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Sample records for bilateral perisylvian syndrome

  1. Bilateral agenesis of arcuate fasciculus demonstrated by fiber tractography in congenital bilateral perisylvian syndrome.

    PubMed

    Kilinc, Ozden; Ekinci, Gazanfer; Demirkol, Ezgi; Agan, Kadriye

    2015-03-01

    Congenital bilateral perisylvian syndrome (CBPS) is a type of cortical developmental abnormality associated with distinctive clinical and imaging features. Clinical spectrum of this syndrome is quite heterogeneous, with different degrees of neurological impairment in affected individuals. High-definition magnetic resonance imaging (MRI) has a great importance in revealing the presence of CBPS, but is limited in elucidating the heterogeneous clinical spectrum. The arcuate fasciculus (AF) is a prominent language tract in the perisylvian region interconnecting Broca and Wernicke areas, and has a high probability of being affected developmentally in CBPS. Herein, we report a case of CBPS with investigation of AF using diffusion tensor imaging (DTI) and fiber tractography in relation to clinical findings. We postulated that proven absence of AF on DTI and fiber tractography would correlate with a severe phenotype of CBPS. PMID:24852949

  2. Congenital bilateral perisylvian syndrome with partial epilepsy. Case report with long-term follow-up.

    PubMed

    Margari, Lucia; Lucia, Margari; Presicci, Anna; Anna, Presicci; Ventura, Patrizia; Patrizia, Ventura; Buttiglione, Maura; Maura, Buttiglione; Andreula, Cosma; Cosma, Andreula; Perniola, Tommaso; Tommaso, Perniola

    2005-01-01

    Congenital bilateral perisylvian syndrome (CBPS) is a rare neurological disorder characterised by pseudobulbar palsy, cognitive deficits and epilepsy associated with bilateral perisylvian cortical dysplasia on neuroimaging studies. We report a long-term follow-up of a 18-years girl diagnosed with CBPS according to the typical clinical and magnetic resonance imaging (MRI) features. The patient showed faciopharyngoglossomasticatory diplegia, severe dysarthria, ataxia, spastic quadriparesis and severe mental retardation. Brain MRI evidenced bilateral perisylvian cortical dysplasia. Since early life she suffered from complex febrile seizures and epilepsy consisting of complex partial attacks with affective manifestations associated with centro-temporal EEG abnormalities. During 18 years of follow-up she was treated with phenobarbital, carbamazepine, lamotrigine, gabapentin but did not show any significant clinical improvement. Subsequently, monotherapy with phenytoin (PHT) was followed by a significant clinical improvement. At age 17, because of adverse effects, PHT was gradually substituted by topiramate (TPM). Full control of seizures was obtained at the age of 17 years with TPM. EEG abnormalities throughout the years have been reduced according to the clinical course. These findings emphasised the importance of long-term follow-up, suggesting that the prognosis for epilepsy may not be predicted based on the early response to treatment or on the presence of structural encephalic abnormalities, as reported in the literature. PMID:15626542

  3. Voxel-based morphometry and intellectual assessment in patients with congenital bilateral perisylvian syndrome.

    PubMed

    Yasuda, Clarissa L; Guimarães, Catarina A; Guerreiro, Marilisa M; Boscariol, Mirela; Oliveira, Ecila P M; Teixeira, Karine C; Costa, André L F; Beltramini, Guilherme C; Cendes, Fernando

    2014-07-01

    Congenital bilateral perisylvian syndrome (CBPS) presents with heterogeneous clinical manifestations such as pseudobulbar palsy, language disorder, variable cognitive deficits, epilepsy, and perisylvian abnormalities (most frequently polymicrogyria) on imaging studies. We investigated the relationship between seizures and extent of gray matter (GM) and white matter (WM) abnormalities using voxel-based morphometry (VBM) of brain magnetic resonance imaging (MRI) as well the association between seizures, structural abnormalities and cognitive ability. In this cross-sectional study, we evaluated 51 healthy volunteers and 18 patients with CBPS with epilepsy (seizure group, n = 7) and without (non-seizure group, n = 11). We used VBM (SPM8/DARTEL) to investigate areas with excess and atrophy of both gray and white matter, comparing groups of patients with controls. Intellectual ability of patients was assessed by the WISC-III or WAIS-III. Both groups with CBPS and the control group were homogeneous with respect to gender (p = 0.07) and age (p = 0.065). Besides perisylvian polymicrogyria, the seizure group exhibited areas with GM and WM reduction including temporal, frontal, parietal and occipital lobes. In contrast, we identified fewer areas with GM and WM reduction in the non-seizure group. The seizure group presented worse intellectual performance (performance IQ and global IQ) than the non-seizure group. The seizure group presented with a more widespread pattern of cortical and sub-cortical abnormalities, as well as worse cognition. Our results suggest that patients with CBPS and epilepsy appear to have widespread neuronal damage that goes beyond the areas with MRI-visible perisylvian polymicrogyria. PMID:24781839

  4. 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

  5. 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

  6. 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

  7. The Syndrome of Perisylvian Polymicrogyria with Congenital Arthrogryposis

    PubMed Central

    Poduri, Annapurna; Chitsazzadeh, Vida; D’Arrigo, Stefano; Fedrizzi, Ermellina; Pantaleoni, Chiara; Riva, Daria; Busse, Claudia; Küster, Helmut; Duplessis, Adre; Gaitanis, John; Sahin, Mustafa; Garganta, Cheryl; Topcu, Meral; Dies, Kira A.; Barry, Brenda J.; Partlow, Jennifer; Barkovich, A. James; Walsh, Christopher A.; Chang, Bernard S.

    2009-01-01

    Background Bilateral perisylvian polymicrogyria (BPP) is a well-recognized malformation of cortical development commonly associated with epilepsy, cognitive impairment, and oromotor apraxia. Reports have suggested the association of BPP with arthrogryposis multiplex congenita. We sought to investigate the clinical, electrophysiological, and neuroradiological features of this combined syndrome to determine if there are unique features that distinguish BPP with arthrogryposis from BPP alone. Methods Cases of BPP with congenital arthrogryposis were identified from a large research database of individuals with polymicrogyria. Clinical features (including oromotor function, seizures, and joint contractures), MR brain imaging, and results of neuromuscular testing were reviewed. Results Ten cases of BPP with congenital arthrogryposis were identified. Most cases had some degree of oromotor apraxia. Only a few had seizures, but a majority of cases were still young children. Electrophysiological studies provided evidence for lower motor neuron or peripheral nervous system involvement. On brain imaging, bilateral polymicrogyria (PMG) centered along the Sylvian fissures was seen, with variable extension frontally or parietally; no other cortical malformations were present. We did not identify obvious neuroimaging features that distinguish this syndrome from that of BPP without arthrogryposis. Conclusions The clinical and neuroimaging features of the syndrome of BPP with congenital arthrogryposis appear similar to those seen in cases of isolated BPP without joint contractures, but electrophysiological studies often demonstrate coexistent lower motor neuron or peripheral nervous system pathology. These findings suggest that BPP with arthrogryposis may have a genetic etiology with effects at two levels of the neuraxis. PMID:19751967

  8. Perisylvian polymicrogyria, infantile spasms and arthrogryposis: the severe end of the spectrum of congenital bilateral perisylvian polymicrogyria.

    PubMed

    De Coene, Anja; Van Coster, Rudy; Verhelst, Helene

    2010-05-01

    Congenital bilateral perisylvian polymicrogyria (CBPP) is the most frequent type of polymicrogyria in children. A 3-month-old male patient is described here with the combination of CBPP, infantile spasms and arthrogryposis. Only four patients have been reported earlier in the literature with this combination. Three of them had epilepsy. These patients represent the more severe phenotype of CBPP, characterized by early onset of symptoms, epilepsy, mental retardation, pseudobulbar palsy and arthrogryposis. PMID:19559633

  9. 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

  10. 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

  11. Bilateral periventricular nodular heterotopia with amniotic band syndrome.

    PubMed

    Ruggieri, Martino; Spalice, Alberto; Polizzi, Agata; Roggini, Mario; Iannetti, Paola

    2007-06-01

    The amniotic (constriction) band syndrome is characterized by distal ring constrictions, intrauterine amputations, and acrosyndactyly. External constriction by amniotic bands is the generally accepted mechanism: early amniotic rupture leads to formation of mesodermal fibrous strands that constrict, entangle, and amputate distal portions of limbs. Etiology is heterogeneous. Anecdotal cases involving central nervous system abnormalities (e.g., acrania, anencephaly, polymicrogyria, congenital bilateral perisylvian syndrome, neuronal heterotopia, septo-optic dysplasia, and spinal cord tethering) have been reported. We describe a 9-year-old girl with typical features of constriction band syndrome localized to the lower limbs who had also profound mental retardation and drug-resistant epilepsy associated with bilateral periventricular nodular heterotopia (a brain malformation of neuronal migration and proliferation caused by mutations in the X-linked filamin 1 gene [FLN1] on chromosome Xq28). The karyotype was normal, as was mutational screening for FLN1. The occurrence of bilateral periventricular nodular heterotopia in the context of amniotic band syndrome is novel (chance occurrence of both: 0.000004%). PMID:17560504

  12. Sequential presentation of bilateral Brown syndrome.

    PubMed

    Sekeroğlu, Hande Taylan; Türkçüoğlu, Peykan; Sanaç, Ali Şefik; Sener, Emin Cumhur

