCCS), is a rare inherited multisystem disorder. This paper presents a 22-years-old Iranian woman with this syndrome whose past history was multiple keratocysts of maxillary bone. She was referred to gynecology clinic with the chief complaint of irregular menses and vaginal spotting. On examination, frontal bossing and hypertelorism were detected. Physical examination of genitalia disclosed bilateral adnexal masses. Pelvic ultrasound showed two solid, echogenous and calcified masses measuring 100*50*10 & 60*50*45 mm in the left and right ovaries, respectively. The patient underwent right oophorectomy and ovarian mass resection with preservation of intact ovarian tissue on the left side. On frozen and permanent histological sections, bilateral and calcified ovarian fibromas were diagnosed. Surprisingly, during the last follow-up one year after the surgery, we found that our patient was expecting a baby. It can be concluded that in the presence of bilateral and calcified ovarian fibromas, the possibility of GS should be considered. Accurate diagnosis is only possible with close attention to the familial and past medical history and physical examination. In these patients, careful follow up for detecting malignancies and other complications is highly recommended. PMID:21772861

  17. Bilateral subclavian steal syndrome with vertigo.

    PubMed

    Yamanaka, Toshiaki; Sawai, Yachiyo; Hosoi, Hiroshi

    2014-06-01

    Subclavian steal syndrome (SSS) is usually caused by unilateral subclavian artery (SA) occlusion, and bilateral SSS is very rare. Takayasu's arteritis (TA) is a chronic granulomatous form of vasculitis that affects the SA, most commonly in women aged 15-40 years. We report a rare case of bilateral SSS due to TA in a 52-year-old woman, who exhibited severe vertigo. Although her blood pressure was within the normal range and did not differ between her arms, Doppler ultrasonography revealed low antegrade blood flow in the right SA and retrograde flow in the left SA. Computed tomography angiography demonstrated complete obstruction of the bilateral SA proximal to the vertebral artery origin. The more marked decrease in the blood flow of the vertebrobasilar artery experienced in bilateral SSS compared with unilateral SSS is considered to have caused the severe rotatory vertigo in the present patient. Since the vascular conditions of TA progressively deteriorate, delayed diagnosis and treatment could result in poor outcomes and unfavorable prognosis. We suggest that SSS with TA might require an early detection and treatment as well as careful follow-up for preventing vertigo and other neurological deficits in the vertebrobasilar arterial region. PMID:24206829

  18. Anton's Syndrome due to Bilateral Ischemic Occipital Lobe Strokes

    PubMed Central

    Zukić, Sanela; Sinanović, Osman; Hodžić, Renata; Mujagić, Svjetlana; Smajlović, Edina

    2014-01-01

    We present a case of a patient with Anton's syndrome (i.e., visual anosognosia with confabulations), who developed bilateral occipital lobe infarct. Bilateral occipital brain damage results in blindness, and patients start to confabulate to fill in the missing sensory input. In addition, the patient occasionally becomes agitated and talks to himself, which indicates that, besides Anton's syndrome, he might have had Charles Bonnet syndrome, characterized by both visual loss and hallucinations. Anton syndrome, is not so frequent condition and is most commonly caused by ischemic stroke. In this particular case, the patient had successive bilateral occipital ischemia as a result of massive stenoses of head and neck arteries. PMID:25530893

  19. CLINICAL OUTCOME IN BILATERAL STURGE-WEBER SYNDROME

    PubMed Central

    Alkonyi, Bálint; Chugani, Harry T.; Karia, Samir; Behen, Michael E.; Juhász, Csaba

    2011-01-01

    Approximately 15% of patients with Sturge-Weber syndrome have bilateral intracranial involvement and the prognosis of these patients is believed to be particularly unfavorable. We reviewed the clinical and neuroimaging features of patients with Sturge-Weber syndrome and bilateral intracranial involvement. Seizure variables, presence of hemiparesis and degree of developmental impairment at last follow-up were compared to imaging abnormalities. Out of 110 Sturge-Weber syndrome patients studied, 14 had bilateral brain involvement, which always showed an asymmetric pattern on glucose metabolism positron emission tomography. Although most patients had frequent seizures initially, associated with frontal hypometabolism on positron emission tomography, six patients (43%) had achieved good seizure-control during follow-up. Bilateral frontal hypometabolism was associated with severe developmental impairment; two children with bitemporal hypometabolism showed autistic features. Hemiparesis was associated with superior frontal (motor cortex) hypometabolism. Three patients had resective surgery, resulting in improved seizure control and/or developmental outcome. The severity of neurological complications and clinical course depend highly on the extent of cortical dysfunction in bilateral Sturge-Weber syndrome; bilateral frontal and temporal hypometabolism is associated with poor developmental outcome. Nevertheless, good seizure control and only mild/moderate developmental impairment can be achieved in about half of bilateral Sturge-Weber syndrome patients, with or without resective surgery. PMID:21555056

  20. Leg Weakness Caused by Bilateral Piriformis Syndrome: A Case Report.

    PubMed

    Moon, Hee Bong; Nam, Ki Yeun; Kwon, Bum Sun; Park, Jin Woo; Ryu, Gi Hyeong; Lee, Ho Jun; Kim, Chang Jae

    2015-12-01

    Piriformis syndrome (PS) is an uncommon neuromuscular disorder caused by the piriformis muscle (PM) compressing the sciatic nerve (SN). The main symptom of PS is sciatica, which worsens with certain triggering conditions. Because the pathophysiology is poorly understood, there are no definite diagnostic and therapeutic choices for PS. This case report presents a young woman who mainly complained of bilateral leg weakness. Electromyography revealed bilateral sciatic neuropathy and magnetic resonance imaging confirmed structural lesions causing entrapment of the bilateral SNs. After a laborious diagnosis of bilateral PS, she underwent PM releasing surgery. Few PS cases present with bilateral symptoms and leg weakness. Therefore, in such cases, a high level of suspicion is necessary for accurate and prompt diagnosis and treatment. PMID:26798622

  1. Leg Weakness Caused by Bilateral Piriformis Syndrome: A Case Report

    PubMed Central

    Moon, Hee Bong; Kwon, Bum Sun; Park, Jin Woo; Ryu, Gi Hyeong; Lee, Ho Jun; Kim, Chang Jae

    2015-01-01

    Piriformis syndrome (PS) is an uncommon neuromuscular disorder caused by the piriformis muscle (PM) compressing the sciatic nerve (SN). The main symptom of PS is sciatica, which worsens with certain triggering conditions. Because the pathophysiology is poorly understood, there are no definite diagnostic and therapeutic choices for PS. This case report presents a young woman who mainly complained of bilateral leg weakness. Electromyography revealed bilateral sciatic neuropathy and magnetic resonance imaging confirmed structural lesions causing entrapment of the bilateral SNs. After a laborious diagnosis of bilateral PS, she underwent PM releasing surgery. Few PS cases present with bilateral symptoms and leg weakness. Therefore, in such cases, a high level of suspicion is necessary for accurate and prompt diagnosis and treatment. PMID:26798622

  2. Catastrophic Antiphospholipid Syndrome Presenting as Bilateral Central Retinal Artery Occlusions

    PubMed Central

    Saraf, Steven S.; Patel, Yogin P.; Desai, Ankit; Desai, Uday R.

    2015-01-01

    A previously healthy 22-year-old African American woman presented with bilateral vision loss associated with headache. Her ocular examination was significant for bilateral retinal arterial “boxcarring,” retinal whitening, retinal hemorrhages, and cherry red spots. She was diagnosed with bilateral central retinal artery occlusions and was hospitalized due to concomitant diagnosis of stroke and hypercoagulable state. She was also found to be in heart failure and kidney failure. Rheumatology was consulted and she was diagnosed with catastrophic antiphospholipid syndrome in association with systemic lupus erythematosus. Approximately 7 months after presentation, the patient's vision improved and remained stable at 20/200 and 20/80. PMID:25722904

  3. Bilateral Renal Dysplasia, Nephroblastomatosis, and Bronchial Stenosis. A New Syndrome?

    PubMed Central

    Rodriguez, Maria Matilde; Correa-Medina, Mayrin; Whittington, Elizabeth E.

    2015-01-01

    Bilateral nephroblastomatosis (NB) is an uncommon renal anomaly characterized by multiple confluent nephrogenic rests scattered through both kidneys, with only a limited number of cases reported in the medical literature. Some of these children may have associated either Perlman or Beckwith–Wiedemann syndrome and others do not demonstrate syndromic features. We report a full-term boy with anteverted nose, bilateral bronchial stenosis due to lack of cartilage, bilateral obstructive renal dysplasia and NB with glomeruloid features. The infant had visceromegaly, but neither gigantism nor hemihypertrophy. Immunohistochemistry for PAX2 (Paired box gene-2) and WT-1 (Wilms Tumor 1) were strongly positive in the areas of NB. GLEPP-1 (Glomerular Epithelial Protein) did not stain the areas of NB with a glomeruloid appearance, but was positive in the renal glomeruli as expected. We found neither associated bronchial stenosis nor the histology of NB resembling giant glomeruli in any of the reported cases of NB. PMID:25871299

  4. Bilateral renal dysplasia, nephroblastomatosis, and bronchial stenosis. A new syndrome?

    PubMed

    Rodriguez, Maria Matilde; Correa-Medina, Mayrin; Whittington, Elizabeth E

    2015-06-01

    Bilateral nephroblastomatosis (NB) is an uncommon renal anomaly characterized by multiple confluent nephrogenic rests scattered through both kidneys, with only a limited number of cases reported in the medical literature. Some of these children may have associated either Perlman or Beckwith-Wiedemann syndrome and others do not demonstrate syndromic features. We report a full-term boy with anteverted nose, bilateral bronchial stenosis due to lack of cartilage, bilateral obstructive renal dysplasia and NB with glomeruloid features. The infant had visceromegaly, but neither gigantism nor hemihypertrophy. Immunohistochemistry for PAX2 (Paired box gene-2) and WT-1 (Wilms Tumor 1) were strongly positive in the areas of NB. GLEPP-1 (Glomerular Epithelial Protein) did not stain the areas of NB with a glomeruloid appearance, but was positive in the renal glomeruli as expected. We found neither associated bronchial stenosis nor the histology of NB resembling giant glomeruli in any of the reported cases of NB. PMID:25871299

  5. Ehlers-Danlos Syndrome Type IV with Bilateral Pneumothorax.

    PubMed

    Nakagawa, Hiroaki; Wada, Hiroshi; Hajiro, Takashi; Nagao, Taishi; Ogawa, Emiko; Hatamochi, Atsushi; Tanaka, Toshihiro; Nakano, Yasutaka

    2015-01-01

    A 17-year-old teen was hospitalized with bilateral pneumothorax. After the bilateral lungs were expanded using catheter tubes, he fully recovered and he was discharged from our hospital. He had a history of colon perforation. Ehlers-Danlos syndrome (EDS) was suspected due to the combination of colon perforation and pneumothorax, and EDS type IV was confirmed after a genetic study identified a c.1511g>a mutation in the COL3A1 gene. This is the first report of bilateral pneumothorax caused by EDS type IV. Clinicians should consider EDS type IV in the differential diagnosis for bilateral pneumothorax in conjunction with distinct previous histories and radiological findings. PMID:26666608

  6. Mounier-Kuhn syndrome and bilateral vocal cord paralysis.

    PubMed

    Dincer, H Erhan; Holweger, Joshua D

    2012-07-01

    Mounier-Kuhn syndrome is a rare disorder of unknown cause that is characterized by atrophy of the elastic and smooth muscle of the tracheobronchial tree leading to tracheobronchomegaly and bronchiectasis. The syndrome is likely underdiagnosed, because the patients usually present with common respiratory symptoms such as productive cough and usually labeled as chronic obstructive pulmonary disease. Diagnosis is established on the basis of radiologic findings. Association with bilateral vocal cord paralysis has not been described. Treatment is mainly supportive. Symptomatic patients may require endobronchial stenting if airway collapse is encountered. Here, we described a patient who presented with hoarseness and pneumonia. Further studies confirmed the diagnosis of Mounier-Kuhn syndrome with bilateral vocal cord paralysis. PMID:23207474

  7. Bilateral adrenalectomy for Cushing's syndrome: Pros and cons

    PubMed Central

    Prajapati, O. P.; Verma, A. K.; Mishra, A.; Agarwal, G.; Agarwal, A.; Mishra, S. K.

    2015-01-01

    Aim: To assess the outcome of patients undergoing bilateral adrenalectomy for Cushing's syndrome (CS). Methods: All patients who underwent bilateral adrenalectomy for CS at the Department of Endocrine Surgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences hospital between 1991 and 2013 were included. Medical records were reviewed to obtain patient characteristics and follow-up data. Results: Twenty-seven patients were studied. Mean age was 28.74 ± 12.95 years (range 9–60), male:female ratio was 1.7:1. About half that is, 48.19% were of Cushing's disease (failed trans-sphenoidal surgery [TSS]), 37.04% were of ectopic CS (ECS), and 14.81% were of CS due to bilateral adrenal pathology. Median follow-up period was 80.5 months. Before surgery, 74.1% patients had body mass index > which after surgery declined to <25 in 75% of them. Hypertension was present in 85.2% and after surgery resolved in 40%. Diabetes mellitus was present in 44.4% and after surgery resolved in 33% of them. Hirsutism and proximal muscle weakness were present in 55.6% and 70.4% patients, respectively, and after surgery improved markedly in all patients. Adrenal crisis developed in 36.3% and Nelson's syndrome in 41.7% patients during follow-up. Three patients died in perioperative period while three succumbed to the disease during follow-up. Two patients developed recurrence of endogenous cortisol production during the follow-up period. Conclusions: Bilateral adrenalectomy is a valid treatment option for palliating severe symptoms in Pituitary Cushing's with failed TSS and unlocalized ECS but the procedure is curative for CS due to bilateral adrenal disease. Overall morbidity and mortality is higher than other endocrine operations. Co-morbidities tend to be more severe and are a risk factor for mortality during the time patient survives. PMID:26693437

  8. Bilateral pial synangiosis in a child with PHACE syndrome.

    PubMed

    Jack, Andrew S; Chow, Michael M; Fiorillo, Loretta; Chibuk, Thea; Yager, Jerome Y; Mehta, Vivek

    2016-01-01

    The acronym PHACE has been used to denote a constellation of abnormalities: posterior fossa anomalies, facial hemangiomas, arterial anomalies, cardiac anomalies, and eye abnormalities. Approximately 30% of patients with large facial hemangiomas have PHACE syndrome, with the vast majority having intracranial arteriopathy. Few reports characterize neurological deterioration from this intracranial arteriopathy, and even fewer report successful treatment thereof. The authors report on a case of a child with PHACE syndrome who presented with an ischemic stroke from a progressive intracranial arteriopathy and describe her successful treatment with bilateral pial synangiosis. An 8-month old girl diagnosed with PHACE syndrome was found to have bilateral internal carotid artery stenosis. Although initially asymptomatic, a few months after diagnosis she suffered a right frontal and parietal stroke. MRI and cerebral angiography investigations demonstrated progressive intracranial arterial stenosis and occlusion. The patient then underwent indirect cerebral revascularization surgery. At 2-year follow-up, she exhibited clinical improvement with persistent speech and motor developmental delay. Follow-up MRI and cerebral angiography showed no new ischemic events and robust extensive vascular collateralization from surgery. PHACE syndrome is an uncommon disease, and affected patients often have cerebral arteriopathy. Although the underlying natural history of cerebral arteriopathy in PHACE remains unclear, cerebral revascularization may represent a potential therapy for symptomatic patients. PMID:26405843

  9. Potocki-Lupski syndrome in conjunction with bilateral clubfoot.

    PubMed

    Dhanaraj, Dinesh; Chu, Alice; Pappas, John G; Moran, Ellen; Lehman, Wallace B

    2015-07-01

    Potocki-Lupski syndrome (PTLS) is a rare chromosomal microduplication syndrome resulting in multiple congenital abnormalities including developmental delays, autistic features, and certain structural anomalies, with cardiovascular being the most common. The phenotype of this contiguous gene duplication syndrome is quite variable and may include musculoskeletal abnormalities. Given the infrequency and novelty of this disorder, full phenotypic characterization of PTLS has not yet been fully elucidated. We present a case of severe bilateral clubfoot in a patient with PTLS. Diagnosis was made by array-based comparative genomic hybridization and confirmed by fluorescence in-situ hybridization. Because clubfoot was also present in an apparently unaffected brother, the presence of PTLS may have acted as a modifier of the phenotype. This report highlights the complex interaction of chromosomal and familial factors that contribute to musculoskeletal birth defects. PMID:25768679

  10. Craniofacial Microsomia: Goldenhar Syndrome in Association with Bilateral Congenital Cataract

    PubMed Central

    Shrestha, U. D.; Adhikari, S.

    2015-01-01

    Craniofacial microsomia (CFM) includes a spectrum of malformations primarily involving structures derived from the first and second branchial arches. Patients with hemifacial microsomia and epibulbar dermoids are said to have Goldenhar syndrome (GHS). Four-month-old boy with whitish pupillary reflex presented with the features of GHS in pediatric ophthalmology clinic. The child had ocular and auricular manifestations. There were no vertebral anomalies, but he had bilateral congenital cataract. The peculiarity of this case is the presence of the bilateral total congenital cataract, in association with CFM. There is absence of epibulbar dermoid or lipodermoid in the eyes, although the child had features of GHS. In addition to it, anesthetic intubation was smooth in this case. Any case diagnosed with CFM and/or GHS needs treatment through multidisciplinary approach, consultation in ophthalmology department is one of them. PMID:26635984

  11. Bilaterally Symmetrical Lower Extremity Compartment Syndrome following Massive Transfusion

    PubMed Central

    Karaoren, Gulsah; Bakan, Nurten; Tomruk, Senay Goksu; Topaç, Zelin; Kurtulmu?, Tuhan; Irkören, Saime

    2016-01-01

    Compartment syndrome is a serious condition characterized by raised intracompartmental pressure, which develops following trauma. Well leg compartment syndrome (WLCS) is a term reserved for compartment syndrome in a nontraumatic setting, usually resulting from prolonged lithotomy position during surgery. In literature, 8 cases have been reported regarding well leg compartment syndrome in a supine position and bilateral symmetrical involvement was observed in only 2 cases. In WLCS etiology, lengthy surgery, lengthy hypotension, and extremity malpositioning have been held responsible but one of the factors with a role in the etiology may have been the tissue oedema and impaired microcirculation formed from the effect of vasoactive mediators expressed into the circulation associated with the massive blood transfusion. The case is presented here regarding symmetrical lower extremity compartment syndrome after surgery in which massive transfusion was made for gross haemorrhage from an abdominal injury. In conclusion, blood transfusion applied at the required time is life-saving but potential risks must always be considered. PMID:26885421

  12. Spontaneous bilateral compartment syndrome in a HIV-positive patient

    PubMed Central

    Davidson, Donald James; Shaukat, Yasir Mehmood; Jenabzadeh, Reza; Gupte, Chinmay M

    2013-01-01

    Spontaneous bilateral compartment syndrome is a very rare condition but one which requires swift diagnosis and urgent surgical decompression by fasciotomies in order to achieve the best outcome. We present the case of a 31-year-old HIV-positive man. The case highlights the perils of being sidetracked by an atypical clinical history instead of acting on the classical clinical examination findings. We will discuss the presentation and management of this patient, review the literature and highlight the key learning points. The most important learning point being that no matter how atypical the history, if a patient presents with limb pain out of proportion to the injury (with or without pain on passive stretch), sensory changes and a loss of motor power, then a diagnosis of acute compartment syndrome must be considered. PMID:24347455

  13. Spontaneous bilateral compartment syndrome in a HIV-positive patient.

    PubMed

    Davidson, Donald James; Shaukat, Yasir Mehmood; Jenabzadeh, Reza; Gupte, Chinmay M

    2013-01-01

    Spontaneous bilateral compartment syndrome is a very rare condition but one which requires swift diagnosis and urgent surgical decompression by fasciotomies in order to achieve the best outcome. We present the case of a 31-year-old HIV-positive man. The case highlights the perils of being sidetracked by an atypical clinical history instead of acting on the classical clinical examination findings. We will discuss the presentation and management of this patient, review the literature and highlight the key learning points. The most important learning point being that no matter how atypical the history, if a patient presents with limb pain out of proportion to the injury (with or without pain on passive stretch), sensory changes and a loss of motor power, then a diagnosis of acute compartment syndrome must be considered. PMID:24347455

  14. Bilateral Type-I Duane Syndrome with Multiple Anamolies: A Case Report

    PubMed Central

    Varma, Chaitanya; Aroor, Shrikiran; Mundkur, Suneel C; Annamalai, Karthick

    2012-01-01

    The Duane syndrome is a strabismus syndrome which is characterized by congenital non-progressive horizontal ophthalmoplegia which primarily affects the abducens nerve. Approximately 70% of the individuals with the Duane syndrome have an isolated disease. We have described here, a case of bilateral Duane syndrome with associated anamolies. PMID:23205369

  15. First Bite Syndrome After Bilateral Temporomandibular Joint Replacement: Case Report.

    PubMed

    Alwanni, Nada; Altay, Mehmet Ali; Baur, Dale A; Quereshy, Faisal A

    2016-03-01

    First bite syndrome (FBS) refers to intense pain in the parotid region, which coincides with the first bite of every meal, gradually subsides over the next several bites, but returns with the first bite of the next meal. The definitive diagnosis can be readily established by the characteristic onset of pain after the first bite of every meal. Pain is typically most intense at the first meal of the day, although some patients experience symptoms when thinking of food or salivating. FBS is a recognized complication of surgery within the parapharyngeal space; however, other surgical procedures involving the upper neck have been associated with this syndrome. The extreme rarity of FBS complicates a thorough understanding of its pathophysiology. Various medical agents have been used, with variable success, for the management of patients with FBS. Although proved effective, more radical treatment modalities are commonly reserved for persistent or refractory cases, because there is potential of spontaneous decrease in the severity of symptoms with time. This report describes the case of a patient presenting with symptoms of FBS after bilateral temporomandibular joint replacement. To the authors' knowledge, this is the first case of FBS in the literature occurring after temporomandibular joint replacement. PMID:26518528

  16. Bilateral Functional Thoracic Outlet Syndrome in a Collegiate Football Player

    PubMed Central

    Robey, Jason H.

    2009-01-01

    Background Thoracic Outlet Syndrome (TOS) involves compression of the brachial plexus, subclavius artery and vein. Many studies discuss efficacy of surgery and few discuss conservative treatment. It is unknown what specific forms of conservative treatment are best. Objective Describe conservative management for TOS using unique exercises. Case Description A collegiate football player reported numbness/tingling down his right arm after a right brachial plexus stretch injury. Seven months later, he was diagnosed with recurrent cervical traction neuropraxia. Two months later, he reported bilateral symptoms and was diagnosed with functional TOS. The athlete began shoulder strengthening (deltoid, middle trapezius, rhomboids, pectoralis major, latissimus dorsi, biceps, upper trapezius and rotator cuff) and stretching (pectoralis, scalene and upper trapezius) which failed to resolve his symptoms after four weeks. Surgical resection of bilateral first ribs and quitting football was recommended by four physicians. Unique therapeutic exercises developed by the Postural Restoration Institute™ were used to optimize respiration/posture via muscle activation and inhibition. After six weeks, the athlete was asymptomatic and returned to football but still experienced paresthesia with contact. Additional exercises were prescribed and remaining symptoms were abolished. Outcomes The Northwick Park Neck Pain Questionnaire was 55.5% at initial and 0% at four weeks and discharge. Discussion Athlete did not demonstrate relief of symptoms from shoulder stretching and strengthening. Intervention designed to optimize respiration/posture by repositioning the pelvis/trunk via specific muscle inhibition and activation resulted in abolishing the athlete's symptoms. Management that aims to optimize respiration via muscle inhibition, activation, and repositioning warrants further research. PMID:21509101

  17. Adrenal Venous Sampling Is Useful for a Definitive Diagnosis in Cushing's Syndrome with Bilateral Adrenal Tumors.

    PubMed

    Seki, Toshiro; Yasuda, Atsushi; Kitajima, Natsumi; Oki, Masayuki; Takagi, Atsushi; Nakamura, Naoya; Hanai, Kazuya; Terachi, Toshiro; Fukagawa, Masafumi

    2015-01-01

    We report three cases of Cushing's syndrome (CS) with bilateral adrenal tumors. When bilateral adrenal tumors are encountered, a differential diagnosis is difficult to make, especially in the case of functioning bilateral adrenocortical adenoma. Adrenal scintigraphy has become a standard technique to determine the laterality of excessive hormone secretion; however, this examination results in bilateral adrenal activity in the functioning bilateral adrenocortical adenoma. Our three patients were diagnosed with adrenocorticotropic hormone (ACTH)-independent CS based on biochemical testing, and an abdominal computed tomography (CT) scan detected bilateral adrenal tumors. Adrenal scintigraphy showed bilateral adrenal activity in all cases. However, adrenal venous sampling (AVS) demonstrated three different hormone-excess patterns (case 1: bilateral cortisol-excess secretions; case 2: unilateral cortisol-excess secretion and bilateral aldosterone-excess secretions; and case 3: bilateral cortisol-excess secretions and bilateral aldosterone-excess secretions). Based on these findings, we could select optimal treatment for each case. Therefore, AVS is useful to obtain a definitive diagnosis and adequate therapy for CS with bilateral adrenal tumors. PMID:26662665

  18. Bilateral Laparoscopic Gonadectomy in a Patient With Complete Androgen Insensitivity Syndrome and Bilateral Sertoli-Leydig Cell Tumor: A Case Report and Brief Review of the Literature

    PubMed Central

    Asl Zare, Mohammad; Kalantari, Mahmood Reza; Asadpour, Amir Abbas; Kamalati, Ali

    2014-01-01

    Introduction: Complete androgen insensitivity syndrome (previously called testicular feminization) is specified by a 46 XY karyotype and negative sex chromatin, bilateral undescended testes, female genitalia appearance, and lack of mullerian derivatives. Case Presentation: A 28-year-old woman with complete (severe) androgen resistance underwent prophylactic laparoscopic bilateral gonadectomy because of the eventually increased risk of gonadal malignancy. Although the gonads appeared grossly normal, microscopic examination revealed bilateral well differentiated sertoli–leydig cell tumor (SLCT). Discussion: Our Medline search revealed that this is the first reported case of bilateral sertoli–leydig cell tumor (SLCT) in androgen insensitivity syndrome. PMID:25032133

  19. Occurrence of bilaterally independent epileptic spasms after a corpus callosotomy in West syndrome.

    PubMed

    Kobayashi, Katsuhiro; Endoh, Fumika; Toda, Yoshihiro; Oka, Makio; Baba, Hiroshi; Ohtsuka, Yoko; Yoshinaga, Harumi

    2016-01-01

    We report a patient with intractable West syndrome whose epileptic spasms (ESs) were initially bilaterally synchronous, as is typical; after a complete corpus callosotomy, however, bilaterally independent ESs originated in either hemisphere. Activity of probable cortical origin associated with ESs was detected by observing ictal gamma oscillations. Brain MRI revealed no structural abnormality before surgery. This case suggests that ESs with a hemispheric origin may appear generalized because of synchronizing effects in the corpus callosum in some patients. PMID:25998967

  20. Single Bilateral Dexamethasone Implant in Addition to Panretinal Photocoagulation and Oral Azathioprine Treatment in IRVAN Syndrome.

    PubMed

    Saatci, Ali Osman; Ayhan, Ziya; Take?, Ömer; Yaman, Aylin; Bajin, F Meltem Söylev

    2015-01-01

    The idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome is a disease characterized by multiple retinal macroaneurysms, neuroretinitis and peripheral capillary nonperfusion. Visual loss may result from either ischemia-related complications or macular involvement. Treatment is not always rewarding. We report a case with stage 2 IRVAN syndrome who was successfully treated with a single bilateral intravitreal dexamethasone implant in addition to panretinal photocoagulation and systemic azathioprine treatment. PMID:25802506

  1. Single Bilateral Dexamethasone Implant in Addition to Panretinal Photocoagulation and Oral Azathioprine Treatment in IRVAN Syndrome

    PubMed Central

    Saatci, Ali Osman; Ayhan, Ziya; Take?, Ömer; Yaman, Aylin; Bajin, F. Meltem Söylev

    2015-01-01

    The idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome is a disease characterized by multiple retinal macroaneurysms, neuroretinitis and peripheral capillary nonperfusion. Visual loss may result from either ischemia-related complications or macular involvement. Treatment is not always rewarding. We report a case with stage 2 IRVAN syndrome who was successfully treated with a single bilateral intravitreal dexamethasone implant in addition to panretinal photocoagulation and systemic azathioprine treatment. PMID:25802506

  2. Bilateral retinopathy, aplastic anaemia, and central nervous system abnormalities: a new syndrome?

    PubMed Central

    Revesz, T; Fletcher, S; al-Gazali, L I; DeBuse, P

    1992-01-01

    A male infant was found to have bilateral exudative retinopathy at 6 months of age. A month later severe aplastic anaemia was diagnosed, eventually leading to the infant's death. Additional features of this seemingly new syndrome were intrauterine growth retardation, fine sparse hair, fine reticulate skin pigmentation, ataxia because of cerebellar hypoplasia, cerebral calcifications, extensor hypertonia, and progressive psychomotor retardation. Images PMID:1404302

  3. Cerebellar Ataxia with Bilateral Vestibulopathy: Description of a Syndrome and Its Characteristic Clinical Sign

    ERIC Educational Resources Information Center

    Migliaccio, Americo A.; Halmagyi, G. Michael; McGarvie, Leigh A.; Cremer, Phillip D.

    2004-01-01

    We report four patients with the syndrome of cerebellar ataxia with bilateral vestibulopathy (CABV) and, using search coil oculography, we validate its characteristic clinical sign, namely impairment of the visually enhanced vestibulo-ocular reflex (VVOR) or doll's head reflex. In our four patients, CABV began in the sixth decade of life; they are…

  4. Cerebellar Ataxia with Bilateral Vestibulopathy: Description of a Syndrome and Its Characteristic Clinical Sign

    ERIC Educational Resources Information Center

    Migliaccio, Americo A.; Halmagyi, G. Michael; McGarvie, Leigh A.; Cremer, Phillip D.

    2004-01-01

    We report four patients with the syndrome of cerebellar ataxia with bilateral vestibulopathy (CABV) and, using search coil oculography, we validate its characteristic clinical sign, namely impairment of the visually enhanced vestibulo-ocular reflex (VVOR) or doll's head reflex. In our four patients, CABV began in the sixth decade of life; they are…

  5. Metachronous Bilateral Posterior Tibial Artery Aneurysms in Ehlers-Danlos Syndrome Type IV

    SciTech Connect

    Hagspiel, Klaus D.; Bonatti, Hugo; Sabri, Saher; Arslan, Bulent; Harthun, Nancy L.

    2011-04-15

    Ehlers-Danlos syndrome type IV is a life-threatening genetic connective tissue disorder. We report a 24-year-old woman with EDS-IV who presented with metachronous bilateral aneurysms/pseudoaneurysms of the posterior tibial arteries 15 months apart. Both were treated successfully with transarterial coil embolization from a distal posterior tibial approach.

  6. Bilateral squamosal suture synostosis: A rare form of isolated craniosynostosis in Crouzon syndrome

    PubMed Central

    Tandon, Yasmeen K; Rubin, Michael; Kahlifa, Mohamed; Doumit, Gaby; Naffaa, Lena

    2014-01-01

    Craniosynostosis is a pathologic condition which is characterized by the premature fusion of cranial sutures. It may occur alone or in association with other anomalies making up various syndromes. Crouzon syndrome is the most common craniosynostosis syndrome. Bicoronal sutures fusion is most commonly involved in Crouzon syndrome. There have only been a handful of cases of squamosal suture synostosis described in the surgery literature with the few ones described in Crouzon syndrome associated with other types of craniosynostosis. To the best of our knowledge, we are presenting the first case of isolated bilateral squamosal suture synostosis in a patient with Crouzon syndrome in a radiology journal with emphasis on its radiological appearance. PMID:25071892

  7. [Bilateral diaphragmatic paralysis due to Parsonage-Turner syndrome].

    PubMed

    Tissier-Ducamp, D; Martinez, S; Alagha, K; Charpin, D; Chanez, P; Palot, A

    2015-09-01

    We report the case of a 49-years-old patient who presented to the accident and emergency department with sudden onset dyspnea associated with acute shoulder pain. He was breathless at rest with supine hypoxemia. He had an amyotrophic left shoulder with localized paresis of the shoulder. Both hemi-diaphragms were elevated on chest X-rays. Pulmonary function tests showed a restrictive pattern and both phrenic nerve conduction velocities were decreased. At night, alveolar hypoventilation was evidenced by elevated mean capnography (PtcCO2: 57mmHg). Neuralgic amyotrophy, Parsonage-Turner syndrome was the final diagnosis. This syndrome is a brachial plexus neuritis with a predilection for the suprascapular and axillary nerves. Phrenic nerve involvement is rare but where present can be the most prominent clinical feature as in our case report. PMID:25534571

  8. Bilateral Giant Coronary Artery Aneurysms Complicated by Acute Coronary Syndrome and Cardiogenic Shock.

    PubMed

    Chiu, Peter; Lynch, Donald; Jahanayar, Jama; Rogers, Ian S; Tremmel, Jennifer; Boyd, Jack

    2016-04-01

    Giant coronary aneurysms are rare. We present a 25-year-old woman with a known history of non-Kawasaki/nonatherosclerotic bilateral coronary aneurysms. She was transferred to our facility with acute coronary syndrome complicated by cardiogenic shock. Angiography demonstrated giant bilateral coronary aneurysms and complete occlusion of the left anterior descending (LAD) artery. Emergent coronary artery bypass grafting was performed. Coronary artery bypass grafting is the preferred approach for addressing giant coronary aneurysms. Intervention on the aneurysm varies in the literature. Aggressive revascularization is recommended in the non-Kawasaki/nonatherosclerotic aneurysm patient, and ligation should be performed in patients with thromboembolic phenomena. PMID:27000621

  9. [A Case of Anti-GQ1b Antibody Syndrome Associated with Pure Bilateral Adie's Pupils].

    PubMed

    Kajikawa, Shunsuke; Ohi, Takekazu; Fujita, Atsushi; Kusunoki, Susumu

    2016-01-01

    A 37 year-old Japanese male felt photophobia of both eyes one week following the onset of the common cold. His neurological examination revealed bilateral Adie's tonic pupils, no extraocular movement disorder, normal deep tendon reflexes, and no cerebellar signs. Based on markedly increased blood levels of anti-GQ1b IgG and anti-GT1a IgG antibodies, we diagnosed him as anti-GQ1b antibody syndrome. Bilateral Adie's tonic pupils were improved by IVIg drip infusion and methylprednisolone pulse therapy. This case suggests that we need to investigate anti ganglioside antibody when a patient presents with bilateral Adie's tonic pupils. (Received February 6, 2015; Accepted September 1, 2015; Published January 1, 2016). PMID:26764303

  10. 46,XY female sex reversal syndrome with bilateral gonadoblastoma and dysgerminoma

    PubMed Central

    DU, XUE; ZHANG, XUHONG; LI, YONGMEI; HAN, YUKUN

    2014-01-01

    Sex reversal syndrome is a rare congenital condition of complete or disordered gonadal development leading to discordance between the genetic, gonadal and phenotypic sexes, including 46,XX and 46,XY. The gonadoblastoma on the Y-chromosome (GBY) region is associated with an increased risk of developing type II germ cell tumors/cancer. The present study reports a unique case of a phenotypically normal female (age 17 years), presenting with primary amenorrhea and later diagnosed with 46,XY female sex reversal syndrome. Following bilateral gonadectomy, bilateral gonadoblastoma and dysgerminoma were diagnosed. Thus, estrogen replacement therapy was administered periodically to promote the development of secondary sexual characteristics and menstruation, and to prevent osteoporosis. A four year follow-up showed no tumor recurrence and a regular menstrual cycle in this patient. PMID:25187804

  11. Acute bilateral useless hand syndrome: a rare presenting manifestation of vitamin B12 deficiency.

    PubMed

    Biyani, Sumant; Jha, Sneh Kumar; Pandey, Suchit; Shukla, Rakesh

    2015-01-01

    We report a case of bilateral useless hand syndrome, a rare presenting manifestation of vitamin B12 deficiency. A 38-year-old man, a strict vegetarian and a teacher by occupation, presented with acute onset clumsiness of both hands while performing fine movements. Detailed history-taking, examination of the patient and relevant investigations (complete blood count, serum vitamin B12 and MRI of the cervical spinal cord) were carried out. Laboratory analysis was suggestive of vitamin B12 deficiency and MRI demonstrated a lesion involving the posterior columns of the cervical cord. The patient was diagnosed as a case of non-compressive cervical myelopathy predominantly involving the posterior column due to vitamin B12 deficiency. Acute bilateral useless hand syndrome can be a rare presenting feature of vitamin B12 deficiency. PMID:26475874

  12. Bilateral De Quervain Syndrome after Aromatase Inhibitor Administration: A Case Report and Review of the Literature

    PubMed Central

    Papadimitriou, Konstantinos; Kountourakis, Panteleimon; Morakis, Emmanouil; Vassiliou, Vassilios; Barbounis, Vasileios; Ardavanis, Alexandros

    2012-01-01

    Aromatase inhibitors are widely used as one of the main treatment options of both early and advanced hormone receptor-positive breast cancer in postmenopausal women. Unfortunately, musculoskeletal symptoms are often presented in patients treated with aromatase inhibitors (AIs), and, although the pathogenesis is unknown, postulated mechanisms have been described. Herein, to our knowledge, we present the first report of bilateral De Quervain syndrome related with AIs therapy with a review of the relevant literature. PMID:22567020

  13. Development of bilateral coronary artery aneurysms in a child with Noonan syndrome.

    PubMed

    Mauro, David M; Flors, Lucia; Hoyer, Andrew W; Norton, Patrick T; Hagspiel, Klaus D

    2016-03-01

    Noonan syndrome is a constellation of congenital malformations including heart defects, facial anomalies and short stature. The cardiovascular defects are variable and extensive, with the most common being pulmonary stenosis and hypertrophic cardiomyopathy. Coronary artery anomalies have only been reported in a few cases. We report a child with Noonan syndrome status post pulmonary stenosis and atrial septal defect repair, who developed bilateral coronary artery aneurysms. The aneurysms were diagnosed with both cardiac magnetic resonance imaging and coronary computed tomography angiography. There had been no evidence of them on a cardiac MR exam 5 years previously. PMID:26515448

  14. The Role of Left Perisylvian Cortical Regions in Spelling

    ERIC Educational Resources Information Center

    Henry, Maya L.; Beeson, Pelagie M.; Stark, Amy J.; Rapcsak, Steven Z.

    2007-01-01

    In order to examine the role of left perisylvian cortex in spelling, 13 individuals with lesions in this area were administered a comprehensive spelling battery. Their spelling of regular words, irregular words, and nonwords was compared with that of individuals with extrasylvian damage involving left inferior temporo-occipital cortex and normal…

  15. Diagnosis and treatment of a patient with bilateral thoracic outlet syndrome secondary to anterior subluxation of bilateral sternoclavicular joints: a case report.

    PubMed

    Nichols, Deidra; Seiger, Cindy

    2013-10-01

    Thoracic outlet syndrome may result from a posterior sternoclavicular (SC) joint subluxation, or an anterior SC joint subluxation after surgical fixation. This case report presents the physical therapy management of a patient with bilateral thoracic outlet syndrome (TOS) secondary to bilateral idiopathic anterior SC joint subluxation. A 16-year-old female presented with a 2-year history of numbness, tingling, and coldness in bilateral upper extremities, and intermittent headaches with occasional vision loss. Ipsilateral upper extremity symptoms were reproduced with cervical rotation and shoulder flexion and abduction from 90° to end of the range. All TOS tests were positive. Passive horizontal abduction, through the plane of scaption, produced anterior subluxation of the ipsilateral SC joint. Sustained posterior glides to the medial clavicle relieved all symptoms during shoulder flexion and the Adson's test. Interventions consisted of manual therapy, therapeutic exercise, and the trial of two orthoses. After 12 treatment sessions, the patient's symptoms resolved and she improved by 10 points on the Upper Extremity Functional Index. She had no reproduction of symptoms with the thoracic outlet special tests. She maintained a static hold for 90 sec at 90° shoulder flexion, 90° shoulder abduction, and full shoulder flexion without symptoms. The outcomes describe a successful intervention for a patient with bilateral TOS secondary to idiopathic bilateral anterior SC joint subluxation. This case suggests that SC joint dysfunction should be considered as a cause of TOS and should be screened during the initial examination. PMID:23343034

  16. Bilateral Sensorineural Hearing Loss and Polyneuropathy in a Patient with Sweet's Syndrome

    PubMed Central

    Cala, Cather M.; Kole, Lauren; Sami, Naveed

    2015-01-01

    Sweet's syndrome is an inflammatory systemic disease which has been associated with various underlying causes. The disease can involve multiple areas of the body including the skin and neurological system. There have been only two cases which have described otological involvement. This report presents a patient who developed loss of hearing secondary to Sweet's syndrome after developing cutaneous involvement along with peripheral neuropathy. Despite the patient's skin and neuropathy noticing improvement with intravenous immunoglobulin and azathioprine, he required bilateral cochlear implants for partial recovery of his hearing loss. This case highlights the need to recognize Sweet's syndrome as a complicated disease process where the role of otolaryngologists is important in the multidisciplinary coordination of care in both diagnosis and treatment. PMID:26770858

  17. Bilateral Sensorineural Hearing Loss and Polyneuropathy in a Patient with Sweet's Syndrome.

    PubMed

    Cala, Cather M; Kole, Lauren; Sami, Naveed

    2015-01-01

    Sweet's syndrome is an inflammatory systemic disease which has been associated with various underlying causes. The disease can involve multiple areas of the body including the skin and neurological system. There have been only two cases which have described otological involvement. This report presents a patient who developed loss of hearing secondary to Sweet's syndrome after developing cutaneous involvement along with peripheral neuropathy. Despite the patient's skin and neuropathy noticing improvement with intravenous immunoglobulin and azathioprine, he required bilateral cochlear implants for partial recovery of his hearing loss. This case highlights the need to recognize Sweet's syndrome as a complicated disease process where the role of otolaryngologists is important in the multidisciplinary coordination of care in both diagnosis and treatment. PMID:26770858

  18. Microform holoprosencephaly with bilateral congenital elbow dislocation; increasing the phenotypic spectrum of Steinfeld syndrome.

    PubMed

    Jones, Gabriela E; Robertson, Lisa; Maniyar, Amit; Shammas, Christos; Phelan, Marie M; Vasudevan, Pradeep C; Tanteles, George A

    2016-03-01

    Steinfeld syndrome (MIM #184705) was first reported in 1982. It is characterised by holoprosencephaly and limb defects, however other anomalies may also be present. Following the initial description, three further cases have been reported in the literature. We report on a 23-year-old girl, with features of microform holoprosencephaly and bilateral congenital elbow dislocation in association with hypoplastic radial heads. She was identified to have a variant in the CDON gene inherited from her father who had ocular hypotelorism, but no other clinical features. We discuss the clinical features of Steinfeld syndrome, and broaden the phenotypic spectrum of this condition. Structural analysis suggests that this variant could lead to destabilisation of binding of CDON with hedgehog proteins. Further work needs to be done to confirm whether mutations in the CDON gene are the cause of Steinfeld syndrome. © 2016 Wiley Periodicals, Inc. PMID:26728615

  19. Bilateral gluteal compartment syndrome complicated by rhabdomyolysis and acute kidney injury in a patient with alcohol intoxication

    PubMed Central

    Young Cho, Jae; Lee, Jae-Won; Jung Cho, Eun; Kim, Myung-Gyu; Jo, Sang Kyung; Yong Cho, Won; Kyu Kim, Hyoung

    2012-01-01

    Bilateral gluteal compartment syndrome is a rare clinical entity that can be complicated by rhabdomyolysis or acute kidney injury (AKI). We report the a case of a 30-year-old woman without any comorbid diseases who was diagnosed with bilateral gluteal compartment syndrome complicated by rhabdomyolysis and dialysis-requiring AKI, which was caused by prolonged immobilization under the influence of alcohol. Although the patient’s renal function recovered fully after 5 sessions of hemodialysis, sciatic neuropathy caused by gluteal compartment syndrome led to permanent foot drop.

  20. Bilateral piriformis syndrome in two elite soccer players: Report of two cases.

    PubMed

    Zeren, B; Canbek, U; Oztekin, H H; İmerci, A; Akgün, U

    2015-12-01

    Piriformis syndrome, a relatively rare condition, is described as entrapment of a sciatic nerve at the level of the piriformis muscle. There have been a few reports of bilateral piriformis syndrome in literature. In this study, we present bilateral piriformis syndrome in two professional soccer players from different teams who are symptom free at last follow-up after surgery. In both patients, resting EMG records were read normal, however EMG recording during the activity revealed prolonged H-reflexes. Both patients had no relief from conservative treatment and rehabilitation, therefore surgical treatment was performed. Preoperative mean visual analogue scale (VAS) value was 7, and decreased to 3 at the sixth month follow-up visit and at the longer term follow-up, mean 85months (74-96) it was valued at 1. Both soccer players returned to their active sports lives in the sixth postoperative month. According to Benson's functional evaluation scale, in long-term follow-up, there have been excellent results and both patients resumed their professional carrier for many years (mean 7 years). PMID:26522381

  1. Parvovirus B19-induced acute bilateral carpal tunnel syndrome in twin girls.

    PubMed

    Sakall?, Hale; Bask?n, Esra; Dener, ?efik

    2015-01-01

    We describe 2 cases of 6-year-old twin girls presenting with acute carpal tunnel syndrome (CTS) associated with human parvovirus B19 (HPV-B19) infection, as evidenced by serological data and detection of HPV-B19 DNA in blood with use of polymerase chain reaction (PCR). To our knowledge, this is the first time that HPV-B19 infection has been suggested as the causal agent of simultaneous acute bilateral CTS in twins, thus presenting the possibility that similar immunologic responses can be observed in twins during viral infections. PMID:26422355

  2. Bilateral subclavian steal syndrome in an intensive care unit. A case report.

    PubMed

    Budincevic, Hrvoje; Friedrich, Latica; Tolj-Karaula, Nikolina; Maric, Nikolina; Sucic, Tena; Bielen, Ivan

    2014-09-01

    We report the case of a 56-year-old man who presented with arterial hypotension, lightheadedness, vomiting, a sense of tingling in his right arm, and a right-beating horizontal nystagmus. He was initially admitted to the Intensive care unit and treated with standard vasopressor agents. A neurosonological examination showed the steal phenomenon on both vertebral arteries; the neuroradiological examination revealed occlusion of the left subclavian artery and subtotal stenosis of the innominate artery. Percutaneous transluminal angioplasty was performed. Our case demonstrates how bilateral subclavian steal syndrome should be taken into account in the case of a seemingly hypotensive patient unresponsive to standard therapy. PMID:25110770

  3. Anterior opercular syndrome induced by Epstein-Barr virus encephalitis.

    PubMed

    Matsushima, Takashi; Nishioka, Kenya; Tanaka, Ryota; Yokoyama, Kazumasa; Hattori, Nobutaka

    2016-02-01

    We report a 19-year-old female presenting with fever, drooling, anarthria, and voluntary facial movement disruption, characteristic of anterior opercular syndrome (AOS). Serological examination revealed Epstein-Barr virus (EBV) infection following acute encephalitis with severe ataxia. A single-photon emission computerized tomography (SPECT) examination indicated hypoperfusion in the left perisylvian region, bilateral thalamus, occipital lobe, and cerebellum. This is the first report of AOS related to EBV encephalitis. SPECT was a useful method for detecting the damaged region of the operculum. In addition, AOS is a clinically distinct entity that may help us understand the mechanisms of language circuits within the operculum. PMID:26027635

  4. Deafness due to bilateral endolymphatic sac tumours in a case of von Hippel-Lindau syndrome.

    PubMed Central

    Kempermann, G; Neumann, H P; Scheremet, R; Volk, B; Mann, W; Gilsbach, J; Laszig, R

    1996-01-01

    A case of bilateral endolymphatic sac tumours is reported. In a patient with von Hippel-Lindau syndrome, tumour growth in the right cerebellopontine angle caused deafness. The tumour was removed and classified as a metastasis from a thyroid carcinoma. However, on thyroidectomy no primary neoplasm could be found. Eight years later a similar tumour was operated on in the left petrosal bone. Histological appearance, immunocytochemical findings, and the clinical context gave evidence that the tumours had to be reclassified as endolymphatic sac tumours--extremely rare entities. The report supports the hypothesis, suggested by the few earlier case reports, that endolymphatic sac tumours could be one of the inherent tumour manifestations in von Hippel-Lindau syndrome. Images PMID:8795608

  5. Genetics of primary bilateral macronodular adrenal hyperplasia: a model for early diagnosis of Cushing's syndrome?

    PubMed

    Drougat, Ludivine; Espiard, Stéphanie; Bertherat, Jerôme

    2015-10-01

    Long-term consequences of cortisol excess are frequent despite appropriate treatment after cure of Cushing's syndrome. This might be due to diagnostic delay, often difficult to reduce in rare diseases. The identification of a genetic predisposing factor might help to improve early diagnosis by familial screening. Primary bilateral macronodular adrenal hyperplasia (PBMAH) is a rare cause of Cushing's syndrome. Hypercortisolism in PBMAH is most often diagnosed between the fifth and sixth decades of life. The bilateral nature of the adrenocortical tumors and the occurrence of rare clear familial forms suggest a genetic origin. Indeed, a limited subset of PBMAH can be observed as part of multiple tumors syndromes due to alterations of the APC, Menin or Fumarate Hydratase genes. Rare variants of the phosphodiesterases PDE11A have been associated with PBMAH. The recent identification of ARMC5 germline alterations in 25-50% of PBMAH patients without obvious familial history or associated tumors opens new perspectives. ARMC5 alterations follow the model of a tumor suppressor gene: a first germline inactivating mutation of this 16p located gene is followed by a somatic secondary hit on the other allele (inactivating mutation or allelic loss). Functional studies demonstrate that ARMC5 controls apoptosis and steroid synthesis. The phenotype of index cases patients with the mutation seems more severe than the one of WT index cases. However, phenotype variability within a family is often observed. This review summarizes the genetics of PBMAH, focusing on ARMC5, which offer new perspectives for early diagnosis of Cushing's syndrome. PMID:26264719

  6. Excessive testosterone production in a patient with Nelson syndrome and bilateral testicular tumors.

    PubMed

    Shekarriz, M; Schneider, C; Sabanegh, E; Kempter, F; Waldherr, R

    1996-01-01

    Bilateral primary testicular tumors are rare and usually consist of either interstitial cells or hypertrophic testicular adrenal remnant tissue. Their differentiation on clinical presentation and histologic examination remains difficult but is essential because of the different therapeutic approaches. We report a rare case of excessive testosterone production by bilateral testicular tumors in a patient with Nelson syndrome (ACTH-secreting pituitary adenoma after bilateral adrenalectomy in patients with Cushing's disease). Increased ACTH stimulation in this patient supports the thesis of pluripotent cells within the testis which can undergo differentiation to cells which are not only morphologically similar to Leydig cells but also have the functional property of these cells. Our clinical findings support the diagnosis of hyperplasia of adrenal remnant or pluripotent cells rather than a true Leydig cell tumor. We emphasize the need for hormonal evaluations which should be assessed in the context of the size of these nodular tumors prior to therapeutic decisions. In cases with elevated serum ACTH and small nodular hyperplasia, we would favor a 'wait-and-see' strategy with appropriate hormonal therapy. In large tumors with clinical signs of hormonal activity, patient noncompliance with steroid replacement regimens or with local symptoms, scrotal exploration and tumor enucleation are indicated. PMID:8860745

  7. Can response time be trained with bilateral limb training in children with Down syndrome?

    PubMed Central

    Rao, Pratiksha Tilak; Solomon, John M.

    2015-01-01

    Aims: Response time (RT), that is, the time taken to respond is known to be delayed in children with Down syndrome (DS). We performed a pilot study to evaluate whether bilateral limb training can be used to train RT, in children with DS. Settings and Design: 10 children with DS (5 males) were recruited from a special school in a suburban region using convenience sampling. Subjects and Methods: Response time was measured using an indigenously developed RT Analyzer, before and after intervention, from right and left hand. Structured bilateral limb training was given for a period of 4 weeks, using low-cost, locally available materials, in community settings. Statistical Analysis Used: The Wilcoxon signed ranks test was used for statistical analysis. Results: Significant improvements in RT following 4 weeks of intervention were seen in the left hand (P = 0.006) but not in the right hand (P = 0.104). Conclusions: Response time can be trained in children with DS using 4 weeks of bilateral limb training activities using low-cost, locally available materials. PMID:26167016

  8. Capgras Syndrome in a Patient with Parkinson's Disease after Bilateral Subthalamic Nucleus Deep Brain Stimulation: A Case Report.

    PubMed

    Kyrtsos, Christina Rose; Stahl, Mark C; Eslinger, Paul; Subramanian, Thyagarajan; Lucassen, Elisabeth B

    2015-01-01

    Capgras syndrome is a delusional misidentification syndrome (DMS) which can be seen in neurodegenerative diseases such as Lewy body dementia and, to a lesser extent, in Parkinson's disease (PD). Here, we report the case of a 78-year-old man with a history of idiopathic PD who developed Capgras syndrome following bilateral subthalamic nucleus deep brain stimulation (DBS) implantation. As the risk of DMS has been related to deficits in executive, memory, and visuospatial function preoperatively, this case highlights the importance of continuing to improve patient selection for DBS surgery. Capgras syndrome is a rare potential complication of DBS surgery in PD patients with preexisting cognitive decline. PMID:26078747

  9. Capgras Syndrome in a Patient with Parkinson's Disease after Bilateral Subthalamic Nucleus Deep Brain Stimulation: A Case Report

    PubMed Central

    Kyrtsos, Christina Rose; Stahl, Mark C.; Eslinger, Paul; Subramanian, Thyagarajan; Lucassen, Elisabeth B.

    2015-01-01

    Capgras syndrome is a delusional misidentification syndrome (DMS) which can be seen in neurodegenerative diseases such as Lewy body dementia and, to a lesser extent, in Parkinson's disease (PD). Here, we report the case of a 78-year-old man with a history of idiopathic PD who developed Capgras syndrome following bilateral subthalamic nucleus deep brain stimulation (DBS) implantation. As the risk of DMS has been related to deficits in executive, memory, and visuospatial function preoperatively, this case highlights the importance of continuing to improve patient selection for DBS surgery. Capgras syndrome is a rare potential complication of DBS surgery in PD patients with preexisting cognitive decline. PMID:26078747

  10. Unilateral or bilateral vagotomy induces ovulation in both ovaries of rats with polycystic ovarian syndrome

    PubMed Central

    2013-01-01

    Background Injecting estradiol valerate (EV) to pre-pubertal or adult female rat results in effects similar to those observed in women with polycystic ovarian syndrome (PCOS). One of the mechanisms involved in PCOS development is the hyperactivity of the sympathetic nervous system. In EV-induced PCOS rats, the unilateral sectioning of the superior ovarian nerve (SON) restores ovulation of the innervated ovary. This suggests that, in addition to the sympathetic innervation, other neural mechanisms are involved in the development/maintenance of PCOS. The aims of present study were analyze if the vagus nerve is one of the neural pathways participating in PCOS development. Methods Ten-day old rats were injected with EV dissolved in corn oil. At 24-days of age sham-surgery, unilateral, or bilateral sectioning of the vagus nerve (vagotomy) was performed on these rats. The animals were sacrificed at 90–92 days of age, when they presented vaginal estrous preceded by a pro-estrus smear. Results In EV-induced PCOS rats, unilateral or bilateral vagotomy restored ovulation in both ovaries. Follicle-stimulating hormone (FSH) levels in PCOS rats with unilateral or bilateral vagotomy were lower than in control rats. Conclusions This result suggests that in EV-induced PCOS rats the vagus nerve is a neural pathway participating in maintaining PCOS. The vagus nerve innervates the ovaries directly and indirectly through its synapsis in the celiac-superior-mesenteric ganglion, where the somas of neurons originating in the SON are located. Then, it is possible that vagotomy effects in EV-induced PCOS rats may be explained as a lack of communication between the central nervous system and the ovaries. PMID:23866168

  11. Bilateral striopallidodentate calcification (Fahr's syndrome) and multiple system atrophy in a patient with longstanding hypoparathyroidism.

    PubMed

    Preusser, Matthias; Kitzwoegerer, Melitta; Budka, Herbert; Brugger, Stefan

    2007-10-01

    Recently, a family with idiopathic brain calcification was reported, in which one family member was diagnosed with multiple system atrophy (MSA) at autopsy. We report here a case showing similar neuropathological features in a patient with longstanding hypoparathyroidism. Our female patient had a history of hypoparathyroidism with hypocalcaemia and tetany since the age of 9 years. In her 50s she developed dementia and parkinsonism. She died of myocardial infarction aged 65 years. Neuropathology showed severe brain calcifications of the Fahr type in the basal ganglia, thalami, cerebral and cerebellar white matter and dentate nuclei. Additionally, there was prominent alpha-synucleinopathy of the multiple system atrophy type (MSA). The patient has a healthy identical twin and there is no family history of hypoparathyroidism or neurological disease. Data on alpha-synuclein accumulation in various cases of Fahr's syndrome are needed to establish the correlation between alpha-synucleinopathy and bilateral striopallidodentate calcification. PMID:18018479

  12. A unilateral purple urine bag syndrome in a patient with bilateral nephrostomy tubes.

    PubMed

    Karim, Adil; Abed, Firas; Bachuwa, Ghassan

    2015-01-01

    A change in the colour of urine is always of clinical significance, and a source of concern for the patient and his physician. Among the different urine colours observed, purple is the least common. Although purple discolouration of a catheter and a urine bag is an uncommon finding, it was reported in the literature as early as 1978, by Barlow and Dickson. We present a unique case of purple urine bag syndrome in a patient with bilateral nephrostomy tubes (NT) and associated urine bags (UB) with only the left nephrostomy tube and urine bag exhibiting the purple colour, which resolved with a course of appropriate antibiotics eradicating the causative bacterial pathogen, and change of NT and UB. PMID:26701992

  13. A rare differential diagnosis to occupational neck pain: bilateral stylohyoid syndrome.

    PubMed

    Kirchhoff, Gertrud; Kirchhoff, Chlodwig; Buhmann, Sonja; Kanz, Karl-Georg; Lenz, Miriam; Vogel, Tobias; Kichhoff, Rainer Maria

    2006-01-01

    Chronic neck pain is widely prevalent and a common source of disability in the working-age population. Etiology of chronic neck pain includes neck sprain, mechanical or muscular neck pain, myofascial pain syndrome, postural neck pain as well as pain due to degenerative changes. We report the case of a 42 year old secretary, complaining about a longer history of neck pain and limited movement of the cervical spine. Surprisingly, the adequate radiologic examination revealed a bilateral ossification of the stylohyoid ligament complex. Her symptoms remained intractable from conservative treatment consisting of anti-inflammatory medication as well as physical therapy. Hence the patient was admitted to surgical resection of the ossified stylohyoid ligament complex. Afterwards she was free of any complaints and went back to work. Therefore, ossification of the stylohyoid ligament complex causing severe neck pain and movement disorder should be regarded as a rare differential diagnosis of occupational related neck pain. PMID:16800878

  14. Age-Related Differences and Heritability of the Perisylvian Language Networks

    PubMed Central

    Dell'Acqua, Flavio; Rijsdijk, Frühling V.; Kane, Fergus; Picchioni, Marco; McGuire, Philip; Toulopoulou, Timothea; Georgiades, Anna; Kalidindi, Sridevi; Kravariti, Eugenia; Murray, Robin M.; Murphy, Declan G.; Craig, Michael C.

    2015-01-01

    Acquisition of language skills depends on the progressive maturation of specialized brain networks that are usually lateralized in adult population. However, how genetic and environmental factors relate to the age-related differences in lateralization of these language pathways is still not known. We recruited 101 healthy right-handed subjects aged 9–40 years to investigate age-related differences in the anatomy of perisylvian language pathways and 86 adult twins (52 monozygotic and 34 dizygotic) to understand how heritability factors influence language anatomy. Diffusion tractography was used to dissect and extract indirect volume measures from the three segments of the arcuate fasciculus connecting Wernicke's to Broca's region (i.e., long segment), Broca's to Geschwind's region (i.e., anterior segment), and Wernicke's to Geschwind's region (i.e., posterior segment). We found that the long and anterior arcuate segments are lateralized before adolescence and their lateralization remains stable throughout adolescence and early adulthood. Conversely, the posterior segment shows right lateralization in childhood but becomes progressively bilateral during adolescence, driven by a reduction in volume in the right hemisphere. Analysis of the twin sample showed that genetic and shared environmental factors influence the anatomy of those segments that lateralize earlier, whereas specific environmental effects drive the variability in the volume of the posterior segment that continues to change in adolescence and adulthood. Our results suggest that the age-related differences in the lateralization of the language perisylvian pathways are related to the relative contribution of genetic and environmental effects specific to each segment. SIGNIFICANCE STATEMENT Our study shows that, by early childhood, frontotemporal (long segment) and frontoparietal (anterior segment) connections of the arcuate fasciculus are left and right lateralized, respectively, and remain lateralized throughout adolescence and early adulthood. In contrast, temporoparietal (posterior segment) connections are right lateralized in childhood, but become progressively bilateral during adolescence. Preliminary twin analysis suggested that lateralization of the arcuate fasciculus is a heterogeneous process that depends on the interplay between genetic and environment factors specific to each segment. Tracts that exhibit higher age effects later in life (i.e., posterior segment) appear to be influenced more by specific environmental factors. PMID:26377454

  15. Single-Session CT-Guided Percutaneous Microwave Ablation of Bilateral Adrenal Gland Hyperplasia Due to Ectopic ACTH Syndrome.