    2012-04-01

    Brown syndrome, characterized by a limitation of elevation in adduction and positive forced duction testing, is usually unilateral but occurs bilaterally in 10% of all cases. It may present as a congenital condition in one eye and develop in the other eye with no apparent cause. We present a case of bilateral Brown syndrome in which the right eye became involved within 1 year of surgery on the left eye for congenital Brown syndrome. PMID:22525185

  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. Gorlin syndrome and bilateral ovarian fibroma

    PubMed Central

    Pirschner, Fernanda; Bastos, Pollyana Marçal; Contarato, George Luiz; Bimbato, Anna Carolina Bon Lima; Filho, Antônio Chambô

    2012-01-01

    INTRODUCTION Gorlin syndrome (GS), also known as nevoid basal cell carcinoma syndrome (NBCCS), is a rare hereditary, autosomal dominant disease that affects various systems. Its prevalence is estimated at 1/57,000 to 1/256,000 of the population. It is characterized by basal cell carcinomas, multiple odontogenic keratocysts, skeletal abnormalities and ovarian fibroma, among other disorders. PRESENTATION OF CASE To report the case of a young patient with Gorlin syndrome and bilateral ovarian fibroma. DISCUSSION A 20-year old patient with Gorlin syndrome presented with facial asymmetry, broad nasal root, dental abnormalities, micrognathism, convergent strabismus, multiple pigmented lesions on the trunk and face, pectus excavatum, kyphoscoliosis and a palpable mass in the abdomen occupying the entire pelvic region. CONCLUSION Gorlin–Goltz syndrome is a hereditary pathology that includes numerous clinical manifestations. Diagnosis is clinical and genetic confirmation is unnecessary. PMID:22771908

  16. Bilateral Parotid Swelling in Polycystic Ovarian Syndrome.

    PubMed

    Yakubov, Yakov; Mandel, Louis

    2016-05-01

    Polycystic ovarian syndrome (PCOS) is recognized by the presence of polycystic ovaries, irregular menstruation, and increased androgen levels. Many patients have insulin resistance or impaired glucose tolerance and an associated development of type 2 diabetes mellitus. A patient with PCOS is presented whose cosmetic concerns centered on the prolonged existence of substantial bilateral parotid swelling. The pathophysiology, diagnosis, and therapy of sialosis are discussed. PMID:26657398

  17. 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

  18. 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

  19. The perisylvian epileptic network. A unifying concept.

    PubMed

    Halász, Péter; Kelemen, Anna; Clemens, Béla; Saracz, Judit; Rosdy, Beáta; Rásonyi, György; Szücs, Anna

    2005-01-20

    In this work the authors provide evidences for a unifying concept of the syndromes of benign focal childhood epilepsies, Landau-Kleffner syndrome, and electrical status epilepticus in sleep treating them as a spectrum of disorders with a common transient, age dependent, non lesional, genetically based epileptogenic abnormality, the nature of which is still not known. The electro-clinical features of these syndromes are congruent with the different degree involvement of the perisylvian cognitive network and with the involvement of the thalamo-cortical associative system of variable degree. These epilepsies are characterized by the abundance of regional epileptiform discharges in sharp contrast with the rare and in several cases lacking seizures. The nature and severity of interictal cognitive symptoms are closely related to localization within the network and amount of epileptic interictal discharges. Spike-wave discharges are attributed to an alternation of overexcitation (spikes) and overinhibition (waves). The recurrent overinhibition represented by the wave of the discharges may interfere with the continuous depolarization of the cells of a large population of neurons, which is a requirement of the overt seizures. The overinhibition also interfere with cognitive processes which are correlated with the continuous presence of the fast (gamma) activity, binding the required cortical areas. Hence the recurrent inhibition works against the existence of the binding fast frequency activity. This is the assumed reason for the co-existence of the relative lack of overt seizures and in the same time for the frequently observed epileptogenic cognitive deficit symptoms ("cognitive epilepsies"). The time course of these syndromes overlaps with important developmental milestones. The frequent epileptic discharges alters the evolution of the perisylvian network developing late after early childhood and is very vulnerable for any interference in this imprinting time for speech and other cognitive functions. This spectrum of disorders represents a type of age linked, mild to severe 'epileptic encephalopathy' limited to the perisylvian network, where the cognitive impairment is caused by epileptic discharges interfering with cognitive development. PMID:15884395

  20. 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

  1. 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

  2. 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

  3. Progressive "vascular" corticobasal syndrome due to bilateral ischemic hemispheric lesions.

    PubMed

    Kim, Young-Do; Kim, Joong-Seok; Lee, Eek-Sung; Yang, Dong-Won; Lee, Kwang-Soo; Kim, Yeong-In

    2009-01-01

    We report a patient that presented with the corticobasal syndrome (CBS), including progressive dementia, asymmetric parkinsonism associated with constructional and ideomotor apraxia, action myoclonus and focal hand dystonia. Magnetic resonance imaging of the brain revealed extensive ischemic lesions in both fronto-temporo-parieto-occipital lobes and steno-occlusion of the middle cerebral arteries, bilaterally. This case illustrates that extensive cortical vascular-ischemic lesions may present with symptoms mimicking the corticobasal syndrome. PMID:19755778

  4. 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

  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. Gorlin Syndrome with Bilateral Polydactyly: A Rare Case Report

    PubMed Central

    Acharya, Sonu; Panda, Swagatika; Sahoo, Sujit Ranjan; Ray, Prayas

    2013-01-01

    ABSTRACT Gorlin's syndrome is a rare disorder transmitted as an autosomal dominant trait. It is characterized by multiple disorders involving multiple systems. We present a case of 11-year-old male child presenting with multiple odontogenic keratocyst to the dental clinic. Retrograde diagnosis of Gorlin-Goltz syndrome was made after clinical and radiological investigation. How to cite this article: Acharya S, Panda S, Dhull KS, Sahoo SR, Ray P. Gorlin Syndrome with Bilateral Polydactyly: A Rare Case Report. Int J Clin Pediatr Dent 2013;6(3):208-212. PMID:25206225

  7. 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

  8. Bilateral Ramsay Hunt syndrome in a diabetic patient.

    PubMed

    Syal, Rajan; Tyagi, Isha; Goyal, Amit

    2004-12-01

    BACKGROUND: Herpes zoster oticus accounts for about 10% cases of facial palsy, which is usually unilateral and complete and full recovery occurs in only about 20% of untreated patients. Bilateral herpes zoster oticus can sometime occur in immunocompromised patients, though incidence is very rare. CASE PRESENTATION: Diabetic male, 57 year old presented to us with bilateral facial palsy due to herpes zoster oticus. Patient was having bilateral mild to moderate sensorineural hearing loss. Patient was treated with appropriate metabolic control, anti-inflammatory drugs and intravenous acyclovir. Due to uncontrolled diabetes, glucocorticoids were not used in this patient. Significant improvement in hearing status and facial nerve functions were seen in this patient. CONCLUSIONS: Herpes zoster causes severe infections in diabetic patients and can be a cause of bilateral facial palsy and bilateral Ramsay Hunt syndrome. Herpes zoster in diabetic patients should be treated with appropriate metabolic control, NSAIDS and intravenous acyclovir, which we feel should be started at the earliest. Glucocorticoids should be avoided in diabetic patients. PMID:15575957

  9. Bilateral Ramsay Hunt syndrome in a diabetic patient

    PubMed Central

    Syal, Rajan; Tyagi, Isha; Goyal, Amit

    2004-01-01

    Background Herpes zoster oticus accounts for about 10% cases of facial palsy, which is usually unilateral and complete and full recovery occurs in only about 20% of untreated patients. Bilateral herpes zoster oticus can sometime occur in immunocompromised patients, though incidence is very rare. Case presentation Diabetic male, 57 year old presented to us with bilateral facial palsy due to herpes zoster oticus. Patient was having bilateral mild to moderate sensorineural hearing loss. Patient was treated with appropriate metabolic control, anti-inflammatory drugs and intravenous acyclovir. Due to uncontrolled diabetes, glucocorticoids were not used in this patient. Significant improvement in hearing status and facial nerve functions were seen in this patient. Conclusions Herpes zoster causes severe infections in diabetic patients and can be a cause of bilateral facial palsy and bilateral Ramsay Hunt syndrome. Herpes zoster in diabetic patients should be treated with appropriate metabolic control, NSAIDS and intravenous acyclovir, which we feel should be started at the earliest. Glucocorticoids should be avoided in diabetic patients. PMID:15575957

  10. 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

  11. Bilaterally Symmetrical Lower Extremity Compartment Syndrome following Massive Transfusion

    PubMed Central

    Karaoren, Gulsah; Bakan, Nurten; Tomruk, Senay Goksu; Topaç, Zelin; Kurtulmuş, 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

    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. 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

  14. Unusual Parsonage-Turner syndrome with relapses and bilateral simultaneous anterior interosseous neuropathy.

    PubMed

    Squintani, Giovanna; Mezzina, Corrado; Lettieri, Christian; Critelli, Adriana; Eleopra, Roberto

    2009-12-01

    We report an unusual case of Parsonage-Turner syndrome with relapses and simultaneous bilateral anterior interosseous neuropathy (AIN). A 66-year-old man, after a typical right brachial amyotrophic neuralgia few months previously, underwent surgery for left carpal tunnel syndrome. The day following surgery, wrist aching and bilateral weakness, even if prevalent on the right side, on thumb and index finger flexion appeared. Neurophysiology was consistent with bilateral AIN neuropathy and serology revealed anti-nucleus antibody positivity. Association of relapses with bilateral acute AIN involvement in the subject with autoantibody detection can suggest an immunological pathogenesis. PMID:19685201

  15. 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

  16. [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. PMID:26764303

  17. Bilateral anterior tarsal tunnel syndrome variant secondary to extensor hallucis brevis muscle hypertrophy in a ballet dancer: a case report.