    PubMed

    Sarma, Asha; Shyn, Paul B; Vivian, Mark A; Ng, Ju-Mei; Tuncali, Kemal; Lorch, Jorchen H; Zaheer, Sarah N; Gordon, Michael S; Silverman, Stuart G

    2015-10-01

    Bilateral adrenalectomy is currently the only available treatment for adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome (ectopic ACTH syndrome) that is refractory to pharmacologic therapy. We describe two patients with refractory ectopic ACTH syndrome who were treated with CT-guided percutaneous microwave ablation of both hyperplastic adrenal glands in a single session: O ne was not a surgical candidate, and the other had undergone unsuccessful surgery. Following the procedure, both patients achieved substantial decreases in serum cortisol, symptomatic improvement, and decreased anti-hypertensive medication requirements. PMID:25762486

  16. Bilateral congenital lacrimal fistulas in an adult as part of ectrodactyly-ectodermal dysplasia-clefting syndrome: A rare anomaly

    PubMed Central

    Ghosh, Debangshu; Saha, Somnath; Basu, Sumit Kumar

    2015-01-01

    Ectrodactyly-ectodermal dysplasia and clefting syndrome or “Lobster claw” deformity is a rare congenital anomaly that affects tissues of ectodermal and mesodermal origin. Nasolacrimal duct (NLD) obstruction with or without atresia of lacrimal passage is a common finding of such a syndrome. The authors report here even a rarer presentation of the syndrome which manifested as bilateral NLD obstruction and lacrimal fistula along with cleft lip and palate, syndactyly affecting all four limbs, mild mental retardation, otitis media, and sinusitis. Lacrimal duct obstruction and fistula were managed successfully with endoscopic dacryocystorhinostomy (DCR) which is a good alternative to lacrimal probing or open DCR in such a case. PMID:26655010

  17. Bilateral congenital lacrimal fistulas in an adult as part of ectrodactyly-ectodermal dysplasia-clefting syndrome: A rare anomaly.

    PubMed

    Ghosh, Debangshu; Saha, Somnath; Basu, Sumit Kumar

    2015-10-01

    Ectrodactyly-ectodermal dysplasia and clefting syndrome or "Lobster claw" deformity is a rare congenital anomaly that affects tissues of ectodermal and mesodermal origin. Nasolacrimal duct (NLD) obstruction with or without atresia of lacrimal passage is a common finding of such a syndrome. The authors report here even a rarer presentation of the syndrome which manifested as bilateral NLD obstruction and lacrimal fistula along with cleft lip and palate, syndactyly affecting all four limbs, mild mental retardation, otitis media, and sinusitis. Lacrimal duct obstruction and fistula were managed successfully with endoscopic dacryocystorhinostomy (DCR) which is a good alternative to lacrimal probing or open DCR in such a case. PMID:26655010

  18. Bilateral stenosis of carotid siphon in Hutchinson-Gilford progeria syndrome.

    PubMed

    Narazaki, Ryo; Makimura, Mika; Sanefuji, Masafumi; Fukamachi, Shigeru; Akiyoshi, Hidetaka; So, Hidenori; Yamamura, Kenichiro; Doisaki, Sayoko; Kojima, Seiji; Ihara, Kenji; Hara, Toshiro; Ohga, Shouichi

    2013-08-01

    Hutchinson-Gilford progeria syndrome (HGPS) is a rare premature aging disease, caused by a de novo mutation of lamin-A gene, LMNA G608G. Accumulation of abnormal lamin-A (progerin) compromises nuclear membrane integrity and results in the accelerated senescence. Affected patients show a typical feature of birdlike face, alopecia, sclerotic skin, loss of subcutaneous fat, and short stature with advancing years. Neonatal scleroderma is the first presentation, although early diagnosis is challenging. The leading cause of death is cardio-/cerebro-vascular accidents associated with atherosclerosis. However, not all findings may recapitulate the aging process. We herein report a 9-year-old Japanese male with HGPS who developed cerebral infarction. The genetic study of peripheral blood-derived DNA determined a heterozygous c.1824C>T mutation, p.G608G. Telomere length of lymphocytes was normal. Bilateral stenosis of carotid siphons was prominent, while systemic arteriosclerosis was unremarkable assessed by the ankle-brachial index, carotid ultrasound imaging and funduscopic study. HGPS patients have marked loss and functional defects in vascular smooth muscle cells, leading to the vulnerability to circulatory stress. Symmetrical stenosis of siphons might occur as a distinctive cerebral vasculopathy of HGPS, rather than simple vascular senescence. Peripheral blood study on LMNA G608G and telomere length could screen progerias in infancy for early therapeutic intervention. PMID:23141186

  19. Bilateral alien hand syndrome in cerebrovascular disease: CT, MR, CT angiography, and 99mTc-HMPAO-SPECT findings.

    PubMed

    Serrano-Vicente, Justo; Duran-Barquero, Carmen; Garcia-Bernardo, Lucia; Dominguez-Grande, Maria Luz; Infante-Torre, Jose Rafael; Rayo-Madrid, Juan Ignacio

    2015-03-01

    We report a 65-year-old man with a right cerebral infarction that occurred 15 years ago and a residual left hemiparesis that began with progressive contralateral hemiparesis. During the hospitalization, the patient developed a bilateral alien hand syndrome. Urgent CT, MR, CT angiography, and brain perfusion SPECT were performed that revealed an old right cerebral infarction and a new ischemic lesion in left parietal lobe and adjacent brain territories. PMID:25546190

  20. A novel missense mutation of NSDHL in an unusual case of CHILD syndrome showing bilateral, almost symmetric involvement.

    PubMed

    König, Arne; Happle, Rudolf; Fink-Puches, Regina; Soyer, Hans Peter; Bornholdt, Dorothea; Engel, Hartmut; Grzeschik, Karl-Heinz

    2002-04-01

    The CHILD syndrome (MIM 308050), an acronym for congenital hemidysplasia with ichthyosiform nevus and limb defects, is an X-linked dominant trait with lethality for male embryos. Recently, we elucidated the underlying gene defect by demonstrating point mutations in NSDHL (NAD[P]H steroid dehydrogenase-like protein) at Xq28 in 6 patients with classic CHILD syndrome. The most striking clinical feature is an inflammatory nevus that usually shows a unique lateralization with strict midline demarcation. Ipsilateral defects involve all skeletal structures and internal organs such as the brain, the lung, the heart, or the kidney. As an exception to this rule, in some cases the CHILD nevus may occur in a more or less bilateral distribution. In 1997 Fink-Puches et al described a case of CHILD nevus with an almost symmetric arrangement. To test the correctness of the diagnosis, we now examined blood lymphocytes of this patient by single-strand conformation analysis and genomic sequencing. We identified a novel missense mutation in NSDHL that potentially may impair protein function. We conclude that a diagnosis of CHILD syndrome can be based on clinical features such as the highly characteristic morphology of the CHILD nevus. A symmetric distribution of this nevus can exceptionally be seen in patients with CHILD syndrome, and this bilateral involvement should not mislead the clinician to any other diagnosis. Apparently, the effect of random X-inactivation is responsible for different patterns of cutaneous involvement in female carriers of NSDHL mutations. PMID:11907515

  1. Perisylvian Functional Connectivity during Processing of Sentential Negation.

    PubMed

    Bahlmann, Jörg; Mueller, Jutta L; Makuuchi, Michiru; Friederici, Angela D

    2011-01-01

    Every language has the means to reverse the truth value of a sentence by using specific linguistic markers of negation. In the present study we investigated the neural processing costs afforded by the construction of meaning in German sentences containing negation in different clause types. We studied negations within and across clause boundaries as well as single and double negations. Participants read German sentences comprising of affirmations, single negations in the main or in the subordinate clause, or double negations. As a result, we found a network including the left inferior frontal gyrus (pars triangularis, BA 45), and the left inferior parietal gyrus (BA 40) to be activated whenever negations in the main clause had to be processed. Additionally, we found increased functional coupling between the left pars triangularis (BA 45), left pars opercularis (BA 44), left SMA (BA 6), and left superior temporal gyrus (BA 42) during the processing of main clause negations. The study shows that in order to process negations that require semantic integration across clause boundaries left BA 45 interplays with other areas that have been related to language processing and/or the processing of cognitive demands and logical/conditional reasoning. Thus, the results indicate that the left perisylvian language network synchronizes in order to resolve negations, in particular, whenever requirements on meaning integration are enhanced. PMID:21687464

  2. Extensive Bilateral Lemierre Syndrome due to Methicillin-Resistant Staphylococcus epidermidis in a Patient with Lung Adenocarcinoma

    PubMed Central

    Choi, Bo Mi; Son, Seong Wan; Park, Chan Kwon; Lee, Sang-Hoon

    2015-01-01

    Lemierre syndrome (LS) is a septic thrombophlebitis of the internal jugular vein (IJV) following an oropharyngeal infection. LS is commonly caused by normal anaerobic flora and treated with appropriate antibiotics and anticoagulation therapy. Although the incidence of disease is very rare, 15% cases of LS are fatal even in the antibiotic era because of disseminated septic thromboemboli. We reported a case of extensive bilateral LS due to methicillin-resistant Staphylococcus epidermidis in a 63-year-old female with lung adenocarcinoma. Initial examination revealed a retropharyngeal abscess; hence, intravenous ceftriaxone and steroid were initiated empirically. However, pulmonary thromboembolism developed and methicillin-resistant S. epidermidis was identified in the bacterial culture. Despite intensive antibiotic and anticoagulation therapies, extensive septic thrombophlebitis involving the bilateral IJV and superior vena cava developed. Adjunctive catheter-directed thrombolysis and superior vena cava stenting were performed and the patient received antibiotic therapy for an additional 4 weeks, resulting in complete recovery. PMID:26175788

  3. Extensive Bilateral Lemierre Syndrome due to Methicillin-Resistant Staphylococcus epidermidis in a Patient with Lung Adenocarcinoma.

    PubMed

    Choi, Bo Mi; Son, Seong Wan; Park, Chan Kwon; Lee, Sang-Hoon; Yoon, Hyung Kyu

    2015-07-01

    Lemierre syndrome (LS) is a septic thrombophlebitis of the internal jugular vein (IJV) following an oropharyngeal infection. LS is commonly caused by normal anaerobic flora and treated with appropriate antibiotics and anticoagulation therapy. Although the incidence of disease is very rare, 15% cases of LS are fatal even in the antibiotic era because of disseminated septic thromboemboli. We reported a case of extensive bilateral LS due to methicillin-resistant Staphylococcus epidermidis in a 63-year-old female with lung adenocarcinoma. Initial examination revealed a retropharyngeal abscess; hence, intravenous ceftriaxone and steroid were initiated empirically. However, pulmonary thromboembolism developed and methicillin-resistant S. epidermidis was identified in the bacterial culture. Despite intensive antibiotic and anticoagulation therapies, extensive septic thrombophlebitis involving the bilateral IJV and superior vena cava developed. Adjunctive catheter-directed thrombolysis and superior vena cava stenting were performed and the patient received antibiotic therapy for an additional 4 weeks, resulting in complete recovery. PMID:26175788

  4. Bilateral lid/brow elevation procedure for severe ptosis in Kearns-Sayre syndrome, a mitochondrial cytopathy

    PubMed Central

    Sebastiá, Roberto; Fallico, Ester; Fallico, Matteo; Fortuna, Eduardo; Lessa, Sergio; Neto, Guilherme Herzog

    2015-01-01

    Background The purpose of this work was to determine the effectiveness and possible complications encountered with bilateral fascia lata lid suspension used to correct blepharoptosis in patients with Kearns-Sayre syndrome. Methods This was a retrospective study of seven patients with Kearns-Sayre syndrome who had a minimum of 1 year of follow-up. A bilateral fascia lata sling was used to correct the ptosis. Preoperative and postoperative measurements of the vertical lid fissure width (VFW) and marginal reflex distance (MRD) were performed. The Student’s t-test was used to analyze the results. Results The mean preoperative VFW and MRD measurements were 4±2.45 mm and 0.14±0.92 mm, respectively. The mean postoperative VFW and MRD measurements were 7.71±1.85 mm, and 2.86±1.69 mm, respectively. All preoperative and postoperative values were considered to be statistically significant (P<0.01). Adequate elevation of the lids was obtained in all patients, both functionally and aesthetically. All of the patients showed a mild symmetric postoperative inferior version lagophthalmos, and one patient developed corneal ulceration and scarring due to corneal exposure and a weak Bell’s phenomenon. Conclusion The surgical technique described to correct the blepharoptosis found in patients with Kearns-Sayre syndrome was found to be efficient and relatively safe. The correction should be conservative to decrease the risk of postoperative corneal damage that occurred in one patient. PMID:25565765

  5. Bilateral ovarian carcinoma with bilateral uveal melanoma.

    PubMed Central

    Mullaney, J; Mooney, D; O'Connor, M; McDonald, G S

    1984-01-01

    A case of bilateral uveal melanoma in a 60-year-old woman in association with primary bilateral ovarian carcinoma is described. This is the first case in which ultrastructural studies have been performed on the ocular tumours. Seven previously described cases are summarised, and the extreme rarity of such reports would suggest that this may indeed be a new syndrome. Images PMID:6704361

  6. Bilateral multiple sialolithiasis of the parotid gland in a patient with Sjögren’s syndrome

    PubMed Central

    Konstantinidis, I; Paschaloudi, S; Triaridis, S; Fyrmpas, G; Sechlidis, S; Constantinidis, J

    2007-01-01

    Summary The presence of multiple calculi in the major salivary glands is an uncommon finding. Sjögren’s syndrome is a chronic autoimmune disease characterized by lymphocyte-mediated destruction of the exocrine glands. The case is presented of a 49-year-old female with Sjögren’s syndrome found to have bilateral multiple sialolithiasis in the parenchyma of the parotid glands. The patient presented with a right sided painful inflamed swelling of the parotid region. Even though she had been diagnosed with primary Sjögren’s syndrome 3 years prior to admission, she did not report any previous episode of sialadenitis. Full blood count showed leukocytosis (white blood cells = 14,900/106L) with neutrophilia (75%). Radiological assessment included ultrasound and computed tomography scan of the parotids which demonstrated intra-parenchymal multiple calculi of both parotid glands and obstruction of the right Stensen’s duct. The patient was treated with intravenous antibiotics and anti-inflammatory drugs. On the second day of hospitalisation, she reported spontaneous extrusion of a calculus during massage of the gland, with immediate relief of symptoms. In patients with Sjögren’s syndrome and radiological findings of calculi in the major salivary glands, close observation is mandatory for better control of recurrent sialadenitis and early recognition of mucosa-associated lymphoid tissue lymphomas. PMID:17601211

  7. Good visual outcome in an immunocompromised patient with bilateral acute retinal necrosis syndrome: A case report

    PubMed Central

    Marrocos de Aragão, Ricardo E.; Barreira, Ieda M.A.; Arrais, Barbara L.A.; Pereira, Leidiane A.; Ramos, Carine S.

    2013-01-01

    Acute retinal necrosis (ARN) is an uncommon necrotizing, fulminant retinopathy caused by the herpes simplex virus types 1 or 2 or by the varicella zoster vírus with visually devastating consequences. Generally it occurs in patients who are systemically healthy, but occasionally occurs in immunocompromised host. We report a case of bilateral ARN in a patient with AIDS with a good final visual outcome. PMID:25278806

  8. White matter microstructural differences linked to left perisylvian language network in children with dyslexia

    PubMed Central

    Rimrodt, Sheryl L.; Peterson, Daniel J.; Denckla, Martha B.; Kaufmann, Walter E.; Cutting, Laurie E.

    2009-01-01

    Studies of dyslexia using diffusion tensor imaging (DTI) have reported fractional anisotropy (FA) differences in left inferior frontal gyrus (LIFG) and left temporo-parietal white matter, suggesting that impaired reading is associated with atypical white matter microstructure in these regions. These anomalies might reflect abnormalities in the left perisylvian language network, long implicated in dyslexia. While DTI investigations frequently report analyses on multiple tensor-derived measures (e.g., FA, orientation, tractography), it is uncommon to integrate analyses to examine the relationships between atypical findings. For the present study, semi-automated techniques were applied to DTI data in an integrated fashion to examine white matter microstructure in 14 children with dyslexia and 17 typically developing readers (ages 7-16 years). Correlations of DTI metrics (FA and fiber orientation) to reading skill (accuracy and speed) and to probabilistic tractography maps of the left perisylvian language tracts were examined. Consistent with previous reports, our findings suggest FA decreases in dyslexia in LIFG and left temporo-parietal white matter. The LIFG FA finding overlaps an area showing differences in fiber orientation in an anterior left perisylvian language pathway. Additionally,a positive correlation of FA to reading speed was found in a posterior circuit previously associated with activation on functional imaging during reading tasks. Overall, integrating results from several complementary semi-automated analyses reveals evidence linking atypical white matter microstructure in dyslexia to atypical fiber orientation in circuits implicated in reading including the left perisylvian language network. PMID:19682675

  9. A case of WAGR syndrome in association with developmental glaucoma requiring bilateral Baerveldt glaucoma implants and subsequent tube repositioning

    PubMed Central

    Akagi, Tadamichi; Yoshikawa, Munemitsu; Nakanishi, Hideo; Yoshimura, Nagahisa

    2015-01-01

    Glaucoma drainage device implantation is efficacious for the treatment of pediatric glaucoma patients when multiple angle surgeries fail. However, tube touching of the corneal endothelium is one of the major postoperative complications to deal with. A 15-month-old male patient with Wilms’ tumor, aniridia, genitourinary anomalies, and mental retardation (WAGR) syndrome was diagnosed with bilateral developmental glaucoma. He underwent Baerveldt glaucoma implant (BGI) surgeries in both eyes after multiple failed trabeculotomies. The tube in his right eye was touching the cornea 15 months after BGI surgery. To avoid corneal endothelium damage, BGI tube repositioning with scleral fixation was performed without serious complications. The bilateral BGI surgeries achieved successful intraocular pressure reduction for over 2 years and tube repositioning with scleral fixation of BGI tube was successful for BGI tube malposition. Although careful attention to intraocular pressure and tube malposition is essential after glaucoma drainage device implantation, especially in pediatric cases, BGI surgery is effective in the management of developmental glaucoma following unsuccessful angle surgeries. PMID:26109842

  10. Synchronous bilateral breast carcinoma in a patient with cowden syndrome: a case report with morphologic, immunohistochemical and genetic analysis.

    PubMed

    Peiró, Gloria; Adrover, Encarna; Guijarro, Jaime; Ballester, Irene; Jimenez, M José; Planelles, María; Catasús, Lluis

    2010-01-01

    Synchronous bilateral breast carcinoma (SBBC) and early onset are important characteristics of hereditary cases. The lifetime risk for breast carcinoma in Cowden syndrome (CS) is estimated to be 25-50%. We reported a 44-year-old woman presenting SBBC and characteristic mucocutaneous lesions of CS, confirmed by PTEN gene mutation analysis. Bilateral modified mastectomy and axillary dissection were performed. Histopathologic examination revealed a moderate-differentiated invasive ductal carcinoma with mixed features of luminal A immunophenotype (Estrogen and/or Progesterone Receptors >50% and/or Ki67 < 30% of positive cells). The skin lesions showed the characteristic findings of tricholemmoma. Lack of PTEN expression was observed in all specimens. Sequencing analysis confirmed the presence of PTEN splice-acceptor site mutation in intron 8 (c.1027-2A>G), a germline mutation which had not been previously reported in CS. The patient received adjuvant chemotherapy and tamoxifen for 5 years. After 5 years of follow-up, she persists recurrence-free. SBBC with early onset suggests a hereditary predisposition. Thus, analysis of PTEN expression abnormality, easily assessed by immunohistochemistry, may be of clinical value to screen those patients with CS. PMID:19968660

  11. Staged, Open, No-Ischemia Nephron-Sparing Surgery for Bilateral-Multiple Kidney Tumors in a Patient with Birt-Hogg-Dubé Syndrome

    PubMed Central

    Tefekli, Ahmet; Akkaya, Ay?e Deniz; Peker, Kamil; Gümü?, Terman; Vural, Metin; Cezayirli, Fatin; Musaoglu, Ahmet; Esen, Tar?k

    2012-01-01

    Hereditary kidney cancer patients with bilateral multiple kidney tumors represent challenges in the era of rapidly growing minimal invasive treatment techniques. Birt-Hogg-Dubé Syndrome (BHDS) is an autosomal dominant genodermatosis characterized by a triad of benign skin tumors (fibrofolliculomas, trichodiscomas, acrochordons) together with an increased risk of developing malignant renal tumors and pulmonary disease such as pneumothoraces and multiple lung cysts. The morbidity and mortality of the affected patients is determined by the presence of the kidney tumors, which tend to be multifocal and bilateral, as observed in other hereditary kidney cancer syndromes like von Hippel-Lindau disease, familial leiomyomatosis, and hereditary papillary renal cell carcinoma. Herein, a patient with BHDS, presenting with synchronous bilateral multiple kidney tumors, is reported. The report describes the management of kidney tumors with two-stage open nephron-sparing surgery in which the nonvascular clamping technique was utilized. PMID:22611408

  12. Staged, open, no-ischemia nephron-sparing surgery for bilateral-multiple kidney tumors in a patient with birt-hogg-dubé syndrome.

    PubMed

    Tefekli, Ahmet; Akkaya, Ay?e Deniz; Peker, Kamil; Gümü?, Terman; Vural, Metin; Cezayirli, Fatin; Musaoglu, Ahmet; Esen, Tar?k

    2012-01-01

    Hereditary kidney cancer patients with bilateral multiple kidney tumors represent challenges in the era of rapidly growing minimal invasive treatment techniques. Birt-Hogg-Dubé Syndrome (BHDS) is an autosomal dominant genodermatosis characterized by a triad of benign skin tumors (fibrofolliculomas, trichodiscomas, acrochordons) together with an increased risk of developing malignant renal tumors and pulmonary disease such as pneumothoraces and multiple lung cysts. The morbidity and mortality of the affected patients is determined by the presence of the kidney tumors, which tend to be multifocal and bilateral, as observed in other hereditary kidney cancer syndromes like von Hippel-Lindau disease, familial leiomyomatosis, and hereditary papillary renal cell carcinoma. Herein, a patient with BHDS, presenting with synchronous bilateral multiple kidney tumors, is reported. The report describes the management of kidney tumors with two-stage open nephron-sparing surgery in which the nonvascular clamping technique was utilized. PMID:22611408

  13. Bilateral granulosa cell tumors: a novel malignant manifestation of multiple endocrine neoplasia 1 syndrome found in a patient with a rare menin in-frame deletion

    PubMed Central

    Hall, Michael J; Innocent, Julie; Rybak, Christina; Veloski, Colleen; Scott, Walter J; Wu, Hong; Ridge, John A; Hoffman, John P; Borghaei, Hossein; Turaka, Aruna; Daly, Mary B

    2015-01-01

    Introduction Multiple endocrine neoplasia 1 (MEN1) is a cancer syndrome resulting from mutations of the MEN1 gene. The syndrome is characterized by neoplasia of the parathyroid and pituitary glands, and malignant tumors of the endocrine pancreas. Other manifestations include benign lipomas, angiofibromas, and carcinoid tumors commonly originating in the colon, thymus, and lung. This is the first report of MEN1 syndrome manifesting as bilateral granulosa cell ovarian tumors, and which is associated with a rare intronic mutation of the MEN1 gene. Case report A 41-year-old woman presented with abdominal pain, increasing abdominal girth, and dysmenorrhea. Ultrasound demonstrated enlarged ovaries and uterine fibroids. After an exploratory laparotomy, she subsequently underwent bilateral salpingo–oophorectomy with hysterectomy where the pathology revealed bilateral cystic granulosa cell tumors of the ovaries. Additional workup including computed tomography imaging discovered a thymic mass, which the pathology showed was malignant, along with a pancreatic mass suspicious for a neuroendocrine tumor. Hyperparathyroidism was also discovered and was found to be secondary to a parathyroid adenoma. Genetic testing revealed an exceedingly rare mutation in the MEN1 gene (c.654 + 1 G>A). Discussion Mutations of the menin gene leading to MEN1 syndrome are classically nonsense or missense mutations producing a dysfunctional protein product. Recently, researchers described a novel mutation of MEN1 (c.654 + 1 G>A) in a male proband meeting the criteria for clinical MEN1 syndrome. Functional analysis performed on the stable mutant protein showed selective disruption of the transforming growth factor beta signaling pathway, yet it maintained its wild-type ability to inhibit nuclear factor kappa B and to suppress JunD transcriptional activity. Conclusion To our knowledge, this is the first report of MEN1 syndrome associated with bilateral granulosa cell malignancy. We postulate that this presentation may be due to the novel menin gene mutation recently described. PMID:25733923

  14. Remission of severe restless legs syndrome and periodic limb movements in sleep after bilateral excision of multiple foot neuromas: a case report

    PubMed Central

    2010-01-01

    Introduction Restless legs syndrome is a sensorimotor neurological disorder characterized by an urge to move the legs in response to uncomfortable leg sensations. While asleep, 70 to 90 percent of patients with restless legs syndrome have periodic limb movements in sleep. Frequent periodic limb movements in sleep and related brain arousals as documented by polysomnography are associated with poorer quality of sleep and daytime fatigue. Restless legs syndrome in middle age is sometimes associated with neuropathic foot dysesthesias. The causes of restless legs syndrome and periodic limb movements in sleep are unknown, but the sensorimotor symptoms are hypothesized to originate in the central nervous system. We have previously determined that bilateral forefoot digital nerve impingement masses (neuromas) may be a cause of both neuropathic foot dysesthesias and the leg restlessness of restless legs syndrome. To the best of our knowledge, this case is the first report of bilateral foot neuromas as a cause of periodic limb movements in sleep. Case presentation A 42-year-old Caucasian woman with severe restless legs syndrome and periodic limb movements in sleep and bilateral neuropathic foot dysesthesias was diagnosed as having neuromas in the second, third, and fourth metatarsal head interspaces of both feet. The third interspace neuromas represented regrowth (or 'stump') neuromas that had developed since bilateral third interspace neuroma excision five years earlier. Because intensive conservative treatments including repeated neuroma injections and various restless legs syndrome medications had failed, radical surgery was recommended. All six neuromas were excised. Leg restlessness, foot dysesthesias and subjective sleep quality improved immediately. Assessment after 18 days showed an 84 to 100 percent reduction of visual analog scale scores for specific dysesthesias and marked reductions of pre-operative scores of the Pittsburgh sleep quality index, fatigue severity scale, and the international restless legs syndrome rating scale (36 to 4). Polysomnography six weeks post-operatively showed improved sleep efficiency, a marked increase in rapid eye movement sleep, and marked reductions in hourly rates of both periodic limb movements in sleep with arousal (135.3 to 3.3) and spontaneous arousals (17.3 to 0). Conclusion The immediate and near complete remission of symptoms, the histopathology of the excised tissues, and the marked improvement in polysomnographic parameters documented six weeks after surgery together indicate that this patient's severe restless legs syndrome and periodic limb movements in sleep was of peripheral nerve (foot neuroma) origin. Further study of foot neuromas as a source of periodic limb movements in sleep and as a cause of sleep dysfunction in patients with or without concomitant restless legs syndrome, is warranted. PMID:20849622

  15. Bilateral Symmetrical Idiopathic Necrotizing Encephalopathy: A New Syndrome in Sprague-Dawley Rats.

    PubMed

    De Jonghe, Sandra; Abbott, David; Vinken, Petra; Moesen, Esther; Feyen, Bianca; Lammens, Lieve; Vynckier, An; De Lahunta, Alexander

    2015-12-01

    This article describes the occurrence of a bilaterally symmetrical encephalopathy in Sprague-Dawley rats, which occurred over the period 2005 to 2012 in our laboratory in both untreated control rats and rats treated with different pharmacologically active compounds. The acute brain lesions consisted of degeneration/necrosis in the ventral areas of the brain mostly with little inflammatory response; in the more rare chronic cases there were numerous lipid-laden macrophages. The areas most consistently affected were the crus cerebri, the ventral midbrain, the pyramids, and the internal capsule. Other areas less frequently affected were the mammillary bodies, the fimbria, the olfactory tubercles, the optic tracts, and the ventral hippocampus. All available data, including clinical signs, gross pathology, clinical pathology, diet, breeding, and housing were collected and are presented. Our investigations did not elucidate the pathogenesis of the lesions, although the infarction-type changes are suggestive of a vascular etiology. To our knowledge, this particular lesion with its consistent distribution pattern has not been reported in the rat literature and its publication is therefore important to the toxicological pathology community, because an unbalanced group distribution in a toxicology study could potentially confound the safety assessment of a compound. PMID:26511844

  16. Bilateral Sturge-Weber and Phakomatosis Pigmentovascularis with Glaucoma, an Overlap Syndrome

    PubMed Central

    Patil, Bharat; Nayak, Bhagabat; Sharma, Reetika; Kumari, Sadhana; Dada, Tanuj

    2015-01-01

    Aim. To report a case of bilateral Sturge-Weber and Phakomatosis pigmentovascularis with secondary glaucoma in a child. Method. Case report. Results. A 4-year-old male child was referred to us for control of intraocular pressure (IOP). Sleeping IOP was 36 mm Hg in right eye and 28 mm Hg in the left eye. The sclera of both the eyes showed bluish black pigmentation—melanosis bulbi. Fundus examination of both eyes showed diffuse choroidal hemangiomas with glaucomatous cupping. Nevus flammeus was present on both sides of face along all the 3 divisions of trigeminal nerve with overlying hypertrophy of skin and on left forearm. Nevus fuscocaeruleus was present on upper trunk. All skin lesions were present since birth and were stationary in nature. CT scan of head revealed left-sided cerebral atrophy. Intraocular pressure was controlled after treatment with topical antiglaucoma medications. Pulsed Dye Laser has been advised by dermatologist for skin lesions. Patient has been advised for regular follow-up. Conclusion. The two overlapping dermatological disorders and their association with glaucoma are a rare entity. Management should be targeted both for dermatological and eye conditions. PMID:26064732

  17. Bilateral Pneumothoraces Following BiV ICD Placement: A Case of Buffalo Chest Syndrome

    PubMed Central

    Rali, Aniket S.; Manyam, Harish

    2015-01-01

    Patient: Female, 73 Final Diagnosis: Buffalo chest syndrome Symptoms: — Medication: — Clinical Procedure: Bi-ventricular ICD Specialty: Cardiology Objective: Rare disease Background: Contralateral pneumothorax following device implantation on the left side has been reported in a few cases. The majority of contralateral pneumothoraces showed evidence of atrial perforation on computed tomography (CT), echocardiography, or chest x-rays and required lead revision. To the best of our knowledge there is only 1 other reported case of contralateral pneumothorax without evidence of macro-displacement of the atrial lead. In that case the patient experienced a right-sided pneumothorax on day 1 after undergoing repositioning of the atrial lead. Case Report: The current case is unique on several accounts, including timing of the contralateral pneumothorax and no evidence of associated atrial lead perforation on device interrogation or CT imaging. Furthermore, the appearance of contralateral pneumothorax within 8 hours of clamping of the ipsilateral chest tube argues in favor of a pleuro-pleural fistula. Conclusions: The term ‘buffalo chest’ refers to a single pleural space, with no anatomical separation of the 2 hemithoraces, as seen in an American buffalo or bison. We believe this to be a case of buffalo chest syndrome. PMID:26431396

  18. Fibrolipomatous hamartoma of the median nerve: A cause of acute bilateral carpal tunnel syndrome in a three-year-old child: A case report and comprehensive literature review

    PubMed Central

    Senger, Jenna-Lynn; Classen, Dale; Bruce, Garth; Kanthan, Rani

    2014-01-01

    A three-year-old boy was investigated for inexplicable incessant crying. On examination, his left wrist was mildly swollen (three to four months) and sensitive. Exploration and carpal tunnel decompression of the left wrist with incisional biopsy was performed for the presence of a fusiform swelling intimately associated with the median nerve. Histopathology revealed the presence of enlarged nerve bundles admixed with mature fat cells and diffuse fibroblastic proliferation. Three months later, he underwent urgent contralateral carpal tunnel decompression for a similar presentation. The final diagnosis was bilateral fibrolipomatous hamartoma (FLH) of the median nerves causing acute bilateral compression neuropathy. FLH of the median nerve is an extremely unusual cause of acute bilateral carpal tunnel syndrome in a young child presenting with ‘incessant crying’. A comprehensive review of FLH including epidemiology, etiology, clinical presentation, differential diagnosis, imaging, pathology, treatment and prognosis is discussed. PMID:25332651

  19. Bilateral motor and premotor cortex hypometabolism in a case of Mills syndrome.

    PubMed

    Scialò, Carlo; Morbelli, Silvia; Girtler, Nicola; Mandich, Paola; Mancardi, Giovanni Luigi; Caponnetto, Claudia; Nobili, Flavio

    2015-01-01

    Mills syndrome is a rare condition characterized by slowly progressive upper motor neuron-predominant hemiparesis, belonging to the motor neuron disorder spectrum. Predominantly unilateral primary degeneration of corticospinal pathways is the supposed underlying pathophysiological mechanism. By means of (18)F-Fluorodeoxyglucose Positron Emission Tomography, we found significant (Statistical Parametric Mapping, SPM, analysis versus controls, uncorrected p < 0.005 at voxel level, p < 0.05 at cluster level, corrected for multiple comparisons) hypometabolism in motor and premotor areas of both hemispheres, mainly contralateral to the limbs weakness in a patient with a 10-year history of slowly progressive left-sided hemiparesis. No significant grey matter loss was found on voxel based morphometry (SPM). This supports the hypothesis of a slowly progressive neurodegenerative process involving primary motor and premotor cortex. PMID:25967545

  20. Surgical-orthodontic management of bilateral multiple impactions in non-syndromic patient

    PubMed Central

    Agrawal, Jiwanasha Manish; Agrawal, Manish Suresh; Nanjannawar, Lalita Girish

    2013-01-01

    Several surgical and orthodontic treatment options are available to disimpact the impacted teeth. But the closed eruption technique has the best long-term prognosis. The tooth is surgically exposed, an attachment is bonded to it, flap is resutured over it and an orthodontic extrusive force is delivered to bring the tooth into occlusion. This case report presents a case with multiple impacted teeth in which no syndrome or systemic conditions were detected. A 20-year-old female patient reported for orthodontic treatment with chief complaint of multiple unerupted permanent teeth and retained primary teeth. Radiographic examination revealed impacted 14, 15, 24, 25, 33, 34, 43 and 44. Surgical exposure of the impacted teeth was done after extraction of retained primary teeth. Forced eruption of these teeth was done by applying traction with closed eruption technique. After careful treatment planning followed by guided eruption of impacted teeth, patient finished with a significantly improved functional and aesthetic result. PMID:23329714

  1. POST?SURGICAL REHABILITATION FOLLOWING FASCIOTOMIES FOR BILATERAL CHRONIC EXERTIONAL COMPARTMENT SYNDROME IN A SPECIAL FORCES SOLDIER: A CASE REPORT

    PubMed Central

    Miller, Joseph

    2013-01-01

    Background and Purpose: The etiology of Chronic Exertional Compartment Syndrome (CECS) is still unclear. The most commonly accepted theory suggests that it is a transient but debilitating process where there is an abnormally increased intracompartmental pressure during exercise/exertion due to non?compliant expansion of the osteofascial tissues. This most commonly occurs in the lower leg. Surgical intervention is often performed for symptom relief. However, there has been limited scientifically?based publication on post?surgical rehabilitation, especially with regard to return to function in the military population. The purpose of this case report is to demonstrate the utilization of a recommended post?operative protocol in a Special Forces Soldier. Case Description: The subject presented as a 25?year?old US Army Special Forces Soldier, who failed 8 weeks of conservative management for the diagnosis of CECS and subsequently underwent bilateral lower leg fasciotomies of the anterior and lateral compartments. Outcomes: Following recommended protocol guidelines he was progressed rapidly and within three months deployed without restriction or complications in a demanding combat zone. Discussion: This case report illustrates that following clearly defined, scientifically?based rehabilitation guidelines helped in addressing all of the involved structures and musculoskeletal dysfunctions that presented following the surgical intervention for CECS in a unique subject. Level of Evidence: 5 PMID:24175149

  2. Chin Wing Osteotomy for Bilateral Goldenhar Syndrome Treated by "Chin Wing Mentoplasty": Aesthetic, Functional, and Histological Considerations.

    PubMed

    Cortese, Antonio; Pantaleo, Giuseppe; Amato, Massimo; Claudio, Pier Paolo

    2015-07-01

    Various treatment strategies have been proposed to perform the aesthetic surgical correction of asymmetric deformities of the mandible. These techniques range from relatively simple to complex procedures including bimaxillary surgery associated with complex mandibular osteotomies. The authors describe a patient with grade III Goldenhar syndrome, treated by a "chin wing" mentoplasty as described by Triaca. These situations are classically treated with a bilateral sagittal splint osteotomy (BSSO) in combination with mentoplasty. However, because of a good occlusion with Angle's class I relation, slight imbalance of the occlusal plane with good function of the mouth opening, the patient refused to be treated with a BSSO, hence, a chin wing mentoplasty was performed. Size and stability of bone regeneration were evaluated by histological examination and dynamic-volume computed tomography (CT). Mature bone in the grafted areas was detected by histology and CT scan with stable results and a significant increase of facial aesthetics 1 year after surgery. The authors also demonstrated that the chin wing technique provided a little but significant breathing improvement as detected on CT scans and lateral X-ray cephalograms by measuring the breathing area. Chin wing mentoplasty is a moderately invasive technique that is able to improve the aesthetics of the face and patient breathing. PMID:26114537

  3. Chromosome 18q-Syndrome and 1p terminal duplication in a patient with bilateral vesico-ureteral reflux: case report and literature revision

    PubMed Central

    2013-01-01

    Background Vesico-ureteral reflux (VUR) is a dynamic event in which a retrograde flow of urine is present into the upper tracts. VUR may occur isolated or in association with other congenital abnormalities or as part of syndromic entities. We present a patient with a bilateral primary VUR, syndromic disease caused by a large deletion of 18q (18q21.3-qter) and terminal duplication of 1p (1p36.32-p36.33). Case report The patient was 8 years old female with a disease including moderate growth retardation, psychomotor retardation, facial dysmorphism, single umbilical artery, umbilical hernia, urachal remnant, bilateral congenital clubfeet and renal-urinary disease. Chromosomal analysis and Array-CGH revealed two heterozygous chromosomal rearrangements: 1p terminal duplication and de novo 18q terminal deletion. She referred to our clinic to evaluation of bilateral hydronephrosis and right renal cortex thinning. Voiding cystourethrography demonstrated bilateral grade IV VUR and dimercaptosuccinic acid renal scintigraphy confirmed right renal cortex thinning and showed a cortical uptake of 75% of the left kidney and 25% of the right kidney. The patient underwent ureterovesical reimplantation after failure of 3 endoscopic submeatal Deflux injections with VUR resolution. Conclusions This is the first report involving a patient with 18q-syndrome and contemporary presence of 1p chromosomal terminal duplication. The coexistence of two chromosomal rearrangements complicates the clinical picture and creates a chimeric disorder (marked by characteristics of both chromosomal anomalies). Kidney problems, primarily VUR is reported in 15% of patients affected by 18-q syndrome and no cases is reported in the literature regarding a correlation between VUR and 1p36 chromosomal duplication. PMID:23343423

  4. Parallel versus Serial Processing Dependencies in the Perisylvian Speech Network: A Granger Analysis of Intracranial EEG Data

    ERIC Educational Resources Information Center

    Gow, David W., Jr.; Keller, Corey J.; Eskandar, Emad; Meng, Nate; Cash, Sydney S.

    2009-01-01

    In this work, we apply Granger causality analysis to high spatiotemporal resolution intracranial EEG (iEEG) data to examine how different components of the left perisylvian language network interact during spoken language perception. The specific focus is on the characterization of serial versus parallel processing dependencies in the dominant…

  5. Beyond the amygdala: Linguistic threat modulates peri-sylvian semantic access cortices.

    PubMed

    Weisholtz, Daniel S; Root, James C; Butler, Tracy; Tüscher, Oliver; Epstein, Jane; Pan, Hong; Protopopescu, Xenia; Goldstein, Martin; Isenberg, Nancy; Brendel, Gary; LeDoux, Joseph; Silbersweig, David A; Stern, Emily

    2015-12-01

    In this study, healthy volunteers were scanned using functional magnetic resonance imaging (fMRI) to investigate the neural systems involved in processing the threatening content conveyed via visually presented "threat words." The neural responses elicited by these words were compared to those elicited by matched neutral control words. The results demonstrate that linguistic threat, when presented in written form, can selectively engage areas of lateral temporal and inferior frontal cortex, distinct from the core language areas implicated in aphasia. Additionally, linguistic threat modulates neural activity in visceral/emotional systems (amygdala, parahippocampal gyrus and periaqueductal gray), and at earlier stages of the visual-linguistic processing stream involved in visual word form representations (ventral occipitotemporal cortex). We propose a model whereby limbic activation modulates activity at multiple nodes along the visual-linguistic-semantic processing stream, including a perisylvian "semantic access network" involved in decoding word meaning, suggesting a dynamic interplay between feedforward and feedback processes. PMID:26575986

  6. Segregation of Lexical and Sub-Lexical Reading Processes in the Left Perisylvian Cortex

    PubMed Central

    Roux, Franck-Emmanuel; Durand, Jean-Baptiste; Jucla, Mélanie; Réhault, Emilie; Reddy, Marion; Démonet, Jean-François

    2012-01-01

    A fundamental issue in cognitive neuroscience is the existence of two major, sub-lexical and lexical, reading processes and their possible segregation in the left posterior perisylvian cortex. Using cortical electrostimulation mapping, we identified the cortical areas involved on reading either orthographically irregular words (lexical, “direct” process) or pronounceable pseudowords (sublexical, “indirect” process) in 14 right-handed neurosurgical patients while video-recording behavioral effects. Intraoperative neuronavigation system and Montreal Neurological Institute (MNI) stereotactic coordinates were used to identify the localization of stimulation sites. Fifty-one reading interference areas were found that affected either words (14 areas), or pseudo-words (11 areas), or both (26 areas). Forty-one (80%) corresponded to the impairment of the phonological level of reading processes. Reading processes involved discrete, highly localized perisylvian cortical areas with individual variability. MNI coordinates throughout the group exhibited a clear segregation according to the tested reading route; specific pseudo-word reading interferences were concentrated in a restricted inferior and anterior subpart of the left supramarginal gyrus (barycentre x?=??68.1; y?=??25.9; z?=?30.2; Brodmann’s area 40) while specific word reading areas were located almost exclusively alongside the left superior temporal gyrus. Although half of the reading interferences found were nonspecific, the finding of specific lexical or sublexical interferences is new evidence that lexical and sublexical processes of reading could be partially supported by distinct cortical sub-regions despite their anatomical proximity. These data are in line with many brain activation studies that showed that left superior temporal and inferior parietal regions had a crucial role respectively in word and pseudoword reading and were core regions for dyslexia. PMID:23226349

  7. Metachronous Bilateral Testicular Leydig-Like Tumors Leading to the Diagnosis of Congenital Adrenal Hyperplasia (Adrenogenital Syndrome)

    PubMed Central

    Vukina, Josip; Chism, David D.; Sharpless, Julie L.; Raynor, Mathew C.; Milowsky, Matthew I.; Funkhouser, William K.

    2015-01-01

    A 33-year-old male with a history of left testis Leydig cell tumor (LCT), 3-month status after left radical orchiectomy, presented with a rapidly enlarging (0.6 cm to 3.7 cm) right testicular mass. He underwent a right radical orchiectomy, sections interpreted as showing a similar Leydig cell-like oncocytic proliferation, with a differential diagnosis including metachronous bilateral LCT and metachronous bilateral testicular tumors associated with congenital adrenal hyperplasia (a.k.a. “testicular adrenal rest tumors” (TARTs) and “testicular tumors of the adrenogenital syndrome” (TTAGS)). Additional workup demonstrated a markedly elevated serum adrenocorticotropic hormone (ACTH) and elevated adrenal precursor steroid levels. He was diagnosed with congenital adrenal hyperplasia, 3β-hydroxysteroid dehydrogenase deficiency (3BHSD) type, and started on treatment. Metachronous bilateral testicular masses in adults should prompt consideration of adult presentation of CAH. Since all untreated CAH patients are expected to have elevated serum ACTH, formal exclusion of CAH prior to surgical resection of a testicular Leydig-like proliferation could be accomplished by screening for elevated serum ACTH. PMID:26351608

  8. Conduction aphasia as a function of the dominant posterior perisylvian cortex. Report of two cases.

    PubMed

    Quigg, Mark; Geldmacher, David S; Elias, W Jeff

    2006-05-01

    Assessment of eloquent functions during brain mapping usually relies on testing reading, speech, and comprehension to uncover transient deficits during electrical stimulation. These tests stem from findings predicted by the Geschwind-Wernicke hypothesis of receptive and expressive cortices connected by white matter tracts. Later work, however, has emphasized cortical mechanisms of language function. The authors report two cases that demonstrate that conduction aphasia is cortically mediated and can be inadequately assessed if not specifically evaluated during brain mapping. To determine the distribution of language on the dominant cortex, electrical cortical stimulation was performed in two cases by using implanted subdural electrodes during brain mapping before epilepsy surgery. A transient isolated deficit in repetition of language was reported during stimulation of the posterior portion of the dominant superior temporal gyrus in one patient and during stimulation of the supramarginal gyrus in the other patient. These cases demonstrate a localization of language repetition to the posterior perisylvian cortex. Brain mapping of this region should include assessment of verbal repetition to avoid potential deficits resembling conduction aphasia. PMID:16703895

  9. Bilateral dacryoadenitis

    PubMed Central

    Derr, Charlotte; Shah, Ankit

    2012-01-01

    Acute dacryoadenitis is an uncommon condition that involves inflammation of the lacrimal gland. In rare instances, dacryoadenitis may be bilateral. A delay in proper treatment of an otherwise simple case of dacryoadenitis may lead to significant soft tissue morbidity such as cellulitis, lacrimal gland abscess, or orbital abscess. We report the case of a 24-year-old male who presented to the emergency department with acute bilateral dacryoadenitis. The patient's symptoms did not respond to oral antibiotics and he subsequently required admission for intravenous antibiotics. During his hospitalization the patient had diagnostic testing to try to determine the etiology for his symptoms. The unique aspects of managing a case of bilateral dacryoadenitis as well as treatment recommendations are discussed in this case report. PMID:22416166

  10. Bilateral gustatory sweating in the submandibular region after bilateral neck dissection successfully treated with botulinum toxin.

    PubMed

    Philouze, Pierre; Vertu, Delphine; Ceruse, Philippe

    2014-10-01

    Gustatory sweating (or chorda tympani syndrome) in the submandibular region is a rare complication of cervical surgery. We report a 66-year-old patient who had been referred for the management of a squamous cell carcinoma of the mobile tongue. She was treated by bilateral neck dissection and bilateral resection of the submandibular glands. Bilateral gustatory sweating in the submandibular region developed 10 years later and was successfully treated with bilateral injections of botulinum toxin type A. PMID:24996697

  11. Bilateral symmetrical adrenal hypermetabolism on FDG PET/CT due to Cushing syndrome in well differentiated neuroendocrine carcinoma.

    PubMed

    Aktas, G E; Soyluoglu Demir, S; Sarikaya, A

    2016-01-01

    The (18)F-FDG PET/CT scan has been suggested for whole-body imaging to identify ectopic adrenocorticotrophic hormone secreting tumours, but there are some challenges involved. The case of a patient is presented, who was admitted with the pre-diagnosis of ectopic ACTH syndrome. On the CT, a nodular lesion was detected in the medial segment of the right lung. The FDG uptake of the lesion seemed to be increased visually, but was not pathological quantitatively (SUVmax: 1.8) on the PET/CT. There was also diffuse increased uptake (SUVmax: 14.2) in the enlarged adrenal glands. The lesion was reported as a possible malignant lesion with low FDG affinity, such as a low grade neuroendocrine tumour, while the diffuse enlarged adrenal glands with high uptake were interpreted as diffusely hyperplasic, due to Cushing's syndrome. The patient was treated with a surgical wedge resection. The histopathological diagnosis confirmed that the tumour was a grade 1 well-differentiated neuroendocrine carcinoma. PMID:26522002

  12. Expression of vasopressin receptors in ACTH-independent macronodular bilateral adrenal hyperplasia causing Cushing's syndrome: molecular, immunohistochemical and pharmacological correlates.

    PubMed

    Louiset, E; Contesse, V; Groussin, L; Cartier, D; Duparc, C; Perraudin, V; Bertherat, J; Lefebvre, H

    2008-01-01

    Cortisol secretion in ACTH-independent macronodular adrenal hyperplasia (AIMAH) causing Cushing's syndrome can be controlled by illegitimate receptors. The aim of the present study was to characterize the molecular, immunohistochemical, and pharmacological profiles of vasopressin receptors in cells derived from three patients with AIMAH (H1-H3), in order to evaluate the role of ectopic vasopressin receptors in the physiopathology of hypercortisolism. Expression of mRNAs encoding the vasopressin receptor types (V(1a), V(1b), and V(2)) were analyzed by RT-PCR in adrenal tissues. The presence of V(1a) and V(2) receptors was studied by immunohistochemistry on adrenal sections. The pharmacological profiles of vasopressin receptors involved in the control of cortisol secretion were investigated using the V(1a) receptor antagonist SR49059 and the V(2) receptor agonist [deamino-Cys(1), Val(4), D-Arg(8)]-vasopressin on cultured cells. The V(1a) receptor protein was present and functional in H1 and H3 tissues, whereas the V(1b) receptor was not expressed in any of the tissues. RT-PCR experiments revealed that V(2) receptor mRNAs were detected in the three tissues. In contrast, immunohistochemical and cell incubation studies showed that the V(2) receptor was involved in the stimulatory effect of AVP on cortisol secretion in H1 and H2, but not in H3 cells. Taken together, these data show that expression of functional ectopic V(2) receptors and repression of eutopic V(1a) receptor can coexist in some hyperplastic corticosteroidogenic tissues. They also reveal that immunohistochemical and incubation studies are essential for the characterization of ectopic receptors actually involved in the control of cortisol secretion by AIMAHs. PMID:18180312

  13. A Case Report of Locked-in Syndrome Due to Bilateral Vertebral Artery Dissection After Cervical Spine Manipulation Treated by Arterial Embolectomy.

    PubMed

    Ke, Jiang-Qiong; Yin, Bo; Fu, Fang-Wang; Shao, Sheng-Min; Lin, Yan; Dong, Qi-Qiang; Wang, Xiao-Tong; Zheng, Guo-Qing

    2016-02-01

    Cervical spine manipulation (CSM) is a commonly spinal manipulative therapies for the relief of cervical spine-related conditions worldwide, but its use remains controversial. CSM may carry the potential for serious neurovascular complications, primarily due to vertebral artery dissection (VAD) and subsequent vertebrobasilar stroke. Here, we reported a rare case of locked-in syndrome (LIS) due to bilaterial VAD after CSM treated by arterial embolectomy.A 36-year-old right-handed man was admitted to our hospital with numbness and weakness of limbs after treating with CSM for neck for half an hour. Gradually, although the patient remained conscious, he could not speak but could communicate with the surrounding by blinking or moving his eyes, and turned to complete quadriplegia, complete facial and bulbar palsy, dyspnea at 4 hours after admission. He was diagnosed with LIS. Then, the patient was received cervical and brain computed tomography angiography that showed bilateral VAD. Aortocranial digital subtraction angiography showed vertebrobasilar thrombosis, blocking left vertebral artery, and stenosis of right vertebral artery. The patient was treated by using emergency arterial embolectomy and followed by antiplatelet therapy and supportive therapy in the intensive care unit and a general ward. Twenty-seven days later, the patient's physical function gradually improved and discharged but still left neurological deficit with muscle strength grade 3/5 and hyperreflexia of limbs.Our findings suggested that CSM might have potential severe side-effect like LIS due to bilaterial VAD, and arterial embolectomy is an important treatment choice. The practitioner must be aware of this complication and should give the patients informed consent to CSM, although not all stroke cases temporally related to SCM have pre-existing craniocervical artery dissection. PMID:26844510

  14. Comparison between Unilateral and Bilateral Ovarian Drilling in Clomiphene Citrate Resistance Polycystic Ovary Syndrome Patients: A Randomized Clinical Trial of Efficacy

    PubMed Central

    Zahiri Sorouri, Ziba; Sharami, Seyede Hajar; Tahersima, Zinab; Salamat, Fatemeh

    2015-01-01

    Background Laparoscopic ovarian drilling (LOD) is an alternative method to induce ovulation in polycystic ovary syndrome (PCOS) patients with clomiphene citrate (CC) resistant instead of gonadotropins. This study aimed to compare the efficacy of unilateral LOD (ULOD) versus bilateral LOD (BLOD) in CC resistance PCOS patients in terms of ovulation and pregnancy rates. Materials and Methods In a prospective randomized clinical trial study, we included 100 PCOS patients with CC resistance attending to Al-Zahra Hospital in Rasht, Guilan Province, Iran, from June 2011 to July 2012. Patients were randomly divided into two ULOD and BLOD groups with equal numbers. The clinical and biochemical responses on ovulation and pregnancy rates were assessed over a 6-month follow-up period. Results Differences in baseline characteristics of patients between two groups prior to laparoscopy were not significant (p>0.05). There were no significant differences between the two groups in terms of clinical and biochemical responses, spontaneous menstruation (66.1 vs. 71.1%), spontaneous ovulation rate (60 vs. 64.4%), and pregnancy rate (33.1 vs. 40%) (p>0.05). Following drilling, there was a significant decrease in mean serum concentrations of luteinizing hormone (LH) (p=0.001) and testosterone (p=0.001) in both the groups. Mean decrease in serum LH (p=0.322) and testosterone concentrations (p=0.079) were not statistically significant between two groups. Mean serum level of follicle stimulating hormone (FSH) did not change significantly in two groups after LOD (p>0.05). Conclusion Based on results of this study, ULOD seems to be equally efficacious as BLOD in terms of ovulation and pregnancy rates (Registration Number: IRCT138903291306N2). PMID:25918587

  15. Moxifloxacin and bilateral acute iris transillumination.

    PubMed

    Knape, Robert M; Sayyad, Fouad E; Davis, Janet L

    2013-01-01

    Recent publications have alerted clinicians to a syndrome of uveitic transilluminating iris depigmentation associated with systemic fluoroquinolones and other antibiotics. Bilateral acute iris transillumination, which is associated with loss of the iris pigment epithelium and results in iris transillumination, differs from the previously described bilateral acute depigmentation of the iris, which is associated with atrophy of the iris stroma without transillumination. We present a case of fluoroquinolone-associated uveitis with anterior segment optical coherence tomography imaging to highlight some observations about this syndrome. We interpret pharmacokinetic data to help explain why oral, but not topical, moxifloxacin may cause fluoroquinolone-associated uveitis. PMID:23514193

  16. Transient neuropsychological abnormalities (including Gerstmann's syndrome) during cortical stimulation.

    PubMed

    Morris, H H; Lüders, H; Lesser, R P; Dinner, D S; Hahn, J

    1984-07-01

    A patient with intractable partial seizures was intensively studied before surgical removal of the epileptogenic focus. A subdural electrode array was surgically placed over the left temporoparietal cortex to better localize the epileptogenic focus and localize cortical function. In addition to speech and sensory findings, acalculia, agraphia, right-left confusion, and finger agnosia were transiently produced by electrical stimulation in the perisylvian area. These findings and their relationship to the controversy surrounding Gerstmann's syndrome are discussed. PMID:6539864

  17. Bilateral proptosis, dilatation of conjunctival veins, and papilloedema: a neuro-ophthalmological syndrome caused by arteriovenous malformation of the torcular herophili.

    PubMed Central

    Buchanan, T A; Harper, D G; Hoyt, W F

    1982-01-01

    A patient with an occipital dural arteriovenous malformation (AVM) developed signs of a carotid-cavernous sinus fistula and raised intracranial pressure. Bilateral transverse sinus occlusion associated with the AVM produced these signs by rerouting intracranial venous drainage anteriorly through the cavernous sinuses and superior ophthalmic veins. Angiography and computerised tomographic reformation techniques were used to define these extraordinary cranio-orbital venous pathways. Images PMID:7066271

  18. Does bilateral pulmonary banding in comparison to Norwood procedure improve outcome in neonates with hypoplastic left heart syndrome beyond second-stage palliation? A review of the current literature.

    PubMed

    Photiadis, Joachim; Sinzobahamvya, Nicodème; Hraška, Viktor; Asfour, Boulos

    2012-04-01

    Best evidence protocol was applied and the question addressed, whether there is an outcome advantage of a hybrid approach including bilateral pulmonary banding (BPB) and ductal stenting over Norwood procedure (NP) to maintain systemic circulation and to restrict pulmonary blood flow in neonates with hypoplastic left heart syndrome (HLHS). Out of 80 articles published since the original description of BPB in 2002, eight were selected to answer the clinical question. All studies were retrospective case-series after BLB, only three compared results with NP controls, but not in a randomized fashion. Only three chose high-risk patients. Mean follow-up interval was mentioned only in one study. Reported mean hospital, interstage, and stage 2 mortalities after BPB was 17.3, 12.4, and 25.3%, respectively. Estimated transplant-free survival beyond stage 2 palliation ranged from 7 to 80%, with no significant differences to NP controls in three studies. Reintervention rate was high, ranging from 20 to 42% at different sites. Due to study design or inhomogeneity of patient groups, current literature does not show evidence that bilateral pulmonary banding improves outcome with respect to survival beyond second-stage HLHS palliation. PMID:22215495

  19. Biomechanical comparison of lumbar spine instability between laminectomy and bilateral laminotomy for spinal stenosis syndrome – an experimental study in porcine model

    PubMed Central

    Tai, Ching-Lung; Hsieh, Pang-Hsing; Chen, Weng-Pin; Chen, Lih-Huei; Chen, Wen-Jer; Lai, Po-Liang

    2008-01-01

    Background The association of lumbar spine instability between laminectomy and laminotomy has been clinically studied, but the corresponding in vitro biomechanical studies have not been reported. We investigated the hypothesis that the integrity of the posterior complex (spinous process-interspinous ligament-spinous process) plays an important role on the postoperative spinal stability in decompressive surgery. Methods Eight porcine lumbar spine specimens were studied. Each specimen was tested intact and after two decompression procedures. All posterior components were preserved in Group A (Intact). In Group B (Bilateral laminotomy), the inferior margin of L4 lamina and superior margin of L5 lamina were removed, but the L4–L5 supraspinous ligament was preserved. Fenestrations were made on both sides. In Group C (Laminectomy) the lamina and spinous processes of lower L4 and upper L5 were removed. Ligamentum flavum and supraspinous ligament of L4–L5 were removed. A hydraulic testing machine was used to generate an increasing moment up to 8400 N-mm in flexion and extension. Intervertebral displacement at decompressive level L4–L5 was measured by extensometer Results The results indicated that, under extension motion, intervertebral displacement between the specimen in intact form and at two different decompression levels did not significantly differ (P > 0.05). However, under flexion motion, intervertebral displacement of the laminectomy specimens at decompression level L4–L5 was statistically greater than in intact or bilateral laminotomy specimens (P = 0.0000963 and P = 0.000418, respectively). No difference was found between intact and bilateral laminotomy groups. (P > 0.05). Conclusion We concluded that a lumbar spine with posterior complex integrity is less likely to develop segment instability than a lumbar spine with a destroyed anchoring point for supraspinous ligament. PMID:18547409

  20. Bilateral Molariform Mandibular Second Premolars

    PubMed Central

    Acharya, Sonu; Kumar Mandal, Pradip; Ghosh, Chiranjit

    2015-01-01

    Macrodontia is a rare dental anomaly that refers to teeth that appear larger than normal. Generalised macrodontia can be associated with certain medical conditions and syndromes. This case report presents clinical and radiographic findings of isolated bilateral macrodontia in a 14-year-old child. The patient was referred to the clinic with local crowding of maxillary and mandibular teeth. Radiographic findings revealed the presence of impacted macrodont mandibular second premolar on one side and erupted macrodontic premolar on the other side and their distinct morphological appearance, characterized by large, multitubercular, and molariform crowns and tapering, single roots. PMID:25685564

  1. Bilateral lower limb polio, chronic obstructive pulmonary disease, and recurrent acute coronary syndrome in a poly tobacco user: a preventable triple tragedy.

    PubMed

    Aggarwal, Ramesh; Dwivedi, Shridhar

    2012-01-01

    Non communicable diseases in most of the developing countries have surpassed the morbidity and mortality arising from communicable diseases. However there are people who continue to suffer from the residual disabilities of some communicable disease acquired at younger age like polio and develop non communicable diseases like COPD and coronary syndrome at older age primarily because of their tobacco habits. Both of these combination of communicable and non communicable diseases are preventable if timely preventive measures and healthy life style is adopted. This case highlights one such case where patient despite suffering from polio and restrictive lung disease started using tobacco and suffered from obstructive lung disease and coronary syndrome. PMID:23897524

  2. Somato-Motor Haptic Processing in Posterior Inner Perisylvian Region (SII/pIC) of the Macaque Monkey

    PubMed Central

    Ishida, Hiroaki; Fornia, Luca; Grandi, Laura Clara; Umiltà, Maria Alessandra; Gallese, Vittorio

    2013-01-01

    The posterior inner perisylvian region including the secondary somatosensory cortex (area SII) and the adjacent region of posterior insular cortex (pIC) has been implicated in haptic processing by integrating somato-motor information during hand-manipulation, both in humans and in non-human primates. However, motor-related properties during hand-manipulation are still largely unknown. To investigate a motor-related activity in the hand region of SII/pIC, two macaque monkeys were trained to perform a hand-manipulation task, requiring 3 different grip types (precision grip, finger exploration, side grip) both in light and in dark conditions. Our results showed that 70% (n?=?33/48) of task related neurons within SII/pIC were only activated during monkeys’ active hand-manipulation. Of those 33 neurons, 15 (45%) began to discharge before hand-target contact, while the remaining neurons were tonically active after contact. Thirty-percent (n?=?15/48) of studied neurons responded to both passive somatosensory stimulation and to the motor task. A consistent percentage of task-related neurons in SII/pIC was selectively activated during finger exploration (FE) and precision grasping (PG) execution, suggesting they play a pivotal role in control skilled finger movements. Furthermore, hand-manipulation-related neurons also responded when visual feedback was absent in the dark. Altogether, our results suggest that somato-motor neurons in SII/pIC likely contribute to haptic processing from the initial to the final phase of grasping and object manipulation. Such motor-related activity could also provide the somato-motor binding principle enabling the translation of diachronic somatosensory inputs into a coherent image of the explored object. PMID:23936121

  3. A rare case of Kearns-Sayre syndrome in a 17-year-old Venezuelan male with bilateral ptosis as the initial presentation.

    PubMed

    Leal, Mayela; Dhoble, Chetan; Lee, Julie; Lopez, Deinymar; Menéndez, Laura Simonne

    2016-03-01

    Kearns-Sayre syndrome (KSS) was first described in 1958 as 'a rare neuromuscular disorder defined by a characteristic triad of progressive external ophthalmoplegia, pigmentary retinopathy, atrioventricular block and cerebellar ataxia'. The prevalence rate of KSS is ∼1-3 per 100 000 individuals. Here, we report a rare case of a 17-year-old Venezuelan male with KSS. PMID:26949540

  4. 17 alpha-hydroxylase deficiency syndrome associated with bilateral streak gonads and impaired development of Müllerian ducts derivatives. Report of a case.

    PubMed

    de Gennes, J L; Jambart, S; Turpin, G; Elkik, F; Roger, M

    1982-05-01

    We report severe 17 alpha-hydroxylase deficiency in a 17 year-old black girl with 46,XX genotype. The diagnosis was suspected because of primary amenorrhoea, absence of sexual maturation, hypertension and hypokalaemia with renal potassium wasting. Endocrine investigation revealed low basal levels of all steroid hormones which require 17 alpha-hydroxylation for biosynthesis (i.e. glucocorticoids, androgens and oestrogens). No increase in their basal levels was seen following adrenal stimulation, indicating a severe block. Plasma concentrations of ACTH, FSH and LH were elevated as were progesterone, 11-deoxycorticosterone and corticosterone. Plasma renin activity was suppressed and aldosterone levels were very low. After 4 months of glucocorticoid replacement therapy, aldosterone was still low, even though the suppression was otherwise effective. Our case is unusual because bilateral streak gonads and impaired development of Müllerian ducts derivatives were also present. To our knowledge, a similar case has never been reported before. PMID:6287777

  5. A rare case of Kearns–Sayre syndrome in a 17-year-old Venezuelan male with bilateral ptosis as the initial presentation

    PubMed Central

    Leal, Mayela; Dhoble, Chetan; Lee, Julie; Lopez, Deinymar; Menéndez, Laura Simonne

    2016-01-01

    Kearns–Sayre syndrome (KSS) was first described in 1958 as ‘a rare neuromuscular disorder defined by a characteristic triad of progressive external ophthalmoplegia, pigmentary retinopathy, atrioventricular block and cerebellar ataxia’. The prevalence rate of KSS is ∼1–3 per 100 000 individuals. Here, we report a rare case of a 17-year-old Venezuelan male with KSS. PMID:26949540

  6. Acute bilateral isolated foot drop: Report of two cases

    PubMed Central

    Kertmen, H.; Gürer, B.; Yimaz, E. R.; Sekerci, Z.

    2015-01-01

    Foot drop is defined as the weakness of the foot and ankle dorsiflexion. Acute unilateral foot drop is a well-documented entity, whereas bilateral foot drop is rarely documented. Slowly progressing bilateral foot drop may occur with various metabolic causes, parasagittal intracranial pathologies, and cauda equina syndrome. Acute onset of bilateral foot drop due to disc herniation is extremely rare. Here we present two cases of acute bilateral foot drop due to disc herniation. The first patient was a 45-year-old man presented with acute bilateral foot drop, without any sign of the cauda equina syndrome. Lumbar magnetic resonance imaging of the patient revealed L4-5 disc herniation. To our knowledge, this is the first presented case of acute bilateral foot drop without any signs of cauda equina syndrome caused by L4-5 disc herniation. The second patient was a 50-year-old man who was also presented with acute bilateral foot drop, and had T12-L1 disc herniation with intradural extension. Also this is the first presented case of T12-L1 disc herniation with intradural extension causing acute bilateral foot drop. We performed emergent decompressive laminectomy to both of the patients and extrude disc materials were excised. Both of the patients were recovered with favorable outcome. PMID:25972945

  7. Bilateral taurodontism in primary dentition with hypodontia.

    PubMed

    Surendar, Marappan Natarajan; Pandey, Ramesh Kumar; Khanna, Richa

    2013-01-01

    Taurodontism is a rare dental anomaly in which there is an enlarged pulp chamber at the expense of roots with apical displacement of the pulpal floor, giving it a rectangular shape. It is caused by the failure of Hertwig's epithelial root sheath to invaginate at the proper horizontal level. Taurodontism has been reported as an intraoral feature of several syndromes like Down syndrome, Klinefelter syndrome, Smith-Magenis syndrome, Hurler syndrome, etc. Association of taurodontism with hypodontia in permanent dentition has also been reported. Taurodontism in primary dentition and its association with hypodontia is very rarely reported in the literature. The present case illustrates bilateral taurodontism of primary mandibular molars with hypodontia in maxilla. PMID:23345504

  8. Bilateral renal infarction.

    PubMed

    Chung, Shiu-Dong; Yu, Hong-Jeng; Huang, Kuo-How

    2009-02-01

    A 34-year-old man was admitted for acute onset of left lower abdominal pain associated with fever. His medical history was unremarkable, and the physical examination revealed bilateral flank tenderness. Bilateral renal infarction was diagnosed and demonstrated by computed tomography. PMID:18829084

  9. Bilateral assymetric epidural hematoma

    PubMed Central

    Pereira, Edmundo Luis Rodrigues; Rodrigues, Daniella Brito; Lima, Lorena Oliveira; Sawada, Luis Armando; Hermes, Mário de Nazareth

    2015-01-01

    Background: Acute bilateral extradural hematoma is a rare presentation of head trauma injury. In sporadic cases, they represent 0.5–10% of all extradural hematomas. However, higher mortality rates have been reported in previous series. Case Description: The authors described the case of a 28-year-old male presenting head injury, comatose, Glasgow Coma Scale of 6, anisocoric pupils without puppilary light reflex. Computed tomography showed asymmetric bilateral epidural hematomas, effacement of the lateral ventricles and sulci, midline shift and a bilateral skull fracture reaching the vertex. Surgical evacuation was performed with simultaneous hematoma drainage. Patient was discharged on the 29th postoperative day with no neurological deficit. Conclusion: The correct approach on bilateral epidural hematomas depends on the volume, moment of diagnosis, and neurological deficit level. Simultaneous drainage of bilateral hematomas has been demonstrated to be an effective technique for it, which soon decreases the intracranial pressure and promotes an efficient resolution to the neurological damage. PMID:25657867

  10. Simultaneous Bilateral or Unilateral Carpal Tunnel Release?

    PubMed Central

    Osei, Daniel A.; Calfee, Ryan P.; Stepan, Jeffrey G.; Boyer, Martin I.; Goldfarb, Charles A.; Gelberman, Richard H.

    2014-01-01

    Background: Over 60% of patients with carpal tunnel syndrome present with symptoms and findings of nerve compression in both hands. Our goal was to compare patient-rated difficulties in performing activities of daily living in the early postoperative period between those undergoing bilateral carpal tunnel release and those undergoing unilateral carpal tunnel release. Methods: This prospective cohort study enrolled consecutive patients with bilateral carpal tunnel syndrome undergoing bilateral carpal tunnel release (n = 47) or unilateral carpal tunnel release (n = 41). Patient function and disease severity were measured by an abbreviated form of the Disabilities of the Arm, Shoulder and Hand questionnaire, QuickDASH, and the Boston Carpal Tunnel Questionnaire at baseline, at postoperative visit 1 at a mean time (and standard deviation) of 10 ± 3 days, and at postoperative visit 2 at a mean time (and standard deviation) of 30 ± 6 days. Patients rated their difficulty in completing fifteen activities of daily living each day for the first postoperative week. Patients reported the factors that influenced their choice of surgery. Results: There was no difference in baseline function or disease severity between the two groups with regard to QuickDASH and the Boston Carpal Tunnel Questionnaire. Patients in both groups improved after carpal tunnel release with no difference between groups either at postoperative visit 1 for QuickDASH (p = 0.97) and the Boston Carpal Tunnel Questionnaire (p = 0.86) or at postoperative visit 2 for QuickDASH (p = 0.43) and the Boston Carpal Tunnel Questionnaire (p = 0.34). Patients undergoing bilateral carpal tunnel release had more difficulty only during postoperative days 1 to 2 in opening jars (p = 0.03), cooking (p = 0.008), and doing household chores (p = 0.02). Patients in the two groups did not differ (p > 0.05) in their abilities to perform activities of daily living necessary for personal hygiene or independence on any day during the first seven days following surgery with regard to using the bathroom, bathing, dressing, or eating. Although the most common reason why patients chose bilateral carpal tunnel release was to avoid two surgical procedures (42%), the most common reason why patients chose unilateral carpal tunnel release was concern for self-care (36%). Conclusions: Patients with bilateral carpal tunnel syndrome can anticipate more severe functional impairment during the first few postoperative days with bilateral carpal tunnel release compared with unilateral carpal tunnel release, but limitations beyond postoperative day 2 or 3 are similar for bilateral and unilateral carpal tunnel release. Level of Evidence: Therapeutic Level II. See Instructions for Authors for a complete description of levels of evidence. PMID:24897736

  11. Bilateral Anterior Shoulder Dislocation

    PubMed Central

    Siu, Yuk Chuen; Lui, Tun Hing

    2014-01-01

    Introduction: Unilateral anterior shoulder dislocation is one of the most common problems encountered in orthopedic practice. However, simultaneous bilateral anterior dislocation of the shoulders is quite rare. Case Presentation: We report a case of a 75-year-old woman presented with simultaneous bilateral anterior shoulder dislocation following a trauma, complicated with a traction injury to the posterior cord of the brachial plexus. Conclusions: Bilateral anterior shoulder dislocation is very rare. The excessive traction force during closed reduction may lead to nerve palsy. Clear documentation of neurovascular status and adequate imaging before and after a reduction should be performed. PMID:25685749

  12. Bilateral lateral periodontal cyst.

    PubMed

    Govil, Somya; Gupta, Vishesh; Misra, Neeta; Misra, Pradyumna

    2013-01-01

    The bilateral lateral periodontal cyst is a rare nasological entity, which despite clinical and radiological presentation is being diagnosed by histological characteristics. It is asymptomatic in nature and is observed in routine radiography. The aim and objective of this article is to present a rare case of bilateral lateral periodontal cyst in a 14-year-old child. The clinical and radiographical findings, along with its management have been discussed. Enucleation of bilateral cyst without extraction of the adjacent tooth was performed. Lesion samples were sent for histopathological analysis. The histopathological analysis revealed a thin, non keratinised stratified squamous epithelium resembling reduced enamel epithelium. Epithelial plaques were also seen. A clinicopathological correlation incorporating the surgical, radiographical and gold standard histopathological findings was obtained to suggest the final diagnosis of the bilateral lateral periodontal cyst. PMID:23667246

  13. Echinoderms have bilateral tendencies.

    PubMed

    Ji, Chengcheng; Wu, Liang; Zhao, Wenchan; Wang, Sishuo; Lv, Jianhao

    2012-01-01

    Echinoderms take many forms of symmetry. Pentameral symmetry is the major form and the other forms are derived from it. However, the ancestors of echinoderms, which originated from Cambrian period, were believed to be bilaterians. Echinoderm larvae are bilateral during their early development. During embryonic development of starfish and sea urchins, the position and the developmental sequence of each arm are fixed, implying an auxological anterior/posterior axis. Starfish also possess the Hox gene cluster, which controls symmetrical development. Overall, echinoderms are thought to have a bilateral developmental mechanism and process. In this article, we focused on adult starfish behaviors to corroborate its bilateral tendency. We weighed their central disk and each arm to measure the position of the center of gravity. We then studied their turning-over behavior, crawling behavior and fleeing behavior statistically to obtain the center of frequency of each behavior. By joining the center of gravity and each center of frequency, we obtained three behavioral symmetric planes. These behavioral bilateral tendencies might be related to the A/P axis during the embryonic development of the starfish. It is very likely that the adult starfish is, to some extent, bilaterian because it displays some bilateral propensity and has a definite behavioral symmetric plane. The remainder of bilateral symmetry may have benefited echinoderms during their evolution from the Cambrian period to the present. PMID:22247765

  14. Changes of symptom and EEG in mal de debarquement syndrome patients after repetitive transcranial magnetic stimulation over bilateral prefrontal cortex: a pilot study.

    PubMed

    Guofa Shou; Han Yuan; Urbano, Diamond; Yoon-Hee Cha; Lei Ding

    2014-01-01

    Mal de debarquement syndrome (MdDS) is a chronic disorder of imbalance characterized by a feeling of rocking and swaying. The medical treatment for MdDS is still limited. Motivated by our previous pilot study that demonstrates the promising clinical efficacy of repetitive transcranial stimulation (rTMS) in MdDS patients, a novel rTMS paradigm, i.e., 1 Hz stimulation over ipsilateral dorsal lateral prefrontal cortex (DLPFC) with respect to the dominant hand followed by 10 Hz stimulation over contralateral DLPFC, was proposed and conducted in MdDS in the present study. To evaluate the potential efficacy, we examined the changes before and after rTMS in both subjective reported symptom using visual analogue scale (VAS) and direct brain activity in resting state electroencephalography (rsEEG). To disentangle activity from distinct brain substrates and/or local networks in rsEEG signals, a group-wise independent component analysis was employed and the corresponding spectral power changes were examined in the identified components. In general, reduction in rocking sensation was reported in five of ten subjects (with dramatic reductions (changes > 30) in three subjects) after rTMS using the present paradigm, while no changes and slight increases in rocking sensation were reported in the remaining subjects. In rsEEG, significant elevated spectral powers in low frequency bands (i.e., theta and alpha) over broad areas of occipital, parietal, motor, and prefrontal cortices were induced by rTMS, reflecting the enhancement of cortical inhibition over these areas. Meanwhile, the significant correlations between changes in rsEEG and VAS scores were detected in the high frequency bands (i.e., high alpha and beta) over posterior parietal and left visual areas, reflecting the suppression of spatial information processing. Therefore, the present findings demonstrate the promising clinical efficacy of a new rTMS paradigm for MdDS, and suggest its merit for further studies in more patients. PMID:25570942

  15. Neuroimaging Aspects of Aicardi Syndrome

    PubMed Central

    Hopkins, Bobbi; Sutton, V. Reid; Lewis, Richard Alan; Van den Veyver, Ignatia; Clark, Gary

    2008-01-01

    Aicardi syndrome is a rare neurodevelopmental disorder characterized by congenital chorioretinal lacunae, corpus callosum dysgenesis, seizures, polymicrogyria, cerebral heterotopias, intracranial cysts, and costovertebral defects. Cerebellar abnormalities have been described occasionally. Aicardi syndrome is sporadic and has been observed only in females and 47,XXY males. Therefore, it is thought to result from a mutation in an X-linked gene. Improved definition of the clinical phenotype should focus the selection of functional candidate genes for mutation analysis. Because central nervous system abnormalities are the most prominent component of the phenotype, we performed a detailed characterization of abnormalities identified on magnetic resonance neuroimaging studies from 23 girls with Aicardi syndrome, the largest cohort to undergo such review by a single group of investigators. All patients had polymicrogyria that was predominantly frontal and perisylvian and often associated with underopercularization. Periventricular nodular heterotopias, present in all patients, were more frequent than previously reported; 10 had single and 11 had multiple intracranial cysts. Posterior fossa abnormalities were also more frequent than previously described. Cerebellar abnormalities were noted in 95% of studies where they could be evaluated. As a novel finding, we noted tectal enlargement in 10 patients. Since mildly-affected girls with variable callosal dysgenesis have now been reported, the constellation of frontal-dominant and perisylvian polymicrogyria, periventricular nodular heterotopias, intracranial cysts, and posterior fossa abnormalities, including tectal enlargement, should prompt consideration of the diagnosis of Aicardi syndrome. We further propose that improved characterization of the neurological phenotype will benefit the selection of candidate genes for mutation analysis. PMID:18925666

  16. Inferior peripheral nonperfusion in bilateral diffuse uveal melanocytic proliferation.

    PubMed

    Yonekawa, Yoshihiro; Shildkrot, Yevgeniy; Eliott, Dean

    2013-01-01

    Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a paraneoplastic syndrome characterized by cataract, photoreceptor loss and subretinal fluid overlying patchy areas of retinal pigment epithelium atrophy, and a diffusely thickened choroid with focal nodules. We present the case of a 64-year-old woman with a history of endometrial adenocarcinoma who developed BDUMP with bilateral exudative retinal detachments with inferior peripheral retinal ischemia. This new finding of peripheral nonperfusion expands the spectrum of BDUMP. PMID:23510044

  17. [Bilateral caudate head infarcts].

    PubMed

    Kuriyama, N; Yamamoto, Y; Akiguchi, I; Oiwa, K; Nakajima, K

    1997-11-01

    We reported a 67-year-old woman with bilateral caudate head infarcts. She developed sudden mutism followed by abulia. She was admitted to our hospital 2 months after ictus for further examination. She showed prominent abulia and was inactive, slow and apathetic. Spontaneous activity and speech, immediate response to queries, spontaneous word recall and attention and persistence to complex programs were disturbed. Apparent motor disturbance, gait disturbance, motor aphasia, apraxia and remote memory disturbance were not identified. She seemed to be depressed but not sad. Brain CT and MRI revealed bilateral caudate head hemorrhagic infarcts including bilateral anterior internal capsules, in which the left lesion was more extensive than right one and involved the part of the left putamen. These infarct locations were thought to be supplied by the area around the medial striate artery including Heubner's arteries and the A1 perforator. Digital subtraction angiography showed asymptomatic right internal carotid artery occlusion. She bad had hypertension, diabetes mellitus and atrial fibrillation and also had a left atrium with a large diameter. The infarcts were thought to be caused by cardioembolic occlusion to the distal portion of the left internal carotid artery. Although some variations of vasculature at the anterior communicating artery might contribute to bilateral medial striate artery infarcts, we could not demonstrate such abnormalities by angiography. Bilateral caudate head infarcts involving the anterior internal capsule may cause prominent abulia. The patient did not improve by drug and rehabilitation therapy and died suddenly a year after discharge. PMID:9503974

  18. Bilateral Parotid Tuberculosis

    PubMed Central

    Thakur, JS; Thakur, A; Mohindroo, NK; Mohindroo, S; Sharma, DR

    2011-01-01

    Tuberculosis of parotid is a rare clinical entity, and cases of bilateral tubercular parotitis are even rarer. We present a case of bilateral primary parotid tuberculosis in a 49-year-old female. The patient received anti-tuberculosis treatment for six months, resulting in complete resolution of the disease. We also review the theories related to the pathogenesis of tubercular parotitis, and propose a novel hypothesis about greater involvement of parotid gland as compared to other salivary glands in primary tuberculosis. PMID:21887065

  19. Bilateral neck paragangliomas.

    PubMed

    Mumoli, N; Cei, M; Pauletti, M; Ferrito, G; Scazzeri, F

    2009-10-01

    Paragangliomas of the head and neck are rare neoplasms presented as cervical mass, generally bilateral, that arise from chemoreceptors located at the carotid bifurcation (carotid body tumors), along the vagus nerve (vagal paragangliomas), and in the jugular fossa and tympanic cavity (jugulotympanic paragangliomas). They are typically asymptomatic at the beginning, highly vascular, slow-growing and compressing the surrounding anatomic structures. Only radical surgery is the curative treatment for paragangliomas. We present a case of a 62- year-old woman with a diagnosis of bilateral neck paragangliomas where surgical removal was judged burdened by excessive risk because of the size of the tumor. PMID:19622673

  20. Bilateral primary choroidal melanoma.

    PubMed Central

    Migdal, C; Macfarlane, A

    1984-01-01

    A case of bilateral primary choroidal melanoma is described in a 51-year-old white male. Histological confirmation followed enucleation of the left eye and local excision in the right. A careful systemic evaluation failed to demonstrate any other primary or secondary melanomata. This case is the first bilateral primary choroidal melanoma reported in the United Kingdom and the first to be described in which histological confirmation was obtained as a result of local excision of one of the tumours. Images PMID:6704362

  1. Articulatory/phonetic sequencing at the level of the anterior perisylvian cortex: a functional magnetic resonance imaging (fMRI) study.

    PubMed

    Riecker, A; Ackermann, H; Wildgruber, D; Meyer, J; Dogil, G; Haider, H; Grodd, W

    2000-11-01

    Damage to the anterior peri-intrasylvian cortex of the dominant hemisphere may give rise to a fairly consistent syndrome of articulatory deficits in the absence of relevant paresis of orofacial or laryngeal muscles (apraxia of speech, aphemia, or phonetic disintegration). The available clinical data are ambiguous with respect to the relevant lesion site, indicating either dysfunction of the premotor aspect of the lower precentral gyrus or the anterior insula in the depth of the Sylvian fissure. In order to further specify the functional anatomic substratum of this syndrome, functional magnetic resonance imaging (fMRI) was performed during reiteration of syllables differing in their demands on articulatory/phonetic sequencing (CV versus CCCV versus CVCVCV). Horizontal tongue movements and a polysyllabic lexical item served as control conditions. Repetition of the CV and CCCV monosyllables elicited a rather bilateral symmetric hemodynamic response at the level of the anterior and posterior bank of the central sulcus (primary sensorimotor cortex), whereas a more limited area of neural activity arose within this domain during production of lexical and nonlexical polysyllables, significantly or exclusively lateralized toward the left hemisphere. There is neurophysiological evidence that primary sensorimotor cortex mediates the "fractionation" of movements. Assuming that the polysyllables considered are organized as coarticulated higher-order units, the observed restricted and lateralized cortical activation pattern, most presumably, reflects a mode of "nonindividualized" motor control posing fewer demands on "movement fractionation." These findings may explain the clinical observation of disproportionately worse repetition of trisyllabic items as compared to monosyllables in apraxia of speech. The various test materials failed to elicit significant activation of the anterior insula. If at all, only horizontal tongue movements yielded a hemodynamic reaction extending beyond the sensorimotor cortex to premotor areas. Since limbic projections target the inferior dorsolateral frontal lobe, the enlarged region of activation during horizontal tongue movements might reflect increased attentional requirements of this task. PMID:11049668

  2. Bilateral renal lymphoangiomatosis

    PubMed Central

    Raed, Alqahtani; Sultan, Alkhateeb; Bader, Al-Mutairi

    2015-01-01

    Introduction Renal lymphangiomatosis is a rare congenital benign disease of renal lymphatic system, here we are presenting a very rare form of disease which is bilateral form. Presentation of the case A young adult presented to our clinic after being referred from primary care clinic with intermittent bilateral flank pain and no other symptoms after extensive radiological investigations diagnosis has been made and confirmed by radiological finding of disease. Active treatment usually preserved for complex cases and for the complications of the disease but in our patient as needed analgesia worked well in controlling his intermittent pain and his wish not to pursue any intervention. The vague presentation with initial imaging rising suspicion of renal tumor or complex renal cyst might cause psychological street on the patient, which our patient had, but reassurance after extensive radiological work up relive that's stress. Discussion Although it is very rare disease to be bilateral but wide variety of other differential diagnoses make importance of disease recognition and accurate diagnosis is the key. Conclusion Renal lymphangiomatosis is a rare benign disease of renal lymphatic, which usually affect one side, but bilateral form is very rare form, which may raise the suspicions of genetic form of renal malignancy. Accurate diagnosis requires work up to role out malignant and other renal tumor, which require active surgical management. PMID:26719997

  3. Bilateral Sagittal Split Osteotomy

    PubMed Central

    Monson, Laura A.

    2013-01-01

    The bilateral sagittal split osteotomy is an indispensable tool in the correction of dentofacial abnormalities. The technique has been in practice since the late 1800s, but did not reach widespread acceptance and use until several modifications were described in the 1960s and 1970s. Those modifications came from a desire to make the procedure safer, more reliable, and more predictable with less relapse. Those goals continue to stimulate innovation in the field today and have helped the procedure evolve to be a very dependable, consistent method of correction of many types of malocclusion. The operative surgeon should be well versed in the history, anatomy, technical aspects, and complications of the bilateral sagittal split osteotomy to fully understand the procedure and to counsel the patient. PMID:24872760

  4. Bilateral Sertoli cell adenoma in gonads, associated with serous cystadenoma.

    PubMed

    Fernandez-Vega, I; Santos-Juanes, J; García-Pravia, C

    2014-06-01

    Complete androgen insensitivity syndrome is an extremely infrequent disease. The patients exhibit female phenotype because of insensitivity to the androgen receptor and may develop tumors, especially in their undescended gonads. We report a case of bilateral Sertoli cell adenoma in gonads with unilateral serous cystadenoma, in an elderly phenotypic woman with primary amenorrhea. We also provide radiological and pathological studies. PMID:25119177

  5. Bilateral Hypermetropia, Myelinated Retinal Nerve Fibers, and Amblyopia

    PubMed Central

    Shenoy, Radha; Bialasiewicz, Alexander A.; Al Barwani, B.

    2011-01-01

    A 14-year-old hyperopic female with poor vision in both eyes was evaluated for ophthalmic and systemic diseases. The patient had bilateral retinal fiber myelination and greater vision loss in the more hyperopic eye. This was a rare case of reverse Straatsma syndrome, the clinical presentation which may be accompanied with significant vision loss. PMID:21572738

  6. Bilateral renal aspergillosis.

    PubMed

    Warshawsky, A B; Keiller, D; Gittes, R F

    1975-01-01

    The recent increased use of steriods, immunosuppressive agents and cytotoxic drugs has been associated with a rise in the incidence of significant fungal disease. The first case of bilateral renal aspergillosis without disseminated involvement is reported. A multitherapeutic approach, including surgical evacuation of masses of hyphae, parenteral antimycotic chemotherapy and topical instillations of amphotericin B, were necessary to clear the kidneys. Newer systemic agents such as 5-fluorocytosine and rifampicin were also used. Treatment of fungal infections of the urinary tract is discussed. PMID:1113403

  7. Synchronous Bilateral Breast Cancers

    PubMed Central

    Subramanyan, Annapurneswari; Radhakrishna, Selvi

    2015-01-01

    Background Bilateral breast cancer (BBC) is not an uncommon entity in contemporary breast clinics. Improved life expectancy after breast cancer treatment and routine use of contra-lateral breast mammography has led to increased incidence of BBC. Our study objective was to define the epidemiological and tumour characteristics of BBC in India. Materials and Methods A total of 1251 breast cancer patients were treated during the period January 2007 to March 2015 and 30 patients were found to have BBC who constituted the study population (60 tumour samples). Synchronous bilateral breast cancers (SBC) was defined as two tumours diagnosed within an interval of 6 months and a second cancer diagnosed after 6 months was labelled as metachronous breast cancer (MBC). Analyses of patient and tumour characteristics were done in this prospective data base of BBC patients. Results Median patient age was 66 years (range 39-85). Majority of the patients had SBC (n=28) and in 12 patients the second tumour was clinically occult and detected only by mammography of the contra-lateral breast. The second tumour was found at lower tumour size compared to the first in 73% of cases and was negative for axillary metastasis in 80% of cases (24/30). Infiltrating ductal carcinoma was the commonest histological type (n=51) and majority of the tumours were ER/PR positive (50/60). Her2 was overexpressed in 13 tumours (21%). Over 70% (22/30) of patients had similar histology in both breasts and amongst them grade concordance was present in about 69% (15/22) of patients. Concordance rates of ER, PR and Her2 statuses were 83%, 80% and 90% respectively. Bilateral mastectomy was the commonest surgery performed in 80% of the patients followed by bilateral breast conservation in 13%. At the end of study period, 26 patients were alive and disease free. Median survival was 29 months (range 3-86 months). Conclusion In most patients with BBC, the second tumour is identified at an early stage than index tumours supporting the importance of contralateral breast cancer screening at the time of primary diagnosis and during follow-up. BBC occurs more frequently in old age group and majority of these tumours are estrogen dependent. There is good pathological concordance between the index tumour and the contralateral breast cancer. PMID:26500995

  8. Idiopathic bilateral male breast abscess

    PubMed Central

    Sinha, Rajan Kumar; Sinha, Mithilesh Kumar; Gaurav, Kumar; Kumar, Amar

    2014-01-01

    A 38-year-old man presented with bilateral breast swelling, along with pain and redness for 7 days. Bilateral axillary nodes were also palpable; which were multiple and discrete. A provisional diagnosis of bilateral breast abscess was made with suspicion of underlying malignancy. Incision and drainage through subareolar incision was performed and the adjoining tissue was excised and sent for histopathological examination. PMID:24614823

  9. Morbidity and Mortality of Bilateral Femur Fractures.

    PubMed

    Lane, Mark K; Nahm, Nickolas J; Vallier, Heather A

    2015-07-01

    Bilateral femur fractures have been associated with frequent morbidity and mortality. Associated injuries and massive hemorrhage contributed to mortality rates that were as high as 27% in previous reports. The goals of this study were to determine the frequency of associated complications, including mortality, and to identify which patient and injury features are associated with increased morbidity and mortality. The authors proposed that some patients with bilateral femur fractures may undergo early definitive fixation with an acceptable rate of complications. Patients who had bilateral femur fractures during the same injury event were retrospectively reviewed. Demographic characteristics, associated injuries, and the type and timing of treatment were determined. Complications were identified. The authors identified 50 men and 22 women, with a mean age of 41.5 years, who had high-energy bilateral femur fractures. These patients accounted for 5.5% of all femur fractures treated at the authors' institution over a period of 11 years. Two patients died before fixation. In addition, 13 other patients (19%) had 21 complications, including pneumonia in 6 (8.6%) and deep venous thrombosis in 7 (10%). No patient had adult respiratory distress syndrome, but 2 died of multiple organ failure. All patients with pulmonary complications had an underlying chest injury (P=.004). The overall mortality rate was 6.9%, and mortality was associated with higher mean age and higher Injury Severity Score (ISS). Of the 60 patients who had definitive fixation within 24 hours of injury, 53 (88%) had no complications. Complication rates were similar to those reported in the literature, with a mortality rate of 6.9%, including 3 patients who died after femoral fixation. Mortality was associated with advanced age and higher ISS. Chest injuries were associated with pulmonary complications. Most patients had early definitive fixation without complications, but it is not possible to predict which patients may be safely treated on an early basis. PMID:26186320

  10. Idiopathic Bilateral Bloody Tearing

    PubMed Central

    Beyazy?ld?z, Emrullah; Özdamar, Yasemin; Beyazy?ld?z, Özlem; Yerli, Hasan

    2015-01-01

    Bloody tear is a rare and distinct clinic phenomenon. We report a case presenting with the complaint of recurrent episodes of bilateral bloody tearing. A 16-year-old girl presented to our clinic with complaint of bloody tearing in both eyes for 3 months. Bloody tearing was not associated with her menses. A blood-stained discharge from the punctum was not observed during the compression of both nasolacrimal ducts. Nasolacrimal passage was not obstructed. Imaging studies such as dacryocystography and gradient-echo magnetic resonance imaging (MRI) of nasolacrimal canal were normal. Intranasal endoscopic evaluation was normal. We collected samples from bloody tears two times and pathological examination was performed. Pathological analysis showed lots of squamous cells and no endometrial cells; dysplastic cells were found. Further evaluations for underlying causes were unremarkable. No abnormalities were found in ophthalmologic, radiologic, and pathologic investigations. This condition is likely a rare abnormality and the least recognized aetiology for the idiopathic phenomenon. PMID:25685572

  11. Idiopathic bilateral bloody tearing.

    PubMed

    Beyazy?ld?z, Emrullah; Özdamar, Yasemin; Beyazy?ld?z, Özlem; Yerli, Hasan

    2015-01-01

    Bloody tear is a rare and distinct clinic phenomenon. We report a case presenting with the complaint of recurrent episodes of bilateral bloody tearing. A 16-year-old girl presented to our clinic with complaint of bloody tearing in both eyes for 3 months. Bloody tearing was not associated with her menses. A blood-stained discharge from the punctum was not observed during the compression of both nasolacrimal ducts. Nasolacrimal passage was not obstructed. Imaging studies such as dacryocystography and gradient-echo magnetic resonance imaging (MRI) of nasolacrimal canal were normal. Intranasal endoscopic evaluation was normal. We collected samples from bloody tears two times and pathological examination was performed. Pathological analysis showed lots of squamous cells and no endometrial cells; dysplastic cells were found. Further evaluations for underlying causes were unremarkable. No abnormalities were found in ophthalmologic, radiologic, and pathologic investigations. This condition is likely a rare abnormality and the least recognized aetiology for the idiopathic phenomenon. PMID:25685572

  12. Severe Acute Orthopnea: Ipilimumab-Induced Bilateral Phrenic Nerve Neuropathy.

    PubMed

    Jinnur, Praveen; Lim, Kaiser G

    2015-08-01

    Ipilimumab is a monoclonal antibody used in the treatment of unresectable or metastatic melanoma. Several immune-related adverse events including potential fatal events have been reported following its use. We report a case of a 66-year-old man who presented with severe acute exertional dyspnea and orthopnea following administration of ipilimumab for metastatic melanoma. Although various peripheral neuropathy syndromes associated with ipilimumab have been reported, bilateral phrenic nerve paralysis has not been previously reported. This case also highlights the clinical features of bilateral phrenic nerve neuropathy. Pulmonologists have to be aware of these unusual immune-related respiratory adverse events in patients being treated with monoclonal antibodies. PMID:25956728

  13. Eagle syndrome.

    PubMed

    Ferreira, Pedro Costa; Mendanha, Mário; Frada, Tiago; Carvalho, Jorge; Silva, Alvaro; Amarante, José

    2014-01-01

    Eagle syndrome, also known as elongated styloid process, is a condition first described by Watt Eagle in 1937. It occurs when an elongated styloid process or calcified stylohyoid ligament causes recurrent throat pain or foreign body sensation, dysphagia, or facial pain. Additional symptoms may include neck or throat pain with radiation to the ipsilateral ear. It is usually hard to diagnose because the symptoms related to this condition can be confused with those attributed to a wide variety of facial neuralgias. In this article, a case of Eagle syndrome exhibiting unilateral symptoms with bilateral elongation of styloid process is reported. PMID:24406612

  14. Bilateral cleft lip reconstruction.

    PubMed

    Noordhoff, M S

    1986-07-01

    Over a period of 8 years 140 bilateral cleft lips were operated using a muscle-repositioning banked fork-flap cheiloplasty. The use of buccal mucosal flaps in the intercartilaginous incision is helpful to decrease scarring and contracture by facilitating alar cartilage repositioning and wound closure without tension. Adding mucosa from the inferior turbinate makes complete wound closure relatively easy without tension. A lateral lip orbicularis muscle flap with white skin roll and vermilion is recommended for reconstruction of the Cupid's bow. Muscle continuity by freeing the muscle in one sheet and repositioning in front of the premaxilla with creation of a buccal alveolar sulcus is stressed to prevent the necessity of reentering the lip in a second procedure. The elongation of the columella is done at 1 to 6 years of age by advancing nasal floor tissue onto the columella and repositioning the alar cartilages superiorly and medially. When nasal floor tissue is inadequate, columellar lengthening is done by the use of a composite free ear graft. PMID:3725954

  15. [Triplegia in bilateral anterior-media watershed infarcts].

    PubMed

    Stillhard, G; Turina, M; Flepp, M; Waespe, W

    1991-02-16

    Deterioration of a neurologic deficit was observed in a 47-year-old patient with high-grade bilateral internal carotid artery stenoses. She presented monoplegia of the right leg with progression to triplegia (bicrural and left arm) following antihypertensive treatment of a suspected "hypertensive crisis". Triplegia is a very rare syndrome and highly suggestive of bilateral pathology of the carotid arteries. Antihypertensive treatment is contraindicated because sufficient blood pressure is needed for adequate perfusion of the brain, especially in the border zones between the great cerebral arteries. Allowing a high blood pressure and low head positioning led to slow recovery from the neurologic deficit, which continued after bilateral carotid endarterectomy. The patient became ambulatory without assistance and with minimal residual paraspasticity. Triplegia and other patterns of motor deficit in cerebrovascular disorders are discussed and the clinical picture of hypertensive encephalopathy is reviewed. PMID:2008602

  16. Bilateral acute iris transillumination (BAIT) initially misdiagnosed as acute iridocyclitis.

    PubMed

    Gonul, Saban; Bozkurt, Banu

    2015-01-01

    Bilateral acute iris transillumination (BAIT) is a relatively new clinical entity characterized by bilateral acute loss of iris pigment epithelium, iris transillumination, pigment dispersion in the anterior chamber, and sphincter paralysis. We report the case of a 30-year-old male who was initially diagnosed with acute iridocyclitis in a different clinic and treated with topical and systemic corticosteroids. He was referred to our clinic to seek another opinion because his symptoms did not improve. An ocular examination revealed bilateral pigment dispersion into the anterior chamber, diffuse iris transillumination, pigment dusting on the anterior lens capsule, atonic and distorted pupils, and increased intraocular pressure, suggesting a diagnosis of BAIT rather than iridocyclitis. Clinicians should be aware of the differential diagnosis of syndromes associated with pigment dispersion from iridocyclitis to avoid aggressive anti-inflammatory therapy and detailed investigation for uveitis. PMID:25945534

  17. A 12-year-old African American girl with subacute bilateral ophthalmoplegia.

    PubMed

    Bar, Amir; Urbine, Jacqueline; Bahora, Yasmine; Berkenstock, Meghan; Vodzak, Jennifer; Guruprasad, Hamalatha; Sinha, Manisha; Abed, Thair; Legido, Agustín

    2014-06-01

    A twelve-year-old African-American female presented with two week history of progressively worsening headache and fatigue, and vision difficulties for the past week. The physical examination was normal. The neurological evaluation was normal, except for cranial nerves (CN) testing, which showed bilateral restriction of adduction (CN III) and up gaze (CN IV) motions, vertical nystagmus, and left side facial paresis of central origin (CN VII). The bilateral exotropia and ophthalmoplegia are characteristics of WEBINO (Wall-Eyed Bilateral Intranuclear Ophthalmoplegia) syndrome, associated to a brain stem structural lesion. The following causes were evaluated and ruled out: tumor, infection, ischemic stroke, non-infectious inflammation. Pediatric Acquired Demyelinating Syndromes were then considered. Neuromyelitis Optica was ruled out in the absence of neuritis and normal spinal cord MRI. The differential diagnosis between Clinically Isolated Syndrome and Acute Demyelinating Encephalomyelitis, causing an isolated brain stem syndrome, is discussed. PMID:25149958

  18. Duane retraction syndrome type 1 with Usher syndrome type 2: an unreported association.

    PubMed

    Khurana, Bhawna Piplani; Khurana, Aruj Kumar; Grover, Sumit

    2015-01-01

    Duane retraction syndrome is characterized by globe retraction and palpebral fissure narrowing on adduction, with restriction of abduction, adduction, or both. Usher syndrome type 2 consists of congenital bilateral sensorineural hearing loss and retinitis pigmentosa. The authors present a case with a yet unreported association between Duane retraction syndrome type 1 and Usher syndrome type 2. PMID:25955520

  19. Bilateral diaphragmatic paralysis after kidney surgery.

    PubMed

    Sozzo, S; Carratù, P; Damiani, M F; Falcone, V A; Palumbo, A; Dragonieri, S; Resta, O

    2012-06-01

    A 57-year-old woman underwent an enucleoresection of her right kidney angiomyolipoma. Two weeks later she was admitted to our hospital because of dyspnea at rest with orthopnea. The chest x-ray showed the elevation of both hemidiaphragms and the measurement of the sniff transdiaphragmatic pressure confirmed the diagnosis of bilateral diaphragmatic paralysis. A diaphragm paralysis can be ascribed to several causes, i.e. trauma, compressive events, inflammations, neuropathies, or it can be idiopathic. In this case, it was very likely that the patient suffered from post-surgery neuralgic amyotrophy. To our knowledge, there are only a few reported cases of neuralgic amyotrophy, also known as Parsonage-Turner Syndrome, which affects only the phrenic nerve as a consequence of a surgery in an anatomically distant site. PMID:23193847

  20. Isolated tuberculous lymphadenitis presenting as bilateral buboes.

    PubMed

    Palanisamy, Arun Prasath; Samuel, Soumya; Vadivel, Sivasubramanian; Kothandapany, Srivenkateswaran

    2015-01-01

    Inguinal and femoral buboes are defined as localized enlargement of lymph nodes in the groin that are painful, and may or may not be fluctuant. We report a case of 42-year-old female who presented with bilateral inguinal swelling of 6 months duration. After a complete evaluation, she was found to be a case of isolated inguinal tuberculous lymphadenitis. There was complete resolution with standard antituberculous therapy. Isolated inguinal tuberculous lymphadenitis though a rare entity in developed countries is not uncommon in developing nations. In this era of syndromic management of sexually transmitted diseases, which carries its own pros and cons, this case report emphasizes the need to look beyond the venereal causes and calls for thorough evaluation and management. PMID:26392662

  1. Isolated tuberculous lymphadenitis presenting as bilateral buboes

    PubMed Central

    Palanisamy, Arun Prasath; Samuel, Soumya; Vadivel, Sivasubramanian; Kothandapany, Srivenkateswaran

    2015-01-01

    Inguinal and femoral buboes are defined as localized enlargement of lymph nodes in the groin that are painful, and may or may not be fluctuant. We report a case of 42-year-old female who presented with bilateral inguinal swelling of 6 months duration. After a complete evaluation, she was found to be a case of isolated inguinal tuberculous lymphadenitis. There was complete resolution with standard antituberculous therapy. Isolated inguinal tuberculous lymphadenitis though a rare entity in developed countries is not uncommon in developing nations. In this era of syndromic management of sexually transmitted diseases, which carries its own pros and cons, this case report emphasizes the need to look beyond the venereal causes and calls for thorough evaluation and management. PMID:26392662

  2. Metachronous Bilateral Extremity Soft Tissue Sarcomas.

    PubMed

    Nowrasteh, Ghodratollah; Aziz, Tanim; Al Assas, Mohammed; Al Nuaimi, Lateefa; Marzouqi, Saeeda; Quadri, Asif A M; Alrawi, Sadir

    2016-01-01

    BACKGROUND Soft tissue sarcomas (STS) account for approximately 1% of adult malignancies, with 50 to 60% occurring in the extremities. Liposarcoma is the most common type of STS and represent about 20% of total adult sarcomas. There are rare syndromes associated with increased risk of developing STS. Further, chemical compounds such as chlorinated phenols and a few chemotherapeutic drugs have been linked to STS, along with ionizing radiation. Nevertheless, the etiology is uncertain for most of these lesions. CASE REPORT This report details 2 cases of metachronous bilateral STS of the lower extremities. The first of these presented as a local recurrence of a previously resected right thigh liposarcoma and a new liposarcoma in the left thigh. As mentioned above, among the different subtypes of STS, liposarcoma has the highest tendency for multifocality. The second patient had multifocal metachronous leiomyosarcoma with lung metastases occurring simultaneously with the second presentation. Leiomyosarcoma is another subtype reported to present with multifocal disease. CONCLUSIONS Despite the rarity of bilateral lesions, their occurrence should not be overlooked in the initial diagnosis and follow-up of the initially detected tumor. Early detection can affect patient survival because their presence predicts unfavorable outcomes. PMID:26744032

  3. Metachronous Bilateral Extremity Soft Tissue Sarcomas

    PubMed Central

    Nowrasteh, Ghodratollah; Aziz, Tanim; Assas, Mohammed Al; Nuaimi, Lateefa Al; Marzouqi, Saeeda; Quadri, Asif A.M.; Alrawi, Sadir

    2016-01-01

    Case series Patient: Male, 44 • Male, 58 Final Diagnosis: Soft tissue sarcomas Symptoms: Discomfort • swelling Medication: — Clinical Procedure: Image guided biopsy • metastatic work up • neoadjuvant radiotherapy • radical resection Specialty: Surgery Objective: Rare disease Background: Soft tissue sarcomas (STS) account for approximately 1% of adult malignancies, with 50 to 60% occurring in the extremities. Liposarcoma is the most common type of STS and represent about 20% of total adult sarcomas. There are rare syndromes associated with increased risk of developing STS. Further, chemical compounds such as chlorinated phenols and a few chemotherapeutic drugs have been linked to STS, along with ionizing radiation. Nevertheless, the etiology is uncertain for most of these lesions. Case Report: This report details 2 cases of metachronous bilateral STS of the lower extremities. The first of these presented as a local recurrence of a previously resected right thigh liposarcoma and a new liposarcoma in the left thigh. As mentioned above, among the different subtypes of STS, liposarcoma has the highest tendency for multifocality. The second patient had multifocal metachronous leiomyosarcoma with lung metastases occurring simultaneously with the second presentation. Leiomyosarcoma is another subtype reported to present with multi-focal disease. Conclusions: Despite the rarity of bilateral lesions, their occurrence should not be overlooked in the initial diagnosis and follow-up of the initially detected tumor. Early detection can affect patient survival because their presence predicts unfavorable outcomes. PMID:26744032

  4. Hemophagocytic Lymphohistiocytosis After Initiation of Chemotherapy for Bilateral Adrenal Neuroblastoma.

    PubMed

    Nascimento, Fábio A; Nery, Juliane; Trennepohl, Joanna; Pianovski, Mara A D

    2016-01-01

    Hemophagocytic lymphohistiocytosis (HLH) is a rare and aggressive syndrome characterized by overactivation of the immune system. Although secondary HLH has been frequently associated with malignancies, this entity is rarely triggered by solid tumors, such as neuroblastomas. Herein, we describe a 14-month-old girl with a late diagnosis of bilateral adrenal neuroblastoma who developed HLH 6 days after the initiation of chemotherapy. On the basis of the large tumoral mass and the time of onset of her symptoms suggestive of HLH, we hypothesize that tumor cell destruction induced by chemotherapy drugs was the trigger to the development of hematophagocytic lymphohistiocytosis syndrome. PMID:26583611

  5. 38 CFR 4.26 - Bilateral factor.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2010-07-01 2010-07-01 false Bilateral factor. 4.26... DISABILITIES General Policy in Rating § 4.26 Bilateral factor. When a partial disability results from disease... disability. The bilateral factor will be applied to such bilateral disabilities before other combinations...

  6. 38 CFR 4.26 - Bilateral factor.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2011-07-01 2011-07-01 false Bilateral factor. 4.26... DISABILITIES General Policy in Rating § 4.26 Bilateral factor. When a partial disability results from disease... disability. The bilateral factor will be applied to such bilateral disabilities before other combinations...

  7. Bilateral superior ophthalmic vein enlargement associated with diffuse cerebral swelling. Report of 11 cases.

    PubMed

    Khanna, R K; Pham, C J; Malik, G M; Spickler, E M; Mehta, B; Rosenblum, M L

    1997-05-01

    Bilateral superior ophthalmic vein (SOV) enlargement has rarely been shown to occur in patients with septic and aseptic cavernous sinus thrombosis, Graves' disease due to obstruction of the SOV by enlarged extraocular muscles, or carotid-cavernous fistulas caused by retrograde flow. The authors describe 11 patients with bilateral SOV enlargement associated with cerebral swelling as detected by computerized tomography scanning. The bilaterally enlarged SOVs returned to a normal size following resolution of cerebral swelling and elevated intracranial pressure. To the authors' knowledge, this is the first report of bilateral SOV enlargement associated with diffuse cerebral swelling that subsequently resolved after treatment of the cerebral edema. The authors believe that the bilateral SOV enlargement was caused by mechanical cavernous sinus venous stagnation due to cerebral swelling, a syndrome that occurs more commonly than currently appreciated. PMID:9126909

  8. Thoracic Outlet Syndrome Following Breast Implant Rupture

    PubMed Central

    Caplash, Yugesh; Giri, Pratyush; Kearney, Daniel; Wagstaff, Marcus

    2015-01-01

    Summary: We present a patient with bilateral breast implant rupture who developed severe locoregional silicone granulomatous lymphadenopathy. Poly Implant Prothese silicone implants had been used for bilateral breast augmentation 5 years prior. Extracapsular implant rupture and bilateral axillary lymphadenopathy indicated explantation, capsulectomy, and selective lymph node excision. Histology demonstrated silicone lymphadenopathy with no evidence of malignancy. Over the subsequent 12 months, she developed progressive locoregional lymphadenopathy involving bilateral cervical, axillary, and internal mammary groups, resulting in bilateral thoracic outlet syndrome. We report the unusual presentation, progression, and the ultimate surgical management of this patient. PMID:25878942

  9. Fraser Syndrome.

    PubMed

    Saleem, Adnan Aslam; Siddiqui, Sorath Noorani

    2015-10-01

    Fraser's Syndrome (FS) is a rare autosomal recessive disorder with a spectrum of malformations. The most consistent features are Cryptophthalmos (CO), syndactyly, genitourinary tract abnormalities, laryngeal and tracheal anomalies, craniofacial dysmorphism, malformations of the ear and nose, orofacial clefting and musculoskeletal defects. FS is genetically heterogeneous; so far mutations in FRAS1, FREM2 and GRIP1 genes have been linked to FS. FS can be diagnosed on clinical examination, pre-natal ultrasound or perinatal autopsy. We present a case of a 3 months old child born to consanguineous healthy parents with bilateral complete CO, unilateral microphthalmia, hypertelorism, syndactyly (hands and feet bilaterally), ambiguous genitalia with cryptorchidism and an umbilical hernia. We also present the criteria for diagnosing FS and the significant features on pre-natal ultrasonography. Around 200 case reports of patients with FS and CO have been published. To our knowledge, this is the first reported case of FS in Pakistan. PMID:26522198

  10. Bilateral cryptorchidism with bilateral synchronous abdominal testicular germ cell tumour

    PubMed Central

    Seetharam, Venkatesh; Hameed, Zeeshan BM; Talengala, Shaila Bhat; Thomas, Joseph

    2014-01-01

    Cryptorchidism or undescended testis is a very common anomaly of the male genitourinary system. It is one of the established risk factors for testicular tumour. The commonest malignancy noted in cryptorchidism is seminoma testis. The presence of bilateral abdominal synchronous testicular tumour in cryptorchidism is very rare. PMID:24521664

  11. Syndrome in question*

    PubMed Central

    Dalapicola, Monique Coelho; Veasey, John Verrinder; Lellis, Rute Facchini

    2016-01-01

    Ross syndrome is a rare disease characterized by peripheral nervous system dysautonomia with selective degeneration of cholinergic fibers. It is composed by the triad of unilateral or bilateral segmental anhidrosis, deep hyporeflexia and Holmes-Adie's tonic pupil. The presence of compensatory sweating is frequent, usually the symptom that most afflicts patients. The aspects of the syndrome are put to discussion due to the case of a male patient, caucasian, 47 years old, with clinical onset of 25 years.

  12. Management and outcomes in massive bilateral Wilms’ tumors

    PubMed Central

    Agarwala, Sandeep; Mittal, Deepak; Bhatnagar, Veereshwar; Srinivas, M; Bakhshi, Sameer; Bajpai, Minu; Gupta, Devendra Kumar; Iyer, V. K.; Mohanti, Bidyut K; Thulkar, Sanjay

    2014-01-01

    Purpose: To evaluate the outcome of children with bilateral Wilms’ tumor (BWT) treated on All India Institute of Medical Sciences-Wilms Tumor-99 (AIIMS-WT-99) protocol. Materials and Methods: All children with BWT, registered in our solid tumor clinic from August 1999 through December 2010 were included. Results: Of the 178 fresh cases of Wilms Tumor (WT) treated during this period, 11 (6.2%) had bilateral involvement. All patients except one (12 and 3 cm), had massive bilateral tumors of more than 10 cm on each side. There were eight boys and three girls in the age range 6–30 months. One patient had Denys-Drash syndrome. Twenty renal units were operated upon (12 tumorectomy, five partial nephrectomy, and three nephrectomies), while one patient with inferior vena cava (IVC) thrombus died of renal failure. Tumor spill occurred in three units, lymphnode was positive in two patients. Local recurrence occurred in four patients (six of 18 renal units (33%)—two bilateral and two unilateral). There was one recurrence in the liver that was treated with radio-frequency ablation. The 5-year overall survival (OS) was 90% (95% confidence interval (CI) = 50.8–98.6) and the relapse free survival (RFS) was 38% (95% CI = 6.1–71.6). Conclusion: Massive BWT respond poorly to preoperative chemotherapy, are often not amenable to partial nephrectomy/tumorectomy and have a higher local recurrence rate, giving a poor RFS. PMID:25336802

  13. Bilateral radial ray hypoplasia with multiple epiphyseal dysplasia.

    PubMed

    Eddy, M C; Steiner, R D; McAlister, W H; Whyte, M P

    1998-05-18

    We describe a 5-4/12-year-old girl with the unique combination of bilateral radial ray hypoplasia and multiple epiphyseal dysplasia (MED). Radial ray hypoplasia was diagnosed at birth. MED was documented at age 4-3/12 years when she presented with leg pain and short stature and was found to have femoral anteversion and tibial torsion giving rise to severe genu valgum deformity and intoeing. She has no facial anomalies and is developmentally normal. Family history is unremarkable and chromosomal analysis was normal. Investigation of mineral metabolism showed idiopathic hypercalciuria. Surgical lengthening of her severely hypoplastic left radius at age 19 months was successful. Bilateral femoral and tibial osteotomies at age 5-4/12 years corrected her lower limb deformities. This combination of two distinctive but rare skeletal abnormalities may represent a new syndrome. PMID:9605584

  14. Kartagener syndrome.

    PubMed

    Skeik, Nedaa; Jabr, Fadi I

    2011-01-01

    Kartagener syndrome is a rare, ciliopathic, autosomal recessive genetic disorder that causes a defect in the action of the cilia lining the respiratory tract and fallopian tube. Patients usually present with chronic recurrent rhinosinusitis, otitis media, pneumonia, and bronchiectasis caused by pseudomonal infection. Situs inversus can be seen in about 50% of cases. Diagnosis can be made by tests to prove impaired cilia function, biopsy, and genetic studies. Treatment is supportive. In severe cases, the prognosis can be fatal if bilateral lung transplantation is delayed. We present a case of a 66-year-old woman with chronic recurrent upper respiratory infections, pseudomonal pneumonia, and chronic bronchiectasis who presented with acute respiratory failure. She was diagnosed with Kartagener syndrome based on her clinical presentation and genetic studies. She expired on ventilator with refractory respiratory and multiorgan failure. PMID:21403791

  15. LUNG EDEMA FOLLOWING BILATERAL VAGOTOMY

    PubMed Central

    Lorber, Victor

    1939-01-01

    1. Small animals (rat and guinea pig) vagotomized in the neck die within a period of hours, the lungs showing extensive congestion and edema. 2. Tracheotomy permits appreciably longer survival with minimal lung changes approximating those seen in the control animals. 3. Intrathoracic vagotomy (sparing the recurrent laryngeal nerve) on one side, and cervical vagotomy on the other, permits almost indefinite survival (guinea pig and rabbit), unless laryngeal paralysis from the unilateral denervation produces respiratory obstruction (rat, guinea pig, and rabbit). 4. Pulmonary edema following bilateral vagotomy probably results primarily from respiratory obstruction. It is suggested that circulatory failure may also be a factor of some importance. The rôle of vagotomy itself is considered in relationship to these two phenomena. 5. The reaction of smaller animals to bilateral vagotomy, with regard to lung changes, apparently differs in no way from that of the larger animals, but is less readily demonstrated because of the smaller diameters of the air passages. PMID:19870894

  16. Bilateral Crossed "Unfused" Renal Ectopia.

    PubMed

    Gambhir, Aashish; James, Vivek M; Kumar, Amit; Jaimini, Abhinav; Mondal, Anupam

    2015-10-01

    Ectopic kidney-uncrossed or crossed, or with and without fusion-is a common congenital developmental anomaly. Commonly employed imaging modalities used for evaluating such condition include ultrasonography, IV pyelography, and contrast-enhanced CT scan. Here in, we describe a rare case report of an incidentally detected bilateral crossed renal ectopia without fusion on Tc-DTPA scan in the setting of inconclusive ultrasonography and deranged renal function. PMID:26204207

  17. Note: Unshielded bilateral magnetoencephalography system using two-dimensional gradiometers

    NASA Astrophysics Data System (ADS)

    Seki, Yusuke; Kandori, Akihiko; Ogata, Kuniomi; Miyashita, Tsuyoshi; Kumagai, Yukio; Ohnuma, Mitsuru; Konaka, Kuni; Naritomi, Hiroaki

    2010-09-01

    Magnetoencephalography (MEG) noninvasively measures neuronal activity with high temporal resolution. The aim of this study was to develop a new type of MEG system that can measure bilateral MEG waveforms without a magnetically shielded room, which is an obstacle to reducing both the cost and size of an MEG system. An unshielded bilateral MEG system was developed using four two-dimensional (2D) gradiometers and two symmetric cryostats. The 2D gradiometer, which is based on a low-Tc superconducting quantum interference device and wire-wound pickup coil detects a magnetic-field gradient in two orthogonal directions, or ∂/∂x(∂2Bz/∂z2), and reduces environmental magnetic-field noise by more than 50 dB. The cryostats can be symmetrically positioned in three directions: vertical, horizontal, and rotational. This makes it possible to detect bilateral neuronal activity in the cerebral cortex simultaneously. Bilateral auditory-evoked fields (AEF) of 18 elderly subjects were measured in an unshielded hospital environment using the MEG system. As a result, both the ipsilateral and the contralateral AEF component N100m, which is the magnetic counterpart of electric N100 in electroencephalography and appears about 100 ms after the onset of an auditory stimulus, were successfully detected for all the subjects. Moreover, the ipsilateral P50m and the contralateral P50m were also detected for 12 (67%) and 16 (89%) subjects, respectively. Experimental results demonstrate that the unshielded bilateral MEG system can detect MEG waveforms, which are associated with brain dysfunction such as epilepsy, Alzheimer's disease, and Down syndrome.

  18. Spontaneous bilateral fracture of patella.

    PubMed

    Moretti, Biagio; Speciale, Domenico; Garofalo, Raffaele; Moretti, Lorenzo; Patella, Silvio; Patella, Vittorio

    2008-03-01

    Bilateral patellae fractures represent a rare entity, accounting for approximately 2.9% of all lesions interesting in this anatomical district. In most cases found in the published work, they are described as stress fractures or as complications of chronic diseases such as osteoporosis, renal failure and secondary hyperparathyroidism. Although many pathogenetic mechanisms have been supposed, none have been proved for certain. Insufficiency fractures of the patellae are rare events and no data has been published on their incidence. We present a case of bilateral fracture of the patellae due to an indirect trauma occurring in an 85-year-old patient affected by Parkinson's disease, osteoporosis and diffuse degenerative osteoarthritis. X-ray of the knees (anteroposterior and lateral) and magnetic resonance imaging evaluation confirmed the fractures. The patient was treated conservatively. She had a good result, returning to her previous autonomous ambulation. This case is unusual because there was no direct trauma to the knees because of bilaterality, but confirmed previous observations about insufficiency fractures of patellae in the presence of comorbidity. Insufficiency fractures of patellae can be an insidious condition in elderly people. Prepatellar pain, a common symptom in the relapse phase of degenerative arthritis of the knee, should not be underestimated, particularly in patients with diseases influencing metabolism of bone and with an elevated risk of fall. A periodical clinical and instrumental follow up should be done in these patient. Moreover, we underline the necessity of a multidisciplinary approach. PMID:18713190

  19. Compensation Following Bilateral Vestibular Damage

    PubMed Central

    McCall, Andrew A.; Yates, Bill J.