    PubMed

    Tennant, Joshua N; Rungprai, Chamnanni; Phisitkul, Phinit

    2014-12-01

    We present a case of bilateral anterior tarsal tunnel syndrome secondary EHB hypertrophy in a dancer, with successful treatment with bilateral EHB muscle excisions for decompression. The bilateral presentation of this case with the treatment of EHB muscle excision is the first of its type reported in the literature. PMID:25457672

  18. Gómez-López-Hernández Syndrome: A Rare Cause of Bilateral Nonscarring Alopecia.

    PubMed

    Saricam, Merve Hatun; Tekin, Burak; Unver, Olcay; Ekinci, Gazanfer; Ergun, Tulin

    2015-01-01

    Gómez-López-Hernández syndrome is a rare neurocutaneous disorder characterized by the triad of rhombencephalosynapsis, parietal alopecia, and trigeminal anesthesia. We report a 16-year-old girl with bilateral parietotemporal alopecia in whom cranial magnetic resonance imaging revealed rhombencephalosynapsis, suggesting a diagnosis of Gómez-López-Hernández syndrome. Neurologic examination and neuroimaging may be warranted in select patients with parietal alopecia to exclude this uncommon entity. PMID:26391554

  19. 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…

  20. Bilateral familial vertical Duane Syndrome with synergistic convergence, aberrant trigeminal innervation, and facial hypoplasia

    PubMed Central

    Gupta, Malvika; Gupta, Om Prakash; Vohra, Vishal

    2014-01-01

    A 5-year-old girl presented with bilateral familial vertical Duane retraction syndrome with alternating esotropia, elevation deficit, Marcus gunn phenomenon, and facial hypoplasia. Abnormal adducting downshoots on attempting abduction suggestive of a synergistic convergence were noted. Hypothesis suggests aberrant innervations or peripheral anatomic connections between inferior and medial recti. PMID:25378878

  1. Bilateral familial vertical Duane Syndrome with synergistic convergence, aberrant trigeminal innervation, and facial hypoplasia.

    PubMed

    Gupta, Malvika; Gupta, Om Prakash; Vohra, Vishal

    2014-09-01

    A 5-year-old girl presented with bilateral familial vertical Duane retraction syndrome with alternating esotropia, elevation deficit, Marcus gunn phenomenon, and facial hypoplasia. Abnormal adducting downshoots on attempting abduction suggestive of a synergistic convergence were noted. Hypothesis suggests aberrant innervations or peripheral anatomic connections between inferior and medial recti. PMID:25378878

  2. Congenital central hypoventilation syndrome. A report of successful experience with bilateral diaphragmatic pacing.

    PubMed Central

    Coleman, M; Boros, S J; Huseby, T L; Brennom, W S

    1980-01-01

    Bilateral diaphragmatic pacing was successfully performed in an 18-month-old child with congenital central hypoventilation syndrome (Ondine's curse) as an alternative to long-term mechanical ventilation. Subsequent complications were related more to cor pulmonale and tracheostomy care than to the pacing itself. PMID:6969060

  3. 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.

  4. 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

  5. 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

  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 multifocal posterior pole lesions in Reiter syndrome

    PubMed Central

    Mansour, Ahmad M; Jaroudi, Mahmoud O; Medawar, Walid A; Tabbarah, Zuhayr A

    2013-01-01

    Reactive arthritis is associated with conjunctivitis or iritis. Rarely reactive arthritis is accompanied by permanent visual loss from macular infarction or foveal scarring. We present the case of a rheumatologist who had a sudden onset of skin lesions, arthritis of several joints and bilateral visual loss. Most of these manifestations resolved after a course of oral corticosteroids. However he was left with decreased vision in the left eye and multiple lesions in the fovea over a follow-up of 2 years. PMID:23576664

  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. Bilateral carpal tunnel syndrome and ulnar neuropathy at the elbow in a pizza chef.

    PubMed

    Vimercati, Luigi; Lorusso, Antonio; L'abbate, Nicola; Assennato, Giorgio

    2009-01-01

    A case of bilateral carpal tunnel syndrome and ulnar neuropathy at the elbow in a 22-year-old pizza chef is described. An on-site analysis revealed that job tasks performed by the worker exposed him to a combination of biomechanical risk factors. Patient history and workplace observations suggest that occupational physical exposure may have caused the bilateral entrapment neuropathies. The present report underlines the advisability of a detailed occupational history in the case of entrapment neuropathies of the upper limbs commonly regarded as being related to biomechanical occupational exposure. PMID:21686375

  10. [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

  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 supplemental permanent maxillary lateral incisors in a non-syndromic patient.

    PubMed

    Bhullar, Mandeep Kaur; Uppal, Amandeep Singh; Kochhar, Gulsheen Kaur; Singh, Ranjit

    2014-01-01

    A supernumerary tooth is an additional entity to the normal series and can be seen in all quadrants of the jaw. Occurrence may be single or multiple, unilateral or bilateral, erupted or impacted in one or in both jaws. Multiple supernumeraries are rare in individuals with no other associated disease or syndromes. The conditions commonly associated with an increased prevalence of supernumerary teeth include cleft lip and palate, cleidocranial dysplasia and Gardner syndrome. The supplemental supernumerary refers to a duplication of teeth in normal series and is found at the end of a tooth series. A supplemental tooth may closely resemble the teeth of the group to which it belongs or it may bear little resemblance in size or shape to the teeth with which it is associated. This report presents a case with bilateral supplemental upper permanent lateral incisors without any symptoms of associated disorders. PMID:25168674

  13. 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

  14. Gonadal function and testicular histology in Noonan's syndrome with bilateral cryptorchidism.

    PubMed

    Sasagawa, I; Nakada, T; Kubota, Y; Sawamura, T; Tateno, T; Ishigooka, M

    1994-01-01

    Testicular histology and gonadal function were studied in 2 patients with Noonan's syndrome accompanied by bilateral cryptorchidism. Plasma FSH level was elevated above the normal range, but plasma levels of LH, prolactin, and testosterone were within normal ranges in both cases. The administration of LH-RH resulted in abnormally high response of plasma gonadotropins in both cases. The response of plasma testosterone to the administration of LH-RH was poor in case 2, but case 1 showed normal response. Testicular histology showed reduction of tubular diameter, Leydig cells per seminiferous tubules, and spermatogonia per tubule in both cases. The results indicate that bilateral cryptorchid patients with Noonan's syndrome have an abnormality in the hypothalamo-pituitary-gonadal axis. PMID:7909426

  15. Bilateral chronic compartment syndrome of the thenar muscles: A case report.

    PubMed

    Haiun, M; Michel, F; Lepage, D

    2015-09-01

    This case report features a patient with chronic bilateral compartment syndrome of the thenar muscles, which had appeared spontaneously 8 years earlier. The condition was progressive and symptoms recently had become worse. Pressure measurements were performed three times to establish the diagnosis: before, immediately after and 10 minutes after hand exertion. Pain relief was achieved by performing a fasciotomy of the right thenar compartment. PMID:26233717

  16. 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

  17. 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

  18. 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

  19. 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

  20. 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

  1. Development of bilateral tension pneumothorax under anesthesia in a Boerhaave's syndrome patient: a case report

    PubMed Central

    Oh, Mi Kyung; Jeon, Woo Jae; Kwon, Yong Deok; Kim, Kyoung Hun

    2016-01-01

    A 33-year-old male visited the emergency room with abdominal pain which developed after a vomiting episode. Based on the pneumomediastinum findings from a chest radiograph and a contrast-enhanced chest and abdominal computed tomography scan, the patient was diagnosed with Boerhaave's syndrome. Preoperative radiologic findings showed no pneumothorax or pleural effusion. Once anesthesia was administered, the patient developed near complete cardiopulmonary collapse due to a bilateral tension pneumothorax, which was treated by bilateral thoracentesis, followed by chest tube insertion. Despite a left side rupture, the damaged right lung was unable to overcome single right ventilation, so the surgery was completed via right thoracotomy. The ruptured site was treated, and the patient was transferred to the intensive care unit. We discuss the anesthetic implications of this disease and how to prevent fatal complications. PMID:27066209

  2. A delayed presentation of bilateral leg compartment syndrome following non-stop dancing.

    PubMed

    Jefferies, James Gordon; Carter, Tom; White, Tim Oliver

    2015-01-01

    We present the case of a young man with a 48 h delayed presentation of bilateral lower limb acute compartment syndrome (ACS) affecting the anterior compartments following an extended period of dancing at a music festival. On making the diagnosis of ACS, the patient was immediately taken to theatre for fasciotomies and compartmental decompression. Repeat look fasciotomies revealed further necrosis to the muscles of the anterior compartments bilaterally and, effectively, all the muscle bellies within the anterior compartments were excised. The patient has been left with a significant functional deficit and disability. This case highlights the importance of timely diagnosis of ACS as delay in presentation can impact significantly on subsequent functional outcome and quality of life. PMID:25786817