    2011-01-01

    Bilateral loss of vestibular inputs affects far fewer patients than unilateral inner ear damage, and thus has been understudied. In both animal subjects and human patients, bilateral vestibular hypofunction (BVH) produces a variety of clinical problems, including impaired balance control, inability to maintain stable blood pressure during postural changes, difficulty in visual targeting of images, and disturbances in spatial memory and navigational performance. Experiments in animals have shown that non-labyrinthine inputs to the vestibular nuclei are rapidly amplified following the onset of BVH, which may explain the recovery of postural stability and orthostatic tolerance that occurs within 10?days. However, the loss of the vestibulo-ocular reflex and degraded spatial cognition appear to be permanent in animals with BVH. Current concepts of the compensatory mechanisms in humans with BVH are largely inferential, as there is a lack of data from patients early in the disease process. Translation of animal studies of compensation for BVH into therapeutic strategies and subsequent application in the clinic is the most likely route to improve treatment. In addition to physical therapy, two types of prosthetic devices have been proposed to treat individuals with bilateral loss of vestibular inputs: those that provide tactile stimulation to indicate body position in space, and those that deliver electrical stimuli to branches of the vestibular nerve in accordance with head movements. The relative efficacy of these two treatment paradigms, and whether they can be combined to facilitate recovery, is yet to be ascertained. PMID:22207864

  20. Common and uncommon bilateral adult renal masses

    PubMed Central

    Roy, Anjali; Silverman, Paul M.; Kundra, Vikas

    2012-01-01

    Abstract Masses can involve the kidney unilaterally or bilaterally. The purpose of this article is to review common and uncommon adult renal masses that present bilaterally. Clinical and imaging findings are described. Renal masses that present in a bilateral fashion can have particular clinical and imaging characteristics and knowledge of their presentation enables appropriate diagnosis and management, especially in a multidisciplinary care setting. More commonly found bilateral renal masses that are discussed include metastasis, lymphoproliferative disorders, adult polycystic kidney disease, angiomyolipomas, renal infracts and renal abscesses. Less common bilateral renal masses include transitional cell carcinoma, oncocytoma, and hematomas. PMID:22750134

  1. Bilateral Ureteral Obstruction in Children after Appendectomy

    PubMed Central

    Grande, M.; Lisi, G.; Bianchi, D.; Bove, P.; Miano, R.; Esser, A.; De Sanctis, F.; Neri, A.; Grande, S.; Villa, M.

    2015-01-01

    Acute renal failure due to bilateral ureteral obstruction is a rare complication after appendectomy in children. We report a case of bilateral ureteric obstruction in a 14-year-old boy nine days after surgery for an acute appendicitis. After saline-filling of the urinary bladder, transabdominal ultrasound demonstrated bilateral hydronephrosis of moderate degree. No abscess was found with CT but presence of millimetric stones on both distal ureters was shown, with bilateral calyceal dilatation. Cystoscopy revealed inflammatory changes in the bladder base. Following introduction of bilateral ureteric stents, there was rapid normalisation of urinary output and serum creatinine. PMID:26295001

  2. A 19-year-old man with relapsing bilateral pneumothorax, hemoptysis, and intrapulmonary cavitary lesions diagnosed with vascular Ehlers-Danlos syndrome and a novel missense mutation in COL3A1.

    PubMed

    Abrahamsen, Bjørg J; Kulseth, Mari Ann; Paus, Benedicte

    2015-05-01

    A 19-year-old sportsman experienced a right-sided pneumothorax and hemoptysis after having had an intermittent cough and blood-tinged sputum for 2 months. A chest CT scan revealed small cavitary lesions in both lungs. The relapsing pneumothorax was treated with a chest tube twice, as well as surgically after the second relapse. Two months after surgery, the patient developed a cough, fever, and high C-reactive protein levels. At that time, large consolidations had developed in the right lung, while the left lung subsequently collapsed due to pneumothorax. The patient's physical appearance and anamnestic information led us to suspect a genetic connective tissue disease. A sequencing analysis of the COL3A1 gene identified a novel, de novo missense mutation that confirmed the diagnosis of vascular Ehlers-Danlos syndrome (EDS). This atypical presentation of vascular EDS with intrathoracic complications shows that enhanced awareness is required and demonstrates the usefulness of the genetic analyses that are clinically available for several hereditary connective tissue disorders. PMID:25940258

  3. Bilateral hippocampal stroke secondary to acute cocaine intoxication

    PubMed Central

    Connelly, Kathryn L.; Chen, Xiao; Kwan, Pei Fun

    2015-01-01

    Hippocampal infarction is a rare complication of cocaine use, with only two cases previously reporting this association. We present a 44-year-old male who developed a persistent amnesic syndrome following cocaine intoxication. Examination identified no other neurological deficits. Subsequent MRI brain revealed high FLAIR signals and diffusion restriction in the hippocampus and centrum semiovale bilaterally, consistent with infarction. These findings were in keeping with the results of formal neuropsychological testing where deficits in both verbal and visual episodic memory and learning capacity were identified, consistent with hippocampal dysfunction. In contrast to previous reports, this presentation occurred in the absence of other vascular risk factors or hypoxic insults. PMID:26634127

  4. Selective cognitive patterns resulting from bilateral hippocampal ischemia

    PubMed Central

    Cachia, David; Swearer, Joan; Ferguson, Warren; Moonis, Majaz

    2011-01-01

    A 54-year-old diabetic, hypertensive man with poorly controlled moderate-severe sleep apnea presented with acute onset of severe anterograde amnesia and well preserved remote memory without additional cognitive impairment. Investigations, including a lumbar puncture, electroencephalogram (EEG) and serology testing ruled out infectious, neoplastic and epilleptogenic causes. MRI taken 10 days after symptom onset, was suggestive of sequential ischemic damage to both hippocampal formations. Neuropyschological evaluation suggested a focal and dense amnestic syndrome with little improvement over time. The bilateral nature of hippocampal ischemia though has been reported, is rare. PMID:22291752

  5. Bilateral adrenalectomy in the 21st century: when to use it for hypercortisolism?

    PubMed

    Guerin, Carole; Taieb, David; Treglia, Giorgio; Brue, Thierry; Lacroix, André; Sebag, Frederic; Castinetti, Frederic

    2016-02-01

    Therapeutic options available for the treatment of Cushing's syndrome (CS) have expanded over the last 5 years. For instance, the efficient management of severe hypercortisolism using a combination of fast-acting steroidogenesis inhibitors has been reported. Recent publications on the long-term efficacy of drugs or radiation techniques have also demonstrated low toxicity. These data should encourage endocrinologists to reconsider the place of bilateral adrenalectomy in patients with ACTH-dependent aetiologies of CS; similarly, the indication of bilateral adrenalectomy is reassessed in primary bilateral macronodular adrenal hyperplasia. The objective of this review is to compare the efficacy and side effects of the various therapeutic options of hypercortisolism with those of bilateral adrenalectomy, in order to better define its indications in the 21st century. PMID:26739832

  6. Multiresolution Bilateral Filtering for Image Denoising

    PubMed Central

    Zhang, Ming; Gunturk, Bahadir K.

    2008-01-01

    The bilateral filter is a nonlinear filter that does spatial averaging without smoothing edges; it has shown to be an effective image denoising technique. An important issue with the application of the bilateral filter is the selection of the filter parameters, which affect the results significantly. There are two main contributions of this paper. The first contribution is an empirical study of the optimal bilateral filter parameter selection in image denoising applications. The second contribution is an extension of the bilateral filter: multiresolution bilateral filter, where bilateral filtering is applied to the approximation (low-frequency) subbands of a signal decomposed using a wavelet filter bank. The multiresolution bilateral filter is combined with wavelet thresholding to form a new image denoising framework, which turns out to be very effective in eliminating noise in real noisy images. Experimental results with both simulated and real data are provided. PMID:19004705

  7. [Therapeutic approach to bilateral hepatoblastoma].

    PubMed

    Babut, J M; Le Gall, E; Bourdelat, D; Jouan, H; Gruel, Y

    1983-01-01

    The treatment of a case of bilateral hepatoblastoma by continuous infusion of adriamycin in the hepatic artery accompanied by ligature of the right hepatic artery enabled the authors to carry out six weeks later an extensive right lobe hepatectomy. Unfortunately the patient died seven weeks after the partial hepatectomy from complication of surgery. Thus it was possible to demonstrate the effectiveness of the intra arterial chemotherapy. No residual tumor could be found at autopsy. As in the case with the adult, arterial ligature combined with carefully targeted chemotherapy would seem to be potentially important therapeutic option in the treatment of malignant liver tumors in children. PMID:6303615

  8. Bilateral inguinal hernias containing ovaries.

    PubMed

    Basrur, Gurudutt Bhaskar

    2015-01-28

    Inguinal hernias are rare in females. The authors report a case of bilateral inguinal hernias in a 10-year-old female. On exploration, the patient was found to be having a sliding hernia containing incarcerated ovary as contents on both sides. Peroperatively the contents were reduced, the sac was transfixed at its base and the redundant sac was excised. The repair of this form of hernias is more difficult because of adhesions between the contents and the wall of the sac and risk of damage during dissection. A description of this clinical presentation in the pre operative assessment and operative management are discussed in this report. PMID:25918632

  9. Amelogenesis imperfecta with bilateral nephrocalcinosis

    PubMed Central

    Poornima, P; Katkade, Shashikant; Mohamed, Roshan Noor; Mallikarjuna, Rachappa

    2013-01-01

    A 12-year-old patient presented with a severe delay of eruption in permanent maxillary and mandibular incisors. On examination, there was over-retained primary teeth and delayed eruption of permanent teeth. Retained primary teeth showed light yellow discolouration whereas permanent teeth were distinct yellow with thin or little enamel. Subsequent imaging revealed all the premolars except maxillary left first premolar showed signs of intra-alveolar coronal resorption, nephrocalcinosis with bilateral multiple calculi and small papillary tip calcifications, marked increase in alkaline phosphatase. Subsequent dental treatment for restoring the functional and aesthetic requirement followed by appropriate treatment for renal problem was undertaken. PMID:23709541

  10. Bilateral Inguinal Hernias Containing Ovaries

    PubMed Central

    Basrur, Gurudutt Bhaskar

    2015-01-01

    Inguinal hernias are rare in females. The authors report a case of bilateral inguinal hernias in a 10-year-old female. On exploration, the patient was found to be having a sliding hernia containing incarcerated ovary as contents on both sides. Peroperatively the contents were reduced, the sac was transfixed at its base and the redundant sac was excised. The repair of this form of hernias is more difficult because of adhesions between the contents and the wall of the sac and risk of damage during dissection. A description of this clinical presentation in the pre operative assessment and operative management are discussed in this report. PMID:25918632

  11. Bilateral peroneal palsy after weightlifting.

    PubMed

    Kyavar, Leila; Heckmann, Josef G

    2013-09-01

    In a 48-year-old otherwise healthy man, a bilateral common peroneal palsy was diagnosed clinically and neurophysiologically. He reported on strength training with weights in both arms, lifting the weights and his upper body from a deep squatting position with broadly positioned legs akimbo in a hitherto unusual intensity. Regarding the pathophysiological mechanisms, 2 options are considered: first, stretching of the nerve at the fascia of the peroneal longus muscle and along the fibula neck, and second, compression of the nerve during squatting with weights loaded and with strongly activated anterior tibial and peroneal muscles. PMID:23615488

  12. [Bilateral chylothorax after gastric surgery].

    PubMed

    Medina, E; Anguiano, M P; Agudo, O; Lobo, J; Tihista, J A; Alonso, I; Calvo, A

    2005-01-01

    Chylothorax is a lymphatic effusion of chylous in the pleural space due to thoracic duct obstruction or injury. The most frequent aetiology is cancer; it is also related to chest trauma and iatrogenic. We describe the case of bilateral chylothorax in a 76-year-old woman, right predominant, during the post-operational phase of gastric surgery. This presented itself with respiratory insufficiency and tachycardia without initial haemodynamic compromise. It presented a favourable evolution after conservative treatment, cessation of oral intake and TPN and chest tube during 10 to 14 days. PMID:16421622

  13. The management of bilateral Wilms tumor

    PubMed Central

    Özyörük, Derya

    2014-01-01

    Wilms tumor (WT) is the most common malignant renal tumor in childhood. Approximately 5-7% of WT patients present with bilateral disease, either synchronously or metachronously. Bilateral WT usually occurs in younger children and more often in girls. Management of a child with bilateral WT is very challenging. In contrast to unilateral WT, there has not been uniform agreement about the therapeutic strategy in the management of bilateral WT. As surgery is a critical component in the treatment of WT, the aim is to achieve a high cure rate while maintaining adequate long-term renal function in patients with bilateral WT. In the past, radical surgical procedures which lead to the patients on dialysis have been traditionally recommended in these patients. After several multicentre trials, bilateral biopsies followed by pre-operative chemotherapy and then renal salvage surgery have been recommended. The management of bilateral WT has evolved from primary surgical extirpation to kidney-preserving resection after preoperative chemotherapy. Preoperative chemotherapy often results in significant reduction in tumor size, thereby facilitating subsequent renal salvage. The analysis of children with bilateral WT shows that preservation of renal parenchyma is possible following initial preoperative chemotherapy. Only centers with experience in bilateral WT should treat the cases with bilateral WT to provide optimal treatment. PMID:26835321

  14. Giant bilateral adrenal myelolipoma with congenital adrenal hyperplasia.

    PubMed

    Al-Bahri, S; Tariq, A; Lowentritt, B; Nasrallah, D V

    2014-01-01

    Myelolipomas are rare and benign neoplasms, predominant of the adrenal glands, consisting of adipose and mature hematopoietic tissue, commonly discovered incidentally with increased use of radiologic imaging. Few cases of giant bilateral adrenal masses are reported, especially in the setting of congenital adrenal hyperplasia (CAH). We report the case of a 39-year-old male with a history of CAH secondary to 21-? hydroxylase deficiency on steroids since childhood, self-discontinued during adolescence, presenting with abdominal distension, fatigue, decreased libido, and easy bruising. Imaging revealed giant bilateral adrenal masses. He subsequently underwent bilateral adrenalectomy found to be myelolipomas measuring 30 × 25 × 20?cm on the left and weighing 4.1?kg and 25 × 20 × 13?cm on the right and weighing 2.7?kg. Adrenal myelolipomas are found to coexist with many other conditions such as Cushing's syndrome, Addison's disease, and CAH. We discuss the association with high adrenocorticotropic hormone (ACTH) states and review the studies involving ACTH as proponent leading to myelolipomas. Massive growth of these tumors, as in our case, can produce compression and hemorrhagic symptoms. We believe it is possible that self-discontinuation of steroids, in the setting of CAH, may have resulted in the growth of his adrenal masses. PMID:25140269

  15. Giant Bilateral Adrenal Myelolipoma with Congenital Adrenal Hyperplasia

    PubMed Central

    Al-Bahri, S.; Tariq, A.; Lowentritt, B.; Nasrallah, D. V.

    2014-01-01

    Myelolipomas are rare and benign neoplasms, predominant of the adrenal glands, consisting of adipose and mature hematopoietic tissue, commonly discovered incidentally with increased use of radiologic imaging. Few cases of giant bilateral adrenal masses are reported, especially in the setting of congenital adrenal hyperplasia (CAH). We report the case of a 39-year-old male with a history of CAH secondary to 21-? hydroxylase deficiency on steroids since childhood, self-discontinued during adolescence, presenting with abdominal distension, fatigue, decreased libido, and easy bruising. Imaging revealed giant bilateral adrenal masses. He subsequently underwent bilateral adrenalectomy found to be myelolipomas measuring 30 × 25 × 20?cm on the left and weighing 4.1?kg and 25 × 20 × 13?cm on the right and weighing 2.7?kg. Adrenal myelolipomas are found to coexist with many other conditions such as Cushing's syndrome, Addison's disease, and CAH. We discuss the association with high adrenocorticotropic hormone (ACTH) states and review the studies involving ACTH as proponent leading to myelolipomas. Massive growth of these tumors, as in our case, can produce compression and hemorrhagic symptoms. We believe it is possible that self-discontinuation of steroids, in the setting of CAH, may have resulted in the growth of his adrenal masses. PMID:25140269

  16. Syndromic Scoliosis

    MedlinePLUS

    ... Neurofibromatosis (NF) Noonan Syndrome VATER/VACTERL Syndrome Angelman Syndrome Rett Prader Willi Osteogenesis Imperfecta Trisomy 21 (Down's Syndrome) Symptoms Highly variable based on underlying syndrome and ...

  17. [Recovery of Cushing syndrome revealing McCune-Albright syndrome].

    PubMed

    Halioui-Louhaichi, S; Dridi, Y; Azzabi, O; Selmi, I; Fetni, I; Siala, N; Maherzi, A

    2016-01-01

    Cushing syndrome (CS) is a rare feature of McCune-Albright syndrome. Treatments consist of bilateral adrenalectomy followed by lifelong glucocorticoid and mineralocorticoid treatment. However, cases of spontaneous remission of CS have been reported in the literature. We report a case of McCune-Albright syndrome with CS treated with metyrapone for 30 months with prolonged remission after a 12-year follow-up. Adrenalectomy may be avoided in some cases of CS caused by McCune-Albright syndrome. Metyrapone could be a good alternative to surgical treatment. PMID:26552628

  18. Bilateral internal thoracic artery grafting

    PubMed Central

    2013-01-01

    The effectiveness of the left internal mammary artery graft to the anterior descending coronary artery as a surgical strategy has been shown to improve the survival rate and decrease the risk of adverse cardiac events in patients undergoing coronary bypass surgery. These clinical benefits appear to be related to the superior short and long-term patency rates of the internal thoracic artery graft. Although the advantages of using of both internal thoracic arteries (ITA) for bypass grafting have taken longer to prove, recent results from multiple data sets now support these findings. The major advantage of bilateral ITA grafting appears to be improved survival rate, while the disadvantages of complex ITA grafting include the increased complexity of operation, and an increased risk of wound complications. While these short-term disadvantages have been mitigated in contemporary surgical practice, they have not eliminated. Bilateral ITA grafting should be considered the procedure of choice for patients undergoing coronary bypass surgery that have a predicted survival rate of longer than ten years. PMID:23977627

  19. SRBF: Speckle reducing bilateral filtering.

    PubMed

    Balocco, Simone; Gatta, Carlo; Pujol, Oriol; Mauri, Josepa; Radeva, Petia

    2010-08-01

    Speckle noise negatively affects medical ultrasound image shape interpretation and boundary detection. Speckle removal filters are widely used to selectively remove speckle noise without destroying important image features to enhance object boundaries. In this article, a fully automatic bilateral filter tailored to ultrasound images is proposed. The edge preservation property is obtained by embedding noise statistics in the filter framework. Consequently, the filter is able to tackle the multiplicative behavior modulating the smoothing strength with respect to local statistics. The in silico experiments clearly showed that the speckle reducing bilateral filter (SRBF) has superior performances to most of the state of the art filtering methods. The filter is tested on 50 in vivo US images and its influence on a segmentation task is quantified. The results using SRBF filtered data sets show a superior performance to using oriented anisotropic diffusion filtered images. This improvement is due to the adaptive support of SRBF and the embedded noise statistics, yielding a more homogeneous smoothing. SRBF results in a fully automatic, fast and flexible algorithm potentially suitable in wide ranges of speckle noise sizes, for different medical applications (IVUS, B-mode, 3-D matrix array US). PMID:20691924

  20. Bilateral zosteriform extragenital lichen sclerosus.

    PubMed

    Kumar, Piyush; Jha, Abhijeet Kumar; Mallik, Sambeet Kumar; Raihan, Mohammed

    2014-01-01

    A 35-year-old man presented with asymptomatic eruption on both forearms and lower aspects of the legs for 6 months. The lesions first appeared on his inner aspects of the wrist, the dorsal surface of the hands, and legs and progressed to involve proximal aspects of the extremities. There was no significant past history. On examination, multiple pearly white papules and depigmented atrophic plaques were found bilaterally on the flexors of the arms and the extensors of the legs. The lesions were arranged in a linear manner, following the lines of Blaschko (Figures 1 and 2). The surface of the atrophic plaques was notable for prominent telangiectasia, giving an erythematous appearance. The genitalia, oral cavity, palms, and soles were spared. Systemic examination was noncontributory. Lichen striatus and extragenital lichen sclerosus (ELS) were considered the differential diagnosis. Clinically, the age of the patient, the absence of scaling, and the presence of atrophic plaques and telangiectasia were in favor of ELS. A punch biopsy from an atrophic plaque was performed, and it revealed hyperkeratosis, atrophic epidermis, basal layer vacuolar degeneration, mild lymphocytic infiltration in the dermis, edema, and homogenization of collagen of the upper portion of the dermis (Figures 3 and Figure 4). Histopathologic findings were consistent with lichen sclerosus. A diagnosis of bilateral zosteriform ELS was made. PMID:24933856

  1. Risk of bilateral idiopathic preretinal macular fibrosis.

    PubMed

    Hikichi, T; Trempe, C L

    1995-01-01

    To ascertain the risk of the development of bilateral idiopathic preretinal macular fibrosis, we retrospectively studied 380 consecutive patients with idiopathic preretinal macular fibrosis. Eighty (21%) patients had bilateral involvement. Sixteen (39%) of 41 patients with diabetes, 40 (28%) of 144 with hypertension, and 12 of 21 (57%) with bilateral high myopia had bilateral involvement. The prevalence of bilateral involvement was significantly higher in patients with these three pathologies than in patients without these conditions (p < 0.01, p < 0.02 and p < 0.01, respectively). In patients with diabetes or hypertension, no significant difference was found in the prevalence of posterior vitreous detachment (PVD) between involved or uninvolved eyes. Diabetes, hypertension even without retinopathy, and high myopia may be risk factors for bilateral involvement of idiopathic preretinal macular fibrosis. Factors other than PVD may be involved in the development of idiopathic preretinal macular fibrosis in patients with diabetes or hypertension. PMID:7713252

  2. Bilateral Adrenocortical Masses Producing Aldosterone and Cortisol Independently

    PubMed Central

    Lee, Seung-Eun; Lee, You-Bin; Seok, Hyeri; Shin, In Seub; Eun, Yeong Hee; Kim, Jung-Han; Oh, Young Lyun

    2015-01-01

    A 31-year-old woman was referred to our hospital with symptoms of hypertension and bilateral adrenocortical masses with no feature of Cushing syndrome. The serum aldosterone/renin ratio was elevated and the saline loading test showed no suppression of the plasma aldosterone level, consistent with a diagnosis of primary hyperaldosteronism. Overnight and low-dose dexamethasone suppression tests showed no suppression of serum cortisol, indicating a secondary diagnosis of subclinical Cushing syndrome. Adrenal vein sampling during the low-dose dexamethasone suppression test demonstrated excess secretion of cortisol from the left adrenal mass. A partial right adrenalectomy was performed, resulting in normalization of blood pressure, hypokalemia, and high aldosterone level, implying that the right adrenal mass was the main cause of the hyperaldosteronism. A total adrenalectomy for the left adrenal mass was later performed, resulting in a normalization of cortisol level. The final diagnosis was bilateral adrenocortical adenomas, which were secreting aldosterone and cortisol independently. This case is the first report of a concurrent cortisol-producing left adrenal adenoma and an aldosterone-producing right adrenal adenoma in Korea, as demonstrated by adrenal vein sampling and sequential removal of adrenal masses. PMID:26248855

  3. Bilateral Adrenocortical Masses Producing Aldosterone and Cortisol Independently.

    PubMed

    Lee, Seung Eun; Kim, Jae Hyeon; Lee, You Bin; Seok, Hyeri; Shin, In Seub; Eun, Yeong Hee; Kim, Jung Han; Oh, Young Lyun

    2015-12-01

    A 31-year-old woman was referred to our hospital with symptoms of hypertension and bilateral adrenocortical masses with no feature of Cushing syndrome. The serum aldosterone/renin ratio was elevated and the saline loading test showed no suppression of the plasma aldosterone level, consistent with a diagnosis of primary hyperaldosteronism. Overnight and low-dose dexamethasone suppression tests showed no suppression of serum cortisol, indicating a secondary diagnosis of subclinical Cushing syndrome. Adrenal vein sampling during the low-dose dexamethasone suppression test demonstrated excess secretion of cortisol from the left adrenal mass. A partial right adrenalectomy was performed, resulting in normalization of blood pressure, hypokalemia, and high aldosterone level, implying that the right adrenal mass was the main cause of the hyperaldosteronism. A total adrenalectomy for the left adrenal mass was later performed, resulting in a normalization of cortisol level. The final diagnosis was bilateral adrenocortical adenomas, which were secreting aldosterone and cortisol independently. This case is the first report of a concurrent cortisol-producing left adrenal adenoma and an aldosterone-producing right adrenal adenoma in Korea, as demonstrated by adrenal vein sampling and sequential removal of adrenal masses. PMID:26248855

  4. Unusual Bilateral Paramolars Associated with Clinical Complications

    PubMed Central

    Sulabha, A. N.; Sameer, C.

    2015-01-01

    Paramolars are rare supernumerary structures of maxillofacial complex that occur buccally or lingually near the molar row. Predominantly these occur singly; bilateral presentation is very rare. This paper reports two unusual bilateral presentations of paramolars with clinical complication and its management. One of the cases in the present paper also documents the cooccurrence of bilateral paramolars and microdontia of single tooth and one of its paramolars presented with multilobed crown with an anomalous buccal tubercle. PMID:26078890

  5. [Massive bilateral adrenal hemorrhage: role of imaging].

    PubMed

    Kably, M I; Zamiati, W; Benkirane, H; Kadiri, R

    2004-05-01

    Bilateral adrenal hemorrhage is a rare and potentially life threatening situation in adults. The clinical presentation is non-specific, and the diagnosis is based on imaging. The purpose of this report is to provide an illustrative case of spontaneous bilateral adrenal hemorrhage that occurred during pregnancy. The sonographic and computed tomographic findings included large bilateral adrenal hematomas with no evidence of underlying malignancy. Since bilateral adrenal hemorrhage is a rare but potentially life threatening situation, prompt laboratory and imaging evaluation are essential and may reduce both morbidity and mortality. PMID:15205660

  6. Bilateral multifocal renal oncocytoma in pregnancy

    PubMed Central

    Wentzel, Schalk W.; Vermeulen, Lodewikus P.

    2012-01-01

    Renal oncocytomas are the most common benign solid renal tumor, accounting for 3–7% of renal neoplasms. Oncocytomas are multifocal in 2–12% and bilateral in 4–14% of cases. Multifocal bilateral oncocytomas represent only 1.4% of renal cases. We present an extraordinary case of a patient with multifocal bilateral renal oncocytomas during pregnancy. An electronic literature search revealed fewer than 30 reports of on cases of bilateral multifocal renal oncocytomas, none of them occurring in pregnancy. The management of this patient differed from the recommended guidelines for renal masses suspected to be malignant because elective caesarean section and nephrectomy in the second trimester was refused. PMID:23372918

  7. Bilateral Neck of Femur Fractures in a Bilateral Below-Knee Amputee: A Unique Case

    PubMed Central

    Lancer, Hannah R.

    2016-01-01

    According to the National Hip Fracture Database, over 64,000 patients were admitted with a hip fracture across England, Wales, and Northern Ireland in 2013, but very few are bilateral, and there are no current cases in the literature of bilateral neck of femur fractures in a patient with bilateral below-knee amputations. We present a case of a 69-year-old bilateral below-knee amputee male admitted to the emergency department with bilateral hip pain and radiological evidence of bilateral displaced neck of femur fractures. The patient subsequently underwent synchronous bilateral total hip replacements under general anaesthetic and an epidural and then went on to make a full recovery. He was discharged 27 days after arrival in hospital. Outpatient follow-up at 3 months has shown that the patient has returned to a similar level of preinjury function and is still able to carry out his daily activities with walking aids and bilateral leg prostheses. PMID:26881162

  8. CUSHING'S SYNDROME

    PubMed Central

    Wilkinson, Allan B.

    1961-01-01

    Sixteen cases of verified Cushing's syndrome, and twelve cases of probable Cushing's syndrome were reviewed and data on them were compared with various reports on Cushing's syndrome in the literature. The diagnosis hinges upon a high index of suspicion, and one or several of the major criteria may be lacking. Ultimate establishment of correct diagnosis should be based largely on the clinical features, although stimulation and suppression tests may help to confirm a clinical diagnosis. In well-established clinical cases, with borderline laboratory confirmation, exploration may be justified, especially if tests fail to identify a specific cause. In cases of adrenal cortical tumor, all pathological tissue should be removed if possible, with great care to support and stimulate the remaining atrophic adrenal gland during and following operation. In cases of bilateral adrenal cortical hyperplasia, the problem is one of how much to remove. At present most investigators advocate radical subtotal resection, leaving less than 10 per cent of one side. ImagesFigure 1.Figure 2.Figure 3.Figure 4.Figure 5.Figure 6. PMID:13785315

  9. "Benign" shaken baby syndrome. Case report.

    PubMed

    Martínez-Lage, J F; Ros de San Pedro, J; Puche, A; Pérez-Espejo, M A

    2006-08-01

    The authors report an infant with clinical and neuroimaging findings of shaken baby syndrome. The pitfalls encountered in the assessment on the cause of the bilateral frontal and interhemispheric subdural hematomas in this child are also briefly discussed. We have called this condition "benign" shaken baby syndrome and emphasize that not always acute subdural hematomas are of non-accidental nature. PMID:16960646

  10. A Rare Entity: Bilateral First Rib Fractures Accompanying Bilateral Scapular Fractures

    PubMed Central

    Gulbahar, Gultekin; Kaplan, Tevfik; Turker, Hasan Bozkurt; Gundogdu, Ahmet Gokhan; Han, Serdar

    2015-01-01

    First rib fractures are scarce due to their well-protected anatomic locations. Bilateral first rib fractures accompanying bilateral scapular fractures are very rare, although they may be together with scapular and clavicular fractures. According to our knowledge, no case of bilateral first rib fractures accompanying bilateral scapular fractures has been reported, so we herein discussed the diagnosis, treatment, and complications of bone fractures due to thoracic trauma in bias of this rare entity. PMID:26175916

  11. Bilateral primary extramedullary orbital plasmacytomas.

    PubMed

    Gonnering, R S

    1987-03-01

    An 81-year-old man presented with bilateral orbital tumors that were found to be extramedullary plasmacytomas. Immunoperoxidase staining of the tissue was positive for IgG-kappa; no lambda chains were seen. Results of serum immunoelectrophoresis were positive for monoclonal IgG-kappa. Test results of the urine were positive for Bence Jones protein; only kappa chains were seen on radial diffusion. Systemic evaluation failed to show tumor elsewhere. The patient was treated with radiation and low-dose melphalan/prednisone, with good response. Five years later, the results of immunoelectrophoresis are normal and there is no clinical evidence of tumor; however, a small amount of kappa light chains remains in the urine. This case supports the important therapeutic and prognostic differences among orbital involvement in extramedullary plasmacytoma, solitary myeloma of bone, and multiple myeloma. PMID:3587904

  12. Bilateral shotgun pellet pulmonary emboli

    PubMed Central

    Huebner, Stephen; Ali, Sayed

    2012-01-01

    Intravascular migration of bullets and other foreign bodies is a rare but known complication of penetrating trauma. Missile embolization can represent a diagnostic challenge because it may present in various and unexpected ways. We present the case of a 54-year-old female who sustained shotgun pellet emboli to the pulmonary arteries following a left upper extremity gunshot wound and related vascular surgery. The case illustrates bilateral embolization, and the embolic events occurred following surgery. Embolization should be considered in evaluating patients with gunshot wounds, particularly if there are anomalous symptoms or the projectile is not found in the original, or expected, location. Close attention to the location of the foreign bodies on serial radiographs may reveal the diagnosis of intravascular embolization. PMID:22690290

  13. Prenatal diagnosis of Bruck syndrome.

    PubMed

    Berg, C; Geipel, A; Noack, F; Smrcek, J; Krapp, M; Germer, U; Bender, G; Gembruch, U

    2005-07-01

    Bruck syndrome is an autosomal recessive connective tissue disorder combining features of osteogenesis imperfecta and arthrogryposis multiplex congenita. There are only few reports describing this rare syndrome of multiple fractures and joint contractures that is thought to be a subtype of osteogenesis imperfecta. We report the first case of prenatal diagnosis of this syndrome in a fetus at 23 weeks of gestation. Ultrasound findings included brachycephaly, retrognathia marked shortening and bowing of both femurs, bilateral fixed flexion of the elbows, bilateral fixed extension of the wrists and partially fixed flexion of the knees. The parents opted for termination of pregnancy. Macroscopic and radiologic examination of the aborted fetus confirmed the prenatal diagnosis, whereas morphological studies of the bone tissue found no hard evidence of osteogenesis imperfecta, probably due to the early stage of pregnancy and the heterogeneity of the syndrome itself. PMID:16034828

  14. Cleidocranial dysplasia with bilateral posterior glenohumeral dislocation: a case-report.

    PubMed

    Hardy, A; Thiong'o, M W; Leroy, A; Hardy, P; Laporte, C

    2015-02-01

    A 31-year-old man experienced bilateral posterior glenohumeral dislocation during seizures. He had cleidocranial dysplasia with complete absence of both clavicles. Cleidocranial dysplasia is a rare inherited disease also known as Marie-Sainton syndrome and responsible for dental abnormalities well-known to stomatologists and dentists. Other manifestations include defective development of the skull bones and hypoplastic or aplastic clavicles. We found no previous reports of bilateral posterior glenohumeral dislocation in patients with cleidocranial dysplasia. The objective of this work was to look for an association between clavicular aplasia and posterior glenohumeral dislocation. PMID:25592053

  15. Endourological treatment of bilateral ureteral stones in bilateral ureteral duplication with right ureterocele

    PubMed Central

    Sen, Volkan; Aydogdu, Ozgu; Yonguc, Tar?k; Bozkurt, Ibrahim Halil; Polat, Salih; Basmaci, Ismail

    2015-01-01

    Bilateral collecting system duplication is a very rare abnormality, including the splitting of the ureteric bud. Complete ureteral duplication with two separate openings in the urinary bladder is also extremely rare. To the best of our knowledge, we present the first case of bilateral ureteral stones in bilateral duplicated collecting system. PMID:26279727

  16. CT demonstration of bilateral adrenal hemorrhage

    SciTech Connect

    Ling, D.; Korobkin, M.; Silverman, P.M.; Dunnick, N.R.

    1983-08-01

    Bilateral adrenal hemorrhage with subsequent adrenal insufficiency is a recognized complication of anticoagulant therapy. Because the clinical manifestations are often nonspecific, the antemortem diagnosis of adrenal hemorrhage has been a difficult clinical problem. Computed tomography (CT) provides detailed images of the adrenal glands that are not possible with conventional imaging methods. The CT findings of bilateral adrenal hemorrhage in an anticoagulated patient are reported.

  17. Submaximal Expression of the Bilateral Deficit

    ERIC Educational Resources Information Center

    McLean, Scott P.; Vint, Peter F.; Stember, Amanda J.

    2006-01-01

    Thirty-six participants performed bilateral and unilateral isometric elbow flexion trials at what they perceived to be 100, 75, 50, and 25% of maximal effort. Absolute bilateral deficits ranged from -16% at 25% effort to -10% at 100% effort. The deficit included a component independent of consciousness and a component inversely related to…

  18. Bilateral microperc in a severe kyphoscoliosis

    PubMed Central

    Dağgülli, Mansur; Penbegül, Necmettin; Dede, Onur; Utanğaç, Mehmet Mazhar

    2016-01-01

    Percutaneous nephrolithotomy is the standard modality for large renal calculi in normal and abnormal renal anatomic situations. This case report describes a 57-year-old male patient who presented with bilateral kidney stones and severe kyphoscoliosis. He had successfully been treated with a bilateral microperc technique.

  19. An evaluation of surgical outcome of bilateral cleft lip surgery using a modified Millard’s (Fork Flap) technique

    PubMed Central

    Adeyemo, W. L.; James, O.; Adeyemi, M. O.; Ogunlewe, M. O.; Ladeinde, A. L.; Butali, A.; Taiwo, O. A.; Emeka, C. I.; Ayodele, A. O. S; Ugwumba, C. U.

    2014-01-01

    Background The central third of the face is distorted by the bilateral cleft of the lip and palate and restoring the normal facial form is one of the primary goals for the reconstructive surgeons. The history of bilateral cleft lip repair has evolved from discarding the premaxilla and prolabium and approximating the lateral lip elements to a definitive lip and primary cleft nasal repair utilising the underlying musculature. The aim of this study was to review surgical outcome of bilateral cleft lip surgery (BCLS) done at the Lagos University Teaching Hospital. Materials and Methods A review of all cases of BCLS done between January 2007 and December 2012 at the Lagos University Teaching Hospital was done. Data analysis included age and sex of patients, type of cleft deformity and type of surgery (primary or secondary) and whether the cleft deformity was syndromic and non-syndromic. Techniques of repair, surgical outcome and complications were also recorded. Results A total of 39 cases of BCLS involving 21 males and 18 females were done during the period. This constituted 10% (39/390) of all cases of cleft surgery done during the period. There were 5 syndromic and 34 non-syndromic cases. Age of patients at time of surgery ranged between 3 months and 32 years. There were 24 bilateral cleft lip and palate deformities and 15 bilateral cleft lip deformities. Thirty-one of the cases were primary surgery, while 8 were secondary (revision) surgery. The most common surgical technique employed was modified Fork flap (Millard) technique, which was employed in 37 (95%) cases. Conclusion Bilateral cleft lip deformity is a common cleft deformity seen in clinical practice, surgical repair of which can be a challenge to an experienced surgeon. A modified Fork flap for repair of bilateral cleft lip is a reliable and versatile technique associated with excellent surgical outcome. PMID:24469478

  20. Meibomian gland dysfunction in floppy eyelid syndrome.

    PubMed

    Gonnering, R S; Sonneland, P R

    1987-01-01

    A 41-year-old obese man presented with bilateral ocular irritation and clinical findings consistent with floppy eyelid syndrome. Light- and electron-microscopic examination of tarsus removed at surgical correction revealed cystic degeneration and squamous metaplasia of the meibomian glands, along with abnormal keratinization and granuloma formation. These findings, not reported before in this syndrome, suggest that meibomian gland dysfunction with attendant qualitative abnormalities of the tear film, may be partly responsible for the keratoconjunctivitis seen in this syndrome. PMID:3154588

  1. [Pseudohypoparathyroidism revealed by Fahr syndrome].

    PubMed

    Kahloul, N; Chaari, W; Boughamoura, L; Charfeddine, L; Khammeri, S; Amri, F

    2009-05-01

    Fahr syndrome is defined by the presence of striopallidal notched bilateral and symmetric calcifications at the base of the skull. We report an observation of a 12-year-old girl who presented gait impairment, seizures, somnolence and aphasia. Brain computed tomodensitometry identified intracranial calcifications. The tests demonstrated pseudohypoparathyroidism. PMID:19339164

  2. Bilateral pneumothorax after orthognatic surgery

    PubMed Central

    Bertossi, Dario; Malchiodi, Luciano; Turra, Matteo; Bondi, Vincenzo; Albanese, Massimo; Lucchese, Alessandra; Carinci, Francesco; Nocini, Pierfrancesco

    2012-01-01

    Among complications in orthognathic surgery, the insurgence of pneumothorax is very rare. Pneumothorax is the presence of air or gas in the pleural cavity and it is rare complications in the postoperative oral and maxillofacial surgery patient. The clinical results are dependent on the degree of collapse of the lung on the affected side. Pneumothorax can impair oxygenation and/or ventilation. If the pneumothorax is significant, it can cause a shift of the mediastinum and compromise haemodynamic stability. While 10% of pneumothoraces are asymptomatic, patients often complain of acute chest pain and difficulty breathing. There is a reduction in vital capacity, tachycardia, tachypnoea and a decrease in partial pressure of oxygen with an inability to maintain oxygen saturations. We observed this unusual surgical consequence in a 28-year-old female with negative clinical history and instrumental evaluation after Le Fort I osteotomy and bilateral sagittal split osteotomy (BSSO). No further consequences, no neurological sequelae, no infections and no other osteotomies sequelae were seen. Sudden post-surgical dispnea associated to sub-cutaneous emphysema of the neck and of the thorax must be adequately observed with the aim of monitoring further severe sequelae. The anaesthetic management of the emergency difficult airway in any post-surgical orthognatic treatment can be extremely difficult requiring a multi-disciplinary approach. PMID:23814593

  3. Retroperitoneal laparoscopic bilateral lumbar sympathectomy.

    PubMed

    Segers, B; Himpens, J; Barroy, J P

    2007-06-01

    The first retroperitoneal lumbar sympathectomy was performed in 1924 by Julio Diez. The classic procedure for sympathectomy is open surgery. We report a unilateral laparoscopic retroperitoneal approach to perform bilateral lumbar sympathectomy. This approach was performed for a 43-year-old man with distal arterial occlusive disease and no indication for direct revascularization. His predominant symptoms were intermittent claudication at 100 metres and cold legs. The patient was placed in a left lateral decubitus position. The optical system was placed first in an intra-abdominal position to check that the trocars were well positioned in the retroperitoneal space. The dissection of retroperitoneum was performed by CO2 insufflation. The inferior vena cava was reclined and the right sympathetic chain was individualized. Two ganglia (L3-L4) were removed by bipolar electro-coagulation. The aorta was isolated on a vessel loop and careful anterior traction allowed a retro-aortic pre-vertebral approach between the lumbar vessels. The left sympathetic chain was dissected. Two ganglia (L3-L4) were removed by bipolar electro-coagulation. PMID:17685269

  4. Bilateral symmetry across Aphrodite Terra

    NASA Technical Reports Server (NTRS)

    Crumpler, L. S.; Head, J. W.; Campbell, D. B.

    1987-01-01

    There are three main highland areas on Venus: Beta Regio, Ishtar Terra and Aphrodite Terra. The latter is least known and the least mapped, yet existing analyses of Aphrodite Terra based on available Pioneer-Venus orbiter data suggest that it may be the site of extensive rifting. Some of the highest resolution (30 km) PV data (SAR) included most of the western half of Aphrodite Terra. Recent analysis of the SAR data together with Arecibo range-doppler topographic profiling (10 X 100 km horizontal and 10 m vertical resolution) across parts of Aphrodite, further characterized the nature of possible tectonic processes in the equatorial highlands. The existence of distinct topographic and radar morphologic linear discontinuities across the nearly east-west strike of Aphrodite Terra is indicated. Another prominent set of linear features is distinctly parallel to and orthogonal to the ground tracks of the PV spacecraft and are not included because of the possibility that they are artifacts. Study of the northwest trending cross-strike discontinuities (CSD's) and the nature of topographic and morphologic features along their strike suggest the presence of bilateral topographic and morphologic symmetry about the long axis of Aphrodite Terra.

  5. Bilateral Tubercular Mastitis - Case Reports.

    PubMed

    Chowdhury, A K; Mubin, S; Yousuf, N A; Rahman, M; Islam, M A; Ahmed, S U

    2015-07-01

    Breast tuberculosis is a rare form of tuberculosis. Moreover the disease is often overlooked and misdiagnosed as carcinoma or pyogenic abscess. Reports on breast tuberculosis have been few; reported incidence of breast tuberculosis amongst the total number of mammary conditions varies between 0.1 and 4 percent. Bilateral involvement is still more uncommon (3%). Here we report 3 cases of adult female ranging from 27 to 35 years who presented with 1 to 4 months history of firm lumps in both breasts and no axillary lymphadenopathy. Fine needle aspiration cytology (FNAC) of breast lump in all 3 cases were done but in 1 case showed evidence of tubercular mastitis and other 2 cases findings were inconclusive. Subsequent Excisional Biopsy of those 2 cases showed features of tuberculosis. All 3 were prescribed with four drug anti-tubercular treatments continued for 12 months in 2 cases and 9 months in other case depending upon their response. The lumps disappeared and ulcer healed after anti-tubercular treatment. PMID:26329964

  6. Non Syndromic Familial Bilateral Decidious Taurodontism – A First Case Report

    PubMed Central

    Panigrahi, Rajat G.; K. T., Srilatha; Bhuyan, Ruchi; Bhuyan, Sanat K.

    2014-01-01

    Taurodontism is anatomical and morphological alteration in the shape of the tooth. In taurodontism the crowns appear larger and the roots shorter compared to the normal anatomy of the respective tooth. The incidence of taurodontism in deciduous is very low and is also not very common in permanent dentition. The endodontic treatment protocol of taurodontic tooth is challenging. Taurodontic tooth requires a very cautious approach in identifying the root canal orientation and presence of any extra or lateral canals. PMID:25654041

  7. Non syndromic familial bilateral decidious taurodontism - a first case report.

    PubMed

    Panigrahi, Antarmayee; Panigrahi, Rajat G; K T, Srilatha; Bhuyan, Ruchi; Bhuyan, Sanat K

    2014-12-01

    Taurodontism is anatomical and morphological alteration in the shape of the tooth. In taurodontism the crowns appear larger and the roots shorter compared to the normal anatomy of the respective tooth. The incidence of taurodontism in deciduous is very low and is also not very common in permanent dentition. The endodontic treatment protocol of taurodontic tooth is challenging. Taurodontic tooth requires a very cautious approach in identifying the root canal orientation and presence of any extra or lateral canals. PMID:25654041

  8. Fahr's disease: bilateral symmetrical striopallidodentate calcification in two brothers with two distinct presentations

    PubMed Central

    Bowirrat, Abdalla; Yassin, Mustafa; Artoul, Faozi; Artul, Suheil

    2013-01-01

    Bilateral striopallidodentate calcinosis, commonly known as Fahr's disease is a rare clinical entity present mainly with extrapyramidal signs and accompanied with metabolic, biochemical, neuroradiological and neuropsychiatric situations. It is characterised by the symmetrical and bilateral intracranial deposition of calcium associated with cell loss in the basal ganglia, cerebral cortex and cerebellum.In this study, we discussed two brothers’ cases of Fahr's diseases who presented with different symptomatology. The first presented with walking difficulty, cramps and dysarthria and moderate memory impairment whereas the second with vertigo, ataxia, forgetfulness and headache. CT scans of both patients revealed intracranial diffuse bilateral calcifications in the basal ganglia and the cerebellum. The second patient revealed progressive cerebral atrophy but reduction in the calcification.Fahr's disease, although encountered rarely, should also be taken into account in the differential diagnosis of cases with abnormal intracranial calcifications along with other familial, congenital and metabolic diseases and syndromes. PMID:24014337

  9. Two case reports of bilateral adrenal myelolipomas.

    PubMed

    Yang, Yu; Ye, Lin-Yang; Yu, Bo; Guo, Jia-Xiang; Liu, Qian; Chen, Yun

    2015-09-16

    Primary adrenal myelolipoma is a rare, non-functioning adrenal benign tumor that is composed of mature adipose tissue and a variable amount of haemopoietic elements. Clinically, it is difficult to get diagnosed with adrenal myelolipoma because the patient usually doesn't have obvious symptoms and signs in early stage. In the present study, two cases of primary bilateral adrenal myelolipomas are reported. Clinical presentation, imaging diagnostic features, histopathological changes and surgical treatments of the two patients are discussed. Preoperative diagnostic imaging examinations (B-mode ultrasonography, computed tomography and magnetic resonance imaging sans) assisted getting a prediction diagnosis of bilateral adrenal myelolipomas. A two-stage surgery was used to successfully excise bilateral adrenal myelolipomas in the two patients. Conventional open adrenalectomy was applied to remove the adrenal myelolipomas greater than 6 cm, and laparoscopic adrenalectomy was performed to excise the adrenal tumors smaller than 6 cm. Bilateral adrenal myelolipomas of the two patients were finally confirmed by postoperative histopathological examinations. Understanding clinical, imaging diagnostic and histopathological features of bilateral adrenal myelolipomas will facilitate timely diagnosis and treatment of this condition. Surgical removal of bilateral adrenal myelolipomas is safe, curative and beneficial. The two-stage surgery appears to be the best treatment option for the patients with bilateral adrenal myelolipomas because it achieves optimal treatment effectiveness with minimized sequelae. PMID:26380835

  10. Heterochronic bilateral ectopic pregnancy after ovulation induction*

    PubMed Central

    Zhu, Bo; Xu, Gu-feng; Liu, Yi-feng; Qu, Fan; Yao, Wei-miao; Zhu, Yi-min; Gao, Hui-juan; Zhang, Dan

    2014-01-01

    Ectopic pregnancy is identified with the widely-applied assisted reproductive technology (ART). Bilateral ectopic pregnancy is a rare form of ectopic pregnancy which is difficult to be diagnosed at the pre-operation stage. In this paper, we presented an unusual case of heterochronic bilateral ectopic pregnancy after stimulated intrauterine insemination (IUI), where there has been a delay of 22 d between the diagnoses of the two ectopic pregnancies. Literature was reviewed on the occurrence of bilateral ectopic pregnancy during the past four years in the MEDLINE database. We found 16 cases of bilateral ectopic pregnancy reported since 2008, and analyzed the characteristics of those cases of bilateral ectopic pregnancy. We emphasize that ovulation induction and other ARTs may increase the risk of bilateral ectopic pregnancy. Because of the difficulty in identification of bilateral ectopic pregnancy by ultrasonography, the clinician should be aware that the treatment of one ectopic pregnancy does not preclude the occurrence of a second ectopic pregnancy in the same patient and should pay attention to the intra-operation inspection of both side fallopian tubes in any ectopic pregnancy case. PMID:25091994

  11. Two case reports of bilateral adrenal myelolipomas

    PubMed Central

    Yang, Yu; Ye, Lin-Yang; Yu, Bo; Guo, Jia-Xiang; Liu, Qian; Chen, Yun

    2015-01-01

    Primary adrenal myelolipoma is a rare, non-functioning adrenal benign tumor that is composed of mature adipose tissue and a variable amount of haemopoietic elements. Clinically, it is difficult to get diagnosed with adrenal myelolipoma because the patient usually doesn’t have obvious symptoms and signs in early stage. In the present study, two cases of primary bilateral adrenal myelolipomas are reported. Clinical presentation, imaging diagnostic features, histopathological changes and surgical treatments of the two patients are discussed. Preoperative diagnostic imaging examinations (B-mode ultrasonography, computed tomography and magnetic resonance imaging sans) assisted getting a prediction diagnosis of bilateral adrenal myelolipomas. A two-stage surgery was used to successfully excise bilateral adrenal myelolipomas in the two patients. Conventional open adrenalectomy was applied to remove the adrenal myelolipomas greater than 6 cm, and laparoscopic adrenalectomy was performed to excise the adrenal tumors smaller than 6 cm. Bilateral adrenal myelolipomas of the two patients were finally confirmed by postoperative histopathological examinations. Understanding clinical, imaging diagnostic and histopathological features of bilateral adrenal myelolipomas will facilitate timely diagnosis and treatment of this condition. Surgical removal of bilateral adrenal myelolipomas is safe, curative and beneficial. The two-stage surgery appears to be the best treatment option for the patients with bilateral adrenal myelolipomas because it achieves optimal treatment effectiveness with minimized sequelae. PMID:26380835

  12. [Bilateral optic atrophy in a drug addict].

    PubMed

    Milet, A; Blaise, P; Andris, C; Rakic, J-M

    2007-11-01

    Bilateral optic neuropathy in a young man is suggestive of hereditary or toxic damage. We describe the case of a 38-year-old man with bilateral optic neuropathy. His best corrected visual acuity was light perception OD and 20/125 OS. The patient's history revealed an addiction to heroin. Three drugs - heroin, quinine, and cocaine - were considered as a possible cause, which is discussed further. We suggest that the condition reported here may be attributed to the use of heroin. This underlines the importance of meticulous history taking in diagnosing a young man with bilateral loss of vision. PMID:18046269

  13. Bilateral sudden hearing loss following habitual abortion: a case report and review of literature.

    PubMed

    Yin, Tuanfang; Huang, Fengying; Ren, Jihao; Liu, Wei; Chen, Xing; Li, Lihua; Xie, Dinghua; Lu, Yongde

    2013-01-01

    Sudden sensorineural hearing loss (SSNHL) is usually unilateral and can be associated with tinnitus and vertigo. The most common causes of this disease are known to be the vascular and viral agents, but immune disorders are involved in the development of sudden deafness. The antiphospholipid syndrome (APS) is an acquired autoimmune system disorder, which is defined as the presence of antiphospholipid antibodies (APA) in the patient's blood, then cause venous and/or arterial thrombosis in various organs of the body, for example, thrombosis can occur in the placenta and/or the inner ear. As a result, it can cause abortion and/or sudden deafness. Bilateral SSNHL following habitual abortion is a rare clinical event. Here, we report a case of 32-year-old woman who presented with bilateral sudden hearing loss following recurrent pregnancy loss (RPL) as the first manifestation of primary antiphospholipid syndrome. Combine the literature, the diagnosis, clinical implication and treatment are discussed. PMID:24040484

  14. Lowe syndrome

    PubMed Central

    Loi, Mario

    2006-01-01

    Lowe syndrome (the oculocerebrorenal syndrome of Lowe, OCRL) is a multisystem disorder characterised by anomalies affecting the eye, the nervous system and the kidney. It is a uncommon, panethnic, X-linked disease, with estimated prevalence in the general population of approximately 1 in 500,000. Bilateral cataract and severe hypotonia are present at birth. In the subsequent weeks or months, a proximal renal tubulopathy (Fanconi-type) becomes evident and the ocular picture may be complicated by glaucoma and cheloids. Psychomotor retardation is evident in childhood, while behavioural problems prevail and renal complications arise in adolescence. The mutation of the gene OCRL1 localized at Xq26.1, coding for the enzyme phosphatidylinositol (4,5) bisphosphate 5 phosphatase, PtdIns (4,5)P2, in the trans-Golgi network is responsible for the disease. Both enzymatic and molecular testing are available for confirmation of the diagnosis and for prenatal detection of the disease. The treatment includes: cataract extraction, glaucoma control, physical and speech therapy, use of drugs to address behavioural problems, and correction of the tubular acidosis and the bone disease with the use of bicarbonate, phosphate, potassium and water. Life span rarely exceeds 40 years. PMID:16722554

  15. Meckel syndrome.

    PubMed Central

    Salonen, R; Paavola, P

    1998-01-01

    Meckel syndrome (MKS) is a lethal syndrome with a central nervous system malformation, usually occipital meningoencephalocele, bilaterally large multicystic kidneys with fibrotic changes of the liver, and polydactyly in most cases. Additional anomalies are frequent. A common characteristic of the parenchymal changes of many organs is a proliferation of the stromal connective tissue and increase and dilatation of the associated epithelial ducts. Autosomal recessive inheritance is well confirmed and the gene locus has been mapped to chromosome 17q21-24 by genome wide linkage study. The locus was later refined to within a less than 1 cM region (17q22), in which most of the Finnish MKS patients share a common chromosomal haplotype suggesting one major and relatively old mutation. However, in most of the non-Finnish MKS families studied, this linkage could not be confirmed. The linkage studies provide evidence that more than one locus is involved in bringing about the combination of CNS malformations, cystic kidneys, and polydactyly, maybe even in typical cases of MKS. Prenatal diagnosis of MKS by vaginal ultrasound scan is possible from 11-12 weeks of pregnancy, especially in families where there is a known risk. In those families where linkage to 17q22 is established, prenatal diagnosis by DNA analysis is possible. Images PMID:9643292

  16. Lowe syndrome.

    PubMed

    Loi, Mario

    2006-01-01

    Lowe syndrome (the oculocerebrorenal syndrome of Lowe, OCRL) is a multisystem disorder characterised by anomalies affecting the eye, the nervous system and the kidney. It is a uncommon, panethnic, X-linked disease, with estimated prevalence in the general population of approximately 1 in 500,000. Bilateral cataract and severe hypotonia are present at birth. In the subsequent weeks or months, a proximal renal tubulopathy (Fanconi-type) becomes evident and the ocular picture may be complicated by glaucoma and cheloids. Psychomotor retardation is evident in childhood, while behavioural problems prevail and renal complications arise in adolescence. The mutation of the gene OCRL1 localized at Xq26.1, coding for the enzyme phosphatidylinositol (4,5) bisphosphate 5 phosphatase, PtdIns (4,5)P2, in the trans-Golgi network is responsible for the disease. Both enzymatic and molecular testing are available for confirmation of the diagnosis and for prenatal detection of the disease. The treatment includes: cataract extraction, glaucoma control, physical and speech therapy, use of drugs to address behavioural problems, and correction of the tubular acidosis and the bone disease with the use of bicarbonate, phosphate, potassium and water. Life span rarely exceeds 40 years. PMID:16722554

  17. Isolation of the Left Subclavian Artery with Right Aortic Arch in Association with Bilateral Ductus Arteriosus and Ventricular Septal Defect

    PubMed Central

    Lee, Ji Seong; Park, Ji Young; Ko, Seong Min; Seo, Dong-Man

    2015-01-01

    Right aortic arch with isolation of the left subclavian artery is a rare anomaly. The incidence of bilateral ductus arteriosus is sporadic, and a right aortic arch with isolation of the left subclavian artery in association with bilateral ductus arteriosus is therefore extremely rare. Since the symptoms and signs of isolation of the left subclavian artery can include the absence or underdevelopment of the left arm, subclavian steal syndrome, or pulmonary artery steal syndrome, the proper therapeutic approach is controversial. We report a case in which surgical reconstruction was used to treat isolation of the left subclavian artery with right aortic arch in association with bilateral ductus arteriosus and a ventricular septal defect. PMID:26665110

  18. Precise circuitry links bilaterally symmetric olfactory maps.

    PubMed

    Yan, Zhiqiang; Tan, Jie; Qin, Chang; Lu, Yao; Ding, Cheng; Luo, Minmin

    2008-05-22

    Olfactory sensory neurons expressing a common receptor gene converge onto one or a few glomeruli with stereotyped positions within the mouse main olfactory bulb (MOB), producing a map of approximately 1800 olfactory columns representing approximately 1000 odorant receptors. Here, we report that this precise olfactory map is maintained over several synapses that ultimately cross MOB hemispheres to link bilateral isofunctional olfactory columns. Focal injection of tracer into genetically identified glomeruli revealed an exquisite topography that involves a bilateral connection via the anterior olfactory nucleus pars externa (AONpE) that links isofunctional olfactory columns in the contralateral MOB. Physiological and behavioral assays revealed an important role for the AONpE in bilateral exchange of odorant-specific information. These results indicate that the interbulbar link through the AONpE integrates bilateral olfactory sensory maps and exchanges olfactory information, positioning it as a unique model system for studying interhemispheric connections. PMID:18498741

  19. Impaired threat prioritisation after selective bilateral amygdala lesions

    PubMed Central

    Bach, Dominik R.; Hurlemann, Rene; Dolan, Raymond J.

    2015-01-01

    The amygdala is proposed to process threat-related information in non-human animals. In humans, empirical evidence from lesion studies has provided the strongest evidence for a role in emotional face recognition and social judgement. Here we use a face-in-the-crowd (FITC) task which in healthy control individuals reveals prioritised threat processing, evident in faster serial search for angry compared to happy target faces. We investigate AM and BG, two individuals with bilateral amygdala lesions due to Urbach–Wiethe syndrome, and 16 control individuals. In lesion patients we show a reversal of a threat detection advantage indicating a profound impairment in prioritising threat information. This is the first direct demonstration that human amygdala lesions impair prioritisation of threatening faces, providing evidence that this structure has a causal role in responding to imminent danger. PMID:25282058

  20. Impaired threat prioritisation after selective bilateral amygdala lesions.

    PubMed

    Bach, Dominik R; Hurlemann, Rene; Dolan, Raymond J

    2015-02-01

    The amygdala is proposed to process threat-related information in non-human animals. In humans, empirical evidence from lesion studies has provided the strongest evidence for a role in emotional face recognition and social judgement. Here we use a face-in-the-crowd (FITC) task which in healthy control individuals reveals prioritised threat processing, evident in faster serial search for angry compared to happy target faces. We investigate AM and BG, two individuals with bilateral amygdala lesions due to Urbach-Wiethe syndrome, and 16 control individuals. In lesion patients we show a reversal of a threat detection advantage indicating a profound impairment in prioritising threat information. This is the first direct demonstration that human amygdala lesions impair prioritisation of threatening faces, providing evidence that this structure has a causal role in responding to imminent danger. PMID:25282058

  1. Bilateral nephrocalcinosis and amelogenesis imperfecta: A case report

    PubMed Central

    Patel, Alok; Jagtap, Chetana; Bhat, Chetan; Shah, Rohan

    2015-01-01

    Amelogenesis imperfecta (AI) is a group of hereditary disorders that affect the quality and/or quantity of dental enamel. This paper describes the clinicopathological features of a patient who was born of nonconsanguineous parents and who presented with oral alterations, including yellow and misshapen teeth, intrapulpal calcifications, delayed tooth eruption, and gum enlargement. Scanning electron microscopy of the teeth revealed hypoplastic enamel, and a renal ultrasound detected bilateral nephrocalcinosis, leading to a diagnosis of AI and nephrocalcinosis syndrome. Since nephrocalcinosis is often asymptomatic and can be associated with impaired renal function, dentists who see children with a generalized and thin hypoplastic AI should consider a renal ultrasound scan and referral to a Nephrologist. Children with nephrocalcinosis should also be considered for a dental check. PMID:26097369

  2. Bilateral obstruction of bilaterally duplicated collecting systems requiring upper and lower moiety drainage.

    PubMed

    Lynch, J O; Cox, A; Rawal, B; Shelmerdine, S; Vasdev, N; Patel, A

    2016-04-01

    A 60-year-old woman with a history of breast cancer presented with bilateral obstruction of bilaterally duplicated renal collecting systems secondary to extrinsic compression from metastatic pelvic lymphadenopathy. Bilateral JJ ureteric stents were inserted, resulting in some improvement of renal function but a failure to normalise completely. Repeat computed tomography demonstrated bilateral duplex collecting systems with persisting obstruction of the undrained moieties. Selective puncture was performed to decompress the obstructed renal moieties for bilateral nephrostomy catheter insertion. This allowed renal function to improve sufficiently for the patient to be discharged and commence chemotherapy. This is the first reported case of bilaterally obstructed partially duplicated collecting systems and it illustrates the importance of recognising anatomical variants to tailor treatment appropriately. It also highlights the important relationship between urology and interventional radiology in the management of such complex patients. PMID:26985816

  3. Magnetic resonance imaging findings in bilateral basal ganglia lesions.

    TOXLINE Toxicology Bibliographic Information

    Lim CC

    2009-09-01

    INTRODUCTION: Radiologists may encounter bilaterally symmetrical abnormalities of the basal ganglia on magnetic resonance imaging (MRI), typically in the context of diffuse systemic, toxic or metabolic diseases. A systematic approach and broad knowledge of pathology causing this uncommon group of conditions would be useful.MATERIALS AND METHODS: This review uses illustrative images to highlight metabolic conditions, such as Leigh's syndrome, citrullinaemia, hypoglycaemia or carbon monoxide poisoning, as well as other causes of bilateral basal ganglia lesions such as osmotic myelinolysis, deep cerebral venous thrombosis and Creutzfeldt-Jakob disease.RESULTS: Careful assessment of radiological findings outside the basal ganglia, such as involvement of the cortex, white matter, thalamus and pons, together with clinical correlation, may be helpful in narrowing the differential diagnosis, and directing further radiological, biochemical or genetic investigations. Recent advances in MR technology have resulted in newer techniques including diffusion-weighted (DW) MR imaging and MR spectroscopy (MRS); these may be helpful if appropriately used.CONCLUSIONS: Abnormal MRI findings in the basal ganglia should not be interpreted in isolation. A systematic approach including DW MR imaging, MRS, and a broad knowledge of diffuse systemic, toxic or metabolic diseases is helpful.