  3. Development of bilateral tension pneumothorax under anesthesia in a Boerhaave's syndrome patient: a case report.

    PubMed

    Oh, Mi Kyung; Jeon, Woo Jae; Cho, Sang Yun; Kwon, Yong Deok; Kim, Kyoung Hun

    2016-04-01

    A 33-year-old male visited the emergency room with abdominal pain which developed after a vomiting episode. Based on the pneumomediastinum findings from a chest radiograph and a contrast-enhanced chest and abdominal computed tomography scan, the patient was diagnosed with Boerhaave's syndrome. Preoperative radiologic findings showed no pneumothorax or pleural effusion. Once anesthesia was administered, the patient developed near complete cardiopulmonary collapse due to a bilateral tension pneumothorax, which was treated by bilateral thoracentesis, followed by chest tube insertion. Despite a left side rupture, the damaged right lung was unable to overcome single right ventilation, so the surgery was completed via right thoracotomy. The ruptured site was treated, and the patient was transferred to the intensive care unit. We discuss the anesthetic implications of this disease and how to prevent fatal complications. PMID:27066209

  4. 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

  5. Bilateral Ptosis as the First Presentation of Guillain-Barre Syndrome

    PubMed Central

    TALEBIAN, Ahmad; SOLTANI, Babak; TALEBIAN, Motahhareh

    2016-01-01

    Objective Guillain-Barre syndrome (GBS) is the most common cause of acute weakness in children. It has multiple variant forms with different presentations. A rare initial sign is ptosis. In this study, we present a 10-year-old girl with bilateral ptosis without opthalmoplegia followed by a weakness in extremities with a favourable response to intravenous immunoglobulin. Due to the patient’s initial eyelid levators, myasthenia gravis was ruled out by a Tensilon test and electrophysiological studies. Our report highlights the possibility of GBS as a cause of isolated ptosis, especially in cases without ophthalmoplegia. PMID:27057192

  6. Monolateral purple urine bag syndrome in a patient with bilateral nephrostomy tubes.

    PubMed

    Sheehan, Michael

    2014-01-01

    Purple urine bag syndrome (PUBS) is a constellation of findings resulting in purple discoloration of the urine and/or urine drainage bag(s) occurring in patients with long-term urinary indwelling catheters. Other causative factors may include constipation, female gender, the presence of bacteria containing sulphatase and phosphatase enzymes, and alkaline urine. While the contributing factors for PUBS are linked with high morbidity, PUBS itself is a benign condition. A case study of monolateral PUBS in a patient with bilateral nephrostomy tubes (NTs) is presented. PMID:25112022

  7. Perisylvian white matter connectivity in the human right hemisphere

    PubMed Central

    Gharabaghi, Alireza; Kunath, Frank; Erb, Michael; Saur, Ralf; Heckl, Stefan; Tatagiba, Marcos; Grodd, Wolfgang; Karnath, Hans-Otto

    2009-01-01

    Background By using diffusion tensor magnetic resonance imaging (DTI) and subsequent tractography, a perisylvian language network in the human left hemisphere recently has been identified connecting Brocas's and Wernicke's areas directly (arcuate fasciculus) and indirectly by a pathway through the inferior parietal cortex. Results Applying DTI tractography in the present study, we found a similar three-way pathway in the right hemisphere of 12 healthy individuals: a direct connection between the superior temporal and lateral frontal cortex running in parallel with an indirect connection. The latter composed of a posterior segment connecting the superior temporal with the inferior parietal cortex and an anterior segment running from the inferior parietal to the lateral frontal cortex. Conclusion The present DTI findings suggest that the perisylvian inferior parietal, superior temporal, and lateral frontal corticies are tightly connected not only in the human left but also in the human right hemisphere. PMID:19257886

  8. 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

  9. Bilateral Painful Ophthalmoplegia: A Case of Assumed Tolosa-Hunt Syndrome

    PubMed Central

    Kamusella, Peter; Andresen, Reimer

    2016-01-01

    We present the case of a man of 47 years with vertical and horizontal paresis of view combined with periorbital pain that developed initially on the right side but extended after 3-4 days to the left. Gadolinum uptaking tissue in the cavernous sinus was shown by MRI of the orbital region in the T1 spin echo sequence with fat saturation (SEfs) with a slice thickness of 2 mm. As no other abnormalities were found and the pain resolved within 72 hours of treatment with cortison a bilateral Tolosa-Hunt Syndrome (THS) was assumed. THS is an uncommon cause for Painful Ophthalmoglegia (PO) and only few cases of bilateral appearance have been reported. Even though the diagnostic criteria for THS oblige unilateral symptoms we suggest that in patients with bilateral PO THS should not be excluded as a differential diagnosis. Further more when using MRI to detect granulomatous tissue in the orbital region the chosen sequence should be T1 SEfs and slice thickness should possibly be as low as 2 mm, as granulomas are often no larger than 1-2 mm. PMID:27134970

  10. Bilateral Painful Ophthalmoplegia: A Case of Assumed Tolosa-Hunt Syndrome.

    PubMed

    Kastirr, Ilko; Kamusella, Peter; Andresen, Reimer

    2016-03-01

    We present the case of a man of 47 years with vertical and horizontal paresis of view combined with periorbital pain that developed initially on the right side but extended after 3-4 days to the left. Gadolinum uptaking tissue in the cavernous sinus was shown by MRI of the orbital region in the T1 spin echo sequence with fat saturation (SEfs) with a slice thickness of 2 mm. As no other abnormalities were found and the pain resolved within 72 hours of treatment with cortison a bilateral Tolosa-Hunt Syndrome (THS) was assumed. THS is an uncommon cause for Painful Ophthalmoglegia (PO) and only few cases of bilateral appearance have been reported. Even though the diagnostic criteria for THS oblige unilateral symptoms we suggest that in patients with bilateral PO THS should not be excluded as a differential diagnosis. Further more when using MRI to detect granulomatous tissue in the orbital region the chosen sequence should be T1 SEfs and slice thickness should possibly be as low as 2 mm, as granulomas are often no larger than 1-2 mm. PMID:27134970

  11. [Two cases of Churg-Strauss syndrome with bilateral wide-spread pulmonary infiltrates].

    PubMed

    Motegi, M; Kitahara, Y; Yamauchi, Y; Fukuda, M; Takino, Y; Yarita, H; Sandou, Y; Takayanagi, N; Suzuki, T; Joshita, T

    1992-08-01

    We report two cases of Churg-Strauss syndrome. The first case was a 25-year-old woman with a one year history of bronchial asthma, who developed fever, skin eruptions, abdominal pain and mononeuritis multiplex. During treatment with prednisolone 40 mg per day, chest X-ray films showed bilateral wide-spread infiltrates. When the dosage of prednisolone was increased to 80 mg per day, these infiltrates disappeared. Skin and lung biopsy specimens demonstrated allergic vasculitis and eosinophilic pneumonia. There was no response to high-dose methylprednisolone pulse therapy for persistent severe abdominal pain and mononeuritis multiplex. Pericardial and pleural effusions with eosinophilia recurred eight months later. The second case was a 31-year-old man with a six year history of bronchial asthma, who developed fever, skin eruptions, myalgia and mononeuritis multiplex. One year later, during treatment with prednisolone 15 mg per day, bronchial asthma with eosinophilia relapsed and chest X-ray films showed bilateral patchy infiltrates. Skin biopsy specimens demonstrated eosinophilic infiltrates and necrotizing vasculitis, while lung biopsy specimens demonstrated eosinophilic infiltrates and small granulomas. With additional administration of cyclophosphamide, he has had no evidence of active disease for six years. In both cases, the neurological symptoms persisted despite treatment with high doses of steroids, and during tapering of prednisolone, vasculitis syndrome relapsed. Therefore, long-term careful surveillance is necessary in this disease. PMID:1434233

  12. Bilateral Coronoidectomy by Craniofacial Approach for Hecht Syndrome-Related Trismus.

    PubMed

    Balkin, Daniel M; Chen, Isaac; Oberoi, Snehlata; Pomerantz, Jason H

    2015-09-01

    Hecht Syndrome is an autosomal dominant distal arthrogryposis caused by mutation in the MYH8 locus characterized by trismus and pseudocamptodactyly. Hecht-associated trismus is thought to result from bilateral hyperplasia of the mandibular coronoid processes. Although several interventions to address trismus have been pursued, no consensus exists regarding optimal management. In this report, the authors present a 7-month-old male with Hecht Syndrome referred for management of trismus. By age 2, interincisal opening had progressively decreased from 12 to 5 mm despite physical therapy. Nutrition was limited to liquids, oral hygiene was compromised, and aspiration risk was present. Computed tomography examination revealed enlarged coronoid processes extending medially and superiorly to the zygomatic arches. To release bony impaction of the coronoid processes against the zygoma and to prevent reossification of the temporalis tendon insertion, resection of the enlarged coronoids and distal temporalis muscles as well as placement of Alloderm spacers were performed via an open craniofacial transzygomatic approach. Jaw motion rehabilitation was used following surgery. Two years postoperatively, the patient had no signs of recurrence and good functional stability of jaw excursion. He was able to chew and swallow solid foods, protrude his tongue, use utensils, and perform regular oral hygiene, none of which were possible before surgery. This case demonstrates that open bilateral coronoidectomy can be a successful and durable management option for trismus in patients with Hecht Syndrome. The open transzygomatic approach is safe, has low morbidity, and provides direct access and adequate exposure for coronoid resection, spacer placement, and prevention of temporalis reinsertion. PMID:26335328

  13. 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

  14. 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

  15. Bilateral frontoparietal polymicrogyria, Lennox-Gastaut syndrome, and GPR56 gene mutations

    PubMed Central

    Parrini, Elena; Ferrari, Anna Rita; Dorn, Thomas; Walsh, Christopher A.; Guerrini, Renzo

    2014-01-01

    Summary Purpose Bilateral frontoparietal polymicrogyria (BFPP) has been reported in sporadic patients and in recessive pedigrees. Eleven mutations in GPR56, a gene encoding an evolutionarily dynamic G-protein–coupled receptor, have been identified in 29 patients from 18 families. The clinical features of BFPP include severe mental retardation, motor and language impairment, and epilepsy. No detailed description of the epilepsy is available for the patients reported to date. We report three consanguineous families in which four affected individuals with BFPP and GPR56 mutations had Lennox-Gastaut syndrome. Methods Family studies, brain magnetic resonance imaging (MRI), electroencephalography (EEG)-video recordings, and mutation analysis. Results In Family 1, with one affected proband, we found an R565W change in the second extracellular loop of GPR56, involving a highly conserved aminoacidic residue. In Family 2, with one affected proband, we found an R79X change affecting the protein N-terminus and predicted to cause a premature truncation with loss of the G-protein–coupled receptor proteolytic site. In family 3, with two affected siblings, we found an R33P substitution in the protein N-terminus, involving a highly conserved aminoacidic residue. Epilepsy, present in all four patients, had started between ages 1 and 8 years, with infantile spasms in one patient and with de novo Lennox-Gastaut syndrome in the remaining three. All patients had Lennox-Gastaut syndrome when last observed, at ages 13 to 32 years. Discussion Several genes, when mutated, can cause malformations of cortical development that have been associated with the Lennox-Gastaut syndrome. BFPP caused by GPR56 mutations represents an additional, although rare, genetically determined cause of Lennox-Gastaut syndrome. PMID:19016831

  16. [Bilateral phrenic nerve paralysis, dysautonomia and restrictive cardiomyopathy in a case of POEMS syndrome].

    PubMed

    Delalande, S; Stojkovic, T; Rose, C; Millaire, A; Hurtevent, J F; Vermersch, P

    2002-07-01

    We report a case of POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M protein and Skin changes) with unusual clinical features. A 62-year-old woman presented a severe polyneuropathy with dysphonia and vegetative symptoms, including bradycardia and sphincterial disorders. The clinical examination showed facial hyperpigmentation, cachexia, anasarca and splenomegaly. She also presented restrictive cardiomyopathy and endocrine disturbances. Nerve conduction studies revealed a severe demyelinating sensorimotor neuropathy. Cerebrospinal fluid analysis showed an elevated protein level. We detected a biclonal gammapathy (Ig G and Ig A with lambda light chain) and lytic pelvic bone lesions. Later, she developed a severe ventilatory failure due to a bilateral phrenic nerve paralysis leading to a mechanical ventilation. Steroids followed by localized radiotherapy partially improved the respiratory status and stabilized the neuropathy. Phrenic nerve paralysis, restrictive cardiomyopathy, vegetative symptoms and cranial nerve palsy are exceptional in POEMS syndrome. Moreover, this case emphasizes the importance of radiological investigations since the discover of plasmocytoma may improve the prognosis of POEMS syndrome. PMID:12486906

  17. 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

  18. 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

  19. Congenital Bilateral Retinal Detachment in Two Siblings with Osteoporosis-Pseudoglioma Syndrome.

    PubMed

    Welinder, Lotte G; Robitaille, Johane M; Rupps, Rosemarie; Boerkoel, Cornelius F; Lyons, Christopher J

    2015-01-01

    The birth of a bilaterally blind child is catastrophic for families and a challenging diagnostic and management problem for ophthalmologists. Early identification of the underlying cause and its genetic basis helps initiate possible treatment, delineate prognosis, and identify risks for future pregnancies. In some cases, an early diagnosis can also influence the treatment of other family members. We report two sisters with bilateral retinal detachment and retro-lental masses from birth with no detectable NDP or FZD4 mutations. They were born to parents without detectable retinal anomalies. At 1 year of age, the elder sister had low impact bone fractures, and further evaluation identified severe osteopenia and multiple spinal compression fractures. Molecular testing identified biallelic lipoprotein receptor-related protein 5 (LRP5) mutations (NM_002335.3:c. [889dupA]; [2827 + 1G > A]) confirming a diagnosis of osteoporosis-pseudoglioma (OPPG) syndrome. After this diagnosis, the father and mother were found to have low bone mass and the father started on therapy. We conclude that early detection of LRP5 mutations is important for initiation of treatment of reduced bone density in the patients and their carrier relatives. PMID:25945592

  20. 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

  1. 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

  2. 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

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

    SciTech Connect

    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.

    2015-10-15

    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: One 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.

  4. High Le Fort I and bilateral split sagittal osteotomy in Crouzon syndrome.

    PubMed

    Tay, Yoong Chuan; Tan, Kian Hian; Yeow, Vincent Kok-Leng

    2013-05-01

    Crouzon syndrome is a rare, autosomal dominant disease from a fibroblast growth factor receptor 2 gene mutation, characterized by premature craniosynostosis, hypertelorism, orbital proptosis, psittichorina, hypoplastic maxilla, and mandibular prognathism. We present an adult 32-year-old Crouzon syndrome patient who underwent an elective High Le Fort I and bilateral split sagittal osteotomy for midface advancement with a background of jaw malocclusion and obstructive respiratory symptoms. The operation features a potential dynamic movement of the secured airway in the surgical field and close proximity to exposed ocular structures. Permissive hypotensive anesthesia was employed to improve the surgical field and reduce intraoperative blood loss and dose of long-acting opioids. He was extubated at the end of an uneventful surgery and was monitored in the high dependency overnight before he was discharged to the general ward. Perioperative issues include potential difficult airway management; ocular, auditory, and neurological injury prevention; surgery-specific anesthetic technique; and postoperative analgesia. Understanding the multisystemic issues facilitates the dynamic anesthetic management during surgery. Good communication among the multidisciplinary team is essential to ensure a successful operation and uneventful recovery. PMID:23714981

  5. Chronic bilateral thalamic stimulation: a new therapeutic approach in intractable Tourette syndrome. Report of three cases.

    PubMed

    Visser-Vandewalle, Veerle; Temel, Yasin; Boon, Peter; Vreeling, Fred; Colle, Henry; Hoogland, Govert; Groenewegen, Henk J; van der Linden, Chris

    2003-12-01

    Based on the results of thalamotomies described by Hassler in 1970, the authors performed bilateral thalamic high-frequency stimulation (HFS) in three patients with intractable Tourette syndrome (TS). In this report they describe the long-term effects. Three male patients (42, 28, and 45 years of age) had manifested motor and vocal tics since early childhood. The diagnosis of TS was made according to the criteria of the Tourette Syndrome Classification Study Group. Any drug or alternative treatment had been either ineffective or only temporarily effective in all three patients. There was no serious comorbidity. The target for stimulation was chosen at the level of the centromedian nucleus, substantia periventricularis, and nucleus ventrooralis internus. After 2 weeks of test stimulation, the pulse generators were implanted. After a follow-up period of 5 years in the patient in Case 1, 1 year in the patient in Case 2, and 8 months in the patient in Case 3, all major motor and vocal tics had disappeared and no serious complications had occurred. When stimulation was applied at the voltage necessary to achieve an optimal result on the tics, a slight sedative effect was noted in all three patients. In the patients in Cases 1 and 3 there were stimulation-induced changes in sexual behavior. Chronic thalamic HFS may be an effective and safe treatment for medically intractable TS in adult patients. Unwanted stimulation-induced side effects may occur. PMID:14705742

  6. 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

  7. 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

  8. Atypical Wernicke's syndrome sans encephalopathy with acute bilateral vision loss due to post-chiasmatic optic tract edema

    PubMed Central

    Desai, Soaham Dilip; Shah, Diva Sidharth

    2014-01-01

    A middle aged male presented with acute bilateral vision loss, 4 weeks after undergoing gastric bypass surgery for gastric carcinoma. He had normal sensorium, fundoscopy, normal pupillary reaction to light, but had mild opthalmoparesis and nystagmus with ataxia. Magnetic resonance imaging of the brain revealed post-chiasmatic optic tract edema along with other classical features of Wernicke's syndrome. Thiamine supplementation leads to complete resolution of clinical as well as imaging findings. In appropriate clinical settings, a high index of suspicion and early treatment are essential for managing Wernicke's syndrome even in patients with atypical clinical and imaging presentation. PMID:24753673

  9. Eagle's syndrome: treatment by intraoral bilateral resection of the ossified stylohyoid ligament. A review and report of two cases.