  4. Bilateral spike-and-wave discharges in a hemi-deafferented cortex.

    PubMed

    Inghilleri, M; Clemenzi, A; Conte, A; Frasca, V; Manfredi, M

    2002-12-01

    We studied a patient with a history of absence attacks in childhood in whom an absence status with bilateral spike-and-wave discharges developed after a top-of-the-basilar syndrome. Surprisingly, even though the ischemic lesion involved the left thalamus alone, spike-and-wave discharges were recorded from the two hemispheres. Three days after antiepileptic treatment (sodium valproate 500mg 3 times a day) began, electroenceplalographic recordings and consciousness became normal. PMID:12464335

  5. Bilateral pneumonia and inappropriate secretion of antidiuretic hormone in a premature infant.

    PubMed Central

    Papageorgiou, A. N.; Moffatt, M.

    1976-01-01

    A 6-week-old infant born prematurely had severe hyponatremia and other features of the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). This disturbance was believed to be secondary to extensive bilateral pneumonia with collapse of the right upper lobe. Although this association has been recognized in adults, this is the first report of its occurrence in an infant. SIADH must be considered in the differential diagnosis of hyponatremia in association with pneumonia in an infant. Images FIG. 1 PMID:945122

  6. Bilateral pheochromocytomas in a child who had hemihypertrophy and alteration in the VHL gene.

    PubMed

    Amini, Zarlasht; Babovic-Vuksanovic, Dusica; Lteif, Aida

    2013-01-01

    The association of hypertrophy with neoplasm is well-known. Pheochromocytoma is a rare neoplasm in children. Isolated hemihypertrophy and hemihypertrophy linked to other genetic disorders have rarely been associated with the development of pheochromocytoma, with only two cases reported to date. We report a novel case of a 4-year-old male with bilateral adrenal pheochromocytomas and lower extremity hemihypertrophy in the setting of von Hippel-Lindau syndrome. PMID:23327821

  7. An Unusual Case of Bilateral Maxillary and Mandibular Para Premolar: A Case Report

    PubMed Central

    Chanagay, Sunil Kumar Vishwanath; Singh, Vikram; Bantwal, Sunil Rao; Muniyappa, Manjunatha

    2013-01-01

    The presence of supernumerary teeth is not uncommon in the general population. They occur more frequently in patients with a family history of such teeth. It is rare to find multiple supernumeraries in individuals with no other associated disease or syndrome. There have been very few documented cases of bilateral maxillary and mandibular supernumeraries in the premolar region. An unusual case of a 35-year-old man with six para premolars and complete dentition is presented. PMID:24396358

  8. Bilateral pneumothorax after unilateral transthoracic puncture.

    PubMed

    Yamaura, Hidekazu; Inaba, Yoshitaka; Sato, Yozo; Najima, Mina; Shimamoto, Hiroshi; Nishiofuku, Hideyuki

    2007-06-01

    Pneumothorax is a common complication following transthoracic interventional procedures. Most often, this occurs unilaterally on the side of the intervention. Bilateral pneumothorax following unilateral puncture is rare, but may mandate emergent chest tube insertion. We describe two cases in which bilateral pneumothorax occurred after unilateral thoracic puncture. One patient had a history of esophageal resection, while the other had no history of thoracic surgery. PMID:17538145

  9. Bilateral Acute Epidural Hematoma with Good Outcome

    PubMed Central

    Paiva, Wellingson Silva; Andrade, Almir Ferreira De; Alves, Aderaldo Costa Junior; Ribeiro, Iuri Neville; Teixeira, Manoel Jacobsen

    2013-01-01

    Epidural haematomas are one of the most common complicated closed-head injuries, but they, rarely show any bilateral localization. We are reporting here a case of a man found unconscious with Glasgow Coma Scale score; 8/15. Computed tomography of skull revealed bilateral epidural hematoma. Two emergency craniotomies were performed simultaneously, with satisfactory radiological control and neurological outcome. We discussed the aspects of a etiology and treatment about this unusual condition. PMID:24392414

  10. Bilateral synchronous plasmacytoma of the testis

    PubMed Central

    Joseph, Rona; Soman, Lali V.

    2016-01-01

    Extramedullary plasmacytoma (EMP) is usually seen in the head and neck regions and in the upper respiratory, gastrointestinal, and central nervous systems. Testis is a rare site for EMP, and bilateral synchronous testicular plasmacytoma occurring as an isolated event at initial presentation has been reported only once previously. We present herein the second such report in a 70-year-old man who underwent bilateral orchidectomy.

  11. Unusual bilateral dentigerous cysts in a nonsyndromic patient assessed by cone beam computed tomography

    PubMed Central

    Imada, Thaís Sumie Nozu; Neto, V. Tieghi; Bernini, G. F.; Silva Santos, P. S.; Rubira-Bullen, I. R. F.; Bravo-Calderòn, D.; Oliveira, D. T.; Gonçales, E. S.

    2014-01-01

    In the absence of syndromes, bilateral dentigerous cysts (DC) located on the jaws are unusual. In English based language literature review, we only found eight reports of nonsyndromic bilateral dentigerous cyst associated with mandibular third molars. Therefore, we report the unusual occurrence of sizable nonsyndromic bilateral DC associated with mandibular impacted third molars in a 42-year-old Caucasian woman. The lesions were assessed by cone beam computed tomography (CBCT) the right lesion showed approximately 23.64 mm and the left one, 16.57 mm diameter, both located intimately next to the mandibular canal. Bilateral surgical enucleation, related teeth excision of both third molars and plate for fixation placement on the right and bigger lesion, under general anesthesia was the final treatment choice. Clinical, radiographic and histopathological features confirmed diagnose of bilateral dentigerous cyst. Now-a-days, the patient is on 18 months radiograph follow-up with favorable osseous formation with no evidence of recurrence of the cysts. PMID:24963255

  12. Visual development in the blepharophimosis syndrome.

    PubMed Central

    Beaconsfield, M; Walker, J W; Collin, J R

    1991-01-01

    One hundred and one cases of the blepharophimosis syndrome presenting over a decade are reviewed with particular attention to the factors influencing their visual development. Three distinct clinical patterns emerge--severe bilateral ptosis, moderate bilateral ptosis, and asymmetric ptosis--and their differing incidence of amblyopia and strabismus is discussed. The risk of amblyopia is much higher than previously believed (56.4% in our series) and preventive management is discussed. Images PMID:1768667

  13. Bilateral Carotid Paraganglioma: Surgery and Radiotherapy

    PubMed Central

    Kiziltan, Huriye S; Ozucer, Berke; Eris, Ali H; Veyseller, Bayram

    2014-01-01

    BACKGROUND Paragangliomas are relatively rare vascular tumors that develop from the neural crest cells of carotid bifurcation. They usually present as slow-growing, painless unilateral neck masses; bilateral presentation is rare and is mostly associated with familial forms. Bilateral total resection is not always possible for high-grade bilateral tumors, and radiotherapy is a good alternative, with cure rates similar to surgery. CASE REPORT A 35-year-old female patient was admitted with a chief complaint of a bilateral, painless mass located on her neck. Subsequent magnetic resonance imaging (MRI) and angiographic imaging revealed bilateral hypervascular masses surrounding her carotid at 360°, and they were interpreted as stage 3 carotid paragangliomas according to the Shamblin classification protocol. Surgery was carried out on the left carotid paraganglioma and the mass was totally resected. It was thought that the patient could not tolerate bilateral surgery. Primary radiotherapy was planned on the right carotid paraganglioma: 59.8 gray (Gy) conformal, Linac-based multileaf collimator radiotherapy with a 180 cGy daily dosage, and five fractions per week were planned. RESULTS Follow-up at 3 months following the conclusion of radiotherapy revealed no significant regression. A follow-up MRI 6 months and 24 months later revealed 59% regression. Grade 2 esophagitis and minimal neck edema were the only complications noted during the course of radiotherapy and during the 24-month follow-up period. No complications or relapse were observed except for edema following neck surgery. PMID:25057243

  14. Cancer problem in Peutz-Jeghers syndrome.

    PubMed

    Taheri, Diana; Afshar-Moghadam, Noushin; Mahzoni, Parvin; Eftekhari, Amin; Hashemi, Seyed Mozafar; Emami, Mohammad Hasan; Fesharakizadeh, Mehdi; Ghasemi-Basir, Hamid Reza

    2013-01-01

    Peutz-Jeghers syndrome is a rare autosomal dominantly inherited condition, characterized by the presence of hamartomatous gastrointestinal polyps and mucocutaneous pigmentation. Patients with this syndrome can be associated with other neoplasms such as ovarian neoplasms known as sex-cord tumor with annular tubules that are associated in one third of the cases with this syndrome and other types of malignancies. We report a 42-year-old woman with a history of Peutz-Jeghers Syndrome and bilateral breast cancer that presented with abnormal uterine bleeding. Total abdominal hysterectomy with bilateral salpino-oophorectomy was done and an ovarian sex cord tumor with annular tubules was incidentally diagnosed. By reviewing literatures and in agreement with previous studies we suggest routine screening for malignancies in patients with Peutz-Jeghers syndrome. PMID:23977663

  15. Visuomotor Learning Generalizes Between Bilateral and Unilateral Conditions Despite Varying Degrees of Bilateral Interference

    PubMed Central

    Mordkoff, J. Toby; Sainburg, Robert L.

    2010-01-01

    Bilateral interference, referring to the tendency of movements of one arm to disrupt the intended movements made simultaneously with the other arm, is often observed in a task that involves differential planning of each arm movement during sensorimotor adaptation. In the present study, we examined two questions: 1) how does the compatibility between visuomotor adaptation tasks performed with both arms affect bilateral interference during bimanual performance? and 2) how do variations in bilateral interference affect transfer of visuomotor adaptation between bilateral and unilateral conditions? To examine these questions, we manipulated visuomotor compatibility using two kinematic variables (direction of required hand motion, direction of an imposed visual rotation). Experiment 1 consisted of two conditions in which the direction of visual rotations for both arms was either in the same or opposing directions, whereas the target direction for both arms was always the same. In experiment 2, we examined the pattern of generalization between the bilateral and unilateral conditions when both the target and rotation directions were opposing between the arms. In both experiments, subjects first adapted to a 30° visual rotation with one arm (preunilateral), then with both arms (bilateral), and finally with the arm that was not used in the first session (postunilateral). Our results show that bilateral interference was smallest when both variables were the same between the arms. Our data also show extensive transfer of visuomotor adaptation between bilateral and unilateral conditions, regardless of degree of bilateral interference. PMID:20881203

  16. An unusual presentation of Kabuki syndrome: clinical overlap with CHARGE syndrome.

    PubMed

    Verhagen, Judith M A; Oostdijk, Wilma; Terwisscha van Scheltinga, Cecilia E J; Schalij-Delfos, Nicoline E; van Bever, Yolande

    2014-09-01

    Kabuki syndrome is a rare genetic disorder characterized by intellectual disability and multiple congenital anomalies, including short stature, peculiar facial appearance, skeletal anomalies, a variety of visceral malformations and abnormal dermatoglyphic patterns. We describe a case of Kabuki syndrome presenting with atypical features, consisting of bilateral microphthalmia, coloboma, anal atresia and panhypopituitarism, showing considerable phenotypic overlap with CHARGE syndrome. This report demonstrates that clinical follow-up and molecular genetic testing can be useful for establishing the correct diagnosis. PMID:24862881

  17. Metabolic Syndrome

    MedlinePLUS

    ... How Can I Help a Friend Who Cuts? Metabolic Syndrome KidsHealth > For Teens > Metabolic Syndrome Print A A ... applies to a condition known as metabolic syndrome. Metabolic Syndrome Is an Early Warning Sign Metabolic syndrome isn' ...

  18. Loin pain hematuria syndrome.

    PubMed

    Zubair, Adeel S; Salameh, Hassan; Erickson, Stephen B; Prieto, Mikel

    2016-02-01

    Loin pain hematuria syndrome (LPHS), first described in 1967, is a rare pain syndrome, which is not well understood. The syndrome is characterized by severe intermittent or persistent flank pain, either unilateral or bilateral, associated with gross or microscopic hematuria. LPHS is a diagnosis of exclusion as there still is not a consensus of validated diagnostic criteria, though several criteria have been proposed. The wide differential diagnosis would suggest a meticulous yet specific diagnostic work-up depending on the individual clinical features and natural history. Several mechanisms regarding the pathophysiology of LPHS have been proposed but without pinpointing the actual causative etiology, the treatment remains symptomatic. Treatment modalities for LPHS are diverse including simple analgesia, opioid analgesic and kidney autotransplantation. This review article summarizes the current understanding regarding the pathophysiology of LPHS along with the steps required for proper diagnosis and a discussion of the different therapeutic approaches for LPHS. PMID:26798473

  19. Loin pain hematuria syndrome

    PubMed Central

    Zubair, Adeel S.; Salameh, Hassan; Erickson, Stephen B.; Prieto, Mikel

    2016-01-01

    Loin pain hematuria syndrome (LPHS), first described in 1967, is a rare pain syndrome, which is not well understood. The syndrome is characterized by severe intermittent or persistent flank pain, either unilateral or bilateral, associated with gross or microscopic hematuria. LPHS is a diagnosis of exclusion as there still is not a consensus of validated diagnostic criteria, though several criteria have been proposed. The wide differential diagnosis would suggest a meticulous yet specific diagnostic work-up depending on the individual clinical features and natural history. Several mechanisms regarding the pathophysiology of LPHS have been proposed but without pinpointing the actual causative etiology, the treatment remains symptomatic. Treatment modalities for LPHS are diverse including simple analgesia, opioid analgesic and kidney autotransplantation. This review article summarizes the current understanding regarding the pathophysiology of LPHS along with the steps required for proper diagnosis and a discussion of the different therapeutic approaches for LPHS. PMID:26798473

  20. Harlequin syndrome as a complication of epidural anesthesia.

    PubMed

    Boling, Bryan; Key, Christopher; Wainscott, Justin; Rebel, Annette

    2014-06-01

    Harlequin syndrome is a rare neurological condition that results in unilateral facial flushing and sweating. Although the syndrome is generally a benign condition with complete resolution if appropriate treatment is initiated, unilateral facial flushing can be a sign of several serious conditions and should be thoroughly investigated. Sudden onset of facial flushing related to harlequin syndrome developed in a patient who had bilateral lung transplant with postoperative epidural anesthesia for pain control. Differential diagnosis includes neurovascular disease (acute stroke), malignant neoplasm of brain or lung, Horner syndrome, idiopathic hyperhidrosis, and Frey syndrome. Harlequin syndrome is often easily treated by discontinuing the anesthetic or adjusting placement of the epidural catheter. PMID:24882829

  1. Bilateral Sequential Pneumolabyrinth Resulting from Nose Blowing

    PubMed Central

    Lee, Joong Seob; Kwon, Sae Young; Kim, Ji Heui

    2015-01-01

    Pneumolabyrinth describes a condition with entrapped air in the labyrinth and usually occurs in temporal bone fractures that involve the otic capsule. While sporadic cases of bilateral pneumolabyrinth have been reported, cases lacking head trauma are very rare. We report the case of a 43-year-old man who had sudden hearing loss bilaterally after blowing his nose at an interval of 1 year. Although conservative management for the right ear and exploratory tympanotomy with sealing of the possible site of perilymphatic leakage in the left ear were performed, hearing outcome was poor in both ears. To our knowledge, this is the first case of bilateral pneumolabyrinth occurring as a result of nose blowing. PMID:26771019

  2. Bilateral Supernumerary Kidney: A Very Rare Presentation

    PubMed Central

    Keskin, Suat; Batur, Abdussamet; Keskin, Zeynep; Koc, Abdulkadir; Firat Ozcan, Irfan

    2014-01-01

    To our knowledge, bilateral supernumerary kidney is a very rare renal abnormality and there are five cases presented in the literature. It is difficult to diagnose supernumerary kidney and clinicians have not detected most cases preoperatively. Laboratory and imaging studies were acquired and carefully examined. The normal laboratory tests were found. Emergency ultrasonography was performed and they revealed no signs of parenchymal abnormality in both kidneys. Serial imaging study including enhanced computed tomography (CT) was performed. An imaging study identified bilateral supernumerary kidney with expanded collecting systems. On each side, significant rotation anomaly was found. In addition, there were two different renal arteries originating from the aorta. This report presents radiological determinations of supernumerary kidney bilaterally in a young man. We think that CT commonly appears to be enough for the diagnosis of supernumerary kidneys. PMID:25780543

  3. Postpartum Bilateral Sacroiliitis caused by Brucella Infection

    PubMed Central

    Yavuz, Ferdi; Altun, Demet; Ulubay, Mustafa; Firatligil, Fahri Burçin

    2015-01-01

    Early diagnosis of this septic sacroiliitis is difficult because symptoms are nonspecific during the postpartum period. In this case we dicscuss about a patient with bilateral buttock pain unresolved with painkillers and rest, after an induction delivery. A 31-year-old woman was presented to our clinic on the second week of postpartum period with bilateral buttock pain. She was subfebrile and had no apparent abnormality on her pelvic X-ray. The pain was so severe that she was unable to walk properly. Sacroiliac MRI during the acute episode of pain showed bone marrow oedema and fluid within the bilateral sacroiliac joint. She was found seropositive for brucellosis and the patient completely recovered with antibiotherapy treatment. We stopped our patient from breastfeeding when the Rose Bengal test turned out positive. Brucella sacroiliitis should be considered in puerperium period women when buttock pain and difficulty in walking are present and pain is unresponsive to analgesics. PMID:26675497

  4. Postpartum Bilateral Sacroiliitis caused by Brucella Infection.

    PubMed

    Ozturk, Mustafa; Yavuz, Ferdi; Altun, Demet; Ulubay, Mustafa; Firatligil, Fahri Burçin

    2015-11-01

    Early diagnosis of this septic sacroiliitis is difficult because symptoms are nonspecific during the postpartum period. In this case we dicscuss about a patient with bilateral buttock pain unresolved with painkillers and rest, after an induction delivery. A 31-year-old woman was presented to our clinic on the second week of postpartum period with bilateral buttock pain. She was subfebrile and had no apparent abnormality on her pelvic X-ray. The pain was so severe that she was unable to walk properly. Sacroiliac MRI during the acute episode of pain showed bone marrow oedema and fluid within the bilateral sacroiliac joint. She was found seropositive for brucellosis and the patient completely recovered with antibiotherapy treatment. We stopped our patient from breastfeeding when the Rose Bengal test turned out positive. Brucella sacroiliitis should be considered in puerperium period women when buttock pain and difficulty in walking are present and pain is unresponsive to analgesics. PMID:26675497

  5. Bilateral cystic nephroma with pleuropulmonary blastoma

    PubMed Central

    Bhardwaj, Anand Kumar; Sharma, Prem Dass; Mittal, Amit; Sharma, Akshay

    2011-01-01

    Cystic nephroma is a rare benign renal neoplasm that is purely cystic and is lined by an epithelium. Bilateral cystic nephromas are even rarer with only a handful cases reported in the literature. A case of a 2-year-old male child who presented with bilateral renal cystic masses later diagnosed as cystic nephromas is presented here. Ultrasound, CT scan and histopathological investigations aided in arriving at the correct diagnosis. The most concerning feature was the presence of a fluid filled cystic mass in the lungs, most probably a pleuropulmonary blastoma which is a rare malignant neoplasm known to be associated with bilateral cystic nephromas. The most common presenting symptoms of cystic nephroma are painless abdominal mass, abdominal or flank pain and haematuria. These tumours usually follow a benign course and nephrectomy alone is curative. A close surveillance of such patients is recommended because of elevated risk of other tumours. PMID:22688934

  6. Bilateral representation in the deep cerebellar nuclei

    PubMed Central

    Soteropoulos, Demetris S; Baker, Stuart N

    2008-01-01

    The cerebellum is normally assumed to represent ipsilateral movements. We tested this by making microelectrode penetrations into the deep cerebellar nuclei (mainly nucleus interpositus) of monkeys trained to perform a reach and grasp task with either hand. Following weak single electrical stimuli, many sites produced clear bilateral facilitation of multiple forelimb muscles. The short onset latencies, which were similar for each side, suggested that at least some of the muscle responses were mediated by descending tracts originating in the brainstem, rather than via the cerebral cortex. Additionally, cerebellar neurones modulated their discharge with both ipsilateral and contralateral movements. This was so, even when we carefully excluded contralateral trials with evidence of electromyogram modulation on the ipsilateral side. We conclude that the deep cerebellar nuclei have a bilateral movement representation, and relatively direct, powerful access to limb muscles on both sides of the body. This places the cerebellum in an ideal position to coordinate bilateral movements. PMID:18187463

  7. A weighted index of bilateral brain lesions.

    PubMed

    Hodos, W; Bobko, P

    1984-11-01

    Attempts to correlate the amount of bilateral brain injury with behavioral changes can be complicated if the lesions are bilaterally unsymmetrical in volume. Further complications are introduced if unilateral lesions are ineffective in producing behavioral changes. An index, W%, is proposed which is based on the product of the volumes of the damage on the right and left sides. The index ranges from 0 to 100 such that conditions of no injury or unilateral injury produce an index of zero. In contrast, relatively large values of the index are generated by symmetrical lesions. Grossly unsymmetrical lesions, in which one side is nearly intact, produce very low index values. For lesions that are bilaterally equivalent in volume, the percentage of tissue destroyed on either side is equal to 10 square root of W%. Some properties of the proposed index are discussed. PMID:6513590

  8. Bilateral Facial Nerve Palsy: A Diagnostic Dilemma

    PubMed Central

    Pothiawala, Sohil; Lateef, Fatimah

    2012-01-01

    Introduction. Bilateral facial nerve palsy (FNP) is a rare condition, representing less than 2% of all cases of FNP. Majority of these patients have underlying medical conditions, ranging from neurologic, infectious, neoplastic, traumatic, or metabolic disorders. Objective. The differential diagnosis of its causes is extensive and hence can present as a diagnostic challenge. Emergency physicians should be aware of these various diagnostic possibilities, some of which are potentially fatal. Case Report. We report a case of a 43-year-old female who presented to the emergency department with sequential bilateral facial nerve paralysis which could not be attributed to any particular etiology and, hence, presented a diagnostic dilemma. Conclusion. We reinforce the importance of considering the range of differential diagnosis in all cases presenting with bilateral FNP. These patients warrant admission and prompt laboratory and radiological investigation for evaluation of the underlying cause and specific further management as relevant. PMID:23326715

  9. Bilateral craniotomies for blunt head trauma.

    PubMed

    Razack, N; Singh, R V; Petrin, D; Villanueva, P; Green, B A

    1997-11-01

    Development of delayed or recurrent intracranial hematomas requiring reexploration or a secondary craniotomy is well known. Patients with bilateral pathology requiring bilateral craniotomies as the initial emergency operative intervention, however, are uncommon. The lack of available literature and the large volume of head trauma seen at our institution prompted us to analyze the retrospective data on blunt head injury requiring bilateral craniotomies. Twenty patients underwent bilateral craniotomies at the University of Miami/Jackson Memorial Medical Center between January 1986 and June 1994. Ages ranged from 18 to 85 years. Mechanism of injury included motor vehicle crash (n = 4), pedestrian hit by automobile (n = 4), assault (n = 8), fall from height (n = 3), and unknown (n = 1). Epidural hematomas, acute subdural hematomas, contusions, and intracerebral hematomas were seen in varying combinations. The preoperative Glasgow Coma Scale (GCS) score ranged from 4 to 14, with a mean of 8.8 (+/-0.82 SE). Sixteen of the 20 patients survived and were discharged from the hospital. The survivors' Rancho Los Amigos Scale score on discharge ranged from 2 to 8, with a mean of 6.1 (+/-0.45 SE). A Fisher's exact test was performed to compare the outcome between the patients with mild (GCS score 13-15) to moderate (GCS score 9-12) head injury and those with severe (GCS score 4-8) head injury. It showed a statistically higher frequency of death in the severe category (p < 0.05). In conclusion, the outcome of patients with bilateral pathology requiring emergency bilateral craniotomy at initial treatment correlated well with their GCS scores at initial presentation. PMID:9390498

  10. Bilateral Metallic Stenting in Malignant Hilar Obstruction

    PubMed Central

    Moon, Jong Ho; Park, Sang-Heum

    2014-01-01

    Endoscopic palliative biliary drainage is considered as a gold standard treatment in advanced or inoperable hilar cholangiocarcinoma. Also, metal stents are preferred over plastic stents in patients with >3 months life expectancy. However, the endoscopic intervention of advanced hilar obstruction is often more challenging and complex than that of distal malignant biliary obstructions. In this literature review, we describe the issues commonly encountered during endoscopic unilateral (single) versus bilateral (multiple) biliary stenting for malignant hilar obstruction. Also, we provide technical guidance to improve the technical success rates and patient outcomes, focusing on bilateral metallic stenting techniques such as stent-in-stent or side-by-side deployment. PMID:25325005

  11. A case of bilateral ophthalmoplegia while diving.

    PubMed

    Lee, Blair C; Young, Colin R

    2015-01-01

    This case report presents a military diver who became dysphoric and lost consciousness during a routine surface-supplied dive. The patient regained consciousness spontaneously, but the physical exam was notable for bilateral ophthalmoplegia. Full eye movement was regained during hyperbaric oxygen (HBO2) therapy, and the patient subsequently made a full recovery. Equipment and dive profile analysis led to the conclusion of hypercapnia and arterial gas embolism as the probable causes of the diver's symptoms. This is a unique case of isolated bilateral ophthalmoplegia presenting in a diving injury. PMID:26403021

  12. Bilateral Multifocal Choroidal Osteoma with Choroidal Neovascularization

    PubMed Central

    MirNaghi, Mousavi; Nasser, Shoeibi; SeyedehMaryam, Hosseini; Ali, Saadat Targhi

    2015-01-01

    Choroidal osteoma has been reported to be unilateral in approximately 80% of cases diagnosed with this condition. Herein we report the clinical characteristics and multimodal imaging findings in a rare case of bilateral multifocal choroidal osteoma. A 32-year-old female presented with decreased visual acuity (VA) in the right eye (20/100), though she had normal VA (20/20) in the left eye. Ophthalmoscopy and multimodal imaging investigation revealed bilateral multifocal choroidal osteoma complicated by choroidal neovascularization (CNV) in the right eye. Following three injections of intravitreal bevacizumab (IVB) for CNV in the right eye, VA improved to 20/40. PMID:26770855

  13. Repair of the Left Pulmonary Artery After Bilateral Banding in Hybrid Procedures: Results Using "Sutureless" Repair Technique.

    PubMed

    Juaneda, Ignacio; Peirone, Alejandro; Diaz, Juan; Kreutzer, Christian

    2016-01-01

    We describe the use of the so-called "sutureless" technique for repair of stenosis of the left pulmonary artery after bilateral banding in the management of two patients with hypoplastic left heart syndrome who had undergone initial palliation with a hybrid procedure. After bilateral pulmonary artery debanding, arterioplasty with autologous pericardial patch and sutureless technique was carried out to decrease the risk of restenosis. Both patients survived the operation, and the exit pulmonary angiography revealed adequate flow across the arterioplasty sites. Patients are alive and well at 17 and 24 months of age, respectively. PMID:26714999

  14. Unusual Presentation of Recurrent Pyogenic Bilateral Psoas Abscess Causing Bilateral Pulmonary Embolism by Iliac Vein Compression

    PubMed Central

    Ijaz, Mohsin; Sakam, Sailaja; Ashraf, Umair; Marquez, Jose Gomez

    2015-01-01

    Patient: Male, 47 Final Diagnosis: Bilateral psoas abscess • acute lower extremity deep vein thrombosis • bilateral pulmonary embolism Symptoms: Progressive left leg swelling • productive cough with whitish sputum • right flank pain Medication: Antibiotics and anticoagulation Clinical Procedure: CT-guided percutaneous drain placement Specialty: Internal Medicine/Critical Care Objective: Unusual presentation Background: Psoas abscesses are a known cause of back pain, but they have not been reported as a cause of acute lower extremity thromboses and bilateral pulmonary emboli. We report a patient with bilateral psoas abscesses causing extensive pulmonary emboli through compression of the iliac vein. Case Report: A 47-year-old man presented with bilateral leg swelling over 4 weeks. Physical examination revealed a thin male with bilateral leg swelling, extending to the thigh on his left side. He had hemoglobin of 10.5 g/dl, leukocytosis of 16 000/ml, and an elevated D-dimer. A computed tomography (CT) angiogram of his chest showed extensive bilateral pulmonary emboli and infarcts. He remained febrile with vague flank pain, prompting a CT of his abdomen and pelvis that showed large, multiloculated, septated, bilateral psoas abscesses with compression of the left femoral vein by the left psoas abscess and a thrombus distal to the occlusion. Two liters of pus was drained from the left psoas abscess by CT-guidance, and although the Gram staining showed Gram-positive cocci in clusters, cultures from the abscess and blood were negative. A repeat CT showed resolution of the abscesses, and the drain was removed. He was discharged to a nursing home to complete a course of intravenous antibiotics and anticoagulation. Conclusions: Although the infectious complications of psoas abscesses have been described in the literature, the mechanical complications of bilateral psoas abscesses are lacking. It is important to assess for complete resolution of psoas abscesses through follow-up imaging to prevent venous thromboembolic events. PMID:26356406

  15. Renal dysfunction due to hydronephrosis by SAPHO syndrome: a case report

    PubMed Central

    Kakoki, Katsura; Miyata, Yasuyoshi; Enokizono, Mikako; Uetani, Masataka; Sakai, Hideki

    2015-01-01

    Key Clinical Message Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis (SAPHO) syndrome shows varied pathological symptoms. This is the first report of hydronephrosis due to the mechanical compression of bilateral ureters as a result of SAPHO syndrome. From our experience, MRI is the most useful imaging examination to check the upper urinary tract in SAPHO syndrome. PMID:26331013

  16. Atraumatic bilateral scapular spine fracture several months after bilateral reverse total shoulder arthroplasty.

    PubMed

    Nicolay, Simon; De Beuckeleer, Luc; Stoffelen, Daniël; Vanhoenacker, Filip; Pouillon, Marc

    2014-05-01

    We report an 89-year-old woman with bilateral atraumatic scapular spine fracture several months after bilateral reverse total shoulder arthroplasty (RTSA). Recently, RTSA has gained popularity in the surgical treatment of complex shoulder disorders such as cuff tear arthropathy. However, scapular fractures may occur several months after surgery as a late complication of this procedure. In this case report we focus on a relatively uncommon subtype, the scapular spine fracture. Although well-known in the orthopedic literature, radiologists are less familiar with this complication. To the best of our knowledge, bilateral scapular fractures have not yet been reported. PMID:24276680

  17. A Bilateral Traumatic Hip Obturator Dislocation

    PubMed Central

    Karaarslan, Ahmet Adnan; Acar, Nihat; Karci, Tolga; Sesli, Erhan

    2016-01-01

    A case of a bilateral simultaneous traumatic obturator dislocation of both hip joints in an 18-year-old young man following a traffic accident is presented. We reduced the dislocated femoral heads immediately under general anesthesia followed by passive and active exercises and early full-weight bearing mobilization. After 5 years, the result was excellent. PMID:26977327

  18. [Quinine-induced renal bilateral cortical necrosis].

    PubMed

    Leroy, F; Bridoux, F; Abou-Ayache, R; Belmouaz, S; Desport, E; Thierry, A; Bauwens, M; Touchard, G

    2008-06-01

    Acute bilateral renal cortical necrosis is a rare cause of renal failure frequently induced by disseminated intravascular coagulation (Dic) following obstetrical complications, sepsis and drugs. We describe a case of Dic with bilateral cortical necrosis after ingestion of only one tablet of quinine. A 41-year-old woman was admitted for severe abdominal pain, melaena, fever and anuria two hours after quinine tablet intake for nocturnal leg cramps. Her medical history included angioneurotic edema caused by chloroquine for malaria prevention. Physical examination was normal. Laboratory data showed acute renal failure, hemolytic anemia without schistocytes and Dic. Platelet antibodies were negative. Ultrasonographic examination showed a complete defect of renal perfusion with permeable renal arteries. Results of abdominal CT scan and MAG3 scintigraphy led to the diagnosis of bilateral renal cortical necrosis. The patient underwent plasma exchanges with fresh frozen plasma which induced rapid resolution of Dic. She remained dependent on chronic hemodialysis. Quinine-induced microangiopathic hemolytic anemia and Dic is a rare described entity. These complications occur typically in quinine-sensitized subjects. The presence of acute renal failure is generally associated with poor prognosis in case of bilateral renal cortical necrosis. Caution is required for the prescription of quinine derivates, which should be avoided in patients experienced on adverse reaction to the drug. PMID:18343736

  19. Synchronous bilateral breast cancer in a male

    PubMed Central

    Rubio Hernández, María Caridad; Díaz Prado, Yenia Ivet; Pérez, Suanly Rodríguez; Díaz, Ronald Rodríguez; Aleaga, Zaili Gutiérrez

    2013-01-01

    Male breast cancer, which represents only 1% of all breast cancers, is occasionally associated with a family history of breast cancer. Sporadic male breast cancers presenting with another primary breast cancer are extremely rare. In this article, we report on a 70-year-old male patient with bilateral multifocal and synchronous breast cancer and without a family history of breast cancer. PMID:24319497

  20. Bilateral synergies in foot force production tasks.

    PubMed

    Sarabon, Nejc; Markovic, Goran; Mikulic, Pavle; Latash, Mark L

    2013-05-01

    We analysed the effects of task symmetry during bilateral accurate force production tasks performed by the two feet. In particular, we tested a hypothesis that bilateral deficit would lead to higher indices of synergies defined as co-varied adjustments in the two forces across trials that reduced total force variability. The subjects produced steady-state force followed by a quick force pulse into the target. The two feet could be acting both into plantar flexion and into dorsiflexion (symmetrical tasks), or in opposite directions (asymmetrical task). We used the framework of the uncontrolled manifold hypothesis to quantify two variance components, one of which did not change total force (V UCM), while the other did (V ORT). Synergy indices during the asymmetrical task were higher than in either symmetrical task. The difference was due to higher V UCM (compared to the symmetrical plantar flexion task) or lower V ORT (compared to the symmetrical dorsiflexion task). The synergy index showed a drop (anticipatory synergy adjustment, ASA) starting 100-150 ms prior to the force pulse initiation. The ASA tended to be shorter and of a smaller magnitude for the asymmetrical task. This is the first demonstration of bilateral synergies during accurate force production by the legs. We conclude that bilateral deficit has no or weak effects on two-leg synergies. The results fit the earlier introduced scheme with two groups of neural variables defining average performance of a redundant system and patterns of co-variation among its elemental variables, respectively. PMID:23568657

  1. Computed tomographic findings in bilateral adrenal tuberculosis

    SciTech Connect

    Wilms, G.E.; Baert, A.L.; Kint, E.J.; Pringot, J.H.; Goddeeris, P.G.

    1983-03-01

    The computed tomographic (CT) features of bilateral adrenal tuberculosis are reported in two cases that demonstrate two typical different clinical and morphological manifestations of the disease. The incidence and CT appearance of adrenal tuberculosis are discussed, with emphasis on differential diagnosis.

  2. Bilateral Retrobulbar Optic Neuritis Caused by Varicella Zoster Virus in a Patient with AIDS

    PubMed Central

    Duda, Jose F.; Castro, Jose G.

    2015-01-01

    Aims To report on a case of bilateral retrobulbar optic neuritis in a patient with acquired immune deficiency syndrome (AIDS) caused by varicella-zoster virus (VZV); and to review the literature focusing on: cases reported, epidemiology, pathophysiology, diagnosis and treatment. Presentation of Case A 38-year-old woman with AIDS presented with a 10-day history of progressive bilateral visual loss and ocular pain. She had bilateral dilated pupils with no light perception; the fundoscopic examination was normal. Facial herpes zoster lesions appeared on the second day of hospitalization Magnetic resonance imaging (MRI) findings were compatible with a bilateral optic neuritis; the cerebrospinal fluid (CSF) showed pleocytosis, increased proteins and a positive VZV-DNA PCR. She was treated with intravenous acyclovir and corticosteroids and was able, when discharged 2 weeks after admission, to carry out activities of daily living. Discussion VZV retrobulbar optic neuritis has previously been reported in 12 patients with AIDS, more than half of the cases had concomitant herpes zoster and an associated retinopathy. A positive VZV-DNA in the CSF is indicative of VZV infection, initial use of intravenous acyclovir is recommended, and the concomitant use of corticosteroids would be a prudent choice; the duration of antiviral therapy remains undefined. Conclusion VZV retrobulbar optic neuritis in AIDS patients can occur with or without herpes zoster. It is a sight-threatening infectious and inflammatory process requiring the advice of specialists in infectious diseases, ophthalmology, neurology and viral microbiology. PMID:26740936

  3. Neglected surgically intervened bilateral congenital dislocation of knee in an adolescent

    PubMed Central

    Kumar, Jaswant; Dhammi, Ish Kumar; Jain, Anil K

    2014-01-01

    Neglected bilateral congenital dislocation of knee is unusual. A 12 year old boy presented with inability to walk due to buckling of the knee. The symptoms were present since the child learnt walking. He preferred not to walk. Bilateral supracondylar femoral osteotomy was done at the age of 6 years. Patient had a fixed flexion deformity of both knees, 30° in the right (range of flexion from 30° to 45°) and 45° fixed flexion deformity in left knee respectively (range of flexion from 45° to 65°) when presented to us. The radiological examination revealed bilateral congenital dislocation of knee (CDK). No syndromic association was observed. He was planned for staged treatment. In stage I, the knee joints were distracted by Ilizarov ring fixators and this was followed by open reduction of both the knee joints in stage II. A bilateral supracondylar extension osteotomy was done 18 months after the previous surgery (stage III). The final followup visit at 4 years the patient presented with range of motion 5-100° and 5-80° on the right and left knee respectively with good functional outcome. The case is reported in view of lack of treatment guidelines for long standing neglected CDK in an adolescent child. PMID:24600070

  4. Metabolic Syndrome

    MedlinePLUS

    ... Th M e etabolic Syndrome What is the metabolic syndrome? The term metabolic syndrome describes a cluster of risk factors that increase ... high blood sugar). The exact cause of the metabolic syndrome is not known but genetic factors, too much ...

  5. Antiphospholipid Syndrome

    MedlinePLUS

    ... Awards Enhancing Diversity Find People About NINDS NINDS Antiphospholipid Syndrome Information Page Synonym(s): Hughes Syndrome Table of Contents ( ... research is being done? Clinical Trials What is Antiphospholipid Syndrome? Antiphospholipid syndrome (APS) is an autoimmune disorder caused ...

  6. Cushing's Syndrome

    MedlinePLUS

    MENU Return to Web version Cushing's Syndrome Overview What is Cushing's syndrome? Cushing's syndrome occurs when your body is exposed to high levels ... they can cause problems with your eyesight. Diagnosis & Tests How is Cushing's syndrome diagnosed? Your doctor may ...

  7. Down Syndrome

    MedlinePLUS

    ... Sledding, Skiing, Snowboarding, Skating Crushes What's a Booger? Down Syndrome KidsHealth > For Kids > Down Syndrome Print A A ... skills. continue Do a Lot of People Have Down Syndrome? Down syndrome is not contagious , so you can' ...

  8. Filippi Syndrome: Report of a Rare Case

    PubMed Central

    Goyal, Lata; Bhakhri, Bhanu Kiran; Chug, Ashi

    2015-01-01

    Filippi syndrome is an autosomal recessive condition characterized by syndactyly of fingers and toes, microcephaly, growth retardation and abnormal facies. We are describing a boy who presented with syndactyly, mental retardation, microcephaly, depressed nasal bridge and growth retardation. In addition he had some dental abnormalities like missing bilateral lateral incisors and delayed eruption of teeth. We concluded it to be Filippi syndrome by studying pathognomic clinical features and reviewed the literature. This is the second case report from India. PMID:26813917

  9. Sturge–Weber syndrome – A case report

    PubMed Central

    Shaikh, Shahid M.; Goswami, Mousumi; Singh, Sanjay; Singh, Darrel

    2015-01-01

    Sturge–Weber syndrome (SWS), also called as encephalotrigeminal angiomatosis is an uncommon congenital neurological disorder & frequent among the neurocutaneous syndromes specifically with vascular predominance. This disorder is characterized by facial capillary malformation & other neurological condition. The oral manifestations are gingival hemangiomatosis restricting to either side in upper and lower jaw, sometimes bilateral. We report a case of SWS with oral, ocular and neurological features. PMID:25853049

  10. Bilateral ureteral obstruction due to primary myelofibrosis caused hyperuricaemia.

    PubMed

    Cvetkovi?, Z P; Cvetkovi?, B R; Celeketi?, D; Milenkovi?, D; Perunici?-Pekovi?, G

    2010-01-01

    In healthy population, uric acid comprises the major component of 10-20% of renal stones. Extreme hiperuricaemia is seen in cancer patients with tumour lysis syndrome (TLS) which is classically associated with haematological malignancies with rapid tumour growth rates such as acute lymphoid leukaemia and high grade lymphomas. Primary melofibrosis (Agnogenic myeloid metaplasia-AMM) is a chronic myeloproliferative disease characterized by splenomegaly, a leukoerythroblastic blood picture, teardrop poikilocytosis and varying degrees of marrow fibrosis. Due to the increased extramedullary haematopoiesis, hiperuricemia may occur. However, TLS in patients with AMM is, according to the available literature, described just in one patient. In this paper we present a case of a 47-year-old male patient who was admitted to the hospital with symptoms of fatigue and small amount of urine, and clinical signs of plethora and enlarged spleen. The laboratory findings showed leuko-and erythrocytosis, increased levels of urea-BUN (32 mmol/l) and creatinine (766 mmol/l) as well as uric acid (920 mmol/l). The immediate abdominal ultrasound confirmed extreme splenomegaly, but also showed bilateral hydronephrosis of grade II-III with two stones in proximal part of right ureter and one in proximal part of left ureter as well as empty bladder. Stones were not seen on plain film. Since the patient was in complete anuria, with further rapid elevation of BUN and creatinine levels, bilateral ureteral stents were applicated together with extensive hydration, urine alkalization and administration of allopurinol which resulted in the complete recovery of kidney function. The bone marrow biopsy was also performed and histopathological diagnosis was: Hypercellulary phase of AMM. PMID:20949707

  11. Bilateral Synergy: A Framework for Post-Stroke Rehabilitation

    PubMed Central

    Sainburg, RL; Good, D; Przybyla, A

    2014-01-01

    Background Unilateral stroke produces debilitating deficits in voluntary control in the contralesional arm, and significant motor coordination deficits in the ipsilesional arm. In addition, patients tend to avoid bilateral arm patterns and during performance of activities of daily living. Nevertheless, upper extremity physical rehabilitation predominantly focuses on motor training activities with only the paretic arm. This can be limiting because of persistent deficits in the ipsilesional arm, and because of the tendency of patients to avoid spontaneous bilateral arm patterns. Proposition Rehabilitation should focus on bilateral training to advance recovery of function in both arms of stroke patients, as well as to facilitate spontaneous bilateral arm use. This paper reviews the rationale for this approach, citing evidence for significant hemisphere specific bilateral motor deficits in stroke patients, which affect both the contralesional and the ipsilesional arm. The rationale for, and advantages of, training both arms simultaneously through bilateral tasks is reviewed. Although bilateral training has been employed to treat stroke patients previously, this has tended to focus on bimanual ‘coupling’ as a rationale for performing parallel, but not cooperative bilateral tasks. Bilateral synergy provides a more functional framework for structuring post-stroke upper extremity rehabilitation. Conclusion Bilateral synergy may be causally linked to spontaneous bilateral arm use, suggesting that rehabilitation should be focused on bilateral cooperative tasks, such as bilateral object transport. Further research is required to determine whether this approach could be efficacious for patients with hemiparesis, and whether both left and right hemisphere strokes can benefit from such intervention. PMID:24729985

  12. Bilateral Facial Paralysis Caused by Bilateral Temporal Bone Fracture: A Case Report and a Literature Review

    PubMed Central

    Eliçora, Sultan ?evik; Dinç, Aykut Erdem; Bi?kin, Sultan; Damar, Murat; Bilgin, Ergin

    2015-01-01

    Bilateral facial paralysis caused by bilateral temporal bone fracture is a rare clinical entity, with seven cases reported in the literature to date. In this paper, we describe a 40-year-old male patient with bilateral facial paralysis and hearing loss that developed after an occupational accident. On physical examination, House-Brackmann (HB) facial paralysis of grade 6 was observed on the right side and HB grade 5 paralysis on the left. Upon temporal bone computed tomography (CT) examination, a fracture line exhibiting transverse progression was observed in both petrous temporal bones. Our patient underwent transmastoid facial decompression surgery of the right ear. The patient refused a left-side operation. Such patients require extensive monitoring in intensive care units because the presence of multiple injuries means that facial functions are often very difficult to evaluate. Therefore, delays may ensue in both diagnosis and treatment of bilateral facial paralysis. PMID:26175920

  13. [Two siblings with bilateral congenital knee dislocations: case report].

    PubMed

    Kubinec, V; Polakovi?ová, L; Kantarská, D

    2015-01-01

    Congenital dislocation of the knee and congenital permanent dislocation of the patella are rare disorders usually associated with complex syndromes. Two cases of siblings, girl and boy, who had the same clinical phenotype of this disorder are presented. The diagnosis of Desbuquois dysplasia was made and its autosomal recessive heredity was confirmed by genetic analysis. DNA samples were sent for a molecular genetic analysis of the skeletal dysplasia. The girl was surgically treated for a complete (grade 3) anterior dislocation of the tibia on the femur in the first year of life. Redressing casts had not previously been applied to avoid the risk of damaging the epiphysis. The left knee was operated on by the method, as described by Curtis and Fisher, at the age of six months. The Kirschner wire was removed after one month and a plaster cast was applied to maintain the flexion required. At seven post-operative weeks physiotherapy was started with temporary use of a knee brace. The right knee was managed by a similar procedure at four months after the first surgery. Normal walking was achieved at the age of 21 months. Knee motion was symmetrically restricted, with 5 to 90 degrees of flexion. The boy was first seen at our out-patient department after his sister had achieved full walking ability. He was 10 years old at that time and presented with walking problems due to nearly 30 degrees of bilateral knee contractures. Permanent dislocation of both patellae was treated by the surgical technique described by Stanisavljevic. Revision surgery of the right knee due to patellar lateralization was required two years after the primary surgery; it was performed using the Campbell's technique. A corrective osteotomy of the left proximal tibia because of progressive genu valgum was carried out at four years following the first operation. At the last follow-up, the boy was 16 years old and the knee range of motion bilaterally was 0 to 120 degrees with good alignment of both knees. As a result of surgical treatment the two patients gained ability to walk without problems. Key words: bilateral congenital knee dislocation, patella, Desbuquois dysplasia, siblings, skeletal dysplasia, case report. PMID:26317189

  14. Hardware Implementation of a Bilateral Subtraction Filter

    NASA Technical Reports Server (NTRS)

    Huertas, Andres; Watson, Robert; Villalpando, Carlos; Goldberg, Steven

    2009-01-01

    A bilateral subtraction filter has been implemented as a hardware module in the form of a field-programmable gate array (FPGA). In general, a bilateral subtraction filter is a key subsystem of a high-quality stereoscopic machine vision system that utilizes images that are large and/or dense. Bilateral subtraction filters have been implemented in software on general-purpose computers, but the processing speeds attainable in this way even on computers containing the fastest processors are insufficient for real-time applications. The present FPGA bilateral subtraction filter is intended to accelerate processing to real-time speed and to be a prototype of a link in a stereoscopic-machine- vision processing chain, now under development, that would process large and/or dense images in real time and would be implemented in an FPGA. In terms that are necessarily oversimplified for the sake of brevity, a bilateral subtraction filter is a smoothing, edge-preserving filter for suppressing low-frequency noise. The filter operation amounts to replacing the value for each pixel with a weighted average of the values of that pixel and the neighboring pixels in a predefined neighborhood or window (e.g., a 9 9 window). The filter weights depend partly on pixel values and partly on the window size. The present FPGA implementation of a bilateral subtraction filter utilizes a 9 9 window. This implementation was designed to take advantage of the ability to do many of the component computations in parallel pipelines to enable processing of image data at the rate at which they are generated. The filter can be considered to be divided into the following parts (see figure): a) An image pixel pipeline with a 9 9- pixel window generator, b) An array of processing elements; c) An adder tree; d) A smoothing-and-delaying unit; and e) A subtraction unit. After each 9 9 window is created, the affected pixel data are fed to the processing elements. Each processing element is fed the pixel value for its position in the window as well as the pixel value for the central pixel of the window. The absolute difference between these two pixel values is calculated and used as an address in a lookup table. Each processing element has a lookup table, unique for its position in the window, containing the weight coefficients for the Gaussian function for that position. The pixel value is multiplied by the weight, and the outputs of the processing element are the weight and pixel-value weight product. The products and weights are fed to the adder tree. The sum of the products and the sum of the weights are fed to the divider, which computes the sum of products the sum of weights. The output of the divider is denoted the bilateral smoothed image. The smoothing function is a simple weighted average computed over a 3 3 subwindow centered in the 9 9 window. After smoothing, the image is delayed by an additional amount of time needed to match the processing time for computing the bilateral smoothed image. The bilateral smoothed image is then subtracted from the 3 3 smoothed image to produce the final output. The prototype filter as implemented in a commercially available FPGA processes one pixel per clock cycle. Operation at a clock speed of 66 MHz has been demonstrated, and results of a static timing analysis have been interpreted as suggesting that the clock speed could be increased to as much as 100 MHz.

  15. Macular dystrophy in Heimler syndrome

    PubMed Central

    Lima, Luiz H.; Barbazetto, Irene A.; Chen, Royce; Yannuzzi, Lawrence A.; Tsang, Stephen H.; Spaide, Richard F.

    2011-01-01

    Purpose To describe the retinal imaging findings in the index patient with Heimler syndrome (OMIM #234580). Design Non-interventional case report. Methods A 29-year-old woman with Heimler syndrome developed bilateral vision loss. Fluorescein angiography (FA), fundus autofluorescence (FAF), spectral domain optical coherence tomography (SD-OCT) and electroretinography (ERG) were performed to assess the retinal anatomy and function. Results FA showed mottling of the retinal pigment epithelium (RPE) in the posterior pole and periphery of the retina. FAF revealed hyper and hypoautofluorescent dots corresponding to the RPE mottling observed on FA. SD-OCT documented loss of the inner/outer segments boundary, and RPE thinning. ERG testing excluded generalized rod-cone dysfunction. Conclusion We report an adult-onset macular dystrophy in one of the previously reported patients with Heimler syndrome and hypothesize that this syndrome is probably an expression of a ciliopathy. PMID:21366429

  16. Bilateral thoracoscopic cervical sympathectomy for the treatment of recurrent polymorphic ventricular tachycardia

    PubMed Central

    Turley, A J; Thambyrajah, J; Harcombe, A A

    2005-01-01

    Congenital long QT syndrome (LQTS) is a disorder of prolonged cardiac repolarisation, manifest by a prolonged QT interval and characterised by recurrent presyncope/syncope, polymorphic ventricular tachycardia (PMVT), or sudden cardiac death. A 46 year old woman with no family history of sudden death or deafness presented with recurrent syncope. Physical examination and electrolytes were normal and a 12 lead ECG showed a corrected QT interval of 458 ms. A monitored syncopal episode documented PMVT. Despite potassium and magnesium supplements, ? blockade, implantation of a single then dual chamber implantable cardioverter defibrillator (ICD), amiodarone, nicorandil, and mexiletine, the patient continued to experience arrhythmia storms, receiving more than 700 ICD discharges over seven months. She was ultimately treated successfully with bilateral thoracoscopic cervicothoracic sympathectomies. This is the first reported bilateral thoracoscopic treatment of a patient with LQTS and symptomatic life threatening ventricular tachyarrhythmias refractory to current pharmacological and pacing techniques. PMID:15604323

  17. Osteogenesis imperfecta with joint contractures: bruck syndrome.

    PubMed

    Blacksin, M F; Pletcher, B A; David, M

    1998-02-01

    We describe an Egyptian boy with osteogenesis imperfecta who was born with thumb contractures and bilateral antecubital pterygia. He was seen at 16 months of age with femur and tibial fractures, thoracic vertebral compression fractures, scoliosis and Wormian bones. The findings are consistent with a diagnosis of Bruck syndrome. PMID:9472060

  18. Marshall syndrome: a condition resembling congenital syphilis.

    PubMed Central

    Onile, B A; Rotowa, A; Osoba, A O; Alausa, O K

    1981-01-01

    Marshall syndrome (ectodermal dysplasia) was diagnosed in a 14-year-old boy and is thought to be the first case reported from West Africa. Because of the saddle-nose deformity and bilateral cataracts all patients suspected of having congenital syphilis should be investigated for ocular or auditory defects, which would confirm the diagnosis of ectodermal dysplasia. Images PMID:7214117

  19. Two Cases of Heerfordt's Syndrome: A Rare Manifestation of Sarcoidosis

    PubMed Central

    Furuta, Yasushi; Fukuda, Satoshi

    2016-01-01

    Heerfordt's syndrome is a rare manifestation of sarcoidosis characterized by the presence of facial nerve palsy, parotid gland enlargement, anterior uveitis, and low grade fever. Two cases of Heerfordt's syndrome and a literature review are presented. Case  1. A 53-year-old man presented with swelling of his right eyelid, right facial nerve palsy, and swelling of his right parotid gland. A biopsy specimen from the swollen eyelid indicated sarcoidosis and he was diagnosed with incomplete Heerfordt's syndrome based on the absence of uveitis. His symptoms were improved by corticosteroid therapy. Case  2. A 55-year-old woman presented with left facial nerve palsy, bilateral hearing loss, and swelling of her bilateral parotid glands. She had been previously diagnosed with uveitis and bilateral hilar lymphadenopathy. Although no histological confirmation was performed, she was diagnosed with complete Heerfordt's syndrome on the basis of her clinical symptoms. Swelling of the bilateral parotid glands and left facial nerve palsy were improved immediately by corticosteroid therapy. Sarcoidosis is a relatively uncommon disease for the otolaryngologist. However, the otolaryngologist may encounter Heerfordt's syndrome as this syndrome presents with facial nerve palsy and swelling of the parotid gland. Therefore, we otolaryngologists should diagnose and treat Heerfordt's syndrome appropriately in cooperation with pneumologists and ophthalmologists. PMID:26885424

  20. Two Cases of Heerfordt's Syndrome: A Rare Manifestation of Sarcoidosis.

    PubMed

    Fujiwara, Keishi; Furuta, Yasushi; Fukuda, Satoshi

    2016-01-01

    Heerfordt's syndrome is a rare manifestation of sarcoidosis characterized by the presence of facial nerve palsy, parotid gland enlargement, anterior uveitis, and low grade fever. Two cases of Heerfordt's syndrome and a literature review are presented. Case  1. A 53-year-old man presented with swelling of his right eyelid, right facial nerve palsy, and swelling of his right parotid gland. A biopsy specimen from the swollen eyelid indicated sarcoidosis and he was diagnosed with incomplete Heerfordt's syndrome based on the absence of uveitis. His symptoms were improved by corticosteroid therapy. Case  2. A 55-year-old woman presented with left facial nerve palsy, bilateral hearing loss, and swelling of her bilateral parotid glands. She had been previously diagnosed with uveitis and bilateral hilar lymphadenopathy. Although no histological confirmation was performed, she was diagnosed with complete Heerfordt's syndrome on the basis of her clinical symptoms. Swelling of the bilateral parotid glands and left facial nerve palsy were improved immediately by corticosteroid therapy. Sarcoidosis is a relatively uncommon disease for the otolaryngologist. However, the otolaryngologist may encounter Heerfordt's syndrome as this syndrome presents with facial nerve palsy and swelling of the parotid gland. Therefore, we otolaryngologists should diagnose and treat Heerfordt's syndrome appropriately in cooperation with pneumologists and ophthalmologists. PMID:26885424

  1. Bilateral Orbital Abscesses After Strabismus Surgery.

    PubMed

    Dhrami-Gavazi, Elona; Lee, Winston; Garg, Aakriti; Garibaldi, Daniel C; Leibert, Michelle; Kazim, Michael

    2015-01-01

    Infectious orbital complications after strabismus surgery are rare. Their incidence is estimated to be 1 case per 1,100 surgeries and include preseptal cellulitis, orbital cellulitis, subconjunctival and sub-Tenon's abscesses, myositis, and endophthalmitis. This report describes the case of an otherwise healthy 3-year-old boy who underwent bilateral medial rectus recession and disinsertion of the inferior obliques. A few days after surgery, the patient presented with bilateral periorbital edema and inferotemporal chemosis. A series of CT scans with contrast revealed inferotemporal orbital collections OU. The patient immediately underwent transconjunctival drainage of fibrinous and seropurulent collections in the sub-Tenon's space and experienced rapid improvement a few days later. The patient is reported to be in stable condition in a follow-up examination performed more than a year after the reported events. PMID:24896771

  2. Bilateral breast cancer after cured Hodgkin's disease

    SciTech Connect

    Anderson, N.; Lokich, J. )

    1990-01-15

    Three patients developed bilateral breast cancer at 10 to 24 years after mantle irradiation for locally or systemically advanced Hodgkin's disease (HD). Four of the six cancers in the three patients were detected only by mammography. Pathologically, five of the cancers were intraductal carcinomas (four with an invasive component) with one being a lobular carcinoma. Five of the six lesions were Stage I pathologically without evidence of axillary nodal involvement. It is recommended that female patients with Hodgkin's disease who have received mantle irradiation as part of the therapy for their Hodgkin's disease and who are observed for 10 or more years after completion of mantle irradiation be considered at risk for the development of breast cancer. Such patients should be monitored appropriately by routine bilateral mammograms to increase the early detection of early stage lesions.

  3. Bilateral idiopathic optic neuritis in pregnancy.

    PubMed

    Liu, Tiffany S; Crow, Robert W

    2013-01-01

    A 33-year-old primiparous woman at 31 weeks gestation with no prior history of ocular disease, autoimmune disease, or recent viral infection presented with rapidly decreasing visual acuity to 20/400 OD and counting fingers OS over 11 days. MRI demonstrated mild bilateral optic nerve enhancement. Humphrey 30-2 visual field testing showed bilateral seco-central scotomas. MR venogram, visual evoked potentials, preeclampsia workup, and serum blood tests for folate, B6, B12, Toxoplasmosis, Bartonella, Lyme disease, ACE levels, RPR, and LFTs were all within normal limits. After one-time treatment of IV 125 mg methylprednisolone and spinal tap, visual acuity improved to 20/20 OD and 20/20 OS. PMID:23305442

  4. Bilateral Breast Masses with a Rare Etiology

    PubMed Central

    Sartorius, Gideon; Kalf, Katrin; Heinzelmann, Viola

    2013-01-01

    Breast masses have a variety of benign and malignant etiologies. We present the case of a 28-year-old woman with bilateral large painful breast masses that developed rapidly in the three weeks before first presentation. Further investigation revealed bilateral ovarian masses. Biopsies of both ovarian masses were taken, and the pathology reported Burkitt's lymphoma. Additional staging with a PET scan was suggestive of bone marrow involvement, but bone marrow biopsy was negative. Examination of the cerebrospinal fluid did not identify malignant cells. The patient underwent CODOX-M/IVAC chemotherapy, and a complete response was demonstrated after one cycle of treatment. Six months after finishing chemotherapy the patient remained in complete remission. To our knowledge this is the first case reporting simultaneous involvement of breast, ovaries, and bones in Burkitt's lymphoma. Gynecologists and oncologists should be aware of this pattern. Polychemotherapy treatment must be initiated rapidly with curative intent. PMID:24066248

  5. Unusual Bilateral Rim Fracture in Femoroacetabular Impingement

    PubMed Central

    Rafols, Claudio; Monckeberg, Juan Edo; Numair, Jorge

    2015-01-01

    This is a report of one case of bilateral acetabular rim fracture in association with femoroacetabular impingement (FAI), which was treated with a hip arthroscopic procedure, performing a partial resection, a labral reinsertion, and a subsequential internal fixation with cannulated screws. Up to date, there are in the literature only two reports of rim fracture and “os acetabuli” in association with FAI. In the case we present, the pincer and cam resection were performed without complications; the technique used was published previously. With this technique the head of the screw lays hidden by the reattached labrum. We removed partially the fractured rim fragment and the internal fixation of the remaining portion was achieved with a screw. In the event of a complete resection of the fragment, it would have ended with a LCE angle of 18° and a high probability of hip instability. We believe that this bilateral case helps establish the efficacy and reproducibility of the technique described by Larson. PMID:25722907

  6. Bilateral calcaneal stress fractures in two cats.

    PubMed

    Cantatore, M; Clements, D N

    2015-06-01

    Two cats that developed bilateral calcaneal stress fractures are reported. One cat developed lameness associated with incomplete fractures at the base of both calcanei, both of which progressed to acute, complete fractures 2 months later. The second cat presented with acute complete calcaneal fracture, with evidence of remodelling of the contralateral calcaneus, which subsequently fractured two years later. The calcaneal fractures were successfully stabilised with lateral bone plates in each case. Stress fractures were suspected because of the bilateral nature, the simple and similar configuration, the consistent location of the fractures, the absence of other signs of trauma in both cases and the suspected insidious onset of the lameness. The feline calcaneus is susceptible to stress fracture, and cats presenting with calcaneal fractures without evidence of trauma should be evaluated for concurrent skeletal pathology. PMID:25929309

  7. U.S. bilateral physical protection program

    SciTech Connect

    Goldman, I.N.; Horton, R.D.

    1993-12-31

    A substantial increase in reported incidents in Europe of smuggling and offers for sale of alleged weapons-usable nuclear materials has demonstrated the importance of ensuring a high level of physical protection on nuclear material and facilities worldwide. Since 1974, the United States has conducted bilateral consultations with more than forty nations, including site visits, on physical protection of nuclear material at fixed sites and during transport. These consultations are designed to share technical information and experience concerning physical protection of civil nuclear activities. International cooperation and collaboration in physical protection is essential to reducing the risk of theft or unauthorized acquisition of special nuclear material. This paper provides an overview of the US bilateral program on physical protection of nuclear materials at facilities and during transportation.

  8. Peribulbar anesthesia causing bilateral orbital hemorrhage

    PubMed Central

    Garft, Kyla; Burt, Peter; Burt, Benjamin

    2016-01-01

    We report a case of bilateral orbital hemorrhage as a complication of peribulbar anesthesia in a 78 year old man. Initially, unilateral orbital hemorrhage occurred but quickly spread to the contralateral side. Neuroophthalmological assessment revealed a proptosed tense globe with normal retinovascular findings. Visual acuity was adversely affected and this was conservatively managed with no lasting ophthalmic sequela. This patient’s case was reported as it illustrates an unusual complication of bilateral spread of orbital hemorrhage secondary to peribulbar anesthesia. It highlights how early ophthalmic assessment can ensure a good visual outcome in the setting of appropriate ophthalmic monitoring. The mechanisms of orbital hemorrhage spread and appropriate management options are discussed. PMID:27013899

  9. [Sturge-Weber syndrome].

    PubMed

    Reith, W; Yilmaz, U; Zimmer, A

    2013-12-01

    Sturge-Weber syndrome (SWS) is a rare congenital disease which affects the brain, the skin and the eyes. It is a sporadically occurring neurocutaneous syndrome affecting the intracerebral veins (venous angiomatosis). The frequency is estimated to be 1 in 50,000 births [1]. The main symptom is intracranial leptomeningeal angiomatosis which mostly affects the occipital and posterior parietal lobes and can occur unilaterally and also bilaterally. Facial cutaneous vascular alterations occur ipsilaterally in the form of port wine stains (nevus flammeus) which are normally found in the catchment area of the trigeminal branch VI. Other clinical symptoms associated with SWS are seizures, glaucoma, headaches, transient neurological stroke-like episodes and cognitive impairment. Neurological cortical symptoms often include hemiparesis, hemiatrophy and hemianopsia. PMID:24292369

  10. Balloon pulmonary valvuloplasty and stent implantation. For peripheral pulmonary artery stenosis in Alagille syndrome.

    PubMed Central

    Saidi, A S; Kovalchin, J P; Fisher, D J; Ferry, G D; Grifka, R G

    1998-01-01

    We report the case of a patient with Alagille syndrome and severe pulmonary valve and bilateral pulmonary artery branch stenosis. In this patient, transcatheter balloon pulmonary valvuloplasty combined with bilateral pulmonary artery angioplasty and stent placement provided excellent immediate results and long-term improvement. Images PMID:9566070

  11. A rare case of bilateral aspergillus endophthalmitis

    PubMed Central

    Gupta, Saurabh; Loudill, Cameron; Tammara, Anita; Chow, Robert T.

    2015-01-01

    Aspergillus endophthalmitis is a devastating inflammatory condition of the intraocular cavities that may result in irreparable loss of vision and rapid destruction of the eye. Almost all cases in the literature have shown an identified source causing aspergillus endophthalmitis as a result of direct extension of disease. We present a rare case of bilateral aspergillus endophthalmitis. A 72-year-old woman with a history of diabetes mellitus, congenital Hirschsprung disease, and recent culture-positive candida pyelonephritis with hydronephrosis status post-surgical stent placement presented with difficulty opening her eyes. She complained of decreased vision (20/200) with pain and redness in both eyes – right worse then left. Examination demonstrated multiple white fungal balls in both retinas consistent with bilateral fungal endophthalmitis. Bilateral vitreous taps for cultures and staining were performed. Patient was given intravitreal injections of amphotericin B, vancomycin, ceftazidime, and started on oral fluconazole. Patient was scheduled for vitrectomy to decrease organism burden and to remove loculated areas of infection that would not respond to systemic antifungal agents. Four weeks after initial presentation, the fungal cultures revealed mold growth consistent with aspergillus. Patient was subsequently started on voriconazole and fluconazole was discontinued due to poor efficacy against aspergillus. Further workup was conducted to evaluate for the source of infection and seeding. Transthoracic cardiogram was unremarkable for any vegetation or valvular abnormalities. MRI of the orbits and sinuses did not reveal any mass lesions or bony destruction. CT of the chest was unremarkable for infection. Aspergillus endophthalmitis may occur because of one of these several mechanisms: hematogenous dissemination, direct inoculation by trauma, and contamination during surgery. Our patient's cause of bilateral endophthalmitis was through an unknown iatrogenic seed. PMID:26653687

  12. Idiopathic Bilateral External Jugular Vein Thrombosis

    PubMed Central

    Hindi, Zakaria; Fadel, Ehab

    2015-01-01

    Patient: Male, 21 Final Diagnosis: Idiopathic bilateral external jugular vein thrombosis Symptoms: Face engorgement • neck swelling Medication: — Clinical Procedure: None Specialty: Hematology Objective: Unknown ethiology Background: Vein thrombosis is mainly determined by 3 factors, which constitute a triad called Virchow’s triad: hypercoagulability, stasis, and endothelial injury. Venous thrombosis commonly occurs in the lower extremities since most of the blood resides there and flows against gravity. The veins of the lower extremities are dependent on intact valves and fully functional leg muscles. However, in case of valvular incompetency or muscular weakness, thrombosis and blood stasis will occur as a result. In contrast, the veins of the neck, specially the jugulars, have distensible walls which allow flexibility during respiration. In addition, the blood directly flows downward towards the heart. Nevertheless, many case reports mentioned the thrombosis of internal jugular veins and external jugular veins with identified risk factors. Jugular vein thrombosis has previously been associated in the literature with a variety of medical conditions, including malignancy. Case Report: This report is of a case of idiopathic bilateral external jugular vein thrombosis in a 21 year-old male construction worker of Southeast Asian origin with no previous medical history who presented with bilateral facial puffiness of gradual onset over 1 month. Doppler ultrasound and computed tomography were used in the diagnosis. Further work-up showed no evidence of infection or neoplasia. The patient was eventually discharged on warfarin. The patient was assessed after 6 months and his symptoms had resolved completely. Conclusions: Bilateral idiopathic external jugular veins thrombosis is extremely rare and can be an indicator of early malignancy or hidden infection. While previous reports in the literature have associated jugular vein thrombosis with malignancy, the present case shows that external jugular vein thrombosis can also be found in persons without malignancy. PMID:26301793

  13. A rare case of bilateral aspergillus endophthalmitis.

    PubMed

    Gupta, Saurabh; Loudill, Cameron; Tammara, Anita; Chow, Robert T

    2015-01-01

    Aspergillus endophthalmitis is a devastating inflammatory condition of the intraocular cavities that may result in irreparable loss of vision and rapid destruction of the eye. Almost all cases in the literature have shown an identified source causing aspergillus endophthalmitis as a result of direct extension of disease. We present a rare case of bilateral aspergillus endophthalmitis. A 72-year-old woman with a history of diabetes mellitus, congenital Hirschsprung disease, and recent culture-positive candida pyelonephritis with hydronephrosis status post-surgical stent placement presented with difficulty opening her eyes. She complained of decreased vision (20/200) with pain and redness in both eyes - right worse then left. Examination demonstrated multiple white fungal balls in both retinas consistent with bilateral fungal endophthalmitis. Bilateral vitreous taps for cultures and staining were performed. Patient was given intravitreal injections of amphotericin B, vancomycin, ceftazidime, and started on oral fluconazole. Patient was scheduled for vitrectomy to decrease organism burden and to remove loculated areas of infection that would not respond to systemic antifungal agents. Four weeks after initial presentation, the fungal cultures revealed mold growth consistent with aspergillus. Patient was subsequently started on voriconazole and fluconazole was discontinued due to poor efficacy against aspergillus. Further workup was conducted to evaluate for the source of infection and seeding. Transthoracic cardiogram was unremarkable for any vegetation or valvular abnormalities. MRI of the orbits and sinuses did not reveal any mass lesions or bony destruction. CT of the chest was unremarkable for infection. Aspergillus endophthalmitis may occur because of one of these several mechanisms: hematogenous dissemination, direct inoculation by trauma, and contamination during surgery. Our patient's cause of bilateral endophthalmitis was through an unknown iatrogenic seed. PMID:26653687

  14. Bilateral leg edema in an older woman.

    PubMed

    Thaler, H W; Pienaar, S; Wirnsberger, G; Roller-Wirnsberger, R E

    2015-01-01

    Bilateral leg edema is a frequent symptom in older people and an important concern in geriatric medicine. Further evaluation is frequently not performed and simple therapy with diuretics is prescribed. Particularly in older patients, long-term use of diuretics can lead to severe electrolyte imbalances, volume depletion, and falls. In this case report we want to focus the physicians' attention on the necessity to determine the cause and show a correspondingly effective treatment for bilateral leg edema in older people. A thorough approach is required to recognize diseases and to avoid adverse drug events as geriatric patients often show an atypical presentation or minor symptoms. The cause of swollen legs is often multifactorial; therefore, the patient's individual history and an appropriate physical examination are important. Depending on the clinical symptoms, evaluation including basic laboratory tests, urinalysis, chest radiography, and echocardiogram may be indicated. The most probable cause of bilateral edema in older patients is chronic venous insufficiency. Heart failure is also a common cause. Other systemic causes such as renal disease or liver disease are much rarer. Antihypertensive and anti-inflammatory drugs can frequently cause leg edema, but the incidence of drug-induced leg swelling is unknown. With the help of this special case we tried to develop an approach to the diagnosis of symmetric leg edema in older patients, a problem frequently neglected in geriatric medicine. PMID:24271146

  15. High Performance, Three-Dimensional Bilateral Filtering

    SciTech Connect

    Bethel, E. Wes

    2008-06-05

    Image smoothing is a fundamental operation in computer vision and image processing. This work has two main thrusts: (1) implementation of a bilateral filter suitable for use in smoothing, or denoising, 3D volumetric data; (2) implementation of the 3D bilateral filter in three different parallelization models, along with parallel performance studies on two modern HPC architectures. Our bilateral filter formulation is based upon the work of Tomasi [11], but extended to 3D for use on volumetric data. Our three parallel implementations use POSIX threads, the Message Passing Interface (MPI), and Unified Parallel C (UPC), a Partitioned Global Address Space (PGAS) language. Our parallel performance studies, which were conducted on a Cray XT4 supercomputer and aquad-socket, quad-core Opteron workstation, show our algorithm to have near-perfect scalability up to 120 processors. Parallel algorithms, such as the one we present here, will have an increasingly important role for use in production visual analysis systems as the underlying computational platforms transition from single- to multi-core architectures in the future.

  16. Multilateral, regional and bilateral energy trade governance

    SciTech Connect

    Leal-Arcas, Rafael; Grasso, Costantino; Rios, Juan Alemany )

    2014-12-01

    The current international energy trade governance system is fragmented and multi-layered. Streamlining it for greater legal cohesiveness and international political and economic cooperation would promote global energy security. The current article explores three levels of energy trade governance: multilateral, regional and bilateral. Most energy-rich countries are part of the multilateral trading system, which is institutionalized by the World Trade Organization (WTO). The article analyzes the multilateral energy trade governance system by focusing on the WTO and energy transportation issues. Regionally, the article focuses on five major regional agreements and their energy-related aspects and examines the various causes that explain the proliferation of regional trade agreements, their compatibility with WTO law, and then provides several examples of regional energy trade governance throughout the world. When it comes to bilateral energy trade governance, this article only addresses the European Union’s (EU) bilateral energy trade relations. The article explores ways in which gaps could be filled and overlaps eliminated whilst remaining true to the high-level normative framework, concentrating on those measures that would enhance EU energy security.

  17. Bruck syndrome - a rare syndrome of bone fragility and joint contracture and novel homozygous FKBP10 mutation.

    PubMed

    Moravej, Hossein; Karamifar, Hamdollah; Karamizadeh, Zohreh; Amirhakimi, Gholamhossein; Atashi, Sepideh; Nasirabadi, Shiva

    2015-01-01

    Bruck syndrome is an autosomal recessive syndrome consisting of bone fragility and congenital joint contractures. According to the genotype, it has been classified into types 1 and 2. Recently, mutations in FKBP10, localised to chromosome 17q21, have been identified in some patients of Bruck syndrome. Twenty-seven patients of this syndrome have been reported so far. We present a new patient of this syndrome, with frequent fractures, congenital joint contractures, kyphoscoliosis, bilateral clubfoot, and pectus carinatum. The clinical and genetic features of all previously reported cases are also reviewed. PMID:25931047

  18. Spared and impaired cognitive abilities after bilateral frontal damage.

    PubMed

    Brazzelli, M; Colombo, N; Della Sala, S; Spinnler, H

    1994-03-01

    We report on a patient (PG) with a severe behavioural disorder following massive bilateral prefrontal damage due to herpetic encephalitis. The lesion involves also the mesial and polar regions of the temporal lobes; the frontal dorso-lateral cortices were relatively spared. The patient's inattentiveness and alternating impulsive and apathetic behaviour led to her social alienation. The only self-motivated activities we observed were those initiated to satisfy her enhanced urge to eat and play games. She did not react to surprise stimuli that consistently elicit a reaction of surprise in healthy people. She also presented "adynamic aphasia" associated with anomia and episodic amnesia. A striking feature of the syndrome was the patient's consistent "utilization behaviour". Her motor behaviour was marked by constant akathisia. She performed surprisingly well on intelligence, "frontal", and visuo-spatial tests. This non-demented patient exhibited a clear dissociation between her relatively spared cognitive abilities and grossly impaired behaviour. In the discussion we explore the extent to which a defect of the supervisory control may be held responsible for the inconsistencies in the patient's coping skill performances. PMID:8004988

  19. Unilateral versus bilateral clubfoot: an analysis of severity and correlation.

    PubMed

    Gray, Kelly; Barnes, Elizabeth; Gibbons, Paul; Little, David; Burns, Joshua

    2014-09-01

    This study compares the severity of unilateral and bilateral clubfoot, and the correlation between right and left feet of bilateral cases. Sixty-six unilateral and 75 bilateral clubfoot patients were assessed for severity using the Pirani score at an average age of 12.9 days (SD 9 days). In bilateral cases, the severity of right and left feet was highly correlated (r=0.68). The odds of being very severe were 2.6 (95% confidence interval 1.3-5.1) times higher in bilateral cases (P=0.007). Bilateral and unilateral clubfeet present with differing severity. Right and left feet from bilateral cases are highly correlated. Researchers need to address these issues during study design and analysis. PMID:24869905

  20. [Familial Wolfram syndrome].

    PubMed

    Bessahraoui, M; Paquis, V; Rouzier, C; Bouziane-Nedjadi, K; Naceur, M; Niar, S; Zennaki, A; Boudraa, G; Touhami, M

    2014-11-01

    Wolfram syndrome (WS) is a rare autosomal recessive progressive neurodegenerative disorder, and it is mainly characterized by the presence of diabetes mellitus and optic atrophy. Other symptoms such as diabetes insipidus, deafness, and psychiatric disorders are less frequent. The WFS1 gene, responsible for the disease and encoding for a transmembrane protein called wolframin, was localized in 1998 on chromosome 4p16. In this report, we present a familial observation of Wolfram syndrome (parents and three children). The propositus was a 6-year-old girl with diabetes mellitus and progressive visual loss. Her family history showed a brother with diabetes mellitus, optic atrophy, and deafness since childhood and a sister with diabetes mellitus, optic atrophy, and bilateral hydronephrosis. Thus, association of these familial and personal symptoms is highly suggestive of Wolfram syndrome. The diagnosis was confirmed by molecular analysis (biology), which showed the presence of WFS1 homozygous mutations c.1113G>A (p.Trp371*) in the three siblings and a heterozygote mutation in the parents. Our observation has demonstrated that pediatricians should be aware of the possibility of Wolfram syndrome when diagnosing optic atrophy in diabetic children. PMID:25282462

  1. [Cushing syndrome: Physiopathology, etiology and principles of therapy].

    PubMed

    Chabre, Olivier

    2014-04-01

    The most frequent cause of Cushing's syndrome is iatrogenic, as Cushing's syndrome is the unavoidable consequence of long-term glucocorticoid treatment using more than 7.5 mg prednisone per day. The most frequent cause of endogenous Cushing's syndrome is Cushing's disease (CD), which is an ACTH dependent hypercortisolism linked to a pituitary corticotroph adenoma. This adenoma is often very small, its diagnosis may require bilateral inferior petrosal sinus sampling and the first line treatment of CD is transsphenoidal surgery by an expert neurosurgeon. The second line treatments include drugs that can act either on the pituitary adenoma or on adrenal steroidogenesis, pituitary radiotherapy or bilateral adrenalectomy. Ectopic ACTH dependent Cushing's syndrome is linked either to poorly differentiated endocrine tumors with a very poor prognosis, such as small cell lung cancer, or to well differentiated endocrine tumors, such as bronchial carcinoid tumors, which have a good prognosis when treated by surgery, but may be very difficult to localize. Adrenal Cushing's syndromes, which are independent of pituitary ACTH secretion, include adrenal cortex carcinoma, which requires abdominal surgery with extended adrenalectomy by an expert surgeon, adrenal adenoma which is treated by laparoscopic unilateral adrenalectomy and bilateral macronodular hyperplasia, whose surgical treatment may require unilateral or bilateral adrenalectomy. Treatment of Cushing's syndrome generally leads to spectacular clinical results, which must not hide the fact that the reversibility of some signs is actually incomplete. This underlines the need for a timely multidisciplinary management of the patients by an expert team. PMID:24656295

  2. Dravet Syndrome

    MedlinePLUS

    ... NINDS Dravet Syndrome Information Page Synonym(s): Severe Myoclonic Epilepsy of Infancy (SMEI) Table of Contents (click to ... Dravet Syndrome? Dravet syndrome, also called severe myoclonic epilepsy of infancy (SMEI), is a severe form of ...

  3. Metabolic syndrome

    MedlinePLUS

    Metabolic syndrome is a name for a group of risk factors that occur together and increase the chance ... Metabolic syndrome is becoming very common in the United States. Doctors are not sure whether the syndrome is ...

  4. Down Syndrome

    MedlinePLUS

    ... NICHD Research Information Clinical Trials Resources and Publications Down Syndrome: Condition Information Skip sharing on social media links Share this: Page Content What is Down syndrome? Down syndrome describes a set of cognitive and ...

  5. Piriformis Syndrome

    MedlinePLUS

    ... Awards Enhancing Diversity Find People About NINDS NINDS Piriformis Syndrome Information Page Table of Contents (click to jump ... is being done? Clinical Trials Organizations What is Piriformis Syndrome? Piriformis syndrome is a rare neuromuscular disorder that ...

  6. Fahr’s Syndrome- An Interesting Case Presentation

    PubMed Central

    Asokan, Athulya Geetha; D’souza, Sydney; Jeganathan, Jayakumar; Pai, Shivananda

    2013-01-01

    Fahr’s syndrome refers to a rare syndrome which is characterized by symmetrical and bilateral intracranial calcification. We are presenting a 63 year old male, who complained of progressive dysarthria of 6 months, which was associated with slowness of movements. His neurological examination revealed Parkinsonian features. His CT scan revealed a symmetrical large area of calcification over the basal ganglia, the thalamus and the cerebellum. The secondary causes of the bilateral calcification were ruled out to make the clinical diagnosis of Idiopathic Bilateral Striopallidodentate Calcinosis, which is otherwise called as the Fahr’s syndrome. Deposits of calcium and minerals cannot be linked to a single chromosomal locus and further genetic studies are in progress for identifying the chromosomal locus of the disease. PMID:23634413

  7. Mechanism of Highly Synchronized Bilateral Hippocampal Activity

    PubMed Central

    Wang, Y.; Toprani, S.; Tang, Y.; Vrabec, T.; Durand, D.M.

    2014-01-01

    In vivo studies of epileptiform discharges in the hippocampi of rodents have shown that bilateral seizure activity can sometimes be synchronized with very small delays (< 2 ms). This observed small time delay of epileptiform activity between the left and right CA3 regions is unexpected given the physiological propagation time across the hemispheres (> 6 ms). The goal of this study is to determine the mechanisms of this tight synchronization with in-vitro electrophysiology techniques and computer simulations. The hypothesis of a common source was first eliminated by using an in-vitro preparation containing both hippocampi with a functional ventral hippocampal commissure (VHC) and no other tissue. Next, the hypothesis that a noisy baseline could mask the underlying synchronous activity between the two hemispheres was ruled out by low noise in-vivo recordings and computer simulation of the noisy environment. Then we built a novel bilateral CA3 model to test the hypothesis that the phenomenon of very small left-to-right propagation delay of seizure activity is a product of epileptic cell network dynamics. We found that the commissural tract connectivity could decrease the delay between seizure events recorded from two sides while the activity propagated longitudinally along the CA3 layer thereby yielding delays much smaller than the propagation time between the two sides. The modeling results indicate that both recurrent and feedforward inhibition were required for shortening the bilateral propagation delay and depended critically on the length of the commissural fiber tract as well as the number of cells involved in seizure generation. These combined modeling/experimental studies indicate that it is possible to explain near perfect synchronization between the two hemispheres by taking into account the structure of the hippocampal network. PMID:24262205

  8. Bilateral Wilms' tumors: changing concepts in management

    SciTech Connect

    Laberge, J.M.; Nguyen, L.T.; Homsy, Y.L.; Doody, D.P.

    1987-08-01

    Bilaterality is uncommon in Wilms' tumor, being present in 4% to 8% of the cases. We report the combined experience of two children's hospitals in one city over a 20-year period. We encountered nine cases of synchronous bilateral nephroblastoma (National Wilms' Tumor Study 3, stage V). Age at diagnosis ranged from 9 to 41 months (mean 23 months). There were five girls and four boys. Associated findings include nephroblastomatosis in three cases (33%), one of which also had a familial history; undescended testis in two cases; and minor anomalies in two other cases. Surgical treatment consisted of unilateral nephrectomy with contralateral partial nephrectomy or tumorectomy in six cases, nephrectomy with contralateral biopsy only in two cases, and the other patient had bilateral biopsies initially, followed at a later date by partial nephrectomy on one side. All patients received chemotherapy; actinomycin D (AMD) only was used in the oldest case, vincristine and AMD in five cases, to which was added cyclophosphamide in one case and adriamycin in two. Seven patients received radiation therapy. Seven out of the nine patients survived more than 2 years (77%); five are well, off chemotherapy, with no evidence of disease from 4 to 11 years after diagnosis. Two patients suffered from chronic renal failure and one died from complications after renal transplantation more than 19 years after diagnosis. The two patients who died from their disease presented with more advanced tumor. Therefore, the agressiveness of multimodal therapy can be tailored according to stage and histology, and effective chemotherapy allows maximal preservation of renal parenchyma in patients with stage I and II tumors.

  9. Mechanism of highly synchronized bilateral hippocampal activity.

    PubMed

    Wang, Y; Toprani, S; Tang, Y; Vrabec, T; Durand, D M

    2014-01-01

    In vivo studies of epileptiform discharges in the hippocampi of rodents have shown that bilateral seizure activity can sometimes be synchronized with very small delays (<2 ms). This observed small time delay of epileptiform activity between the left and right CA3 regions is unexpected given the physiological propagation time across the hemispheres (>6 ms). The goal of this study is to determine the mechanisms of this tight synchronization with in-vitro electrophysiology techniques and computer simulations. The hypothesis of a common source was first eliminated by using an in-vitro preparation containing both hippocampi with a functional ventral hippocampal commissure (VHC) and no other tissue. Next, the hypothesis that a noisy baseline could mask the underlying synchronous activity between the two hemispheres was ruled out by low noise in-vivo recordings and computer simulation of the noisy environment. Then we built a novel bilateral CA3 model to test the hypothesis that the phenomenon of very small left-to-right propagation delay of seizure activity is a product of epileptic cell network dynamics. We found that the commissural tract connectivity could decrease the delay between seizure events recorded from two sides while the activity propagated longitudinally along the CA3 layer thereby yielding delays much smaller than the propagation time between the two sides. The modeling results indicate that both recurrent and feedforward inhibition were required for shortening the bilateral propagation delay and depended critically on the length of the commissural fiber tract as well as the number of cells involved in seizure generation. These combined modeling/experimental studies indicate that it is possible to explain near perfect synchronization between the two hemispheres by taking into account the structure of the hippocampal network. PMID:24262205

  10. Primary amenorrhea with bilateral endometriotic cysts.

    PubMed

    Jabeen, Sadaqat; Raees, Mehnaz

    2015-05-01

    Primary amenorrhea is a common problem. Diagnosis is usually by going through systematic approach of history, examination and investigations. This case had bilateral large endometriotic cysts in the adnexal region. Uterus was normal sized with well-formed endometrium. She underwent laparotomy followed by drainage of endometriotic cysts, stripping and reconstruction of ovaries was performed. Patient was given a trial of combined oral contraceptive pills for two consecutive cycles to observe withdrawal bleeding, but it failed. Till now we are unable to find out such case in literature. Exact case of primary amenorrhea could not be found. PMID:26008666

  11. Bilateral temporal bone xanthoma. Case report.

    PubMed

    Muthusamy, Kalai A; Azmi, Khairul; Narayanan, Prepageran; Rajagopalan, Raman; Rahman, Nazarina Abdul; Waran, Vicknes

    2008-02-01

    Xanthoma formation is frequently seen over the subcutaneous tissue of extensor surfaces and tendons that have received minor trauma or friction in patients with hypercholesterolemia. However, temporal bone xanthomas with intracranial extension are uncommon. To the best of the authors' knowledge, this is the second report in the literature in which bilateral extension of a xanthoma is described. Xanthomas of the temporal bone are benign lesions, and complete or even partial removal is effective. The predisposing cause of the lesion should also be treated. PMID:18240935

  12. Bilateral nodular sarcoid choroiditis with vitreous haemorrhage.

    PubMed Central

    Stone, L S; Ehrenberg, M

    1984-01-01

    A 21-year-old black man with presumed systemic sarcoidosis had bilateral choroidal nodules, unilateral retinal neovascularisation and vitreous haemorrhage, and non-caseating granulomas on percutaneous liver biopsy. The choroidal nodules were serially documented by fundus photography and fluorescein angiography over a 22-month period. Fluorescein angiography was more accurate than ophthalmoscopy in demonstrating choroidal inflammation. The choroidal nodules resolved after systemic corticosteroid therapy. A vitreous haemorrhage occurred probably secondary to neovascularisation related to occlusion of an inferotemporal branch vein. The non-resolving vitreous haemorrhage and associated traction retinal detachment were treated with vitrectomy and membrane sectioning. Images PMID:6205681

  13. Arthroscopic treatment of bilateral humeral head osteonecrosis.

    PubMed

    Hardy, P; Decrette, E; Jeanrot, C; Colom, A; Lortat-Jacob, A; Benoit, J

    2000-04-01

    A 37-year-old woman with a renal transplant was treated by arthroscopic debridement for bilateral steroid-induced humeral head osteonecrosis. Radiologically, the right shoulder had been categorized as stage III and the left as stage IV according to Arlet and Ficat. Relief of pain and improved range of motion were obtained especially on the right shoulder. Arthroscopy is an efficient procedure for treatment of humeral head osteonecrosis in the renal transplant recipient including radiological stages III with episodes of locking. PMID:10750016

  14. Progressive bilateral thinning of the parietal bones

    SciTech Connect

    Cederlund, C.G.; Andren, L.; Olivecrona, H.

    1982-03-01

    Observation of a case of progressive bilateral parietal thinning within a period of 14 years induced us to study skull films of 3 636 consecutive patients. Parietal thinning was found in 86 patients (2.37%). It was more common in women, with a sex ratio of 1:1.9. The mean age of the females was 72 years, and that of the males 63 years. Previous skull films of 25 of these patients were available and showed progression in 10. It is concluded that parietal thinning is a slowly progressive disease of middle-aged and old patients and is not an anatomical variant or congenital dysplasia of the dipole.

  15. Topiramate induced bilateral angle-closure glaucoma.

    TOXLINE Toxicology Bibliographic Information

    Singh SK; Thapa SS; Badhu BP

    2007-04-01

    Topiramate is a sulfamate-substituted monosaccharide and is used as an antiepileptic medication. It can also be used as a prophylactic agent for headache from migraine. The main purpose of this article is to create awareness among medical colleagues regarding severe ophthalmic side effect of topiramate. This is the first reported case of topiramate induced bilateral angle-closure glaucoma in Nepal. This case was managed successfully by discontinuing topiramate and by starting anti-glaucoma medication along with atropine eye drops which were tapered and eventually discontinued.

  16. Topiramate induced bilateral angle-closure glaucoma.

    PubMed

    Singh, S K; Thapa, S S; Badhu, B P

    2007-01-01

    Topiramate is a sulfamate-substituted monosaccharide and is used as an antiepileptic medication. It can also be used as a prophylactic agent for headache from migraine. The main purpose of this article is to create awareness among medical colleagues regarding severe ophthalmic side effect of topiramate. This is the first reported case of topiramate induced bilateral angle-closure glaucoma in Nepal. This case was managed successfully by discontinuing topiramate and by starting anti-glaucoma medication along with atropine eye drops which were tapered and eventually discontinued. PMID:18604026

  17. Second-Side Surgery in Superior Canal Dehiscence Syndrome

    PubMed Central

    Agrawal, Yuri; Minor, Lloyd B.; Schubert, Michael C.; Janky, Kristen L.; Davalos-Bichara, Marcela; Carey, John P.

    2014-01-01

    Objective Bilateral superior canal (SC) dehiscence syndrome poses a challenge because bilateral SC dehiscence (SCD) plugging might be expected to result in oscillopsia and disability. Our aims were as follows: 1) to evaluate which symptoms prompted patients with bilateral SCD syndrome (SCDS) to seek second-side surgery, and 2) to determine the prevalence of disabling imbalance and oscillopsia after bilateral SC plugging. Study Design Prospective observational study. Setting Tertiary referral center. Patients Five patients with bilateral SCDS based on history, audiometric and physiologic testing, and computed tomographic findings. This includes all of our patients who have had second-side plugging surgery to date. Intervention(s) Bilateral sequential middle fossa craniotomy and plugging of SCs. Main Outcome Measure(s) Cochleovestibular symptoms, cervical and ocular vestibular-evoked myogenic potential testing, dizziness handicap inventory, short-form 36 Health Survey, dynamic visual acuity testing. Results The most common symptoms prompting second-side surgery were sound- and pressure-induced vertigo and autophony. Three of the 5 patients reported that symptoms shifted to the contralateral ear immediately after plugging the first side, whereas in 2 patients, contralateral symptoms developed several years after the first SC plugging. Two of 4 patients experienced ongoing oscillopsia after bilateral SCDS surgery; however, all patients reported relief from their SCD symptoms and were glad that they had pursued bilateral surgery. Conclusion In patients with bilateral SCDS, sound- and pressure-induced vertigo most commonly prompted second-side surgery. Despite some degree of oscillopsia after bilateral SCDS surgery, patients were very satisfied with second-side surgery, given their relief from other SCDS symptoms. PMID:22158019

  18. Generalization of Visuomotor Learning Between Bilateral and Unilateral Conditions

    PubMed Central

    Sainburg, Robert L.

    2009-01-01

    A long history of behavioral and physiological research has suggested that bilateral coordination invokes unique neural processes that are not involved in unilateral movements. This hypothesis predicts that motor learning should show limited transfer between unilateral and bilateral conditions, which is consistent with a recent finding that indicated partial, but not complete, transfer of learning between the two conditions. However, during learning of new motor skills, transformations must also be made between visual and proprioceptive coordinate systems, a process that may occur upstream to the processes that differentiate bilateral from unilateral movements. We now investigate whether visuomotor adaptations are shared between unilateral and bilateral movement conditions. Our results indicate substantial transfer from bilateral to subsequent unilateral conditions for both arms. Interestingly, whereas the nondominant arm never showed complete adaptation to visual rotation under bilateral conditions, this interference, or lack of improvement, in bilateral performance did not disturb the visuomotor adaptation process or transfer, as reflected by superb unilateral performances immediately following the bilateral conditions. These findings unambiguously indicate that visuomotor adaptation can extensively generalize between bilateral and unilateral conditions, thus suggesting a substantial overlap in the neural processes underlying visuomotor transformations between the two movement conditions. Our findings provide support for a two-stage model of motor planning, in which the visuomotor transformation process precedes the processes that convert the visuomotor plan into effector-specific commands that incorporate bilateral synergies and that result in the forces that determine motion. PMID:19759325

  19. Interictal slow-wave focus in left medial temporal lobe during bilateral electroconvulsive therapy.

    PubMed

    Neuhaus, Andres H; Gallinat, Juergen; Bajbouj, Malek; Reischies, Friedel M

    2005-01-01

    The interictal state between two electroconvulsive therapy (ECT) sessions is clinically characterised by possible cognitive adverse effects like mild amnestic syndrome. ECT-induced amnestic deficits can persist for several weeks after ECT. Electrophysiologically, slowing of brain electrical activity in the interictal state has often been reported. Especially, for bilateral ECT a correlation between enhanced left frontotemporal theta activity and retrograde amnesia has been demonstrated. This study focuses on the topographic distribution of cortical slow-wave oscillations during the interictal state of a bilateral ECT cycle. Twelve patients with major depression have been investigated with 32-channel resting EEG 24 h after the 6th ECT session. As controls, 8 major depressive patients were investigated prior to antidepressive treatment. The generating sources of slow-wave activity are estimated within the theta frequency band with low-resolution brain electromagnetic tomography. Source analysis revealed a distinct pattern of theta activity in the depth of the left temporal lobe (fusiform and parahippocampal gyri, Brodmann areas 37 and 36, respectively; p< 0.05) during the interictal state. This finding suggests a dysfunction of the left medial temporal lobe memory system during the interictal state of a bilateral ECT cycle. It will further be discussed whether it is possible to obtain information about activity of deep brain structures like the hippocampal formation from scalp-recorded signals. PMID:16220024

  20. Bilateral adrenal hemorrhage: The unrecognized cause of hemodynamic collapse associated with heparin-induced thrombocytopenia

    PubMed Central

    Rosenberger, Laura H.; Smith, Philip W.; Sawyer, Robert G.; Hanks, John B.; Adams, Reid B.; Hedrick, Traci L.

    2011-01-01

    Objective Heparin-induced thrombocytopenia is a common adverse effect of treatment with heparin resulting in paradoxical thromboses. An immunoglobulin G class “heparin-induced thrombocytopenia antibody” attaches to a heparin—platelet factor 4 protein complex. The antibody then binds to the Fc?IIa receptor on the surface of a platelet, resulting in activation, consumption, and thrombocytopenia in the clinical syndrome of heparin-induced thrombocytopenia. In contradistinction to other drug-induced thrombocytopenias that lead to a risk of hemorrhage, the state of thrombocytopenia in heparin-induced thrombocytopenia leads to an acquired hypercoagulability syndrome. Bilateral adrenal hemorrhage associated with heparin-induced thrombocytopenia has become an increasingly documented association. The adrenal gland has a vascular construction that lends itself to venous thrombus in the setting of heparin-induced thrombocytopenia and subsequent arterial hemorrhage. A literature search revealed 17 reported cases of bilateral adrenal hemorrhage in the setting of heparin-induced thrombocytopenia uniformly presenting with complete hemodynamic collapse. Data Sources An Ovid MEDLINE search of the English-language medical literature was conducted, identifying articles describing cases of bilateral adrenal hemorrhage in the setting of heparin-induced thrombocytopenia. Study Selection All cases with this association were included in the review. Data Extraction and Data Synthesis A total of 14 articles were identified, describing 17 individual case reports of bilateral adrenal hemorrhage associated with heparin-induced thrombocytopenia. All cases confirmed known characteristics of heparin-induced thrombocytopenia and uniformly revealed hypotension due to adrenal insufficiency. There were five deaths, resulting in an overall mortality rate of 27.8%, and 100% mortality in the three cases where adrenal insufficiency went unrecognized. Conclusions The secondary complication of adrenal vein thrombosis leading to bilateral adrenal hemorrhage remains insufficiently recognized and undertreated. The nonspecific presentation of adrenal hemorrhage and insufficiency as a complication of heparin-induced thrombocytopenia, coupled with the catastrophic clinical course of untreated adrenal collapse, requires a high index of suspicion to achieve rapid diagnosis and provide life-saving therapy. PMID:21242799

  1. Bilateral clavicle osteomyelitis: A case report

    PubMed Central

    Saglam, Fevzi; Saglam, Seymanur; Gulabi, Deniz; Eceviz, Engin; Elmali, Nurzat; Yilmaz, Mustafa

    2014-01-01

    INTRODUCTION Osteomyelitis of the clavicle is rare. Infection occurs from hematogenous spread or trauma. In adults infection is usually secondary due to an exogenous cause such as open fractures, surgery (iatrogenic) or spread from local tissue with infection. PRESENTATION OF CASE The case is presented here of a 50-year old female with bilateral clavicular fractures, who was operated on with open reduction and internal fixation. At the 6-month follow-up, she had complaints of bilateral osteomyelitis which was successfully treated with resection of the infected segment of the bone, and antibiotic impregnated collagen. DISCUSSION Predisposing factors include diabetes, intravenous drug abuse, tuberculosis or immune suppression. Management involves the removal of bone fixation, debridement of the bone and if there is a defect, coverage with a muscle flap is applied. CONCLUSION In cases of clavicular osteomyelitis where infection continues despite debridement and antibiotic therapy, excision of the sequestered clavicular section is a successful treatment approach and has been seen to improve quality of life without any functional loss. PMID:25460439

  2. Hiatus Hernia Repair with Bilateral Oesophageal Fixation

    PubMed Central

    Martin, David

    2015-01-01

    Background. Despite advances in surgical repair of hiatus hernias, there remains a high radiological recurrence rate. We performed a novel technique incorporating bilateral oesophageal fixation and evaluated outcomes, principally symptom improvement and hernia recurrence. Methods. A retrospective study was performed on a prospective database of patients undergoing hiatus hernia repair with bilateral oesophageal fixation. Retrospective and prospective quality of life (QOL), PPI usage, and patient satisfaction data were obtained. Hernia recurrence was assessed by either barium swallow or gastroscopy. Results. 87 patients were identified in the database with a minimum of 3 months followup. There were significant improvements in QOL scores including GERD HRQL (29.13 to 4.38, P < 0.01), Visick (3 to 1), and RSI (17.45 to 5, P < 0.01). PPI usage decreased from a median of daily to none, and there was high patient satisfaction (94%). 57 patients were assessed for recurrence with either gastroscopy or barium swallow, and one patient had evidence of recurrence on barium swallow at 45 months postoperatively. There was an 8% complication rate and no mortality or oesophageal perforation. Conclusions. This study demonstrates that our technique is both safe and effective in symptom control, and our recurrence investigations demonstrate at least short term durability. PMID:26065030

  3. Bilateral Comparison in Chemosensory-Mediated Foraging

    NASA Astrophysics Data System (ADS)

    Webster, D. R.; Rahman, S.; Dasi, L. P.

    2000-11-01

    Motivation is drawn from the need to determine the sensory cues that animals such as blue crabs and lobsters use to track chemical odor plumes to locate food or mates. Major steps forward with this difficult problem can only be achieved through an appreciation of the spatial and temporal variation of concentration fields and the information content available to a forager in the plume. Here we discuss the usefulness of bilateral comparison to an animal tracking a turbulent plume. Instantaneous concentration fields of a chemical plume diffusing in a fully-developed turbulent open channel flow are measured using planar laser-induced fluorescence (PLIF). The plume is released iso-kinetically 25 mm above the smooth bed (z+ = 90), thus transport is mainly due to advection and ambient turbulence. A spatial correlation function in the spanwise direction is a dramatic indicator of the relative position of the centerline and distance from the source. The relative direction of the plume centerline can be estimated from an instantaneous bilateral comparison provided the sensors are separated by a distance that is relatively large compared to the spanwise integral length scale based on the spatial correlation function.

  4. Bihemispheric processing of redundant bilateral lexical information.

    PubMed

    Hasbrooke, R E; Chiarello, C

    1998-01-01

    Cerebral asymmetries in lexical ambiguity resolution were studied. In 2 experiments, targets related to the dominant and subordinate meanings of ambiguous word primes were presented for lexical decision after a 750-ms stimulus onset asynchrony. Experiment 1 compared presentation of target words to the left visual field/right-hemisphere (LVF/RH), to the right visual field/left-hemisphere (RVF/LH), or after redundant bilateral visual field (BVF) presentation. Experiment 2 examined unilateral priming in the absence of a BVF condition. On unilateral trials, priming was observed for dominant meanings in both the LVF/RH and RVF/LH, whereas subordinate priming was obtained only in the RVF/LH. These results suggest a possible role of hemispheric interaction in the availability of ambiguous word meanings. BVF performance evidenced a bilateral redundancy gain and priming that resembled that obtained on RVF/LH trials. Additional BVF analyses were not consistent with a strict race model interpretation and appear to implicate hemispheric cooperation in the bihemisperic processing of lexical information. PMID:9460737

  5. Noninvasive testing of asymptomatic bilateral hilar adenopathy

    SciTech Connect

    Carr, P.L.; Singer, D.E.; Goldenheim, P.; Bernardo, J.; Mulley, A.G. )

    1990-03-01

    The diagnostic strategy for asymptomatic patients with persistent bilateral bilar adenopathy often involves invasive procedures. The authors used Bayesian analysis to: (1) estimate the relative prevalences of diseases causing bilateral bilar adenopathy; (2) assess changes in the prevalence of disease by race, the presence of other clinical symptoms, and geography; and (3) determine the value of relevant noninvasive tests, including the angiotensin-converting enzyme (ACE) assay, gallium scan, and purified protein derivative (PPD), in order to assess when a strategy of watchful waiting is appropriate. The analysis indicated that the ACE assay, particularly when paired with the PPD, can identify many patients who might safely be managed without immediate invasive biopsy. Patients who are ACE+ and PPD- have an estimated probability of sarcoidosis of 0.95 or greater; patients who are ACE- and PPD+ have a probability of tuberculosis of 0.86 if black, 0.79 if white. In contrast, gallium scanning has no diagnostic role in this clinical situation. Bronchoscopic or mediastinoscopic biopsy has a limited role for patients who are ACE+ PPD- or ACE- PPD+ because of limited sensitivity. Patients who are both ACE- and PPD-, particularly if white, may have a high enough risk of lymphoma to consider invasive biopsy.

  6. Influence of unilateral weight on bilateral cyclograms

    NASA Astrophysics Data System (ADS)

    Pellicer Costa, Juan José; Dusza, Jacek J.

    2014-11-01

    The paper presents the results of gait parameters as a function of unilateral weight. The object of the research was a woman walking on a stationary surface and carrying in his hand weights from 0 to 15 kg. Her movement was recorded by 6 cameras recording the location of 34 markers placed at appropriate points in the body. 3D reconstruction was performed for each of the reflecting markers. Tested signals were changes in the value the joint angles of ankle, knee and hip. On the basis of about 6 cycles of movement of each load, a model for the average gait cycle was developed. The result of the experiments are graphs of changes the joint angles as a function of time, bilateral cyclograms, synchronized bilateral cyclograms and regression lines. The conclusion of the study is to determine how one-sided load affects gait asymmetry. Simple and easy to interpret method of presentation of results were also shown. Studies were conducted using VICON system.

  7. Anaesthetic consideration during laparoscopic bilateral simultaneous nephrectomy

    PubMed Central

    Modi, Manisha P; Vora, Kalpana S; Parikh, Geeta P; Modi, Pranjal R; Shah, Veena R

    2014-01-01

    STUDY OBJECTIVE: To assess outcome from anaesthesia during laparoscopic bilateral simultaneous nephrectomy. DESIGN: Retrospective study. MEASUREMENTS: Preoperative Hb%, serum potassium, coagulation profile electrocardiography (ECG) changes, 2D Echography, x-ray chest, haemodynamic changes, end-tidal carbon dioxide (EtCO2), fluid management and postoperative analgesia. RESULTS: The mean age was 24.75 ± 14.35 years. The mean duration of surgery was 120 ± 80 minutes. The Hb%, serum creatinine and serum potassium were 9.4 ± 1.04%, 6.79 ± 4.91 meq/L and 3.61 ± 0.51 meq/L, respectively. Pulse rate mean blood pressure and EtCO2 were recorded after creation of pneumoperitoneum and at 15, 30, 45 and after exsufflation of pneumoperitoneum. After pneumoperitoneum, there was increase in pulse rate, systolic blood pressure, diastolic blood pressure and EtCO2. After 30 minutes and throughout the surgery, these variables remained stable. Four patients required nitroglycerine infusion for intraoperative hypertention. Only one patient required packed cell volume (PCV) transfusion and total intravenous fluid was 1 ± 0.5 L. At the time of exsufflation, there was decrease in pulse rate, systolic and diastolic blood pressure and EtCO2. CONCLUSION: Because of advancement in anaesthetic agents and muscle relaxant, there is safe outcome from anaesthesia during laparoscopic bilateral simultaneous nephrectomy. PMID:24501502

  8. Upper frequency limits of bilateral coordination patterns.

    PubMed

    Morrison, S; Hong, S L; Newell, K M

    2009-05-01

    This study examined what changes occur in upper-limb bilateral coordination during clapping as the movement frequency requirements were increased to the maximum. Subjects were required to begin the clapping action at approximately 1Hz and gradually increase the movement speed until their maximal frequency was achieved. Hand and finger displacement and surface electromyograms (EMG) from finger flexor/extensor muscles were recorded. The results showed that the maximal attainable movement frequency was between 7 and 8Hz. As the action approached the ceiling frequency (>5Hz), there was a significant reduction in movement amplitude of the non-preferred limb accompanied by increased co-activation of the muscles within this limb. The movement amplitude of preferred limb was maintained. Subsequently, there was a decrease in coupling between the two limbs with the bilateral coordination pattern transitioning from an in-phase pattern to an asymmetric mode, where only the preferred limb was moving. These findings reveal that there is a frequency induced transition to single-limb motion that reflects a stability boundary at the upper frequency limits of bimanual coordination. PMID:19429090

  9. Atypical Bilateral Fuchs Uveitis: Diagnostic Challenges

    PubMed Central

    Couto, Cristóbal; Hurtado, Erika; Faingold, Dana; Demetrio, Carmen; Schlaen, Ariel; Zas, Marcelo; Zarate, Jorge; Rosetti, Silvia; de Lima, Andrea Paes; Croxatto, Juan Oscar; Chiaradía, Pablo; Burnier, Miguel N.

    2015-01-01

    Bilateral Fuchs uveitis associated with vitreous infiltration and posterior segment involvement requires a thorough diagnostic evaluation. The lack of well-defined diagnostic criteria makes identification of this entity difficult. The aim of this case report was to present the characteristics of a patient with atypical Fuchs uveitis and the procedures needed to rule out the differential diagnosis with specific attention to the utility of in vivo confocal microscopy (IVCM). Case Report One case of chronic bilateral uveitis with severe vitreous opacities is presented. After extensive systemic workup, including vitrectomy, the case had no identifiable systemic etiology. IVCM of the cornea revealed the presence of dendritiform keratic precipitates. Conclusion The diagnosis of Fuchs uveitis is based on clinical findings as no confirmatory laboratory tests are available. A high index of suspicion is key to an early diagnosis, especially in the cases with vitreous opacities and posterior segment manifestations. Auxiliary tests such as IVCM may aid the clinician in the diagnosis of Fuchs uveitis. PMID:26483668

  10. Improvement of sudden bilateral hearing loss after vertebral artery stenting.

    PubMed

    Kim, Ji Hwa; Roh, Kyung Jin; Suh, Sang Hyun; Lee, Kyung-Yul

    2016-03-01

    Bilateral deafness is a rare but possible symptom of vertebrobasilar ischemia. We report a case of sudden bilateral sensorineural hearing loss caused by bilateral vertebral artery (VA) occlusion which dramatically improved after stenting. A 54-year-old man was admitted with sudden onset of bilateral deafness, vertigo, and drowsy mental status. Brain diffusion-weighted MRI showed acute infarction involving both the posterior inferior cerebellar artery and left posterior cerebral artery territory. Cerebral angiography showed bilateral distal VA occlusion, and emergency intracranial stenting was performed in the left VA. After reperfusion therapy his symptoms gradually improved, including hearing impairment. Endovascular stenting may be helpful in a patient with sudden deafness caused by bilateral VA occlusion. PMID:25712982

  11. Multispectral image denoising with optimized vector bilateral filter.

    PubMed

    Peng, Honghong; Rao, Raghuveer; Dianat, Sohail A

    2014-01-01

    Vector bilateral filtering has been shown to provide good tradeoff between noise removal and edge degradation when applied to multispectral/hyperspectral image denoising. It has also been demonstrated to provide dynamic range enhancement of bands that have impaired signal to noise ratios (SNRs). Typical vector bilateral filtering described in the literature does not use parameters satisfying optimality criteria. We introduce an approach for selection of the parameters of a vector bilateral filter through an optimization procedure rather than by ad hoc means. The approach is based on posing the filtering problem as one of nonlinear estimation and minimization of the Stein's unbiased risk estimate of this nonlinear estimator. Along the way, we provide a plausibility argument through an analytical example as to why vector bilateral filtering outperforms bandwise 2D bilateral filtering in enhancing SNR. Experimental results show that the optimized vector bilateral filter provides improved denoising performance on multispectral images when compared with several other approaches. PMID:24184727

  12. Acquired personality disturbances associated with bilateral damage to the ventromedial prefrontal region.

    PubMed

    Barrash, J; Tranel, D; Anderson, S W

    2000-01-01

    Personality changes in 7 participants with bilateral ventromedial prefrontal lesions (PF-BVM), 14 participants with prefrontal lesions but not bilateral ventromedial involvement (PF-NBVM), and 36 with nonprefrontal lesions (NPF) were investigated with the Iowa Rating Scales of Personality Change. Informants rated 30 specific characteristics for degree of disturbance and change from premorbid personality. PF-BVM participants showed a higher rate of acquired disturbances than NPF participants in blunted emotional experience, apathy, low emotional expressiveness, inappropriate affect, poor frustration tolerance, irritability, lability, indecisiveness, poor judgment, social inappropriateness, lack of planning, lack of initiation and persistence, and lack of insight. Differences between the PF-BVM and PF-NBVM groups were significant for several of these characteristics. All 7 PF-BVM participants developed a syndrome, including general dampening of emotional experience; poorly modulated emotional reactions; defective decision making, especially in the social realm; impaired goal-directed behavior; and striking lack of insight. Similarities between this syndrome of "acquired sociopathy" and developmental psychopathy in characteristic personality disturbances and psychophysiological abnormalities suggest that diminished emotionality, impaired decision making, and psychosocial dysfunction may be related to ventromedial prefrontal dysfunction in both groups. PMID:11385830

  13. Successful Reconstruction of Asymptomatic Bilateral External Carotid Artery Aneurysms.

    PubMed

    Loja, Melissa N; Pevec, William C

    2016-04-01

    True aneurysms of the external carotid artery (ECA) are extremely rare with an unknown incidence and natural history. We present the successful operative management of an asymptomatic 65-year-old man found to have bilateral internal carotid artery stenosis and bilateral ECA aneurysms. His bilateral carotid arteries were reconstructed with bifurcated interposition grafts in a staged fashion. The patient recovered without sequelae and continues to be asymptomatic 1 year after reconstruction. We present the operative management of this rare case. PMID:26802292

  14. Bilateral chylothorax: an unusual complication of cervical rib resection

    PubMed Central

    Aslam, Imran; Johnson, Brian; Greenstone, Michael A.; Kastelik, Jack A.

    2015-01-01

    Bilateral chylothorax is a rare cause of pleural effusions. Here we report an unusual acute presentation of bilateral chylothorax following thoracic outlet surgery. Unique to this case was the disparate characteristics of pleural fluid analyses with an exudate on the left and a transudate on the right. This report describes the recognition and management of bilateral chylothoraces, an uncommon but potentially serious complication of this frequently performed surgical procedure. PMID:25553240

  15. Unusual anatomic variation of bilateral ectopic ureters in a dog.

    PubMed

    Hosgood, G; Salisbury, K; Blevins, W E; Widmer, W R

    1989-12-01

    An unusual anatomic variation of bilateral ectopic ureters was diagnosed in a 6-week-old female Siberian Husky with urinary incontinence. Assessment during surgery revealed bilateral ectopic ureters with a common opening in the proximal portion of the urethra. Ureteroneocystostomy was performed bilaterally. After surgery, intermittent urinary incontinence continued, but was less severe. After resection of a persistent hymen in the 6-month-old dog, incontinence appeared to resolve, but then recurred, and has been controlled by phenylpropanolamine administration. PMID:2599944

  16. Primary bilateral malignant melanoma of the lower limbs

    PubMed Central

    Lembo, Serena; Megna, Matteo; Maddalena, Paolo; Monfrecola, Ambra; Balato, Anna; Baldo, Antonello

    2011-01-01

    Malignant melanoma is the eighth most common cancer in European women. Its incidence is increasing rapidly and it has been demonstrated that this is related to sun exposure. Although few cases of bilateral uveal or choroidal melanoma are described in literature, there are no cases reporting bilateral cutaneous malignant melanoma. We report a singular case of a 43-year-old woman with two primary bilateral melanomas localized on the flexural surface of both legs. PMID:25386272

  17. Spermatozoa protein alterations in infertile men with bilateral varicocele.

    PubMed

    Agarwal, Ashok; Sharma, Rakesh; Durairajanayagam, Damayanthi; Cui, Zhihong; Ayaz, Ahmet; Gupta, Sajal; Willard, Belinda; Gopalan, Banu; Sabanegh, Edmund

    2016-01-01

    Among infertile men, a diagnosis of unilateral varicocele is made in 90% of varicocele cases and bilateral in the remaining varicocele cases. However, there are reports of under-diagnosis of bilateral varicocele among infertile men and that its prevalence is greater than 10%. In this prospective study, we aimed to examine the differentially expressed proteins (DEP) extracted from spermatozoa cells of patients with bilateral varicocele and fertile donors. Subjects consisted of 17 men diagnosed with bilateral varicocele and 10 proven fertile men as healthy controls. Using the LTQ-orbitrap elite hybrid mass spectrometry system, proteomic analysis was done on pooled samples from 3 patients with bilateral varicocele and 5 fertile men. From these samples, 73 DEP were identified of which 58 proteins were differentially expressed, with 7 proteins unique to the bilateral varicocele group and 8 proteins to the fertile control group. Majority of the DEPs were observed to be associated with metabolic processes, stress responses, oxidoreductase activity, enzyme regulation, and immune system processes. Seven DEP were involved in sperm function such as capacitation, motility, and sperm-zona binding. Proteins TEKT3 and TCP11 were validated by Western blot analysis and may serve as potential biomarkers for bilateral varicocele. In this study, we have demonstrated for the first time the presence of DEP and identified proteins with distinct reproductive functions which are altered in infertile men with bilateral varicocele. Functional proteomic profiling provides insight into the mechanistic implications of bilateral varicocele-associated male infertility. PMID:25999357

  18. Spermatozoa protein alterations in infertile men with bilateral varicocele

    PubMed Central

    Agarwal, Ashok; Sharma, Rakesh; Durairajanayagam, Damayanthi; Cui, Zhihong; Ayaz, Ahmet; Gupta, Sajal; Willard, Belinda; Gopalan, Banu; Sabanegh, Edmund

    2016-01-01

    Among infertile men, a diagnosis of unilateral varicocele is made in 90% of varicocele cases and bilateral in the remaining varicocele cases. However, there are reports of under-diagnosis of bilateral varicocele among infertile men and that its prevalence is greater than 10%. In this prospective study, we aimed to examine the differentially expressed proteins (DEP) extracted from spermatozoa cells of patients with bilateral varicocele and fertile donors. Subjects consisted of 17 men diagnosed with bilateral varicocele and 10 proven fertile men as healthy controls. Using the LTQ-orbitrap elite hybrid mass spectrometry system, proteomic analysis was done on pooled samples from 3 patients with bilateral varicocele and 5 fertile men. From these samples, 73 DEP were identified of which 58 proteins were differentially expressed, with 7 proteins unique to the bilateral varicocele group and 8 proteins to the fertile control group. Majority of the DEPs were observed to be associated with metabolic processes, stress responses, oxidoreductase activity, enzyme regulation, and immune system processes. Seven DEP were involved in sperm function such as capacitation, motility, and sperm-zona binding. Proteins TEKT3 and TCP11 were validated by Western blot analysis and may serve as potential biomarkers for bilateral varicocele. In this study, we have demonstrated for the first time the presence of DEP and identified proteins with distinct reproductive functions which are altered in infertile men with bilateral varicocele. Functional proteomic profiling provides insight into the mechanistic implications of bilateral varicocele-associated male infertility. PMID:25999357

  19. Bilateral inverted and impacted maxillary third molars: A case report

    PubMed Central

    Barakat, Ali; Al-Turkmani, Tareq; Al-Yousef, Abdulaziz

    2015-01-01

    Bilateral inverted third molar impaction is an extremely rare condition. We reported the case of a 50-year-old female patient with bilateral inverted and impacted maxillary third molars. Both were asymptomatic and pathology free clinically and radiographically. Surgical extraction of these inverted third molars with inaccessible positions requires an aggressive bone removal on the tuberosity bilaterally. Moreover, it contains a high risk of displacement of the inverted third molar into the maxillary sinus. Conservative management was the choice, with the patient’s agreement, and the inverted third molars were left in situ. Key words:Bilateral inverted, maxillary third molar, upper impacted tooth. PMID:26330945

  20. Weighted bilateral filtering using relative difference between pixels

    NASA Astrophysics Data System (ADS)

    Ma, Li; Zhang, Yu-qi; Hu, Bin-bin; Zheng, Ming-yang

    2015-07-01

    The similarity measurement of the bilateral filtering can't indicate the difference between pixels accurately in the dense texture region. It causes the smoothing effect seriously, thereby reducing the edge preserving properties of the bilateral filtering. This paper presents a new weighted function of the bilateral filtering. It involves an additional range kernel using the relative difference between pixels. The range kernel operates differently by acting on the pixel gray intensities or colors. And it uses the reciprocal kernel for the approximation of the standard Gaussian weight value. Experimental results suggest that it significantly preserves more details than the classical bilateral filtering in edge or dense texture regions.

  1. Catel-Manzke palatodigital syndrome in a second trimester female foetus with nuchal oedema, costovertebral anomalies and radial ray defect.

    PubMed

    Petit, P; Moerman, P; Legius, E; Fryns, J P

    1994-01-01

    We present a female fetus with combination of Pierre Robin anomaly and nuchal oedema, bilateral radial defects, multiple hand malformations including bilateral hyperphalangy, brachymesophalangy, costovertebral abnormalities, and complex cardiac malformation. The present findings constitute a true MCA syndrome with uncertain pattern of inheritance. PMID:7888142

  2. [Proteus syndrome].