    PubMed

    Martins, Wilson Denis; Ribas, Marina de Oliveira; Bisinelli, Julio; França, Beatriz Helena Sottile; Martins, Guilherme

    2013-07-01

    The complexities of Eagle's syndrome are examined according to anatomical, historical, clinical, and treatment aspects. There appears to be little correlation between the extent, form, and size of the anomalies of the styloid process and the stylohyoid ligament and the predictability of patients with related symptoms. Surgical treatment and the advantages and disadvantages of each surgical approach (intra- and extraoral) are discussed. The probable causes of enlargement of the styloid process and ossification of the stylohyoid ligament are addressed. Two cases of Eagle's syndrome are presented: one unilateral and the other bilateral. An intraoral modified surgical technique is presented. PMID:23971163

  10. Changes in leg pain after bilateral fasciotomy to treat chronic compartment syndrome: a case series study

    PubMed Central

    2013-01-01

    Background Intracompartmental pressure (ICP) as the diagnostic gold standard in the management of chronic compartment syndrome (CCS) is debated. We present a diagnostic protocol in which the decision to operate can be based upon clinical findings alone. The aim of this study was to examine whether patients who underwent surgery for CCS based on clinical findings experienced significant long-term pain relief. Methods A standardized clinical examination, including skin sensitivity, was performed in patients with bilateral leg pain and/or cramps. Before and after a symptom-provoking step test, ICPs were measured. The primary outcome was self-reported leg pain measured on a visual analogue scale. Secondary outcomes were satisfaction with the treatment result and health-related quality of life (HRQL) measured with the SF-8 questionnaire. Postoperative data were collected after 2 years. Results Follow-up was completed for 37 of 40 patients. ICP was increased in 80.5% of the compartments examined before surgery, but did not correlate with the degree of leg pain. The remaining compartments were diagnosed as CCS based on clinical findings, despite ICPs below the threshold. Leg cramps occurred in 32 of 37 (86.5%) patients during physical activity and at night. Leg pain improved from a score of 8.0 ± 1.5 to 2.3 ± 2.1, P < 0.001. Satisfaction with the treatment result was reported by 81.1% of the patients, accompanied by normalized HRQL. Conclusions The diagnostic protocol led to a fasciotomy in all compartments of both legs, which was associated with substantial and sustained relief of leg pain, improved HRQL, and patient satisfaction. PMID:23561303

  11. Bilateral vocal cord paralysis and hypothyroidism as presenting symptoms of Williams-Beuren syndrome: a case report.

    PubMed

    Koren, Ilana; Kessel, Ira; Rotschild, Avi; Cohen-Kerem, Raanan

    2015-09-01

    Williams-Beuren syndrome is a rare neurodevelopmental disorder caused by deletion of 1.5-1.8Mb genes on chromosome 7q11.23. The syndrome was first described as a triad of supra-valvular aortic stenosis, mental retardation, and distinctive facial features. Our patient was referred due to audible inspiratory stridor when he was seven days old. Following endoscopy he was diagnosed with bilateral vocal cord paralysis and was eventually intubated due to respiratory de-compensation followed by tracheotomy. On further workup he was diagnosed with hypothyroidism. Genetic workup supported the diagnosis of Williams-Beuren syndrome. We report here a case with an unusual clinical presentation. PMID:26143126

  12. Lower extremity anterior compartment syndrome complicating bilateral mastectomy and immediate breast reconstruction: A case report and literature review

    PubMed Central

    Tashakkor, A Yashar; Macadam, Sheina A

    2012-01-01

    ‘Well leg compartment syndrome’ refers to compartment syndrome occurring in a nontraumatic setting. This occurs most commonly in the lower limb during surgery performed with the patient in an anatomically vulnerable position. While this complication is well documented in the setting of orthopedic, urological and gynecological surgeries, it is an exceptionally rare complication in plastic surgery; only seven cases have been published on compartment syndrome complicating an operation performed on a supine patient. A case involving a 56-year-old woman who developed an anterior compartment syndrome of her right lower leg following a bilateral mastectomy with immediate breast reconstruction is presented. A detailed literature review is also included. PMID:23730157

  13. Colonic adenocarcinoma and bilateral malignant ovarian sex cord tumor with annular tubules in Peutz-Jeghers syndrome.

    PubMed

    Ayadi-Kaddour, A; Bouraoui, S; Bellil, K; Bellil, S; Kchir, N; Zitouna, M M; Haouet, S

    2004-06-01

    Peutz-Jeghers syndrome is characterized by multiple polyps throughout the gastrointestinal tract in association with mucocutaneous pigmentation. Although Peutz-Jeghers syndrome polyps are hamartomas, frequent association of this syndrome with both gastrointestinal and non-gastrointestinal tumours had led to reassessment of the cancer risk in this hereditary disorder. The most common gynaecological tumors in this syndrome are adenoma malignum of the uterine cervix and ovarian sex cord tumor, particularly sex cord tumor with annular tubules. The question of malignant change in a polyp or of the association of gastro intestinal carcinomas still discuss. The authors report a case of Peutz-Jeghers syndrome in a young patient who developed a colonic adenocarcinoma in a hamartomatous polyp together with an incidentally discovered bilateral malignant sex cord tumours. We discuss its association with certain benign and malignant tumors and the risk of rare complications of these hamartomatous polyps. Although malignant tumors are increasingly reported in association with the Peutz-Jeghers syndrome, to our knowledge, there have been no previous reports of such an association in the literature. PMID:15524052

  14. 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

  15. 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

  16. Disseminated cervical adenoma malignum and bilateral ovarian sex cord tumors with annular tubules associated with Peutz-Jeghers syndrome.

    PubMed

    Srivatsa, P J; Keeney, G L; Podratz, K C

    1994-05-01

    Adenoma malignum is a highly differentiated mucinous adenocarcinoma of the cervix with a deceptively innocent histologic appearance but a highly aggressive behavior. We describe a patient who had adenoma malignum associated with Peutz-Jeghers syndrome (PJS) and bilateral ovarian sex cord tumor with annular tubules (SCTAT). The relatively frequent occurrence of cervical adenoma malignum in women with PJS warrants close surveillance by gynecologists for early detection and treatment of this cancer. We report the 16th known case of PJS with adenoma malignum and only the 8th known case of PJS with adenoma malignum and bilateral SCTAT. The highly aggressive nature of cervical adenoma malignum is exemplified, and the difficulties associated with early diagnosis are discussed. The clinical significance of cervical and ovarian tumors associated with PJS is reviewed. PMID:8188091

  17. Brain dysplasia associated with Larsen-like syndrome.

    PubMed

    Yamaguchi, K; Ogawa, Y; Handa, T

    1996-01-01

    We present an autopsy case of Larsen-like syndrome with unusually severe neurologic complications. The patient, a 3-year-old girl, manifested severe psychomotor retardation, tetraplegia, and intractable partial seizures as well as multiple joint dislocations with other skeletal deformities, minor external anomalies, and laryngotracheomalacia. Neuropathological examination of the brain revealed (1) cortical dysgenesis in the bilateral perisylvian region; (2) protrusions of the brain parenchyma into the subarachnoid space, (3) abnormal arrangement of olivary neurons, (4) dilation of the lateral ventricles with subventricular gliosis and multiple glial nodules, (5) hypoplasia of the cerebral white matter with subcortical astrocytosis, and (6) necrotic change in Sommer sector of the hippocampus. There were no microscopic abnormalities in the mesenchymal tissue of the brain (i.e., vascular walls and the meninges). Cortical dysgenesis in the perisylvian region was characterized by a zonal heterotopia of pyramidal and granule neurons in the molecular layer, which clinically may be closely related to intractable partial seizures in the orofacial area. Findings 1, 2, and 3 may represent a disturbance of neuroblast migration, speculated to have occurred during the latest stage of migration (around 20-25 weeks gestation). There are few published reports describing the combination of Larsen-like syndrome and brain dysplasia. Correlation of brain dysplasia with congenital skeletal abnormalities is unclear in our patient. We speculate that systemic hypoxic-ischemic insults during the second half of gestation and/or some genetic factors might be possible causes of brain dysplasia. PMID:8652025

  18. 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

  19. Bilateral Common Carotid Artery Occlusion in Spontaneously Hypertensive Rats: A Feasible Animal Model for Ocular Ischemic Syndrome.

    PubMed

    Wang, Yacong; Fan, Yuhua; Zhang, Lihong; Wang, Yi-Xiang J; Qi, Wei; Liang, Willmann; Wang, Chunmei; T W Yew, David; Ye, Cunxi; Sha, Ou

    2016-06-01

    The purpose of this study was to investigate the feasibility of inducing ocular ischemic syndrome in spontaneously hypertensive rats. Hypertensive and normotensive Wistar-Kyoto rats had bilateral occlusion or sham surgery. They were divided into 4 groups: (1) hypertensive-ischemia, (2) hypertensive-sham, (3) normotensive-ischemia, and (4) normotensive-sham. Four months after the operation, the global changes of the eye and pupillary light reflex were assessed. Then each rat was perfused, and randomly one of the bulbuses oculi was prepared as retinal flat mounts for investigation of vascular changes. The opposite eyeball was prepared as a paraffin section for observation of the linear density of retinal ganglion cells and for thickness measurement. One hypertensive-ischemia rat had a cataract in one eye and another rat in the same group had bulbus oculi collapse in one eye. The light reflex disappeared in 13.33% of hypertensive-ischemia rats, and the rest of the hypertensive-ischemia rats and normotensive-ischemia rats had slow reflex. Compared with the respective controls, the peripheral retinal vascular network in hypertensive-ischemia and normotensive-ischemia rats was sparse; linear density of the retinal ganglion cells was significantly reduced; and the retinal thickness was reduced. Compared with normotensive-ischemia rats, the hypertensive-ischemia rats demonstrated more severe changes. After bilateral common carotic artery occlusion, the eyes of hypertensive rats developed various pathological changes similar to those of ocular ischemic syndrome. In conclusion, an animal model for ocular ischemic syndrome can be created by bilateral common carotid artery occlusion in spontaneously hypertensive rats. Anat Rec, 299:806-814, 2016. © 2016 Wiley Periodicals, Inc. PMID:26917224

  20. Pure sensory syndromes and post-stroke pain secondary to bilateral thalamic lacunar infarcts: a case report

    PubMed Central

    2012-01-01

    Introduction Patients often complain about sensory symptoms that appear to the doctor as harmless, and reassurances are often given. Sensory strokes may easily be ignored. Case presentation A 48-year-old Caucasian woman with insulin-dependent diabetes and hyperlipidemia experienced symptoms that progressed within hours to a complete left-sided hemisensory syndrome. This was caused by a lacunar infarct in the ventral posterior tier nuclei of the right thalamus. A few days later she gradually developed an almost identical, but incomplete hemisensory syndrome on the opposite side caused by a corresponding lacune in the left thalamus. Severe persistent and paroxysmal pain on both sides of the body became disabling. Conclusion Small strokes only affecting the somatosensory system should not be underestimated. Neuropathic pain may result. Probably unique in the present case is the demonstration of bilateral thalamic pain secondary to two almost identical thalamic infarcts. Small vessel disease (microatheroma or lipohyalinosis) was the most likely cause of the lacunes. One can only speculate if there was an occlusion in two separate thalamic perforators, or in a single dominant artery supplying the bilateral thalami. PMID:23095785

  1. 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

  2. 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

  3. Bilateral Proliferative Retinopathy Associated With Hoyeraal-Hreidarsson Syndrome, a Severe Form of Dyskeratosis Congenita.

    PubMed

    Allingham, Michael J

    2016-04-01

    Dyskeratosis congenita (DC) is the prototypical member of a family of diseases caused by defective telomere maintenance. These "telomeropathies" also include Hoyeraal-Hreidarsson syndrome (HH) and Revesz syndrome, which are severe forms of dyskeratosis congenita, as well as a subset of idiopathic pulmonary fibrosis, aplastic anemia, and Coats' plus syndrome. Retinopathy has only rarely been reported in DC and HH, but is universally present in Coats' plus and Revesz syndromes. The care of these patients is typically a multidisciplinary effort, and this should include monitoring by an ophthalmologist. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:366-368.]. PMID:27065378

  4. [A case of Peutz-Jeghers syndrome combined with bilateral breast cancer, an adenocarcinoma of the cervix and ovarian genital cord neoplasms with annular tubules].

    PubMed

    Gloor, E

    1978-05-13

    The clinico-pathological findings are presented in a case of Peutz-Jeghers syndrome associated with a bilateral mammary invasive ductal carcinoma, a well-differentiated mucinous adenocarcinoma of the cervix and microscopic, bilateral ovarian sex cord tumors with annular tubules. The sex cord tumor with annular tubules was described in 1970 by SCULLY, who recognized its striking association with the Peutz-Jeghers syndrome. Two cases of adenocarcinoma of the cervix and another case of uterine adenocarcinoma of unspecified localization associated with Peutz-Jeghers syndrome were found in the literature. It is possible that women with Peutz-Jeghers syndrome run an increased risk of developing adenocarcinoma of the uterine cervix. PMID:644282

  5. A Case of Acute Bilateral Irvine-Gass Syndrome following Uncomplicated Phacoemulsification, Demonstrated with Optical Coherence Tomography

    PubMed Central

    Shields, Melissa K.; Adler, Paul A.; Fuzzard, Dujon R.W.; Chalasani, Rajeeve; Teong, Joanne M.Y.

    2015-01-01

    Purpose To report a case of acute bilateral Irvine-Gass syndrome. Methods This is an observational case report. Results An 82-year-old man with no significant ocular history developed postsurgical pseudophakic cystoid macular edema (CME; Irvine-Gass syndrome) on consecutive phacoemulsification cataract surgeries. His initial first-eye (left) CME developed 25 days after surgery and was managed with topical preparations of dexamethasone 0.1% and ketorolac 0.4%, in addition to a routine post-cataract surgery drop regime. His left CME resolved completely on optical coherence tomography (OCT) by day 100, and he subsequently (after extensive discussion of CME risks) underwent cataract surgery on his right eye. He was commenced prophylactically on dexamethasone, ketorolac and oral indomethacin 25 mg t.d.s. immediately after surgery; however, he later developed CME (OD) on day 32 postoperatively. Within 6 months, he achieved complete resolution of his CME in both eyes. His clinical course was documented with serial OCT studies. Conclusion Irvine-Gass syndrome remains an important differential diagnosis in the evaluation of blurred vision after cataract surgery, despite decreasing incidence. Those who experience CME following their first cataract operation should be counseled about the risks of developing the condition in the contralateral eye, despite prophylactic measures. PMID:26034486

  6. 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

  7. Horner's syndrome associated with a mandibular symphyseal fracture and bilateral temporomandibular luxation.

    PubMed

    Baines, S J; Langley-Hobbs, S

    2001-12-01

    Interruption of the postganglionic neuron in the sympathetic pathway to the eye is reported to be a common cause of Horner's syndrome in the cat, although there are few clinical reports identifying the site and nature of the lesion responsible. A case of Horner's syndrome resulting from trauma to the periorbital structures following reduction of temporomandibular joint luxation is presented. In this case, the ophthalmic abnormalities resolved spontaneously over eight days. PMID:11791777

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

    PubMed

    Peir, Gloria; Adrover, Encarna; Guijarro, Jaime; Ballester, Irene; Jimenez, M Jos; Planelles, Mara; Catass, 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

  9. Multiple osteoblastomas in a child with Cushing syndrome due to bilateral adrenal micronodular hyperplasias

    PubMed Central

    Yu, Hyeoh Won; Cho, Won Im; Choi, Keun Hee; Yun, Sumi; Cho, Hwan Seong; Shin, Choong Ho; Yang, Sei Won

    2016-01-01

    Adrenocorticotropin-independent adrenal hyperplasias are rare diseases, which are classified into macronodular (>1 cm) and micronodular (≤1 cm) hyperplasia. Micronodular adrenal hyperplasia is subdivided into primary pigmented adrenocortical disease and a limited or nonpigmented form 'micronodular adrenocortical disease (MAD)', although considerable morphological and genetic overlap is observed between the 2 groups. We present an unusual case of a 44-month-old girl who was diagnosed with Cushing syndrome due to MAD. She had presented with spotty pigmentation on her oral mucosa, lips and conjunctivae and was diagnosed with multiple bone tumors in her femur, pelvis and skull base at the age of 8 years. Her bone tumor biopsies were compatible with osteoblastoma. This case highlights the importance of verifying the clinicopathologic correlation in Cushing syndrome and careful follow-up and screening for associated diseases. PMID:27104180

  10. Multiple osteoblastomas in a child with Cushing syndrome due to bilateral adrenal micronodular hyperplasias.

    PubMed

    Yu, Hyeoh Won; Cho, Won Im; Chung, Hye Rim; Choi, Keun Hee; Yun, Sumi; Cho, Hwan Seong; Shin, Choong Ho; Yang, Sei Won

    2016-03-01

    Adrenocorticotropin-independent adrenal hyperplasias are rare diseases, which are classified into macronodular (>1 cm) and micronodular (≤1 cm) hyperplasia. Micronodular adrenal hyperplasia is subdivided into primary pigmented adrenocortical disease and a limited or nonpigmented form 'micronodular adrenocortical disease (MAD)', although considerable morphological and genetic overlap is observed between the 2 groups. We present an unusual case of a 44-month-old girl who was diagnosed with Cushing syndrome due to MAD. She had presented with spotty pigmentation on her oral mucosa, lips and conjunctivae and was diagnosed with multiple bone tumors in her femur, pelvis and skull base at the age of 8 years. Her bone tumor biopsies were compatible with osteoblastoma. This case highlights the importance of verifying the clinicopathologic correlation in Cushing syndrome and careful follow-up and screening for associated diseases. PMID:27104180

  11. 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

  12. 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

  13. 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

  14. 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

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

    PubMed

    Scial, 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

  16. 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

  17. 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

  18. 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

  19. Perfusion imaging of the right perisylvian neural network in acute spatial neglect.

    PubMed

    Zopf, Regine; Fruhmann Berger, Monika; Klose, Uwe; Karnath, Hans-Otto

    2009-01-01

    Recent studies have suggested a tightly connected perisylvian neural network associated with spatial neglect. Here we investigated whether structural damage in one part of the network typically is accompanied with functional damage in other, structurally intact areas of this network. By combining normalized fluid-attenuated inversion-recovery (FLAIR) imaging, diffusion-weighted imaging (DWI), and perfusion-weighted imaging (PWI) we asked whether or not lesions centering on fronto-temporal regions co-occur with abnormal perfusion in structurally intact parietal cortex. With thresholds applied to delineate behaviourally relevant malperfusion of brain tissue, the analysis of normalized time-to-peak (TTP) and maximal signal reduction (MSR) perfusion maps did not reveal significant changes outside the area of structural damage. In particular, we found no abnormal perfusion in the structurally intact inferior parietal lobule (IPL) and/or the temporo-parietal junction (TPJ). The present results obtained in three consecutively admitted neglect patients with fronto-temporal lesions indicate that structural damage in one part of the right perisylvian network associated with spatial neglect does not necessarily require dysfunction by malperfusion in other, structurally intact parts of the network to provoke spatial neglect. The neural tissue in the fronto-temporal cortex appears to have an original role in processes of spatial orienting and exploration. PMID:19680470

  20. Huge bilateral ovarian cysts in adulthood as the presenting feature of Van Wyk Grumbach syndrome due to chronic uncontrolled juvenile hypothyroidism.