    PubMed

    Benichou, J J; Labrune, B; Formanek, A; Denoix, C; Oger, P

    1990-01-01

    Two new cases of Proteus syndrome are reported. This congenital syndrome, first described in 1983, comprises gigantism of extremities, body hemihypertrophy, pigmented nevi and multiple tumors (subcutaneous, lipomas, hamartomas). This syndrome belongs to the same group as Recklinghausen disease, Maffucci or Klippel-Trenaunay syndromes. The prognosis is not well known but mostly depends on functional and psychologic consequences of important deformations. PMID:2206106

  3. Interocular symmetry analysis of bilateral eyes.

    PubMed

    Li, Yi; Bao, Fang Jun

    2014-05-01

    The purpose of this study was to evaluate the interocular symmetry of several biometric parameters between both eyes. The symmetry between the right and left eye of 397 subjects in 14 biometric parameters, spherical equivalent of refractive error (SE), Jackson crossed cylinder power of refractive error astigmatism with axes at 90° and 180° (RJ0) and at 45° and 135° (RJ45), best-corrected visual acuity (BCVA), average corneal curvature (CC), Jackson crossed cylinder power of corneal astigmatism (CJ0 and CJ45), corneal asphericity coefficient (Q), intraocular pressure (IOP), central corneal thickness (CCT), axial length (AL), anterior chamber depth (ACD), lens thickness (LT) and vitreous chamber depth (VCD), was assessed by comparative data analysis. Aside from RJ0 (p?=?0.00), RJ45 (p?=?0.02) and Q (p?=?0.00), the overall interocular differences of other biometric parameters between fellow eyes were not significant (p?>?0.05). The interocular correlation and Bland-Altman plots showed a good agreement between fellow eyes in 14 biometric parameters. Correlations between interocular differences in SE and that in RJ0 (p?=?0.03), CC (p?=?0.00), AL (p?=?0.00) and VCD (p?=?0.00) were statistically significant. There were similar strong linear relationships between refractive error astigmatism vectors and corneal astigmatism vectors in bilateral eyes. There were negative correlations of RJ45 and CJ45 between bilateral eyes. A potentially clinically important interocular symmetry in SE, BCVA, CC, CJ0, CJ45, IOP, CCT, AL, ACD, LT and VCD is found in this research, while the differences of RJ0, RJ45 and Q between left and right eyes seem a bit large. The negative interocular relationships of RJ45 and CJ45 demonstrate moderate mirror symmetry exists among fellow eyes. High interocular symmetry in bilateral eyes may be helpful in intraocular lens power calculation, intraocular pressure evaluation, post-operative visual acuity and refraction prediction at the time the fellow eye is undergoing refractive surgery. PMID:24734876

  4. Bilateral foveal retinoschisis accompanying unilateral peripheral retinoschisis

    PubMed Central

    Kocak, Nilufer; Ozturk, Taylan A; Kaynak, Suleyman

    2014-01-01

    X-linked juvenile retinoschisis is a rare hereditary retinal disease characterized by a tangential splitting of the neurosensory retina which may cause early-onset visual impairment. Existence of the retinal neurosensory layer splitting on cross-sectional images of optical coherance tomography (OCT) and the absence of leakage on fluorescein angiography (FA) help confirming the diagnosis. Such diagnostic tests are also helpful in determining the management of the disease. However, most of the retinoschisis cavities remain stable and rarely extend to the posterior pole, many authors suggest laser prophylaxis to avoid the potential risk of retinal detachment due to holes in the outer retinal layer. Herein, we report a case with bilateral foveal retinoschisis accompanying unilateral peripheral retinoschisis who was evaluated with detailed ophthalmologic examination. Visual acuity, fundoscopy, OCT, and FA remained stable in the second year of follow-up after prophylactic argon laser treatment. PMID:23571248

  5. Patellofemoral arthroplasty in a bilateral Syme's amputee.

    PubMed

    Kanna, Raj; Barrett, David S

    2016-01-01

    We present the case of a 50-year-old woman, who is a bilateral Syme's amputee and subsequently underwent patellofemoral arthroplasty (PFA) for osteoarthritis primarily involving the patellofemoral (PF) joint. History and physical examination were suggestive of severe PF arthritis without patellar instability of the right knee. The diagnosis was confirmed by roentgenogram and a PFA was performed. A slightly modified rehabilitation program was implemented and the patient was followed until 6 years after surgery. In comparison to the preoperative scores, the Oxford, the Western Ontario and McMaster Universities (WOMAC), the Knee Society, and the Hospital for Special Surgery Knee Scores improved significantly (p < 0.05) at the time of final follow-up. PF problems specific to below-knee amputees, factors to be considered before performing PFA, and the drawbacks of other treatment modalities in these patients have been discussed. PMID:26908975

  6. [Unilateral to bilateral pleurisy: Pleural tuberculosis?].

    PubMed

    Ben Ameur, S; Smaoui, S; Kamoun, F; Chabchoub, I; Kamoun, T; Messaadi, F; Aloulou, H; Hachicha, M

    2016-04-01

    Pleural tuberculosis is the first or second most common form of extrapulmonary tuberculosis as well as the main cause of pleural effusion in many countries. It is rare in young infants and is more common in children over 10 years of age. We report the case of a 19-month-old girl admitted for prolonged fever with unilateral pleural effusion. The mother reported a history of lymph node tuberculosis 6 years previously. Intravenous antibiotics with cefotaxime and vancomycin were started. Thoracocentesis yielded a serosanguinous exudate fluid with a lymphocyte predominance. The tuberculin skin test and PCR GeneXpert(©) on pleural fluid were negative. The initial outcome was favorable, but the chest X-rays 10 days after discharge showed bilateral pleural effusion. Pleural biopsy was proposed but the culture of pleural fluid was positive for Mycobacterium tuberculosis. The child was put under standard treatment for tuberculosis. The outcome was favorable. PMID:26922570

  7. An Unusual Case of Bilateral Granulomatous Mastitis

    PubMed Central

    Pistolese, C. A.; Di Trapano, R.; Girardi, V.; Costanzo, E.; Di Poce, I.; Simonetti, G.

    2013-01-01

    Idiopathic granulomatous mastitis (IGM) is an uncommon benign disorder of the breast. At clinical examination, IGM is characterized by an inflammatory process of the breast, usually unilateral. Possible clinical findings are palpable mass with erythematous skin, pain, sterile abscesses, fistula and nipple retraction. Mammography and ultrasound findings are not specific for IGM. Magnetic resonance imaging (MRI) is a useful tool for the differential diagnosis; it is also necessary to delineate the exact extension of the disease and to plan the correct treatment. Final diagnosis is histological. We described an unusual case of IGM with bilateral involvement in a patient with history of pacemaker implantation and IGM typical clinical symptoms. Mammography, ultrasound, and MRI examinations were performed to identify the inflammatory disorder and to plan the correct therapy. Imaging features were correlated with final histological diagnosis of IGM. PMID:23781373

  8. Bilateral pigmented villonodular synovitis of the knee.

    PubMed

    Shah, Samir H; Porrino, Jack A; Green, John R; Chew, Felix S

    2015-12-01

    Pigmented villonodular synovitis is a disorder resulting in a villous, nodular, or villonodular proliferation of the synovium, with pigmentation related to the presence of hemosiderin. These lesions are almost exclusively benign with rare reports of malignancy. Pigmented villonodular synovitis can occur in a variety of joints and at any age but most often occurs within the knee in the young adult. Pigmented villonodular synovitis is a rare disease entity, and bilateral synchronous or metachronous involvement of a joint is even more uncommon, with few reports previously described in the literature. We present a case of pigmented villonodular synovitis involving both the right and left knee in the same patient, with radiographic imaging, magnetic resonance imaging, photograph and video intraoperative imaging, and pathologic correlation. PMID:26649121

  9. Bilateral mandibular fracture related to osteoradionecrosis.

    PubMed

    Goyal, Shikha; Mohanti, Bidhu Kalyan

    2015-01-01

    Mandible is the most frequently affected bone during head and neck irradiation. Late changes in the mandible may manifest in the form of reduced bone density, dental caries, loss of spongiosa trabeculations, delayed healing following dental extraction, pathologic fractures, osteoradionecrosis, trismus, growth defects in children or second malignancies. Pathologic fractures of mandibular bone are rare and may be spontaneous or traumatic (following dental extraction). We report the case of a 55-year lady, who had undergone surgery and adjuvant radiotherapy for carcinoma oral tongue T2N0M0 on a cobalt-60 unit and was disease-free. After a follow-up of 8 years post-irradiation, she presented with sudden onset oral pain and inability to open mouth. Pantomogram showed fracture at the junction of body and ramus of the mandible bilaterally. PMID:26097342

  10. The Baller-Gerold syndrome.

    PubMed Central

    Van Maldergem, L; Verloes, A; Lejeune, L; Gillerot, Y

    1992-01-01

    A case of severe craniosynostosis-radial aplasia (Baller-Gerold) syndrome is described in a newborn male, following a pregnancy complicated by polyhydramnios and intrauterine growth retardation. Death occurred after two hours owing to a prolonged apnoeic spell. Extensive agenesis of the frontal and parietal bones, resulting in a very large fontanelle, in addition to coronal bilateral craniosynostosis was observed at necropsy. There was also bilateral radial agenesis, oligodactyly of the hands and feet, a midline facial angioma, and a scrotally positioned anus, all of which have been described in some of the 10 previously reported cases. Microcephaly, erythroblastosis of the liver, and pancreatic islet cell hypertrophy were also noted. Images PMID:1583650

  11. Conscious attention, meditation, and bilateral information transfer.

    PubMed

    Bob, Petr; Zimmerman, Elizabeth M; Hamilton, Elizabeth A; Sheftel, Jenna G; Bajo, Stephanie D; Raboch, Jiri; Golla, Megan; Konopka, Lukasz M

    2013-01-01

    Recent findings indicate that conscious attention is related to large-scale information integration of various brain regions, including both hemispheres, that enables integration of parallel distributed modalities of processed information. There is also evidence that the level of information transference related to integration or splitting among brain regions, and between hemispheres, establishes a certain level of efficiency of the information processing. The level of information transference also may have modulatory influences on attentional capacity that are closely linked to the emotional arousal and autonomic response related to a stimulus. These findings suggest a hypothesis that changes in conscious attention, specifically during meditation could be reflected in the autonomic activity as the left-right information transference calculated from bilateral electrodermal activity (EDA). With the aim to compare conscious attention during meditation with other attentional states (resting state, Stroop task, and memory task), we performed bilateral EDA measurement in 7 healthy persons during resting state, Stroop task, neurofeedback memory test, and meditation. The results indicate that the information transference (ie, transinformation) is able to distinguish those attentional states, and that the highest level of the transinformation has been found during attentional processing related to meditation, indicating higher level of connectivity between left and right sides. Calculations other than pointwise transinformation (PTI) performed on EDA records, such as mean skin conductance level or laterality index, were not able to distinguish attentional states. The results suggest that PTI may present an interesting method useful for the assessment of information flow, related to neural functioning, that in the case of meditation may reflect typical integrative changes in the autonomic nervous system related to brain functions and focused attentional processing. PMID:23171535

  12. Red Ear Syndrome.

    PubMed

    Raieli, V; Compagno, A; D'Amelio, M

    2016-03-01

    The Red ear syndrome (RES) is an intriguing syndrome originally described for the first time nearly 20 years ago. RES is characterized by unilateral/bilateral episodes of pain and burning sensation of the ear, associated with ipsilateral erythema. RES episodes are indeed isolated in some patients, but they can occur in association with primary headaches, including in particular migraine in the developmental age. Although the underlying pathophysiological mechanisms are still uncertain, in the recent years the described comorbidities have aroused increasing interest because of possible clinical implications. Moreover, RES seems to be more often associated with clinical features of migraine partially provoked by the involvement of the parasympathetic system. This clinical association has shed new light on the pathophysiology of RES, supporting the hypothesis of a shared pathophysiological background, for example, through the activation of the trigeminal autonomic reflex. Current therapies of RES will be also discussed. Finally, we will resume the more controversial aspects of this relatively new and probably underestimated neurological syndrome. PMID:26879877

  13. UNITED STATES/GERMAN TECHNICAL BILATERAL AGREEMENT: PAST, PRESENT & FUTURE

    EPA Science Inventory

    The U.S. Environmental Protection Agency (EPA) and the German Federal Ministry of Education and Research (BMBF) entered into a Bilateral Agreement in 1990 to study each country's efforts in developing and demonstrating remedial technologies. The bilateral agreement is being impl...

  14. Bilateral Cochlear Implantation in Children: Experiences and Considerations

    ERIC Educational Resources Information Center

    Bohnert, Andrea; Spitzlei, Vera; Lippert, Karl L.; Keilmann, Annerose

    2006-01-01

    Between 2000 and 2006, the University Clinic for Ear Nose and Throat and Communication Disorders in Mainz, Germany, performed 41 bilateral cochlear implantations in children. This article addresses some of the factors to be considered in a decision to bilaterally implant a child, including the age of the child at the first implant, the length of…

  15. Bilateral temporomandibular joint dislocation with locked mandibular impaction.

    PubMed

    Hynes, Sally L; Jansen, Leigh A; Brown, D Ross; Courtemanche, Douglas J; Boyle, James C

    2012-02-01

    Bilateral anterior temporomandibular joint dislocation is very rare, with only 2 reported cases published. In the present report, we describe a healthy 25-year-old man from Haida Gwaii, in British Columbia, Canada, who was transferred to our tertiary trauma center with life-threatening complications of a bilateral anterior temporomandibular joint dislocation with locked mandibular impaction. PMID:22260912

  16. Chronic Cough and Bilateral Pneumothoraces in a Nonsmoker.

    PubMed

    O'Beirne, Sarah L; Escalon, Joanna G; Arkin, Jordan E; Stiles, Brendon M; Kaner, Robert J; Legasto, Alan C; Narula, Navneet; King, Thomas C

    2016-02-01

    An 82-year-old Japanese nonsmoking man presented with persistent dry cough and small left apical pneumothorax. High resolution CT scan of the chest demonstrated bilateral upper lobe pleuroparenchymal thickening and architectural distortion. Serial imaging revealed mild progression and development of small bilateral pneumothoraces, and pneumomediastinum. A surgical lung biopsy was required to confirm the diagnosis. PMID:26867855

  17. Bilateral congenital ureteral strictures in a young cat

    PubMed Central

    Lee, Namsoon; Choi, Mihyun; Keh, Seoyeon; Oh, Yein; Seo, Jimin; Choi, Heeyeon; Kim, Hyunwook; Yoon, Junghee

    2014-01-01

    An 8-month-old cat was presented with bilateral hydronephrosis. Bilateral ureteral obstructions were identified by diagnostic imaging and confirmed by necropsy. Histopathologic findings revealed polypoid transitional epithelial hyperplasia with chronic lymphoplasmacytic inflammation. This report documents congenital ureteral strictures as a cause of ureteral obstruction in a young cat. PMID:25183890

  18. Bilateral osteomas and exostoses of the internal auditory canal.

    PubMed

    Schutt, Christopher A; Guo, Jennifer N; Bagwell, Kenneth A; Bulsara, Ketan R; Malhotra, Ajay; Michaelides, Elias

    2015-01-01

    Osteomas and exostoses are benign tumors of the bone that occur in the head and neck region but are rarely found within the internal auditory canal (IAC). In this report, we review the literature on bony lesions of the IAC and present two cases: one case of bilateral compressive osteomas and one case of bilateral compressive exostoses of the IAC. PMID:25896777

  19. Cryoglobulinaemic neuropathy: a further cause of bilateral sciatic neuropathy

    PubMed Central

    Pérez, Desireé; de la Torre, Ricardo Gómez; Carrio, Isabel; Pinto, Jesús; Morís, Germán

    2008-01-01

    Bilateral sciatic neuropathy is a rare condition and it has been described as a compression or entrapment neuropathy but it is an uncommon clinical manifestation due to necrotizing vasculitis. We report an unusual case of cryoglobulinaemic neuropathy in an elderly woman with no underlying infectious or neoplastic cause; acute bilateral sciatic mononeuropathy was the presenting clinical manifestation of the cryoglobulinaemia. PMID:18834546

  20. Bilateral hyperplasia of the coronoid processes: clinical report.

    PubMed

    Tieghi, Riccardo; Galiè, Manlio; Piersanti, Luigi; Clauser, Luigi

    2005-07-01

    Monolateral or bilateral hyperplasia of the coronoid processes of the mandible is a rare disorder resulting in reduction of mouth opening because of the unnatural contact of the coronoid process with the zygomatic bones. The authors describe two cases of bilateral hyperplasia of the coronoid processes that were successfully treated. PMID:16077327

  1. Laparoscopic Management of Synchronous Bilateral Ovarian Torsion in a Neonate

    PubMed Central

    Alkan, Murat; Elbek, Ali; Evruke, Cuneyt; Memec, Ahmet Eray; Ozkan, Bulent Aziz; Sucak, Hatice Gülin; Erkan, Ozgur Talat

    2016-01-01

    Synchronous bilateral ovarian torsion is an uncommon entity of which both ovaries twist at the same time or observed twisted during the surgical intervention. Herein, we present a neonate with bilateral ovarian torsion, which successfully managed by laparoscopic approach. PMID:26793599

  2. Bilateral Symmetrical Congenital Giant Becker's Nevus: A Rare Presentation

    PubMed Central

    Rao, Angoori Gnaneshwar

    2015-01-01

    Becker's nevus is a focal epidermal hypermelanotic disorder. It morphologically presents as unilateral, hyperpigmented, hypertrichotic patch on upper trunk, proximal upper extremities and arms. However, Becker's nevus presenting as bilateral, symmetrical patches is rare. Herein, we report a rare case of giant Becker's nevus with bilateral symmetrical presentation in an adult male. PMID:26538733

  3. Ultrasound Diagnosis of Bilateral Quadriceps Tendon Rupture After Statin Use

    PubMed Central

    Nesselroade, Ryan D.; Nickels, Leslie Connor

    2010-01-01

    Simultaneous bilateral quadriceps tendon rupture is a rare injury. We report the case of bilateral quadriceps tendon rupture sustained with minimal force while refereeing a football game. The injury was suspected to be associated with statin use as the patient had no other identifiable risk factors. The diagnosis was confirmed using bedside ultrasound. PMID:21079697

  4. Idiopathic Bilateral Adrenal Hemorrhage in a 63-Year-Old Male: A Case Report and Review of the Literature

    PubMed Central

    Bodukam, Vijay Kumar; Thakur, Kshitij; Singh, Amandeep; Jenkins, Donald; Bahl, Jaya

    2015-01-01

    Adrenal hemorrhage is a largely uncommon condition typically caused by a number of factors including infection, MI, CHF, anticoagulants, trauma, surgery, and antiphospholipid syndrome. Yet, idiopathic bilateral hemorrhage is rare. The authors present a case of a 63-year-old male who presented with abdominal pain that was eventually diagnosed as bilateral adrenal hemorrhages due to an unknown origin. Abdominal CT revealed normal adrenal glands without enlargement, but an MRI displayed enlargement due to hemorrhage in both adrenals. There was no known cause; the patient had not suffered from an acute infection and was not on anticoagulants, and the patient's history did not reveal any of the other known causative factors. The case underscores the importance of keeping bilateral adrenal hemorrhages on the list of differentials even when a cause is not immediately clear. It also raises the question of whether CT is the most sensitive test in the diagnosis of adrenal hemorrhage and whether the diagnostic approach should place greater weight on MRI. The case highlights the need for prompt therapy with steroids once bilateral hemorrhage is suspected to avert the development or progression of adrenal insufficiency. PMID:25973281

  5. Bilateral thoracic ganglion cyst : a rare case report.

    PubMed

    Kazanci, Burak; Tehli, Ozkan; Türkoglu, Erhan; Guclu, Bulent

    2013-05-01

    Ganglion cysts usually arise from the tissues around the facet joints. It is usually associated with degenerative cahanges in facet joints. Bilateral thoracic ganglion cysts are very rare and there is no previous case that located in bilateral intervertebral foramen compressing the L1 nerve root associated with severe radiculopathy. We report a 53 years old woman who presented with bilateral groin pain and severe numbness. Magnetic resonance imaging revealed bilateral cystic mass in the intervertebral foramen between 12th thoracal and 1st lumbar vertebrae. The cystic lesions were removed after bilateral exposure of Th12-L1 foramens. The result of hystopathology confirmed the diagnosis as ganglion cyst. The ganglion cyst may compromise lumbar dorsal ganglion when it located in the intervertebral foramen. The surgeon should keep this rare entity in their mind for differential diagnosis. PMID:23908708

  6. Rare cause of paraparesis: bilateral obturator neuropathy after hysterosalpingectomy.

    PubMed

    López-Blanco, Roberto; Mejía-Jiménez, Inmaculada; de Fuenmayor-Fernández de la Hoz, Carlos Pablo; Ruiz-Morales, Juan

    2015-01-01

    Bilateral obturator nerve injury during pelvic surgery is an infrequent cause of lower limb paraparesis. We report the case of a 45-year-old woman with a large uterine leiomyoma who underwent simple total hysterectomy and bilateral salpingectomy. At 24?h after the surgery, the patient noticed loss of muscle strength when adducting both legs. She had no problem with other movements and no sensory or sphincter abnormalities. Neurological examination confirmed that there was loss of strength only in the adductor muscles, with preserved sensory function and reflexes, suggesting bilateral obturator nerve involvement. Pelvic MRI showed a small postsurgical haematoma in the Douglas recess, but far from the obturator nerves. 2?weeks later, electromyography showed positive sharp waves and low motor unit recruitment in the adductor magnus muscles, confirming acute, bilateral obturator nerve neuropathy. The few cases of bilateral obturator neuropathy that have been reported were mostly related to abdominopelvic interventions. PMID:26689250

  7. Kearns-Sayre syndrome: An unusual ophthalmic presentation.

    PubMed

    Ahmad, Syed S; Ghani, Shuaibah A

    2012-05-01

    Kearns-Sayre syndrome (KSS) belongs to the group of neuromuscular disorders known as mitochondrial encephalomyopathies. It has characteristic syndromal features, which include: chronic progressive external ophthalmoplegia, bilateral atypical pigmentary retinopathy, and cardiac conduction abnormalities. So far, only a single case has been reported where a patient with KSS had a normal retina. Herein, we report this extremely rare variant of KSS, which not only presented later than the normal age of presentation, but also had minimal pigmentary retinopathy. PMID:22993469

  8. The Syndrome of Familial Hypoparathyroidism, Sensorineural Deafness and Renal Dysplasia.

    PubMed

    Meena, Ratti Lal; Maloo, Sudheer Kumar; Samar, Neera; Ruhela, Asim; Saini, Subhash

    2015-06-01

    The syndrome of familial hypoparathyroidism, sensorineural deafness, and renal dysplasia (HDR syndrome) is inherited as an autosomal dominant trait, caused by haploinsufficiency of the GATA3 gene in chromosome 10p. Although first described years ago, but the disease is considered to be very rare. Patients usually present with hypocalcemia, tetany, or afebrile convulsions at any age. Hearing loss is usually bilateral, range from mild to profound impairment. Renal disease includes dysplasia, hypoplasia or aplasia. PMID:26710407

  9. Becker's Nevus Syndrome

    PubMed Central

    Dasegowda, Sathyanarayana B; Basavaraj, GB; Nischal, KC; Swaroop, MR; Umashankar, NP; Swamy, Suchetha S

    2014-01-01

    Becker's nevus is a cutaneous hamartoma characterized by circumscribed hyperpigmentation with hypertrichosis. Becker's nevus syndrome is an association of Becker's nevus with unilateral breast hypoplasia and muscle, skin, and/or skeletal abnormalities. We here report a case of a 15 year-old female who presented with bilateral Becker's nevus over her groins, thighs, vulva, and in front of the neck from the age of 5 years. She had associated mental retardation, delayed development of mile stones, delayed puberty, dwarfism, depressed nasal bridge, long slender digits, crowding of lateral toes, valgus deformity of first metatarsophalangeal joint, mitral valve prolapse, muddy conjunctiva with hypertrophic and hyperpigmented caruncle of both eyes, ichthyosis, brownish hair, and absence of axillary and pubic hair. On histopathological examination collagen hamartoma underneath the Becker's nevus was found. PMID:25071279

  10. Becker's Nevus Syndrome.

    PubMed

    Dasegowda, Sathyanarayana B; Basavaraj, Gb; Nischal, Kc; Swaroop, Mr; Umashankar, Np; Swamy, Suchetha S

    2014-07-01

    Becker's nevus is a cutaneous hamartoma characterized by circumscribed hyperpigmentation with hypertrichosis. Becker's nevus syndrome is an association of Becker's nevus with unilateral breast hypoplasia and muscle, skin, and/or skeletal abnormalities. We here report a case of a 15 year-old female who presented with bilateral Becker's nevus over her groins, thighs, vulva, and in front of the neck from the age of 5 years. She had associated mental retardation, delayed development of mile stones, delayed puberty, dwarfism, depressed nasal bridge, long slender digits, crowding of lateral toes, valgus deformity of first metatarsophalangeal joint, mitral valve prolapse, muddy conjunctiva with hypertrophic and hyperpigmented caruncle of both eyes, ichthyosis, brownish hair, and absence of axillary and pubic hair. On histopathological examination collagen hamartoma underneath the Becker's nevus was found. PMID:25071279

  11. Comparative gait initiation kinematics between simulated unilateral and bilateral ankle hypomobility: Does bilateral constraint improve speed performance?

    PubMed

    Delafontaine, A; Honeine, J-L; Do, M-C; Gagey, O; Chong, R K

    2015-08-31

    Improvement of motor performance in unilateral upper limb motor disability has been shown when utilizing inter-limb coupling strategies during physical rehabilitation. This suggests that 'default' bilateral central motor commands are facilitated. Here, we tested whether this bilateral motor control principle may be generalized to the lower limbs during gait initiation, which involves alternate bilateral actions. Disability was simulated by strapping to produce ankle hypomobility. Healthy adult subjects initiated gait at a self-paced speed with no ankle constraint (control), or with the stance, swing or bilateral ankles strapped. The duration of the anticipatory postural adjustments lengthened and the center of mass instantaneous progression velocity at foot-off decreased when the ankle was strapped. During the step execution phase, progression velocity at foot-contact was higher when both ankles were strapped compared to unilateral strapping of the stance ankle. These findings suggest that bilateral central motor commands are favored during walking tasks. Indeed, unilateral constraint of the stance ankle should compel the central nervous system to adapt specific commands to the constraint and normal sides whereas the 'default' bilateral motor commands would be utilized when both ankles are strapped leading to better kinematics performance. Bilateral in-phase upper limb coordination and bilateral alternating lower limb locomotor movements may share similar control mechanisms. PMID:26197055

  12. Tuberculous pneumonia with the syndrome of inappropriate secretion of antidiuretic hormone: cause of the adult respiratory distress syndrome.

    PubMed

    Sahn, S A; Skeff, K M

    1977-11-01

    Bilateral tuberculous pneumonia with the syndrome of inappropriate secretion of antidiuretic hormone was the cause of the adult respiratory distress syndrome in an elderly patient. Early recognition and prompt therapy enabled the patient to make a complete recovery without the necessity for mechanical ventilation. With the shift of care of tuberculous patients out of the sanitorium, the practicing physician should be aware of the varied manifestations of tuberculosis. PMID:913160

  13. [Poland-Möbius syndrome associated with lacrimal punctal and canalicular agenesis].

    PubMed

    Aydin, A; Ayata, A; Sabahyildizi, M; Ersanli, D

    2010-02-01

    We report the case of a 21-year-old patient who presented with epiphora in the left eye and a bilateral ocular motility problem. The clinical examination revealed bilateral moderate abduction limitation (impairment of the VIth cranial nerve), facial diplegia with amimic face (impairment of the VIIth cranial nerve), punctal agenesis of the left eye, absence of the left pectoralis major muscle, left breast aplasia, and hypoplasia of the left upper limb and hand. Neither the lacrimal puncta nor the canaliculi could be found during surgical exploration of left lacrimal system using operating microscope. Based on these findings, the patient was diagnosed with Poland-Möbius syndrome associated with punctal and canalicular agenesis. Poland-Möbius syndrome is a rare entity characterized by the association of two different syndromes: Poland syndrome and Möbius syndrome. Several abnormalities in association with this syndrome have been published. However, punctal and canalicular agenesis is not among these reported abnormalities. PMID:20061050

  14. Otolaryngologic manifestations of Maffucci's syndrome.

    PubMed

    Sun, Gordon H; Myer, Charles M

    2009-07-01

    Maffucci's syndrome is an uncommon congenital disease marked by multiple vascular malformations and enchondromas throughout the body. Otolaryngologic manifestations are relatively rare but potentially can cause symptoms such as respiratory distress and dysphagia if the lesions appear in the upper aerodigestive tract or larynx. We report a case of a young woman who demonstrated a wide range of head and neck pathology associated with this unique disease, including sphenoid enchondroma, bilateral petrous apex lesions, and rarely reported laryngeal and hypopharyngeal vascular malformations, over a period of nearly 20 years. PMID:19394093

  15. Imaging findings of Gorlin-Goltz syndrome.

    PubMed

    Hajalioghli, Parisa; Ghadirpour, Ali; Ataie-Oskuie, Reza; Kontzialis, Marinos; Nezami, Nariman

    2015-01-01

    A 15-year-old girl was referred to a dentist complaining of parageusia, bad taste in the mouth, which started 9 months ago. Panoramic X-ray and non-enhanced computed tomography scan revealed multiple bilateral unilocular cysts in the mandible and maxilla, along with calcification of anterior part of the falx cerebri. She was eventually diagnosed with Gorlin-Goltz syndrome based on imaging and histopathologic finding of keratocystic odontogenic tumor. PMID:25610614

  16. [Rare ocular manifestation with suspect alport syndrome].

    PubMed

    Krejčířová, I; Varadyová, B; Doležel, Z; Autrata, R; Matúšová, J; Gregorová, E

    2014-06-01

    The authors mention a case report of a 13 year old girl with renal disease, who visited the outpatient Department of Pediatric Ophthalmology, University Hospital Brno with subjective complaints on decreased vision of both eyes. Ophthalmologic examination showed physiological foveolar reflex on fundus and very discrete changes of the retinal pigment epithelium in macula, the fundus periphery was without pathology. OCT images showed bilateral atrophy of central macula and changes at the level of the photoreceptors. The authors describe a rare ocular manifestation of macular atrophy with suspect Alport syndrome, which strengthened the suspicion of this disease. The authors also mention other possible ocular manifestations of Alport syndrome and compare the findings with the up to date international references. Key words: Alport syndrome, X heterozygot Alport syndrome, macular atrophy, lentikonus. PMID:25032798

  17. [Fahr syndrome discovered following a bacterial meningitis].

    PubMed

    Sbai, H; Smail, L; Hamdani, S; Essatara, Y; Harrandou, M; Khatouf, M; Kanjaa, N

    2008-05-01

    Fahr's disease refers to a rare syndrome characterized by symmetrical and bilateral intracranial calcifications. The basal ganglia and dentate nucleus are the most common site of involvement and most cases present extrapyramidal symptoms. This disease is mostly associated with a phosphocalcic metabolism disorder, especially to hypoparathyroidism. The authors report a case of Fahr syndrome (FS) discovered when a young patient with hypocalcemia and bacterial meningitis had a cerebral CT scan disclosing intracerebral calcifications. She fully recovered after both meningitis and hypocalcemia were treated. PMID:18329142

  18. Spontaneous bilateral extrapleural hematoma: a case report.

    PubMed

    Hu, Sheng-I; Lee, Shih-Chun; Chang, Hung; Kuo, Yen-Shou

    2015-01-01

    Extrapleural hematoma (EPH) is a rare condition characterized by the accumulation of blood in the extrapleural space. EPH is generally identified by computed tomography (CT), which shows an inward displacement of extrapleural fat due to intrathoracic peripheral fluid accumulation (Ann Ital Chir 75(83): 5, 2004; J Korean Radiol Soc 49: 89-97, 2003; Monaldi Arch Chest Dis 63(3): 166-169, 2005). EPH has been reported to be associated with chest trauma and injuries. However, the correlation between hemodialysis and EPH has not yet been reported. The causes of EPH in a hemodialysis patient have been postulated, which include high venous flow through the arteriovenous fistula that results in an increase in venous pressure stenosis and/or thrombosis of the brachiocephalic and/or subclavian veins. These conditions thereby induce an increase in venous pressure in the intercostals and bronchial veins of the chest. Pleural fluid resorption is rare and excess pleural fluid formation commonly occurs (J Thoracic Imaging 26(3): 218-223, 2011). The occurrence of pleuritis with fusion of the two pleuric layers results in hematoma development in the extrapleural space instead of the pleural space. We present a chronic hemodialysis patient with spontaneous unilateral EPH. The progression to bilateral EPH was noted after VATS procedure. Awareness of EPH and the use of conservative management are key points for the treatment of this rare clinical condition. PMID:26169787

  19. From bilateral to multi-countries cooperation

    NASA Astrophysics Data System (ADS)

    Gueye, Paul; G4TRAP Collaboration

    2011-04-01

    In 2010, the Office for Science and Technology of the French Embassy in Washington DC provided seed funding to enable a new scientific collaboration between the US and France. The work primarily focuses on the understanding of the interaction of particles with the DNA. The National Society of Black Physicists managed the funds. This so-called G4TRAP work is an extension of the ESA-funded G4DNA project that uses the Geant4 Monte Carlo toolkit and is spearheaded by the University of Bordeaux 1 in France. This initiative spawns to new areas (ultrafast lasers, nuclear/high energy physics, radiation biology and space science) and includes US industries, thanks to the interdisciplinary activities needed to advance this project. In addition, the bi-lateral collaboration was expanded to two other countries: Canada and Senegal. Subsequent funding requests were made to US agencies (NSF and DoE) as well as other French programs. More are anticipated that will also include NIH, NASA and the Canadian programs. A review of this multidisciplinary effort will be presented along with the present status on the various aspects of the G4TRAP project.

  20. Mutual entrainment of bilaterally distributed circadian pacemaker.

    PubMed Central

    Page, T L; Caldarola, P C; Pittendrigh, C S

    1977-01-01

    The interactions between the bilaterally distributed components of the circadin system that controls the locomotor activity rhythm of the cockroach Leucophaea maderae were investigated in a series of surgical lesion experiments. Complete excision of one optic lobe (either right or left) or its surgical isolation from the central nervous system had no effect on the animals' ability to free-run in constant darkness nor was there any indication, as judged by postoperative pi values of any difference between left and right lobe pacemakers. However, these surgical procedures consistently resulted in a significant increase in tau over preoperative value while optic nerve section had no effect on tau. The propostion is developed that the left and right pacemakers in the two optic lobes are mutally coupled and that the compound pacemaker's period is shorter than either of its constituent pacemakers. It was also found that the integrity of either compound eye is sufficient to assure entrainment of both left and right pacemakers. Images PMID:265571

  1. Mutual entrainment of bilaterally distributed circadian pacemaker.

    PubMed

    Page, T L; Caldarola, P C; Pittendrigh, C S

    1977-03-01

    The interactions between the bilaterally distributed components of the circadin system that controls the locomotor activity rhythm of the cockroach Leucophaea maderae were investigated in a series of surgical lesion experiments. Complete excision of one optic lobe (either right or left) or its surgical isolation from the central nervous system had no effect on the animals' ability to free-run in constant darkness nor was there any indication, as judged by postoperative pi values of any difference between left and right lobe pacemakers. However, these surgical procedures consistently resulted in a significant increase in tau over preoperative value while optic nerve section had no effect on tau. The propostion is developed that the left and right pacemakers in the two optic lobes are mutally coupled and that the compound pacemaker's period is shorter than either of its constituent pacemakers. It was also found that the integrity of either compound eye is sufficient to assure entrainment of both left and right pacemakers. PMID:265571

  2. Bilateral psoas haematomata complicating renal transplantation.

    PubMed

    Akoh, Jacob A; Rana, Tahawar A; Higgs, Daniel

    2014-01-01

    Background. The challenge in managing patients undergoing renal transplantation is how to achieve optimum levels of anticoagulation to avoid both clotting and postoperative bleeding. We report a rare case of severe postoperative retroperitoneal bleeding including psoas haematomata complicating renal transplantation. Case Report. SM, a 55-year-old female, had a past history of aortic valve replacement, cerebrovascular event, and thoracic aortic aneurysm and was on long-term warfarin that was switched to enoxaparin 60?mg daily a week prior to her living donor transplantation. Postoperatively, she was started on a heparin infusion, but this was complicated by a large retroperitoneal bleed requiring surgical evacuation on the first postoperative day. Four weeks later, she developed features compatible with acute femoral neuropathy and a CT scan revealed bilateral psoas haematomata. Following conservative management, she made steady progress and was discharged home via a community hospital 94 days after transplantation. At her last visit 18 months after transplantation, she had returned to full fitness with excellent transplant function. Conclusion. Patients in established renal failure who require significant anticoagulation are at increased risk of bleeding that may involve prolonged hospitalisation and more protracted recovery and patients should be carefully counselled about this. PMID:25374958

  3. Psychosocial Outcomes after Bilateral Hand Transplantation

    PubMed Central

    Singh, Mansher; Oser, Megan; Zinser, Jennifer; Sisk, Geoffroy; Carty, Matthew J; Sampson, Christian; Pribaz, Julian J; Pomahac, Bohdan

    2015-01-01

    Background: Since the first successful hand transplantation in 1998, there have been multiple reports about surgical technique, transplant survival, and immunosuppression. However, very limited published data exist on psychosocial outcomes following hand transplantation. Methods: We report psychosocial outcomes in a patient with bilateral hand transplants at the midforearm level with serial follow-ups over 3.5 years. Different metrics used to study psychosocial outcomes included the following: SF-12, CES-D, Dyadic Adjustment Scale, Rosenberg SE, and EQ-5D. Result: Preoperatively, our patient did not have any evidence of depression (CES-D = 3), had a nonstressful relationship with his spouse (Dyadic Adjustment Scale = 100), and self-esteem was in the normal range (Rosenberg SE = 21). These metrics and his additional scales (SF-12 MCS, EQ-5D, and EQ-VAS) did not change appreciably and were within the normal range for the entire duration of 3.5-year follow-up at all different time points. Conclusion: With the increasing popularity of hand transplantation and the increasing awareness of the importance of psychosocial parameters in overall success, appropriate, comprehensive, and standardized measurements are important. These should be an integral part of patients’ screening and follow-up. PMID:26579339

  4. Rehabilitation for bilateral amputation of fingers

    USGS Publications Warehouse

    Stapanian, Martin A.; Stapanian, Adrienne M.P.; Staley, Keith E.

    2010-01-01

    We describe reconstructive surgeries, therapy, prostheses, and adaptations for a patient who experienced bilateral amputation of all five fingers of both hands through the proximal phalanges in January 1992. The patient made considerable progress in the use of his hands in the 10 mo after amputation, including nearly a 120% increase in the active range of flexion of metacarpophalangeal joints. In late 1992 and early 1993, the patient had "on-top plasty" surgeries, in which the index finger remnants were transferred onto the thumb stumps, performed on both hands. The increased web space and functional pinch resulting from these procedures made many tasks much easier. The patient and occupational therapists set challenging goals at all times. Moreover, the patient was actively involved in the design and fabrication of all prostheses and adaptations or he developed them himself. Although he was discharged from occupational therapy in 1997, the patient continues to actively find new solutions for prehension and grip strength 18 yr after amputation.

  5. Paraneoplastic Syndromes

    MedlinePLUS

    ... NINDS Funding Information Research Programs Training & Career Awards Enhancing Diversity Find People About NINDS NINDS Paraneoplastic Syndromes ... done? Research on paraneoplastic syndromes is aimed at enhancing scientific understanding and evaluating new therapeutic interventions. Researchers ...

  6. Angelman Syndrome

    MedlinePLUS

    ... heads, jerky movements, protruding tongues, and bouts of laughter." Infants with Angelman syndrome appear normal at birth, ... develop techniques to diagnose, treat, prevent, and ultimately cure them. NIH Patient Recruitment for Angelman Syndrome Clinical ...

  7. Marfan Syndrome

    MedlinePLUS

    ... Like for Kids With Marfan Syndrome? en español Síndrome de Marfan Evan couldn't wait for school ... for Marfan syndrome runs in families, getting passed down to children from parents who have the disease. ...

  8. Brown Syndrome

    MedlinePLUS

    ... poor binocular vision (which can result in poor depth perception) and/or amblyopia. Are there different kinds of Brown syndrome? Brown syndrome can be classified according to severity. In mild cases there is a reduced ability to look up ...

  9. LEOPARD syndrome

    MedlinePLUS

    LEOPARD syndrome is a very rare inherited disorder in which there are problems with the skin, face, ... LEOPARD syndrome is inherited as an autosomal dominant trait. This means the person only needs the abnormal ...

  10. Asperger syndrome

    MedlinePLUS

    Asperger syndrome is often considered a high functioning form of autism. It can lead to difficulty interacting socially, repeat behaviors, and clumsiness. Asperger syndrome is a part of the larger developmental ...

  11. Pseudoaminopterin syndrome.

    PubMed

    Kraoua, Lilia; Capri, Yline; Perrin, Laurence; Benmansour, Abdelmajjid; Verloes, Alain

    2012-09-01

    Pseudoaminopterin syndrome or aminopterin syndrome-like sine aminopterin (ASSA syndrome--OMIM 600325] is a rare autosomal recessive syndrome defined by characteristic dysmorphic features, skeletal defects, limb anomalies, cryptorchidism, and growth retardation. The syndrome owes its name to the fact that patients resemble the children exposed to aminopterin or to methotrexate, two dihydrofolate reductase inhibitors used for chemotherapy, or as an abortificient in early pregnancy. Ten patients have been described with pseudoaminopterin syndrome. Their phenotype is variable, and differs from the phenotype resulting from folic acid deprivation, leading to the notion that the pathogenesis may be more complex than simple vitamin deficiency. We report on an Algerian patient with pseudoaminopterin syndrome, review the previously reported cases and confirm that pseudoaminopterin syndrome does not result from a detectable contiguous gene imbalance as high resolution CGH array was normal in this child. PMID:22811276

  12. Fahr's Syndrome

    MedlinePLUS

    ... is Fahr's Syndrome? Fahr's Syndrome is a rare, genetically dominant, inherited neurological disorder characterized by abnormal deposits ... the NINDS or the NIH is appreciated. Last Modified February 13, 2007 National Institute of Neurological Disorders ...

  13. Pendred Syndrome

    MedlinePLUS

    ... Pendred syndrome will show vestibular weakness when their balance is tested. However, the brain is very good at making up for a weak vestibular system, and most children and adults with Pendred syndrome don't have a problem ...

  14. Rett Syndrome

    MedlinePLUS

    Rett syndrome is a rare genetic disease that causes developmental and nervous system problems, mostly in girls. It's related to autism spectrum disorder. Babies with Rett syndrome seem to grow and develop normally at first. ...

  15. Rett Syndrome

    MedlinePLUS

    ... NICHD Research Information Clinical Trials Resources and Publications Rett Syndrome: Overview Skip sharing on social media links Share this: Page Content Rett syndrome is a neurological and developmental genetic disorder that ...

  16. Craniofacial Syndrome Descriptions

    MedlinePLUS

    ... Goldenhar/Hemifacial Moebius syndrome Pfeiffer syndrome Pierre Robin Sequence Treacher Collins syndrome Other syndromes Wonder News & Events ... of the radial limb. Pfeiffer syndrome Pierre Robin Sequence Saethre-Chotzen Saethre-Chotzen syndrome is a condition ...

  17. Globus Pallidus Interna Deep Brain Stimulation in a Patient with Medically Intractable Meige Syndrome

    PubMed Central

    Bae, Dae-Woong; Son, Byung-chul; Kim, Joong-Seok

    2014-01-01

    Medical therapies in patients with Meige syndrome, including botulinum toxin injection, have been limited because of incomplete response or adverse side effects. We evaluated a patient with Meige syndrome who was successfully treated with deep brain stimulation (DBS) in the globus pallidus interna (GPi). This case report and other previous reports suggest that bilateral GPi DBS may be an effective treatment for medically refractory Meige syndrome, without significant adverse effects. PMID:25360233

  18. Speckle reducing bilateral filter for cattle follicle segmentation

    PubMed Central

    2010-01-01

    Background Ultrasound imaging technology has wide applications in cattle reproduction and has been used to monitor individual follicles and determine the patterns of follicular development. However, the speckles in ultrasound images affect the post-processing, such as follicle segmentation and finally affect the measurement of the follicles. In order to reduce the effect of speckles, a bilateral filter is developed in this paper. Results We develop a new bilateral filter for speckle reduction in ultrasound images for follicle segmentation and measurement. Different from the previous bilateral filters, the proposed bilateral filter uses normalized difference in the computation of the Gaussian intensity difference. We also present the results of follicle segmentation after speckle reduction. Experimental results on both synthetic images and real ultrasound images demonstrate the effectiveness of the proposed filter. Conclusions Compared with the previous bilateral filters, the proposed bilateral filter can reduce speckles in both high-intensity regions and low intensity regions in ultrasound images. The segmentation of the follicles in the speckle reduced images by the proposed method has higher performance than the segmentation in the original ultrasound image, and the images filtered by Gaussian filter, the conventional bilateral filter respectively. PMID:21047390

  19. Exophthalmos: A Forgotten Clinical Sign of Cushing's Syndrome.

    PubMed

    Giugni, Aldo Schenone; Mani, Shylaja; Kannan, Subramanian; Hatipoglu, Betul

    2013-01-01

    Exophthalmos is typically associated with Graves' ophthalmopathy. Although originally described by Harvey Cushing, exophthalmos is an underappreciated sign of Cushing's syndrome. We present a case of a 38-year-old female who presented with severe bilateral proptosis and was subsequently diagnosed with Cushings disease. We discuss the possible mechanisms causing exophthalmos in patients with either endogenous or exogenous hypercortisolemia. PMID:23555062

  20. Dance Therapy with Physical Therapy for Children with Down Syndrome.

    ERIC Educational Resources Information Center

    Dupont, Blanche Burt; Schulmann, Diana

    This study sought to investigate effects of a dance program on bilateral toe-standing balance and single-point static balance skills of a group of children with Down Syndrome. Thirteen experimental and 10 control group students between the ages of 3 and 13 years were assessed on toe-standing balance and single-point standing balance on the right…

  1. Exercise-Related Bilateral Leg Atypical Claudication in Female Olympic Taekwondo Player: A Case Report

    PubMed Central

    Vilás, Ramón Olivé; Rodriguez, Lorenzo Álvarez; Campos, Montserrat Yeste; Moran, Antonio De la Torre; Mas, Ferran Latorre

    2011-01-01

    We report the case of an Olympic taekwondo athlete with an atypical bilateral intermittent claudication that represented a handicap in her performance during competition fight. Diagnosis of chronic compartment syndrome was established by confirmation of compartment hypertension and the patient was submitted to fasciectomy. Recurrence of symptoms like numbness or tingling after 6 months raised the suspicion of deep compartmental hypertension that, once confirmed, was treated by a repeat deep fasciectomy. New symptoms appeared 4 months after surgery but no hypertension in the compartment was detected. Functional popliteal artery entrapment syndrome (FPAES) was suspected, an unusual form of this syndrome, in which a hypertrophic plantaris tendon as the cause of the entrapment was observed. Resection of the band was performed in a third operation. The patient evolved favourably, maintaining high competition level. Key points We need a thorough clinical examination to reach a satisfactory clinical diagnosis. FPAES is an uncommon disease that is frequently misdiagnosed and overlooked. Dynamic tests are essential for diagnosis de FPAES. PMID:24149572

  2. Exercise-related bilateral leg atypical claudication in female olympic taekwondo player: a case report.

    PubMed

    Vilás, Ramón Olivé; Rodriguez, Lorenzo Álvarez; Campos, Montserrat Yeste; Moran, Antonio De la Torre; Mas, Ferran Latorre

    2011-01-01

    We report the case of an Olympic taekwondo athlete with an atypical bilateral intermittent claudication that represented a handicap in her performance during competition fight. Diagnosis of chronic compartment syndrome was established by confirmation of compartment hypertension and the patient was submitted to fasciectomy. Recurrence of symptoms like numbness or tingling after 6 months raised the suspicion of deep compartmental hypertension that, once confirmed, was treated by a repeat deep fasciectomy. New symptoms appeared 4 months after surgery but no hypertension in the compartment was detected. Functional popliteal artery entrapment syndrome (FPAES) was suspected, an unusual form of this syndrome, in which a hypertrophic plantaris tendon as the cause of the entrapment was observed. Resection of the band was performed in a third operation. The patient evolved favourably, maintaining high competition level. Key pointsWe need a thorough clinical examination to reach a satisfactory clinical diagnosis.FPAES is an uncommon disease that is frequently misdiagnosed and overlooked.Dynamic tests are essential for diagnosis de FPAES. PMID:24149572

  3. Velocardiofacial Syndrome

    ERIC Educational Resources Information Center

    Gothelf, Doron; Frisch, Amos; Michaelovsky, Elena; Weizman, Abraham; Shprintzen, Robert J.

    2009-01-01

    Velocardiofacial syndrome (VCFS), also known as DiGeorge, conotruncal anomaly face, and Cayler syndromes, is caused by a microdeletion in the long arm of Chromosome 22. We review the history of the syndrome from the first clinical reports almost half a century ago to the current intriguing molecular findings associating genes from the…

  4. Piriformis Syndrome

    MedlinePLUS

    ... the symptoms of piriformis syndrome? The most common symptom of piriformis syndrome is sciatica. This term describes pain, tingling or numbness that ... such as a car accident or a fall. ... something other than piriformis syndrome is causing your sciatica, he or she may order additional tests. Computerized ...

  5. Turcot Syndrome

    MedlinePLUS

    ... of colorectal cancer , and an increased risk of brain cancer . The type of brain cancer generally depends on whether the Turcot syndrome ... Lynch syndrome or FAP. The two most common types of brain tumors in Turcot syndrome are: Glioblastoma . This type ...

  6. Rowell syndrome

    PubMed Central

    Bhat, Ramesh Y; Varma, Chaitanya; Bhatt, Sonia; Balachandran, C

    2014-01-01

    Rowell syndrome is a rare disease consisting of erythema multiforme-like lesions associated with lupus erythematosus. The syndrome occurs mostly in middle-aged women. The authors describe the syndrome in a 15-year-old boy who responded well to systemic steroids and hydroxychloroquine. PMID:25506561

  7. Velocardiofacial Syndrome

    ERIC Educational Resources Information Center

    Gothelf, Doron; Frisch, Amos; Michaelovsky, Elena; Weizman, Abraham; Shprintzen, Robert J.

    2009-01-01

    Velocardiofacial syndrome (VCFS), also known as DiGeorge, conotruncal anomaly face, and Cayler syndromes, is caused by a microdeletion in the long arm of Chromosome 22. We review the history of the syndrome from the first clinical reports almost half a century ago to the current intriguing molecular findings associating genes from the…

  8. Aase syndrome

    MedlinePLUS

    Aase-Smith syndrome; Hypoplastic anemia/Triphalangeal thumb syndrome ... Jones KL, ed. Aase syndrome. In: Smith's Recognizable Patterns Of Human Malformation. 6th ed. Saunders. 2005. Clinton C, Gazda HT. Diamond-Blackfan Anemia. 2009 Jun 25 [Updated 2013 Jul ...

  9. Chronic bilateral dislocation of temporomandibular joint.

    PubMed

    Shakya, S; Ongole, R; Sumanth, K N; Denny, C E

    2010-01-01

    Dislocation of the condyle of the mandible is a common condition that may occur in an acute or chronic form. It is characterised by inability to close the mouth with or without pain. Dislocation has to be differentiated from subluxation which is a self reducible condition. Dislocation can occur in any direction with anterior dislocation being the commonest one. Various predisposing factors have been associated with dislocation like muscle fatigue and spasm, the defect in the bony surface like shallow articular eminence, and laxity of the capsular ligament. People with defect in collagen synthesis like Ehler Danlos syndrome, Marfan syndrome are said to be genetically predisposed to this condition. Various treatment modalities have been used ranging from conservative techniques to surgical methods. Acute dislocations can be reduced manually or with conservative approach and recurrent and chronic cases can be reduced by surgical intervention. Though the dislocation in our case was 4 months a simple manual reduction proved to be successful. We believe that manual reduction can be attempted as first line of treatment prior to surgical intervention. PMID:21209547

  10. Bilateral BRVO in a patient with recurrent prostate cancer.

    PubMed

    Castro-Navarro, Veronica; Odaibo, Stephen G; Ghodasra, Devon H; Besirli, Cagri G

    2015-01-01

    Bilateral cases of branch retinal vein occlusions (BRVO) are infrequent and often related to systemic disease. A 72-year-old man with biochemical recurrence of prostate cancer was referred for decreased vision in his left eye. Fundus examination and fluorescein angiography disclosed bilateral BRVO with patches of peripheral non-perfusion and macular oedema in the left eye. A systemic work up revealed elevated fibrinogen and reduced free protein S antigen, consistent with an underlying hypercoagulable state. Cancer is a well-known cause of hypercoagulability. We report the first case of bilateral BRVO related to biochemical recurrence of prostate cancer and a proven coagulation derangement. PMID:26491002

  11. Unilateral Direct Carotid Cavernous Fistula Causing Bilateral Ocular Manifestation

    PubMed Central

    Demartini Jr., Zeferino; Liebert, Fernando; Gatto, Luana Antunes Maranha; Jung, Thiago Simiano; Rocha Jr., Carlos; Santos, Alex Marques Borges; Koppe, Gelson Luis

    2015-01-01

    Unilateral carotid cavernous fistula presents with ipsilateral ocular findings. Bilateral presentation is only seen in bilateral fistulas, usually associated with indirect (dural) carotid cavernous fistulas. Direct carotid cavernous fistulas are an abnormal communication between the internal carotid artery and the cavernous sinus. They typically begin with a traumatic disruption in the artery wall into the cavernous sinus, presenting with a classic triad of unilateral pulsatile exophthalmos, cranial bruit and episcleral venous engorgement. We report the case of a 38-year-old male with traumatic right carotid cavernous sinus fistula and bilateral ocular presentation successfully treated by interventional neuroradiology.

  12. Bilateral topographic symmetry patterns across Aphrodite Terra, Venus

    NASA Technical Reports Server (NTRS)

    Crumpler, L. S.; Head, James W.

    1988-01-01

    Topographic profiles have been obtained across Aphrodite Terra to test for bilateral symmetry of the type associated with thermal boundary layer topography at divergent plate boundaries on earth. In addition to a broad bilateral symmetry at a range of angles across Aphrodite Terra, detailed bilateral symmetry is noted within domains between linear discontinuities in directions parallel to the strike of the discontinuities. The results suggest that western Aphrodite Terra is similar to terrestrial oceanic divergent plate boundary environments, and that the cross-strike discontinuities are analogous to oceanic fracture zones rather than strike-slip faults.

  13. Reconstruction of bilateral tendoachilles with fascia lata graft

    PubMed Central

    Saxena, Vikas; Pradhan, Pavan; Yadav, Ashok; Nathani, Neeraj

    2013-01-01

    A 19 year old male presented with progressive enlargement of both tendoachilles for 2 years and difficulty in walking for 3 months. The neurological history and examination revealed progressive mental deterioration and ataxia. The blood investigation revealed hypercholesterolemia. We report this rare case of cerebrotendinous xanthomatosis with bilateral tendoachilles enlargement, which was treated by excision of bilateral tendoachilles and reconstruction with fascia lata. The American Orthopedic Foot and Ankle Society hindfoot score was 93/100 bilaterally and the subjective evaluation of the patient showed very good results. PMID:24379473

  14. A case of Ross syndrome presented with Horner and chronic cough.

    PubMed

    Baran, Aslihan; Balbaba, Mehmet; Demir, Caner F; Ozdemir, Hasan H

    2014-10-01

    Ross syndrome is a rare sweating disorder associated with Adie's tonic pupil, decreased or diminished tendon reflex and unknown etiology. Although autonomic disturbances affecting sudomotor and vasomotor functions are seen commonly, they are rarely symptomatic. While Ross syndrome is typically characterized with dilated tonic pupil, it may be rarely manifested with miotic pupils (little old Adie's pupil), which can make diagnosis difficult. In this article, we aim to specify the atypical clinical manifestations of syndrome by means of Ross syndrome manifested by autonomic symptoms, Horner syndrome, chronic cough together with bilateral little old Adie's pupil. PMID:25288844

  15. Wolfram syndrome maps to distal human chromosome 4p

    SciTech Connect

    Polymeropoulos, M.H.; Swift, R.; Swift, M.

    1994-09-01

    Wolfram syndrome (MIM 222300) is an autosomal recessive disorder defined by the occurrence of diabetes mellitus and progressive bilateral optic atrophy. Wolfram syndrome homozygotes develop widespread nervous system abnormalities; in particular, they exhibit severe behavioral difficulties that often lead to suicide attempts or psychiatric hospitalizations. The Wolfram syndrome gene also predisposes heterozygous carriers to psychiatric disorders. Since these heterozygotes are common in the general population, the Wolfram syndrome gene may contribute significantly to the overall burden of psychiatric illness. Based on a linkage analysis of 11 families segregating for this syndrome, using microsatellite repeat polymorphisms throughout the human genome, we found the Wolfram syndrome gene to be linked to markers on the short arm of human chromosome 4, with Zmax=6.46 at {theta}=0.02 for marker D4S431.

  16. Mutational analysis of steroidogenic factor 1 (NR5a1) in 24 boys with bilateral anorchia: a French collaborative study†

    PubMed Central

    Philibert, Pascal; Zenaty, Delphine; Lin, Lin; Soskin, Sylvie; Audran, Françoise; Léger, Juliane; Achermann, John C.; Sultan, Charles

    2010-01-01

    BACKGROUND Steroidogenic factor 1 (SF1/AdBP4/FTZF1, NR5A1) is a nuclear receptor transcription factor that plays a key role in regulating adrenal and gonadal development, steroidogenesis and reproduction. Recently, haploin-sufficiency of SF1 has been described in several 46,XY individuals with mild gonadal dysgenesis and impaired androgenization, but normal adrenal function, suggesting that dosage-sensitive or domain-specific effects of SF1 action are important in human testicular development and function. Our objective was to investigate whether partial defects in SF1 function might be associated with milder male reproductive phenotypes, such as bilateral anorchia (‘vanishing testis syndrome’) and micropenis. METHODS This study involved mutational analysis of NR5A1 in 24 individuals with bilateral anorchia and micropenis from the French Collaborative Anorchia study, as well as in vitro functional studies of SF1-dependent transcriptional activation and computer modeling. RESULTS A novel heterozygous missense mutation (V355M) in SF1 was found in one boy with a micropenis and testicular regression syndrome. This non-synonymous change was found to affect a highly conserved amino acid within helix 7 of the ligand-binding domain of SF1. This V355M mutation did not affect stability or nuclear localization, but did result in an ~50% reduction in SF1 activity in several different assay systems. CONCLUSIONS In conclusion, heterozygous partial loss of function mutations in SF1 may be associated with bilateral anorchia (‘vanishing testis syndrome’) and micropenis in humans. PMID:17940071

  17. Connectivity-Based Parcellation of the Thalamus Explains Specific Cognitive and Behavioural Symptoms in Patients with Bilateral Thalamic Infarct

    PubMed Central

    Serra, Laura; Cercignani, Mara; Carlesimo, Giovanni A.; Fadda, Lucia; Tini, Nadia; Giulietti, Giovanni; Caltagirone, Carlo; Bozzali, Marco

    2013-01-01

    A novel approach based on diffusion tractography was used here to characterise the cortico-thalamic connectivity in two patients, both presenting with an isolated bilateral infarct in the thalamus, but exhibiting partially different cognitive and behavioural profiles. Both patients (G.P. and R.F.) had a pervasive deficit in episodic memory, but only one of them (R.F.) suffered also from a dysexecutive syndrome. Both patients had an MRI scan at 3T, including a T1-weighted volume. Their lesions were manually segmented. T1-volumes were normalised to standard space, and the same transformations were applied to the lesion masks. Nineteen healthy controls underwent a diffusion-tensor imaging (DTI) scan. Their DTI data were normalised to standard space and averaged. An atlas of Brodmann areas was used to parcellate the prefrontal cortex. Probabilistic tractography was used to assess the probability of connection between each voxel of the thalamus and a set of prefrontal areas. The resulting map of corticothalamic connections was superimposed onto the patients’ lesion masks, to assess whether the location of the thalamic lesions in R.F. (but not in G. P.) implied connections with prefrontal areas involved in dysexecutive syndromes. In G.P., the lesion fell within areas of the thalamus poorly connected with prefrontal areas, showing only a modest probability of connection with the anterior cingulate cortex (ACC). Conversely, R.F.’s lesion fell within thalamic areas extensively connected with the ACC bilaterally, with the right dorsolateral prefrontal cortex, and with the left supplementary motor area. Despite a similar, bilateral involvement of the thalamus, the use of connectivity-based segmentation clarified that R.F.’s lesions only were located within nuclei highly connected with the prefrontal cortical areas, thus explaining the patient’s frontal syndrome. This study confirms that DTI tractography is a useful tool to examine in vivo the effect of focal lesions on interconnectivity brain patterns. PMID:23755128

  18. "Rescue" of bilateral subthalamic stimulation by bilateral pallidal stimulation: case report.

    PubMed

    Matias, Caio M; Silva, Danilo; Machado, Andre G; Cooper, Scott E

    2016-02-01

    Deep brain stimulation (DBS) of the subthalamic nucleus (STN) orglobus pallidus pars interna (GPi) is well established as a treatment for advanced Parkinson's disease. In general, one of the 2 targets is chosen based on the clinical features of each patient. Stimulation of both targets could be viewed as redundant, given that the 2 targets are directly connected. However, it is possible that each target has different mechanisms, with clinical effects mediated by orthodromic or antidromic stimulation. The authors report the case of a patient with severe Parkinson's disease who had previously undergone bilateral subthalamic stimulation with excellent benefits. However, he presented with significant worsening associated with disease progression and pharmacological treatment, and then underwent bilateral GPi DBS. Follow-up assessment was conducted clinically as well as through blinded ratings of video recordings. Pallidal DBS may be a safe and useful strategy to manage dystonic features and behavioral complications of subthalamic stimulation and pharmacological management. While combined stimulation was quite successful in the reported patient, further studies with larger samples and longer follow-up periods will be necessary before recommending the addition of pallidal DBS as a routine strategy for patients previously implanted with STN DBS. PMID:26230477

  19. Early Deprivation Impairs the Development of Balance and Bilateral Coordination

    PubMed Central

    Roeber, Barbara J.; Gunnar, Megan R.; Pollak, Seth D.

    2014-01-01

    This study examined balance and bilateral coordination skills in a sample of internationally adopted, post-institutionalized (PI) children. We compared the performance of these PI children to two age-matched groups. One was a group of children who were internationally adopted from foster care (FC). The second group consisted of non-adopted children being raised in their birth families, who served as controls (Control). Both PI and FC children scored lower than control children on balance, while PI children scored lower than both FC and control children on bilateral coordination. These results suggest that aspects of institutional rearing impact the development of bilateral coordination, while factors common to internationally adopted children other than institutionalization impact the development of balance. Region of birth (Asia, Latin/South America, Russia/Eastern Europe) did not moderate associations between institutional duration and bilateral coordination. PMID:24014461

  20. Spontaneous Unruptured Bilateral Tubal Pregnancy: A Case Report.

    PubMed

    Ghomian, Nayereh; Lotfalizadeh, Marziyeh

    2015-11-01

    Bilateral spontaneous tubal ectopic pregnancy is the rarest form of extra uterine pregnancy. The diagnosis is usually made intraoperatively and levels of serum BHCG and ultrasound has not been useful in the diagnosis of bilateral tubal ectopic pregnancy. A 33-year-old woman with 8 weeks amenorrhea and sever lower abdominal pain was admitted. A transvaginal pelvic ultrasound revealed left adnexal mass and massive fluid collection in the pelvis and abdomen. The serum BHCG was 5,700 mIU/ml and in laparotomy bilateral unruptured tubal pregnancy was noted. Left salpingectomy and right salpingostomy were performed. The diagnosis of bilateral spontaneous tubal ectopic pregnancy is usually made intraoperatively. Both tubes at the time of surgery should be closely examined in order to prevent maternal morbidity and mortality. PMID:26538784

  1. Radiotherapy for Langerhans Cell Histiocytosis of Bilateral Eyelids

    PubMed Central

    Bourque, Jean-Marc; Lukovic, Jelena; Dar, A. Rashid

    2016-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder with numerous clinicopathological variants with differing clinical courses, treatment methods, and prognoses. We report one patient with atypical LCH of the bilateral lower eyelids and subsequent successful treatment with local radiation therapy.

  2. Bilateral Ankle and Subtalar Joint Fusion Secondary to Guillain Barré-Induced Foot Drop.

    PubMed

    Seidel, Jay; Mathew, Bindu; Marks, Jeffrey

    2016-01-01

    Guillain-Barré syndrome is a serious disorder that occurs when the body's immune system mistakenly attacks the peripheral nervous system. This leads to nerve inflammation and damage that can cause muscle weakness and/or paralysis, including foot drop. Therapy ranges from supportive measures, such as physical therapy, to surgical intervention. It appears from the published data that these patients, generally, have been left to their own devices, because no known cure is available, necessitating reliance on others for their daily activities and ambulation. We present a unique surgical option, with a follow-up period of 24 months, in which bilateral subtalar and ankle fusion allowed unassisted ambulation to a patient who might otherwise have never walked again. PMID:25128306

  3. Unilateral Arm Urticaria Presenting as a Paraneoplastic Manifestation of Metachronous Bilateral Breast Cancer.

    PubMed

    Kasi, Pashtoon Murtaza; Hieken, Tina J; Haddad, Tufia C

    2016-01-01

    Various paraneoplastic syndromes (PNS) are reported to be associated with breast cancer and can range from mild dermatological symptoms to severe neurological complications. Neurological and dermatological manifestations tend to be the more commonly seen paraneoplastic manifestations, albeit both are relatively rare. Diagnosis of the underlying malignancy is often delayed since the presence and severity of paraneoplastic manifestations are not dependent on the tumor size or stage. Herein, we describe a unique case of unilateral arm urticaria presenting as a paraneoplastic manifestation of metachronous bilateral breast cancer. Similar reports and other PNS associated with breast cancer are described. Recognition of PNS associated with underlying malignancies and age-appropriate screening can facilitate diagnosis of the underlying occult malignancy. Resection of the underlying malignancy can lead to resolution and/or improvement of the PNS for some patients. PMID:26933416

  4. Bilateral congenital lumbar hernias in a patient with central core disease - A case report.

    PubMed

    Lazier, Joanna; Mah, Jean K; Nikolic, Ana; Wei, Xing-Chang; Samedi, Veronica; Fajardo, Carlos; Brindle, Mary; Perrier, Renee; Thomas, Mary Ann

    2016-01-01

    Congenital lumbar hernias are rare malformations caused by defects in the development of the posterior abdominal wall. A known association exists with lumbocostovertebral syndrome; however other associated anomalies, including one case with arthrogryposis, have been previously reported. We present an infant girl with bilateral congenital lumbar hernias, multiple joint contractures, decreased muscle bulk and symptoms of malignant hyperthermia. Molecular testing revealed an R4861C mutation in the ryanodine receptor 1 (RYR1) gene, known to be associated with central core disease. This is the first reported case of the co-occurrence of congenital lumbar hernias and central core disease. We hypothesize that ryanodine receptor 1 mutations may interrupt muscle differentiation and development. Further, this case suggests an expansion of the ryanodine receptor 1-related myopathy phenotype to include congenital lumbar hernias. PMID:26684984

  5. Unilateral Arm Urticaria Presenting as a Paraneoplastic Manifestation of Metachronous Bilateral Breast Cancer

    PubMed Central

    Kasi, Pashtoon Murtaza; Hieken, Tina J.; Haddad, Tufia C.

    2016-01-01

    Various paraneoplastic syndromes (PNS) are reported to be associated with breast cancer and can range from mild dermatological symptoms to severe neurological complications. Neurological and dermatological manifestations tend to be the more commonly seen paraneoplastic manifestations, albeit both are relatively rare. Diagnosis of the underlying malignancy is often delayed since the presence and severity of paraneoplastic manifestations are not dependent on the tumor size or stage. Herein, we describe a unique case of unilateral arm urticaria presenting as a paraneoplastic manifestation of metachronous bilateral breast cancer. Similar reports and other PNS associated with breast cancer are described. Recognition of PNS associated with underlying malignancies and age-appropriate screening can facilitate diagnosis of the underlying occult malignancy. Resection of the underlying malignancy can lead to resolution and/or improvement of the PNS for some patients. PMID:26933416

  6. Anesthetic management of emergent laparoscopic bilateral adrenalectomy in a patient with a life-threatening cortisol crisis.

    PubMed

    Sharma, Ankur; Subramaniam, Rajeshwari; Misra, Mahesh; Joshiraj, Bandi; Krishnan, Gopi; Varma, Prerna; Kishore, Shyam

    2015-01-15

    Cushing syndrome may rarely present with life-threatening hypercortisolism, manifested by hypertension, hypokalemia, hyperglycemia, and edema. If medical treatment proves ineffective in ameliorating the symptoms, emergent rescue adrenalectomy may be the only way to relieve the crisis. We describe the anesthetic management of a patient with an ectopic adrenocorticotropic hormone-secreting tumor, whose condition was rapidly deteriorating due to severe cortisol excess, and emergent adrenalectomy was the only available therapeutic modality. Despite severe metabolic derangement, edema, and incipient respiratory failure, emergent bilateral laparoscopic adrenalectomy was performed and the patient improved sufficiently to undergo surgery for the ectopic lesion without incident. PMID:25611000

  7. Bilateral sensorineural deafness and hydrocephalus due to foramen of Monro obstruction in sibs: A newly described autosomal recessive disorder

    SciTech Connect

    Chudley, A.E.; McCullough, C.; McCullough, D.W.

    1997-01-31

    We identified a Canadian-Mennonite family in which a brother and sister have hydrocephalus due to obstruction at the foramen of Monro and profound bilateral sensorineural deafness. This appears to be a unique combination of anomalies and, to our knowledge, has not been reported previously. Both parents and a brother are phenotypically normal. The parents are second cousins. Thus, on the basis of consanguinity, affected sibs of both sexes, and in the absence of evidence for intrauterine infections or other adverse perinatal events, this syndrome is likely inherited in an autosomal recessive fashion. 37 refs., 5 figs.

  8. [Bilateral carotid dissection. A not to underestimate cause of neurological loss after road accident].

    PubMed

    Stock, K; Peters, S; Behrmann, C; Spielmann, R P

    2002-10-01

    The dissection of the internal carotid artery is a rare complication of acceleration traumas of the upper spine. 30% of these dissections are caused by road accidents and again less than 30% of these occur bilateral as shown here. The symptoms are fronto-temporal and periorbital starting headaches spreading out to the occiput and Horner's syndrome. Complete hemiplegia as in our case is an impressive exception but the doctor in attendance should think of the carotid dissection. The exclusion of this complication is obligatory because treatment and outcome depend on it.The dynamic effects of bilateral carotid dissections may, as shown here, lead to relapsing cerebral infarctions with persisting neurologic deficits up to manifest hemiparesis. But restitution can be accomplished if early diagnosed by DSA and/or MRI. Therapy of choice is early prevention of persisting neurologic deficits using effective dosed heparin and depending on the residual lumen of the vessel oral anticoagulants or platelet antagonists for one year. PMID:12376897

  9. Role of bilateral inferior petrosal sinus sampling (BIPSS) in the diagnosis of Cushing’s disease in a patient with double superior vena cava

    PubMed Central

    Tashi, Sonam; Ng, Keng Sin

    2015-01-01

    Cushing’s syndrome is known to have a wide spectrum of clinical presentation with debilitating consequences and morbidity if not diagnosed and treated in time. Sometimes the diagnosis of Cushing’s syndrome can be challenging to the endocrinologist, especially when the usual battery of biochemical tests and advanced cross-sectional imaging is negative or inconclusive. We described a case in which the use of bilateral inferior petrosal sinus sampling (BIPSS) was conclusive albeit being technically challenging (due to a rare incidental finding of double superior vena cava) and invasive in nature. PMID:26629301

  10. Bilateral benign paroxysmal positional vertigo following a tooth implantation.

    PubMed

    Kaplan, Daniel M; Attal, Uriel; Kraus, Mordechai

    2003-04-01

    Benign paroxysmal positional vertigo (BPPV) is a common cause of vertigo and may occur following recent head trauma. Bilateral involvement in BPPV is considered rare and has received little attention in the medical literature. We describe an unusual case of bilateral BPPV in a middle-aged woman that presented during a dental implantation, performed with the use of osteotomes. We discuss the diagnostic and therapeutic challenge of this entity. PMID:12816224

  11. Bilateral keratocystic odontogenic tumor: A report of two cases

    PubMed Central

    Srivatsan, K. S.; Kumar, Vikas; Mahendra, Ashish; Singh, Preeti

    2014-01-01

    The designation “keratocyst” was used to describe any jaw cyst in which keratin was formed to a large extent. A rare incidence of bilateral mandibular cysts (odontogenic keratocysts) was related to third molar teeth. Herein, we report two cases of bilateral keratocystic odontogenic tumor in a 22-year-old male and 15-year-old female, which was diagnosed by a series of investigations and treated appropriately. PMID:25298727

  12. Superior gluteal artery perforator flap in bilateral breast reconstruction.

    PubMed

    Werdin, Frank; Peek, Alberto; Martin, Nicolas C S; Baumeister, Steffen

    2010-01-01

    The incidence of bilateral breast reconstruction is increasing particularly due to genetic counseling and the option for bilateral prophylactic mastectomies. The decision to undergo a prophylactic mastectomy depends on the achievable outcomes of breast reconstruction. The free superior gluteal artery perforator flap (sGAP) flap is one option for autologous bilateral reconstruction which has rarely been reported.All bilateral sGAP flaps performed in the department of plastic surgery at the Behandlungszentrum Vogtareuth over a period of 4.5 years were retrospectively analyzed for indication, success rate, and complications.Thirty sGAP flaps were performed for bilateral breast reconstruction. The average age of the 15 women was 42 years and the average body mass index was 20.8. Indications for breast reconstruction were predominantly prophylactic mastectomies (60%). Indication for a sGAP flap was either a thin patient with insufficient abdominal tissue or a 2-staged bilateral reconstruction. 83% of the breast reconstructions were performed secondarily and 93% in 2 stages. The average operating time was 7 hours 12 minutes. Twenty-nine flaps (97%) were successful. Complications were fat necrosis (n = 3), hematoma (n = 3), and breast seroma (n = 1). Donor site complications were seroma (n = 8), infection (n = 1), and wound dehiscence (n = 1).Our results with bilateral breast reconstruction with the sGAP flap show cosmetically appealing results with high success and low complication rates on the breast. However, seromas on the donor site occurred in 27%. In addition, the sGAP flap is a technically demanding and time consuming operation. We recommend the sGAP flap when the abdomen has not enough tissue bulk to perform a deep inferior epigastric perforator flap or for a 2-staged bilateral reconstruction. This is often the case in women with a hereditary high risk of breast cancer who often present as young and slim patients. PMID:20010408

  13. [Bilateral hydatid cyst of the fallopian tubes: a case report].

    PubMed

    Ben Rejeb, C; Dhifallah, S; Bibi, M; Nouira, M; Memmi, A; Chaieb, A; Saidi, A; Khairi, H

    2001-02-01

    We report an exceptional case of bilateral hydatid cysts that developed solely in the fallopian tubes of a young girl. The diagnosis was suggested by the clinical history, the patient's residence in an endemic area, and the ultrasound findings and was confirmed peroperatively. Due to the extensive damage bilateral salpingectomy was required compromising future reproduction potential. We discuss the pathogenesis of pelvic localizations and the ultrasound aspects of pelvic hydatid cysts. PMID:11240508

  14. ERP correlates of the bilateral redundancy gain for words.

    PubMed

    Mohr, Bettina; Endrass, Tanja; Hauk, Olaf; Pulvermüller, Friedemann

    2007-05-15

    Neurophysiological correlates of hemispheric asymmetry and interhemispheric interaction in lexical processing were investigated in a lexical decision task with tachistoscopic stimulus presentation either unilaterally, to the right or left visual field, or bilaterally, with identical stimulus copies to each visual hemi-field. Behavioral data confirmed both right visual field advantage and bilateral redundancy gain for words but not for pronounceable orthographically regular pseudowords. ERPs showed a significant amplitude increase 160-200 after stimulus presentation specifically for words after bilateral redundant stimulation, which was present in the recordings from both hemispheres. Localization of cortical sources using minimum norm estimation indicated stronger cortical activity for words in temporal regions of both hemispheres after bilateral presentation compared with each of the unilateral stimulation conditions individually. Pseudoword presentation did not lead to a general increase of cortical activation in the bilateral condition compared with unilateral presentation. The specific activation increase for words in the bilateral redundant condition relative to unilateral stimulation and the absence of this effect for pseudowords, which became manifest in a significant interaction of the factors lexicality and presentation mode, is best explained by summation of neuronal activation from both hemispheres within distributed lexical circuits. Source estimation indicates that temporal areas, particularly in the left hemisphere, are the primary cortical loci where such stimulus-specific activity increases occurred. PMID:17336345

  15. Proteus Syndrome: Report of a Case with Developmental Glaucoma

    PubMed Central

    Yuksel, Nursen; Sarman, Hakan; Bayramgurler, Dilek

    2014-01-01

    The purpose of this study was to report developmental glaucoma and pseudopapilledema in a patient with Proteus syndrome. We defined the presence of developmental glaucoma, right pseudopapilledema and myopia in a 4.5-year-old patient with Proteus syndrome. Marked right hemihypertrophy, lipoma, macrodactyly, and asymmetry of the limbs were observed on systemic examination. A cavernoma was also detected in magnetic resonance imaging of the brain. The patient underwent bilateral goniotomy surgery due to glaucoma. The surgical outcomes were satisfactory in both eyes. In conclusions developmental glaucoma and pseudopapilledema might be associated with Proteus syndrome. PMID:24882963

  16. Proteus syndrome: report of a case with developmental glaucoma.

    PubMed

    Sarman, Zuleyha Sik; Yuksel, Nursen; Sarman, Hakan; Bayramgurler, Dilek

    2014-06-01

    The purpose of this study was to report developmental glaucoma and pseudopapilledema in a patient with Proteus syndrome. We defined the presence of developmental glaucoma, right pseudopapilledema and myopia in a 4.5-year-old patient with Proteus syndrome. Marked right hemihypertrophy, lipoma, macrodactyly, and asymmetry of the limbs were observed on systemic examination. A cavernoma was also detected in magnetic resonance imaging of the brain. The patient underwent bilateral goniotomy surgery due to glaucoma. The surgical outcomes were satisfactory in both eyes. In conclusions developmental glaucoma and pseudopapilledema might be associated with Proteus syndrome. PMID:24882963

  17. Bilateral osteochondritis dissecans of the lateral femoral condyle following bilateral total removal of lateral discoid meniscus: a case report.

    PubMed

    Hashimoto, Yusuke; Yoshida, Gen; Tomihara, Tomohiro; Matsuura, Takeshi; Satake, Shinji; Kaneda, Kunikazu; Shimada, Nagakazu

    2008-11-01

    Osteochondritis dissecans (OCD) of the lateral femoral condyle sometimes occurs with a discoid lateral meniscus. Recently, it was reported that OCD of the lateral femoral condyle occurred after total removal of the lateral meniscus. We report the case of a 12-year-old boy with bilateral OCD of the lateral femoral condyle following bilateral total removal for discoid lateral meniscus. Valgus deviation of the knee after total removal and increased sporting activity might have concentrated excessive stress on the lateral condyles in the standing position. As a result, bilateral OCD might have occurred. Drilling of the areas of OCD on the bilateral lateral femoral condyles was done and the patient wore inner wedge arch supports postoperatively. After 2 years, neither knee pain nor arthrosis has occurred so far, but long-term follow-up of this patient is considered to be necessary. PMID:17985146

  18. Impaired Sertoli cell function in males diagnosed with Noonan syndrome.

    PubMed

    Marcus, K A; Sweep, C G J; van der Burgt, I; Noordam, C

    2008-11-01

    In order to study male gonadal function in Noonan syndrome, clinical and laboratory data, including inhibin B, were gathered in nine pubertal males diagnosed with Noonan syndrome. Bilateral testicular maldescent was observed in four, and unilateral cryptorchidism occurred in two. Puberty was delayed in three patients. Luteinising hormone (LH) levels were normal in all patients in our series, while follicle stimulating hormone (FSH) levels were raised in seven. Inhibin B was low in six males and just above the lower limit of normal in two. Importantly, all three men with normal testicular descent displayed signs of Sertoli cell dysfunction, indicating, in contrast to earlier reports, that bilateral cryptorchidism does not seem to be the main contributing factor to impairment of testicular function in Noonan syndrome. These findings suggest different mechanisms of disturbance in male gonadal function, which is frequently associated with Sertoli dysfunction. PMID:19189703

  19. EphA4-Mediated Ipsilateral Corticospinal Tract Misprojections Are Necessary for Bilateral Voluntary Movements But Not Bilateral Stereotypic Locomotion

    PubMed Central

    Serradj, Najet; Paixão, Sónia; Sobocki, Tomasz; Feinberg, Mitchell; Klein, Rüdiger; Kullander, Klas

    2014-01-01

    In this study, we took advantage of the reported role of EphA4 in determining the contralateral spinal projection of the corticospinal tract (CST) to investigate the effects of ipsilateral misprojections on voluntary movements and stereotypic locomotion. Null EphA4 mutations produce robust ipsilateral CST misprojections, resulting in bilateral corticospinal tracts. We hypothesize that a unilateral voluntary limb movement, not a stereotypic locomotor movement, will become a bilateral movement in EphA4 knock-out mice with a bilateral CST. However, in EphA4 full knock-outs, spinal interneurons also develop bilateral misprojections. Aberrant bilateral spinal circuits could thus transform unilateral corticospinal control signals into bilateral movements. We therefore studied mice with conditional forebrain deletion of the EphA4 gene under control by Emx1, a gene expressed in the forebrain that affects the developing CST but spares brainstem motor pathways and spinal motor circuits. We examined two conditional knock-outs targeting forebrain EphA4 during performance of stereotypic locomotion and voluntary movement: adaptive locomotion over obstacles and exploratory reaching. We found that the conditional knock-outs used alternate stepping, not hopping, during overground locomotion, suggesting normal central pattern generator function and supporting our hypothesis of minimal CST involvement in the moment-to-moment control of stereotypic locomotion. In contrast, the conditional knock-outs showed bilateral voluntary movements under conditions when single limb movements are normally produced and, as a basis for this aberrant control, developed a bilateral motor map in motor cortex that is driven by the aberrant ipsilateral CST misprojections. Therefore, a specific change in CST connectivity is associated with and explains a change in voluntary movement. PMID:24719100

  20. Acro-dermato-ungual-lacrimal-tooth (ADULT) syndrome: report of a child with phenotypic overlap with ulnar-mammary syndrome and a new mutation in TP63.

    PubMed

    Slavotinek, Anne M; Tanaka, June; Winder, Alison; Vargervik, Karin; Haggstrom, Anita; Bamshad, Michael

    2005-10-01

    We report on a new patient with clinical findings consistent with acro-dermato-ungual-lacrimal-tooth (ADULT) syndrome. The child had sparse hair, extensive freckling, lacrimal duct stenosis, oligodontia, dystrophic nails, reduced sweating, and bilateral athelia. Examination of his hands showed ulnar ray hypoplasia with bilateral fifth finger brachydactyly and camptodactyly. He also had surgical repair of an imperforate anus. Mutation analysis of TP63 showed a single nucleotide substitution, c.G518A, predicting a novel missense mutation, p.V114M in exon 4. This is the third mutation to be reported in TP63 in ADULT syndrome. PMID:16114047

  1. Acquired retinal pigmentary degeneration in a child with 13q deletion syndrome.

    PubMed

    Aguilera, Zenia P; Belin, Peter J; Cavuoto, Kara M; Jayakar, Parul; McKeown, Craig A

    2015-10-01

    Orbeli syndrome, or 13q deletion syndrome, is a rare condition caused by a distal deletion in the long arm of chromosome 13. The syndrome is characterized by severe physical malformations and developmental delays and has been associated with numerous ocular manifestations. We report the case of a 10-year-old boy with 13q deletion syndrome, who was evaluated for impaired vision and found to have bilateral retinal pigmentary changes resembling those seen in retinitis pigmentosa. There has only been one other case of retinal pigment variation in association with 13q deletion syndrome; however, this represents the first case of bilateral symmetric retinal pigmentary changes with corresponding rod and cone dysfunction on electroretinography. PMID:26486039

  2. Bilateral femoral neck fractures secondary to chronic carbamazepine use treated by bilateral dynamic hip screw: A case report

    PubMed Central

    Sariyilmaz, Kerim; Gulenc, Baris; Ozkunt, Okan; Dikici, Fatih; Yazicioglu, Onder

    2014-01-01

    Introduction Bilateral femoral neck fractures without major trauma are rare and related to several conditions. Insufficiency fractures due to the use of anti-epileptic drug are one of the rare causes. This case study is about bilateral femoral neck insufficiency fractures resulting from chronic use of anti-epileptic drug. Presentation of case A 26-year-old woman was referred to our emergency department with a complaint of bilateral groin pain and a 12-year history of irregular carbamazepine use. The diagnosis was bilateral femoral neck insufficiency fractures due to irregular long-term carbamazepine use. One-stage bilateral dynamic hip screw osteosynthesis was performed. After 2 years of follow up, good result was obtained. Discussion There are several risk factors for insufficiency fracture, and antiepileptic drug related osteoporosis is one of the reason. These drugs have negative effect on bone methabolism and bone mineral density. Conclusion To our knowledge, this is the first case in the literature of bilateral femoral neck insufficiency fracture due to chronic carbamazepine use. Joint and bone pain with a history of long-term use of anti-epileptic drug should be investigated carefully, and insufficiency fractures should be kept in mind. PMID:25528039

  3. Moebius syndrome and narcolepsy: A case dissertation☆

    PubMed Central

    Sabaneeff, Lídia; Mendes Motta, Henrique; Castro, Juliana; Tufik, Sergio; Santos Coelho, Fernando Morgadinho

    2014-01-01

    Moebius syndrome (MS) is a congenital syndrome characterized by unilateral or bilateral aplasia of the VI and VII cranial nerves, with consequent convergent strabismus and bilateral peripheral facial paralysis. This syndrome might be associated with diurnal excessive sleepiness and muscular hypotony, mimetizing in this manner, narcolepsy. The diagnostic criteria for narcolepsy depend on the presence of REM sleep during the day. As with patients with MS we do not have ocular movements due to the VI nerve paralysis, the absence of horizontal ocular movements might make it difficult to confirm narcolepsy in these patients. The common clinical characteristics of these patients are due to a possible impairment of the same structures that are affected in the central nervous system. However, the mechanism by which it occurs remains to be fully understood. Further electrophysiological researches are necessary to better clarify the association of these two diseases. The objective of this dissertation is to describe and discuss a case of Moebius syndrome with diurnal excessive sleepiness as a differential diagnosis for narcolepsy. PMID:26483899

  4. Moebius syndrome and narcolepsy: A case dissertation.

    PubMed

    Sabaneeff, Lídia; Mendes Motta, Henrique; Castro, Juliana; Tufik, Sergio; Santos Coelho, Fernando Morgadinho

    2014-03-01

    Moebius syndrome (MS) is a congenital syndrome characterized by unilateral or bilateral aplasia of the VI and VII cranial nerves, with consequent convergent strabismus and bilateral peripheral facial paralysis. This syndrome might be associated with diurnal excessive sleepiness and muscular hypotony, mimetizing in this manner, narcolepsy. The diagnostic criteria for narcolepsy depend on the presence of REM sleep during the day. As with patients with MS we do not have ocular movements due to the VI nerve paralysis, the absence of horizontal ocular movements might make it difficult to confirm narcolepsy in these patients. The common clinical characteristics of these patients are due to a possible impairment of the same structures that are affected in the central nervous system. However, the mechanism by which it occurs remains to be fully understood. Further electrophysiological researches are necessary to better clarify the association of these two diseases. The objective of this dissertation is to describe and discuss a case of Moebius syndrome with diurnal excessive sleepiness as a differential diagnosis for narcolepsy. PMID:26483899

  5. Nonlethal multiple pterygium syndrome: Escobar syndrome.

    PubMed

    Bissinger, Robin L; Koch, Frances R

    2014-02-01

    Nonlethal Escobar is a rare disorder that is a variant of multiple pterygium syndromes. It is a form of arthrogryposis multiplex congenita characterized by excessive webbing (pterygia), congenital contractures (arthrogryposis), and scoliosis. It is usually diagnosed in utero on fetal ultrasound and then confirmed in the neonatal period. A case of nonlethal neonatal Escobar is reported in a 35-week-and-6-day old infant who presented in utero with decreased fetal movement, oligohydramnios, and arthrogryposis. The etiologies from maternal causes were excluded prior to birth. Subsequent workup after birth led to a highly suspected diagnosis of nonlethal Escobar by the geneticist. The diagnosis was confirmed by a positive CHRNG gene sequence analysis after discharge. The infant demonstrated contractures and bilateral hip subluxation but was feeding well and was discharged home with outpatient follow-up. Treatment after discharge has been extensive secondary to difficulties associated with this disease. The clinical presentation of nonlethal Escobar, as well as diagnosis and treatment strategies, is provided with caregiving strategies. PMID:24472885

  6. Gorlin syndrome.

    PubMed

    Devi, Basanti; Behera, Binodini; Patro, Sibasish; Pattnaik, Subhransu S; Puhan, Manas R

    2013-05-01

    Gorlin Syndrome, a rare genodermatosis, otherwise known as Nevoid basal cell carcinoma syndrome (NBCCS) is a multisystem disease affecting skin, nervous system, eyes, endocrine glands, and bones. It is characterized by multiple basal cell carcinomas, palmoplantar pits, jaw cysts, and bony deformities like kyphoscoliosis and frontal bossing. We would like to report a case of Gorlin syndrome with classical features, as this is a rare genodermatosis. PMID:23723494

  7. Single-Session Radiofrequency Ablation of Bilateral Lung Metastases

    SciTech Connect

    Palussiere, Jean Gomez, Fernando; Cannella, Matthieu; Ferron, Stephane; Descat, Edouard; Fonck, Marianne; Brouste, Veronique; Avril, Antoine

    2012-08-15

    Purpose: This retrospective study examined the feasibility and efficacy of bilateral lung radiofrequency ablation (RFA) performed in a single session. Methods: From 2002-2009, patients with bilateral lung metastases were treated by RFA, where possible in a single session under general anesthesia with CT guidance. The second lung was punctured only if no complications occurred after treatment of the first lung. Five lung metastases maximum per patient were treated by RFA and prospectively followed. The primary endpoint was the evaluation of acute and delayed complications. Secondary endpoints were calculation of hospitalization duration, local efficacy, median survival, and median time to tumor progression. Local efficacy was evaluated on CT or positron emission tomography (PET) CT. Results: Sixty-seven patients were treated for bilateral lung metastases with RFA (mean age, 62 years). Single-session treatment was not possible in 40 due to severe pneumothoraces (n = 24), bilateral pleural contact (n = 14), and operational exclusions (n = 2). Twenty-seven (41%) received single-session RFA of lesions in both lungs for 66 metastases overall. Fourteen unilateral and four bilateral pneumothoraces occurred (18 overall, 66.7%). Unilateral (n = 13) and bilateral (n = 2) chest tube drainage was required. Median hospitalization was 3 (range, 2-8) days. Median survival was 26 months (95% confidence interval (CI), 19-33). Four recurrences on RFA sites were observed (4 patients). Median time to tumor progression was 9.5 months (95% CI, 4.2-23.5). Conclusions: Although performing single-session bilateral lung RFA is not always possible due to pneumothoraces after RFA of first lung, when it is performed, this technique is safe and effective.

  8. Persistent Mullerian Duct Syndrome with Embryonal Cell Carcinoma along with Ectopic Cross Fused Kidney

    PubMed Central

    Bharath, NR Manju; Narayana, V; Raja, V Om Pramod Kumar; Jambula, Pranav Reddy

    2016-01-01

    Persistent Mullerian Duct Syndrome (PMDS) is a form of internal male pseudohermaphroditism, where there is normal development of male secondary sexual characters, along with the presence of bilateral fallopian tubes and uterus. Majority of these cases go undetected and some cases are accidentally diagnosed while investigating for other problems. Cross fused renal ectopia is a condition where one kidney lies in the opposite side, fused to the other kidney. We present an extremely rare case of a phenotypical male presenting with mass per abdomen and bilateral cryptorchidism, turned out to have uterus with bilateral fallopian tubes, ectopic cross fused right kidney and Embryonal cell carcinoma of left undescended testis. PMID:26894123

  9. Irritable Bowel Syndrome

    MedlinePLUS

    ... How Can I Help a Friend Who Cuts? Irritable Bowel Syndrome KidsHealth > For Teens > Irritable Bowel Syndrome Print ... intestinal disorder called irritable bowel syndrome. What Is Irritable Bowel Syndrome? Irritable bowel syndrome (IBS) is a common ...

  10. Bilateral parotid enlargement due to malnutrition under the influence of the media in an adolescent in Lithuania.

    PubMed

    Mieliauskaite, Diana; Venalis, Algirdas; Graziene, Vida; Kirdaite, Gailute

    2007-07-01

    The elimination of censorship for the media in post-communist countries in transition has contributed to increases in the prevalence of several medical problems. Children and adolescents are particularly vulnerable to the messages conveyed through the media, which influence their perceptions and behaviour. We describe a case of bilateral parotid enlargement due to malnutrition under the influence of self-prescribed diet in an adolescent. A 15-year-old girl reported to our institution under suspicion of Sjögren's syndrome for medical advice. Two months ago she developed persistent bilateral parotid enlargement and a dry mouth. Her medical history revealed a weight loss due to "self-prescribed" reduce diet. Social questioning clarified high use of the media and influence on the body concept and self image. On extra oral examination, a diffuse parotid enlargement was seen bilaterally. The examination of the mouth showed a low moisture level of the intraoral mucosa. The unstimulated whole salivary flow rate was 2 ml in 15 min. Laboratory findings evidenced anemia (107 g/l). The serum albumin concentration indicated a reduced level (28 g/l). Search for antinuclear antibodies, anti-SSA antibodies, anti-SSB, -Sm, -RNP and anti-double-stranded DNA antibodies was negative. Evaluation for antibodies against hepatitis C, cytomegalovirus and Epstein-Barr virus infection and HIV rendered negative results. A histopathologic examination of labial salivary gland biopsy revealed a picture of sialoadenosis. From the above investigations, a diagnosis of sialoadenosis due to malnutrition was made. PMID:17198741

  11. Bilateral filtering of diffusion tensor magnetic resonance images.

    PubMed

    Hamarneh, Ghassan; Hradsky, Judith

    2007-10-01

    We extend the well-known scalar image bilateral filtering technique to diffusion tensor magnetic resonance images (DTMRI). The scalar version of bilateral image filtering is extended to perform edge-preserving smoothing of DT field data. The bilateral DT filtering is performed in the Log-Euclidean framework which guarantees valid output tensors. Smoothing is achieved by weighted averaging of neighboring tensors. Analogous to bilateral filtering of scalar images, the weights are chosen to be inversely proportional to two distance measures: The geometrical Euclidean distance between the spatial locations of tensors and the dissimilarity of tensors. We describe the noniterative DT smoothing equation in closed form and show how interpolation of DT data is treated as a special case of bilateral filtering where only spatial distance is used. We evaluate different recent DT tensor dissimilarity metrics including the Log-Euclidean, the similarity-invariant Log-Euclidean, the square root of the J-divergence, and the distance scaled mutual diffusion coefficient. We present qualitative and quantitative smoothing and interpolation results and show their effect on segmentation, for both synthetic DT field data, as well as real cardiac and brain DTMRI data. PMID:17926929

  12. Successful management of bilateral patellar tendon rupture in a dog.

    PubMed

    Shipov, A; Shahar, R; Joseph, R; Milgram, J

    2008-01-01

    A seven-year-old, 41 kg, intact, cross breed dog, was presented with a history of bilateral hind limb lameness after falling from a height of 1 m. Clinical and radiographic findings were consistent with bilateral patellar tendon rupture. Surgical repair was performed bilaterally. The tendons were sutured primarily, and an internal splint of nylon leader was added. Good apposition of the severed tendon ends had been achieved intraoperatively; however, post operative radiographs showed supra-trochlear displacement of both patellae. The casts used to immobilize the stifle joints slipped distally and three days post operatively the tendon repair had broken down, bilaterally. Revision surgery was undertaken and the tendons were re-sutured. Nylon leader was placed through holes that had been drilled in the patellae and tibiae. The stifle joints were immobilized with type I external skeletal fixators (ESFs). Both freeform polymethylmethacrylate (PMMA) connecting bars were found to be broken at the level of the stifle joints two days later, without any disruption of the primary tendon repair. Each connecting bar was replaced with two connecting bars of PMMA reinforced with 3 mm steel wire. The dog was fully weight-bearing with a reduced range of motion in flexion immediately after removal of the ESFs at six weeks and was still sound 18 months post-operatively. Primary tendon repair in combination with adequate immobilization allowed for an excellent outcome in a complicated bilateral pathology. PMID:18545725

  13. Directional bilateral filters for smoothing fluorescence microscopy images

    NASA Astrophysics Data System (ADS)

    Venkatesh, Manasij; Mohan, Kavya; Seelamantula, Chandra Sekhar

    2015-08-01

    Images obtained through fluorescence microscopy at low numerical aperture (NA) are noisy and have poor resolution. Images of specimens such as F-actin filaments obtained using confocal or widefield fluorescence microscopes contain directional information and it is important that an image smoothing or filtering technique preserve the directionality. F-actin filaments are widely studied in pathology because the abnormalities in actin dynamics play a key role in diagnosis of cancer, cardiac diseases, vascular diseases, myofibrillar myopathies, neurological disorders, etc. We develop the directional bilateral filter as a means of filtering out the noise in the image without significantly altering the directionality of the F-actin filaments. The bilateral filter is anisotropic to start with, but we add an additional degree of anisotropy by employing an oriented domain kernel for smoothing. The orientation is locally adapted using a structure tensor and the parameters of the bilateral filter are optimized for within the framework of statistical risk minimization. We show that the directional bilateral filter has better denoising performance than the traditional Gaussian bilateral filter and other denoising techniques such as SURE-LET, non-local means, and guided image filtering at various noise levels in terms of peak signal-to-noise ratio (PSNR). We also show quantitative improvements in low NA images of F-actin filaments.

  14. Unilateral versus Bilateral Instrumentation in Spinal Surgery: A Systematic Review

    PubMed Central

    Molinari, Robert W.; Saleh, Ahmed; Molinari, Robert; Hermsmeyer, Jeff; Dettori, Joseph R.

    2015-01-01

    Study Design Systematic review. Clinical Questions (1) What is the comparative efficacy of unilateral instrumentation compared with bilateral instrumentation in spine surgery? (2) What is the safety of unilateral instrumentation compared with bilateral instrumentation in spine surgery? Methods Electronic databases and reference lists of key articles were searched up to September 30, 2014, to identify studies reporting the comparative efficacy and safety of unilateral versus bilateral instrumentation in spine surgery. Studies including recombinant human bone morphogenetic protein 2 as adjunct therapy and those with follow-up of less than 2 years were excluded. Results Ten randomized controlled trials met the inclusion criteria: five compared unilateral with bilateral instrumentation using open transforaminal or posterior lumbar interbody fusion (TLIF/PLIF), one used open posterolateral fusion, and four used minimally invasive TLIF/PLIF. There were no significant differences between unilateral and bilateral screw instrumentation with respect to nonunion, low back or leg pain scores, Oswestry Disability Index, reoperation, or complications. Conclusions The existing literature does not identify significant differences in clinical outcomes, union rates, and complications when unilateral instrumentation is used for degenerative pathologic conditions in the lumbar spine. The majority of published reports involve single-level lumbar unilateral instrumentation. PMID:26131385

  15. Asymmetric pelvic and hip rotation in children with bilateral cerebral palsy: uni- or bilateral femoral derotation osteotomy?

    PubMed

    Niklasch, M; Döderlein, L; Klotz, M C; Braatz, F; Wolf, S I; Dreher, T

    2015-02-01

    Internal rotation gait is common among children with bilateral cerebral palsy. However, despite bilaterally increased femoral anteversion asymmetric internal rotation gait is often found. Femoral derotation osteotomy (FDO) is commonly performed bilaterally. Variable functional outcomes are reported especially in cases with mild internal hip rotation during gait and abnormal preoperative pelvic rotation. A major question is if a unilateral treatment of the more involved side in asymmetric cases leads to a comparable or even superior outcome. One hundred and nine children with spastic bilateral CP treated with FDO with pre- and 1-year postoperative 3D gait analysis were retrospectively collected. The asymmetry was calculated from the preoperative difference between both limbs in hip rotation obtained by 3D gait analysis. Twenty-eight children with asymmetry larger than 20° were selected and classified into two groups, according to whether they obtained a unilateral or bilateral FDO. Preoperative clinical examination and pre- and postoperative hip and pelvic rotation in gait analysis on the more and the less involved side did not differ significantly between both groups. Interestingly, in both groups, hip rotation did not change significantly in less-involved limbs, although intraoperative derotation averaged 25°. After unilateral FDO a significant change in pelvic rotation resulted, whereas this was not found after bilateral FDO. The results of this study suggest that unilateral FDO in children with asymmetric internal rotation gait leads to a comparable functional outcome compared to bilateral treatment. Furthermore, it was shown for the first time that considering the asymmetry has a positive effect on pelvic rotation. PMID:25698350

  16. Ruptured celiac artery aneurysm mimicking Boerhaave syndrome.

    PubMed

    Liu, Chien-Yu; Yang, Kai-Wei; Chen, Wei-Kung; Huang, Chen-Hsiung

    2013-09-01

    Ruptured celiac artery aneurysm is a rare cause for epigastric pain and is usually detected incidentally. Atypical presentation with postemetic epigastralgia and pleural effusion usually leads physicians to make the diagnosis of Boerhaave syndrome. Herein, we report a 32-year-old woman who was diagnosed with Boerhaave syndrome initially after presenting with acute postemetic epigastralgia and predominant left side pleural effusion. Diagnostic left thoracentesis yielded bloody fluid with similar amylase level to serum. The chest computed tomographic scan showed no evidence of esophageal rupture. However, a ruptured celiac artery aneurysm with retroperitoneal hematoma extending to the posterior mediastinum and bilateral pleural space was found incidentally. Although ruptured celiac artery aneurysm is an uncommon cause for postemetic epigastralgia, acute vascular events such as the previously stated cause should be the first impression rather than Boerhaave syndrome if the patient also presents with isolated pleural effusion containing unelevated amylase. PMID:23773770

  17. Nystagmus in laurence-moon-biedl syndrome.

    PubMed

    Janati, A Bruce; ALGhasab, Naif Saad; Haq, Fazal; Abdullah, Ahmad; Osman, Aboubaker

    2015-01-01

    Introduction. Laurence-Moon-Biedl (LMB) syndrome is a rare autosomal-recessive ciliopathy with manifold symptomatology. The cardinal clinical features include retinitis pigmentosa, obesity, intellectual delay, polydactyly/syndactyly, and hypogenitalism. In this paper, the authors report on three siblings with Laurence-Moon-Biedl syndrome associated with a probable pseudocycloid form of congenital nystagmus. Methods. This was a case study conducted at King Khaled Hospital. Results. The authors assert that the nystagmus in Laurence-Moon-Biedl syndrome is essentially similar to idiopathic motor-defect nystagmus and the nystagmus seen in optic nerve hypoplasia, ocular albinism, and bilateral opacities of the ocular media. Conclusion. The data support the previous hypothesis that there is a common brain stem motor abnormality in sensory-defect and motor-defect nystagmus. PMID:25984376

  18. Nystagmus in Laurence-Moon-Biedl Syndrome

    PubMed Central

    Janati, A. Bruce; ALGhasab, Naif Saad; Haq, Fazal; Abdullah, Ahmad; Osman, Aboubaker

    2015-01-01

    Introduction. Laurence-Moon-Biedl (LMB) syndrome is a rare autosomal-recessive ciliopathy with manifold symptomatology. The cardinal clinical features include retinitis pigmentosa, obesity, intellectual delay, polydactyly/syndactyly, and hypogenitalism. In this paper, the authors report on three siblings with Laurence-Moon-Biedl syndrome associated with a probable pseudocycloid form of congenital nystagmus. Methods. This was a case study conducted at King Khaled Hospital. Results. The authors assert that the nystagmus in Laurence-Moon-Biedl syndrome is essentially similar to idiopathic motor-defect nystagmus and the nystagmus seen in optic nerve hypoplasia, ocular albinism, and bilateral opacities of the ocular media. Conclusion. The data support the previous hypothesis that there is a common brain stem motor abnormality in sensory-defect and motor-defect nystagmus. PMID:25984376

  19. Renal failure in Hay-Wells syndrome.

    PubMed

    Mohan, Dhanya; Sugathan, Dileep K; Railey, Mohammed; Alrukhaimi, Mona

    2013-11-01

    Ectodermal dysplasia (ED) is a group of disorders involving developmental defects of the ectodermal structures, with abnormalities in the skin, hair, nails and sweat glands. Ankyloblepharon - ectodermal defects-cleft lip/palate (AEC) syndrome, also known as Hay-Wells syndrome, is characterized by eyelid fusion, ED and cleft lip/palate. We report a 15-year-old Omani boy with AEC syndrome who presented with end-stage renal disease (ESRD) requiring renal replacement therapy. Genitourinary anomalies including hypospadiasis, posterior urethral stricture and bilateral vesicoureteric reflux were noted. These led to secondary focal segmental glomerulosclerosis and ESRD. Screening for genitourinary anomalies and renal function tests must be performed in all patients with ED to facilitate early detection of renal disease and appropriate management. PMID:24231488

  20. Intraocular Lens Subluxation in Marfan Syndrome

    PubMed Central

    Rodrigo, Bolaños-Jiménez; Paulina, López-Lizárraga E; Francesc, March de R; Eduardo, Telich-Tarriba J; Alejandro, Navas

    2014-01-01

    Purpose : Ectopia lentis (EL) is a major criteria for the diagnosis of Marfan syndrome, it may vary from an asymptomatic mild displacement to a significant subluxation that places the equator of the lens in the pupillary axis. The purpose of this work is to present the case of a patient with Marfan syndrome who received treatment for subluxation at our institution. Case Report : A 51-year-old female diagnosed with Marfan syndrome presented to the emergency department with bilateral eye redness, foreign body sensation and crusting around the eyes on awakening. She had the following history of cardiac and ophthalmologic complications, including: 1. Lens subluxation 2. High myopia 3. Aortic root dilation, 4. Mitral valve prolapse and 5. Tricuspid insufficiency. Conclusion : The ophthalmological management of Marfan patients is challenging and periodical follow-up is needed. Surgical versus conservative management is controversial, each case needs to be evaluated individually to analyze the risks and benefits of the procedures. PMID:25279020

  1. [Shy-Drager syndrome].

    PubMed

    Miron, E; Yagana, S; Ben-Asouli, S

    1995-02-01

    An 81-year-old man with a history of chronic pulmonary disease due to heavy smoking and ischemic heart disease had been suffering for the past few years from chronic constipation and urinary incontinence and was receiving medication for cardiopulmonary symptoms and urinary incontinence. He was admitted for repeated falling for a few months prior to admission. When put in the supine position, his blood pressure fell. He had bilateral pulmonary rales, consistent with lung disease, eccentricity of the left pupil (after cataract surgery), constriction of the right pupil, and absence of the pupillary light reflex. There was generalized hyperreflexia and a bilateral Babinski sign. He had normocytic, normochromic anemia; B12, folic acid and ferritin were within normal ranges, ESR was rapid, there was hyperglobulinemia (IgA and IgG), urea nitrogen and creatinine were increased but returned to normal after rehydration. ECG and chest X-ray were consistent with his cardiopulmonary status. Bone-marrow biopsy showed hypocellularity. IVP and barium enema were normal. Echocardiography revealed a possible old posterior wall myocardial infarction. CT-scan showed moderate cerebral and cerebellar atrophy, calcifications in the carotid and vertebral arteries, and small infarcts in both hemispheres. At this point, after an extensive survey of the literature, the diagnosis of Shy-Drager syndrome was proposed and proved by monitoring ECG and serum levels of noradrenaline during postural changes. He was treated with Fluorinef and there were no more episodes of postural hypotension. Several weeks after discharge he reported that he was feeling well and had not fallen since discharge. PMID:7759002

  2. A Case of Neuromyelitis Optica Masquerading as Miller Fisher Syndrome

    PubMed Central

    Furutani, Yuka; Hata, Masayuki; Miyamoto, Kazuaki; Moribata, Yusaku; Yoshimura, Nagahisa

    2014-01-01

    A 22-year-old woman presented with double vision that she had experienced since an infection 2 weeks previously. A neurological examination showed limited bilateral eye abduction, mimicking Miller Fisher syndrome. However, T2-weighted magnetic resonance imaging of her brain revealed hyperintense areas in the tegmentum of the pons, including the abducens nucleus, and her serum anti-aquaporin-4 antibody test was positive. She was finally diagnosed with neuromyelitis optica. Intravenous high-dose steroid therapy immediately improved the patient's abduction palsy, but bilateral optic neuritis manifested during the treatment. Subsequent treatment with plasma exchange improved her optic neuritis symptoms. PMID:25408664

  3. Reversible man-in-the-barrel syndrome in myasthenia gravis

    PubMed Central

    Shah, Poornima A; Wadia, Pettarusp Murzban

    2016-01-01

    Man-in-the-barrel syndrome (MBS) is an uncommon presentation due to bilateral, predominantly proximal muscle weakness that has not been described to be associated with myasthenia gravis. We describe a case of myasthenia gravis presenting as MBS. Additionally, he had significant wasting of the deltoids bilaterally with fibrillations on electromyography (EMG) at rest and brief duration (3-6 ms) bi/triphasic motor unit potentials (MUPs) on submaximal effort apart from a decremental response on repetitive nerve stimulation (RNS) at 2 Hz. While electrophysiology is an important tool in the diagnosis of myasthenia gravis, pathological EMG patterns do not exclude the diagnosis of myasthenia gravis. PMID:27011638

  4. [A case of epilepsy with ring chromosome 20 syndrome].

    PubMed

    Tanaka, Atsuko; Ohtake, Michiko; Yoshimi, Tamami; Suzuki, Tsuneo; Abe, Ikuro; Iwasaki, Hiroshi; Sue, Hironari; Kaito, Ken

    2012-09-01

    A case of epilepsy associated with ring chromosome 20 [r(20)] syndrome was first reported in 1972. Relatively few cases of [r(20)] syndrome have been reported. We report here a middle-aged female with this syndrome. She had two types of seizures characterized by complex partial seizure with complex motor automatism mainly occurred while asleep and episodes of fluctuating consciousness (non-convulsive status epilepticus) lasting 40-60 min. The ictal electroencephalography (EEG) findings of the latter showed almost continuous diffuse spike and wave complexes or high voltage slow waves. The interictal EEG findings showed spike or sharp waves located in bilateral frontal regions or 3-6Hz diffuse high voltage slow waves intermingled with spikes in left posterior areas. We reviewed and discussed the characteristics of [r(20)] syndrome reported in the literature, intractable seizures, behavioral problems and some degree of mental retardation or dysmorphism, especially variable EEG findings characterized this syndrome. PMID:23157113

  5. Orthopedic Manifestations of Mobius Syndrome: Case Series and Survey Study

    PubMed Central

    McClure, Philip; Booy, David; Katarincic, Julia; Eberson, Craig

    2016-01-01

    Background. Mobius Syndrome is a rare disease defined by bilateral congenital 7th nerve palsy. We focus on reporting the prevalence of orthopedic disease in this population. Methods. Twenty-three individuals with Mobius Syndrome underwent orthopedic physical examination, and additional 96 patients filled out a survey for self-reported orthopedic diagnoses. Results. Clubfoot was present in 60% of individuals in the physical exam series and 42% of those in the survey. Scoliosis was present in 26% and 28%, respectively. Poland's Syndrome was present in 17% and 30%. In addition to these findings, 27% of patients reported having difficulty with anesthesia, including difficulty in intubation and airway problems. Conclusion. An increased prevalence of scoliosis, clubfoot, transverse limb deficiencies, and Poland's Syndrome is identified in the setting of Mobius Syndrome. In the setting of several deformities often requiring surgical correction, a high incidence of anesthetic difficulty is noted and should be discussed with patients and other providers during surgical planning. PMID:26977161

  6. Baller-Gerold syndrome associated with congenital portal venous malformation.

    PubMed Central

    Savarirayan, R; Tomlinson, P; Thompson, E

    1998-01-01

    We report a 4 year old boy in whom the clinical features of craniosynostosis and bilateral absent radii led to a diagnosis of Baller-Gerold syndrome. Additional congenital abnormalities included midface hypoplasia, atrial and ventricular septal defects, right hydronephrosis, partial sacral agenesis, and anterior ectopic anus. Evidence of portal venous hypertension was present from 8 months and a congenital portal venous malformation was discovered at 2 years. This is the first reported case of Baller-Gerold syndrome associated with a congenital portal venous malformation. We discuss the diagnostic confusion between this syndrome and other overlapping malformation syndromes and propose optimal evaluation strategies aimed at clarifying the nosology of these syndromes. Images PMID:9733037

  7. Prostatitis Syndromes

    PubMed Central

    Nickel, J. Curtis

    1991-01-01

    The many prostatitis syndromes remain a frustrating enigma to family physicians as well as specialists. An understanding of the etiology and pathophysiology of these syndromes and a rigorous diagnostic plan to properly classify the patients at first presentation are essential to a successful treatment outcome. ImagesFigure 1 PMID:21229071

  8. HELLP Syndrome

    MedlinePLUS

    ... get HELLP syndrome if you're white and older than 25 years of age. You are also more likely to get it if you have had children before or if you had a problem with a pregnancy in the past. Treatment How is HELLP syndrome ...

  9. Marfan Syndrome

    MedlinePLUS

    ... Connective tissue helps support all parts of your body. It also helps control how your body grows and develops. Marfan syndrome most often affects ... A mutation, or change, in the gene that controls how the body makes fibrillin causes Marfan syndrome. Fibrillin is a ...

  10. Marfan Syndrome

    MedlinePLUS

    ... even exercise — they just have to be a little more careful. Kids should always check with their doctors about what's ... activities during gym class. But other than that, kids with Marfan syndrome are just like everyone else — only a little taller. If you have Marfan syndrome, you probably ...

  11. Bloom's Syndrome

    MedlinePLUS

    ... Niemann-Pick Disease, Type A Spinal Muscular Atrophy Tay-Sachs Disease Usher Syndrome, Type 1F and Type III Walker- ... Niemann-Pick Disease, Type A Spinal Muscular Atrophy Tay-Sachs Disease Usher Syndrome, Type 1F and Type III Walker- ...

  12. Alder-Reilly Anomaly in Hurler's Syndrome in a Neonate: A Rare Case Report.

    PubMed

    Bhuyan, Pallavi; Singh, Bipsa; Chakrabarty, Sukumar; Mohanty, Niranjan; Agarwal, Mukesh; Satpathy, Sanghamitra

    2013-09-01

    A 22-days-old male newborn baby presented with persistence of neonatal jaundice since birth. On clinical examination he had coarse facial features, a prominent forehead, enlarged tongue, icterus, hepatosplenomegaly, skeletal deformities and bilateral inguinal hernia. On investigation the peripheral smear revealed Alder-Reilly anomaly in the neutrophils suggesting mucopolysaccharidosis. Mucopolysaccharide excretion spot test of the urine was positive; and an assay for glycosaminoglycans in the urine was also high, which confirmed the clinical diagnosis of Hurler's syndrome. We present this rare case to highlight the association of Alder-Reilly anomaly and bilateral inguinal hernia in Hurler's syndrome even in neonates. PMID:24426371

  13. Acardiac twin fetus with severe hydrops fetalis and bilateral talipes varus deformity.

    PubMed

    Thelmo, Mary Lou C; Fok, Randolph Y; Shertukde, Savita P

    2007-01-01

    Twin reversed-arterial-perfusion syndrome (TRAPS) is a rare complication of monochorionic twin pregnancies. TRAPS is characterized by the hemodynamic dependence of a "recipient" twin from a "pump" twin. The "recipient" twin exhibits lethal abnormalities, such as acardia and acephaly. Circulatory failure of the normal twin derives from the existence of arterio-arterial and veno-venous anastomoses within the placenta that allow retrograde perfusion of the acardiac twin by blood coming from the normal twin. Acardiac twinning is the most extreme manifestation of the twin-twin transfusion syndrome. This occurs in 1 in 100 monozygotic twin pregnancies and 1 in 35,000 births. We report a case of diamnionic monochorionic female twins in which the acardiac twin demonstrated severe hydrops fetalis and bilateral talipes varus deformity. Cesarean section was performed on a 27-year-old hypertensive gravida 2, para 1 mother for fetal indications at 32 6/7 weeks gestation. The acardiac fetus had a two-vessel umbilical cord measuring 43.5 cm in length and 0.8 cm in diameter. The proximal end inserted into the root of the normal twin's umbilical cord in an acute angle forming a "v" close to the placental disc. Structures rostral to the thorax were absent except for a round mass of flesh with three small buds in place of the head and neck, and bilateral upper extremities. Only the kidneys, right adrenal, small and large intestine, and rudimentary urinary bladder were present. Both feet demonstrated talipes varus deformity. The fetus was severely hydropic. The subcutaneous fat measured 4.5 cm in maximum thickness. The normal twin had a protracted course complicated with respiratory distress syndrome, moderate secundum atrial septal defect with left to right shunt, and thrombocytopenia of prematurity. The baby was eventually discharged after approximately 1 month. At the time of this report, 5 months postpartum, the neonate is growing and developing normally. To our knowledge, this is the first report of severe hydrops fetalis and talipes varus deformity in an acardiac twin. PMID:18363156

  14. Bilateral Pedicle and Crossed Translaminar Screws in C2

    PubMed Central

    Mendelsohn, Daniel; Lee, Robert; Boyd, Michael C.

    2015-01-01

    Multiple techniques exist for the fixation of C2, including axial pedicle screws and bilateral translaminar screws. We describe a novel method of incorporating both the translaminar and pedicle screws within C2 to improve fixation to the subaxial spine in patients requiring posterior cervical instrumentation for deformity correction or instability. We report three cases of patients with cervical spinal instability, who underwent cervical spine instrumentation for stabilization and/or deformity correction. Bilateral C2 pedicle screws were inserted, followed by bilateral crossed laminar screws. The instrumentation method successfully achieved fixation in all three patients. There were no immediate postoperative complications, and hardware positioning was satisfactory. Instrumenting C2 with translaminar and pedicle screws is technically feasible, and it may improve fixation to the subaxial spine in patients with poor bone quality or severe subaxial deformity, which require a stronger instrumentation construct. PMID:26435799

  15. Bilateral chylothorax as a complication of internal jugular vein cannulation

    PubMed Central

    Saxena, Puneet; Shankar, Subramanian; Kumar, Vivek; Naithani, Nardeep

    2015-01-01

    Central venous catheterization is one of the most prevalent procedures in the Intensive Care Unit. Complications are reported in about 15% of the patients and usually comprise of infection, arterial puncture, malpositioning, pneumothorax, local hematoma, hemothorax, and so on. Chylothorax is a rare complication of this procedure. We present a 42-year-old lady, who developed bilateral massive chylothorax after cannulation of her left internal jugular vein (IJV), due to direct injury to the thoracic duct during the procedure. The patient was successfully managed with bilateral chest tube drainage and omission of oral feeds for four days. Development of bilateral chylothorax as a complication of IJV cannulation is rare, but merits reporting, in view of a large number of central venous cannulations being undertaken. Critical care professionals should be aware of this rare complication of a common procedure to facilitate early identification and institute appropriate therapy. PMID:26180388

  16. Bilateral Inflammatory Aural Polyps: A Manifestation of Samter's Triad

    PubMed Central

    Brobst, Robert; Suss, Nichole; Joe, Stephanie; Redleaf, Saadia

    2009-01-01

    We report an unusual case of bilateral inflammatory aural polyps in a patient with Samter's triad. This 52-year-old patient had a history of chronic rhinosinusitis with sinonasal polyps, asthma, and aspirin sensitivity, with progressive right-sided hearing loss, otorrhea, and aural fullness. She was found to have bilateral aural polyps, with the larger obstructing lesion on the right. A computed tomography supported these findings and revealed bilateral opacification of the middle ear cleft and mastoid air cells. An initial right tympanomastoidectomy was performed with the specimen histologically resembling a typical sinonasal polyp. We speculate that this patient's middle ear polyposis is secondary to the inflammatory changes of Samter's triad. This has not been described previously in the literature. PMID:20182629

  17. Bilateral seminoma with hepatic metastasis in a cockatiel (Nymphicus hollandicus).

    PubMed

    Saied, Ahmad; Beaufrère, Hugues; Tully, Thomas N; Wakamatsu, Nobuko

    2011-06-01

    Abstract: An 18-year-old, intact male cockatiel (Nymphicus hollandicus) presented with a clinical history of dyspnea, lethargy, and reluctance to perch. Coelomic ultrasonographic examination revealed hypoechoic nodules in the proximity of, and within, the liver. The bird did not respond to supportive care and was euthanatized. At necropsy, the testes were bilaterally enlarged, whitish-tan, firm, and multilobulated. The right hepatic lobe contained a mass that had a similar color and consistency to those in the testes. On histologic and ultrastructural examinations, the diagnosis was bilateral testicular seminoma with hepatic metastasis. To our knowledge, this is the first case of a metastatic bilateral testicular seminoma reported in a cockatiel. PMID:21877450

  18. A rare presentation of bilateral spontaneous superficial femoral arteriovenous fistulas.

    PubMed

    Hwang, Inyong; Bomb, Kavita; Askari, Raza; Khouzam, Rami N

    2016-01-01

    Spontaneous arteriovenous fistulas are a rare clinical entity with very few cases reported in the literature. Prompt diagnosis and treatment is crucial and can prevent further complications in such patients. We report a case of a patient who presented with progressive bilateral claudication, discolouration of feet, hypaesthesia and non-healing ulcers. The patient had no history of trauma, aneurysm or surgery. After abnormal non-invasive studies, a peripheral angiogram revealed significant disease and obstruction of bilateral superficial femoral arteries. Spontaneous bilateral femoral arteriovenous fistulas were also found incidentally. After discussing the risks and benefits of multiple treatment options with the patient, percutaneous intervention with orbital atherectomy, balloon angioplasty and covered stent placements were performed. This treatment improved the patient's symptoms significantly. PMID:26759400

  19. Blindness after bilateral neck dissection: case report and review.

    PubMed

    Pazos, G A; Leonard, D W; Blice, J; Thompson, D H

    1999-01-01

    The primary objective of this review of the literature is to identify the probable causes of blindness after bilateral radical neck dissections. This case report and literature review also discusses possible preventive measures that may avert this catastrophic outcome. Cases of blindness after bilateral radical neck dissection were identified by an electronic literature search, as well as cross-checking all references of the above-identified papers. Eleven previous cases of blindness after bilateral neck dissection were identified. The most common cause was posterior ischemic optic neuropathy (PION), which was permanent. We present the only case in the literature in which blindness occurred after radical neck dissections separated by a span of 9 years. The cause of blindness in our patient was posterior ischemic optic neuropathy. Contributing factors included anemia, hypotension, and disruption of collateral venous return from the neck. PMID:10512147

  20. Bilateral orthokeratinized odontogenic cyst: A rare case report and review

    PubMed Central

    Pimpalkar, Rahul Devidas; Barpande, Suresh R; Bhavthankar, Jyoti D; Mandale, Mandakini S

    2014-01-01

    Orthokeratinized odontogenic cyst (OOC) is a developmental cyst of jaw and was initially considered by the World Health Organization (1992) as the uncommon orthokeratinized variant of odontogenic keratocyst (OKC). However, studies have shown that OOC has peculiar clinicopathologic aspects when compared with other developmental odontogenic cysts, especially OKC. So orthokeratinized odontogenic cyst now stands out to be a distinct entity. Clinically, it occurs as a single cyst, shows a predilection for males and is most often found in the second to the fifth decade. Its bilateral occurrence is extremely rare. The purpose of the article is to present a rare case of bilateral OOC arising in the mandible and review the literature on bilateral occurrence of this lesion. PMID:25328309