    PubMed

    Shivaprasad, K S; Dutta, Deep; Jain, Rajesh; Kumar, Manoj; Maisnam, Indira; Biswas, Dibakar; Ghosh, Sujoy; Mukhopadhyay, Satinath; Chowdhury, Subhankar

    2013-10-01

    Juvenile primary hypothyroidism causing cystic ovaries and pseudoprecocious puberty (Van-Wyk Grumbach syndrome (VWGS)) is well documented in literature. There are only a few reports of primary hypothyroidism presenting as ovarian cysts in adults. Here we present a case of huge bilateral ovarian cysts in adulthood as the presenting feature of VWGS due to chronic uncontrolled juvenile hypothyroidism. Large uniloculor right ovarian cyst (119 × 81 × 90 mm) and a multicystic left ovary (55 × 45 × 49 mm) were detected in a 24 year lady with secondary amenorrhea, galactorrhea, and palpable abdominal mass with history of neonatal jaundice, delayed milestones, short stature, and precocious menarche at age of 7.5 years age. She had elevated levels of cancer antigen (CA)-125 which normalized post levothyroxine supplementation. Elevated CA-125 may lead to misdiagnosis of ovarian carcinoma and inadvertent treatment. Bilateral ovarian cysts in adults are a rare presentation of juvenile hypothyroidism. It is necessary to screen for primary hypothyroidism in patients presenting with bilateral ovarian cysts to prevent unnecessary evaluation and treatment. PMID:24251145

  1. 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

  2. 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…

  3. 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

  4. Mapping phonemic processing zones along human perisylvian cortex: an electro-corticographic investigation

    PubMed Central

    Molholm, Sophie; Mercier, Manuel R.; Liebenthal, Einat; Schwartz, Theodore H.; Ritter, Walter; Foxe, John J.; De Sanctis, Pierfilippo

    2015-01-01

    The auditory system is organized such that progressively more complex features are represented across successive cortical hierarchical stages. Just when and where the processing of phonemes, fundamental elements of the speech signal, is achieved in this hierarchy remains a matter of vigorous debate. Non-invasive measures of phonemic representation have been somewhat equivocal. While some studies point to a primary role for middle/anterior regions of the superior temporal gyrus (STG), others implicate the posterior STG. Differences in stimulation, task and inter-individual anatomical/functional variability may account for these discrepant findings. Here, we sought to clarify this issue by mapping phonemic representation across left perisylvian cortex, taking advantage of the excellent sampling density afforded by intracranial recordings in humans. We asked whether one or both major divisions of the STG were sensitive to phonemic transitions. The high signal-to-noise characteristics of direct intracranial recordings allowed for analysis at the individual participant level, circumventing issues of inter-individual anatomic and functional variability that may have obscured previous findings at the group level of analysis. The mismatch negativity (MMN), an electro-physiological response elicited by changes in repetitive streams of stimulation, served as our primary dependent measure. Oddball configurations of pairs of phonemes, spectro-temporally matched non-phonemes, and simple tones were presented. The loci of the MMN clearly differed as a function of stimulus type. Phoneme representation was most robust over middle/anterior STG/STS, but was also observed over posterior STG/SMG. These data point to multiple phonemic processing zones along perisylvian cortex, both anterior and posterior to primary auditory cortex. This finding is considered within the context of a dual stream model of auditory processing in which functionally distinct ventral and dorsal auditory processing pathways may be engaged by speech stimuli. PMID:23708059

  5. 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

  6. Birt-Hogg-Dubé syndrome detected incidentally by asymptomatic bilateral pneumothorax in health screening: a case of a young Japanese woman.

    PubMed

    Miura, Kentaro; Kondo, Ryoichi; Kurai, Makoto; Ishii, Keiko

    2015-12-01

    Birt-Hogg-Dubé syndrome (BHD) is an autosomal dominant disease caused by mutations of germline folliculin (FCLN) mapped in the chromosome 17p11.2 region. BHD commonly accompanies renal tumors, fibrofolliculomas, multiple pulmonary cysts, and spontaneous pneumothorax. We report a case of a young Japanese woman in whom asymptomatic bilateral pneumothorax was found incidentally in a health screening, which led to the diagnosis of BHD. She had developed neither renal tumors nor fibrofolliculomas. However, her father, uncle, and aunt also experienced pneumothorax. In Japan, BHD is not yet well known because skin-related symptoms of fibrofolliculomas are sometimes absent unlike in most cases in Europe and the United States. On the basis of this case, we propose that BHD should be considered at the time of pneumothorax examination. PMID:26943385

  7. 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

  8. 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

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

    PubMed Central

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

    2016-01-01

    Abstract 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

  10. Bilateral patellar tendon-bearing Symes-type prostheses in a severe case of Freeman-Sheldon syndrome in a 21-year-old woman presenting with uncorrectable equinovarus.

    PubMed

    McCormick, Rodger J; Poling, Mikaela I; Chamberlain, Robert L

    2015-01-01

    Described is the use of bilateral patellar tendon-bearing Symes-type prostheses in a severe case of Freeman-Sheldon syndrome (FSS) in a 21-year-old woman presenting with uncorrectable equinovarus and multiple surgical sequela of the lower extremities. FSS is considered a type of myopathic distal arthrogryposis; diagnosis requires the following: microstomia, whistling-face appearance, H-shaped chin dimpling, nasolabial folds, and multiple contractures of the hands and feet. Spinal deformities, metabolic and gastroenterological problems, other dysmorphic craniofacial characteristics, and visual and auditory impairments, are frequent findings. We highlight what we believe to be a unique approach to enhancing the patient's ability to ambulate and engage in physical activity, particularly critical determinates of wellness and long-term functional outcome in FSS, when the feet are non-correctable but not amputated. Important implications exist for physiatrists, other rehabilitation providers and surgeons regarding this likely under-diagnosed syndrome. PMID:26178231

  11. 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

  12. [Bilateral renal agenesis or Potter's sequence. A case of recurrent bilateral renal agenesis].

    PubMed

    Néophytou, O

    1994-01-01

    Bilateral renal agenesis (Potter's sequence) is usually a sporadic occurrence. Recurrence in the same family is rare. A family study of bilateral and unilateral renal agenesis was undertaken in three cases of renal agenesis in the same family. The grand-father and the father have a unilateral renal agenesis and his two stillborn infants had a Potter's syndrome. The author considered the familial nature of congenital absence of the kidneys and the relationship between hereditary bilateral renal agenesis and unilateral renal agenesis. PMID:8157895

  13. Forme fruste of Proteus syndrome: A limited form with bilateral plantar cerebriform collagenomas and varicose veins secondary to a mosaic AKT1 mutation

    PubMed Central

    Wee, Jamie S.; Mortimer, Peter S.; Lindhurst, Marjorie J.; Chong, Heung; Biesecker, Leslie G.; Holden, Colin A.

    2014-01-01

    Importance Proteus syndrome is an extremely rare disorder of mosaic postnatal overgrowth affecting multiple tissues including bone, soft tissue and skin. It typically presents in early childhood with asymmetric and progressive skeletal overgrowth that leads to severe distortion of the skeleton and disability. The genetic basis has recently been identified as a somatic activating mutation in the AKT1 gene, which encodes an enzyme mediating cell proliferation and apoptosis. Observations We present a 33-year-old man that developed plantar cerebriform collagenomas on the soles of both feet and varicose veins in early childhood, in the absence of any skeletal or other connective tissue abnormality. Although the patient did not meet the diagnostic criteria for Proteus syndrome, he was found to have the c.49G>A, p.Glu17Lys AKT1 mutation in lesional skin but not in his blood. Conclusions and Relevance To our knowledge, this is the mildest molecularly confirmed patient with Proteus syndrome, occurring in the absence of the characteristic skeletal overgrowth. These findings extend the spectrum of Proteus syndrome pathology and suggest that somatic mutations late in development and restricted in distribution cause subtle clinical presentations that do not meet the published clinical criteria. PMID:24850616

  14. 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

  15. 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

  16. 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

  17. 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

  18. 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

  19. Bilateral dentigerous cyst in a nonsyndromic patient: case report and literature review.

    PubMed

    Cury, Sérgio Elias Vieira; Cury, Maria Dorotéa Pires Neves; Cury, Sérgio Elias Neves; Pontes, Flávia Siroteau Corrêa; Pontes, Hélder Antonio Rebelo; Rodini, Camila; Pinto, Décio dos Santos

    2009-01-01

    Dentigerous cysts are the most common developmental cysts of the jaws and are associated with impacted teeth. Bilateral dentigerous cysts are rare and typically occur in association with a developmental syndrome. The occurrence of bilateral dentigerous cysts in the absence of a developmental syndrome is very rare. The purpose of this paper was to report on a nonsyndromic, 5-year-old boy who presented mandibular bilateral dentigerous cysts. PMID:19341587

  20. Bilateral diffuse uveal melanocytic proliferation: a management dilemma

    PubMed Central

    Alrashidi, Salah; Aziz, Ayman A; Krema, Hatem

    2014-01-01

    A female patient suffered from gradual decline of vision for few months. She presented with bilateral multiple pigmented choroidal tumours, associated with overlying retinal changes. The clinical presentation suggested bilateral diffuse uveal melanocytic proliferation (BDUMP) syndrome, which is a paraneoplastic disease, although there was no evidence of any concurrent malignancy. The periodic systemic surveillance that was undertaken for the following 4 years failed to reveal any occult cancer. Nevertheless, there has been relentless progressive deterioration in vision as a consequence of BDUMP syndrome. The management of the declining vision in BDUMP syndrome is challenging and controversial. PMID:24855079

  1. 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

  2. Bilateral Integrative Medicine, Obviously

    PubMed Central

    Stumpf, Steven H.; Shapiro, Simon J.

    2006-01-01

    Unstated and unacknowledged bias has a profound impact on the nature and implementation of integrative education models. Integrative education is the process of training conventional biomedical and traditional Chinese medicine practitioners in each tradition such that patient care may be effectively coordinated. A bilateral education model ensures that students in each tradition are cross-taught by experts from the ‘other’ tradition, imparting knowledge and values in unison. Acculturation is foundational to bilateral integrative medical education and practice. Principles are discussed for an open-minded bilateral educational model that can result in a new generation of integrative medicine teachers. PMID:16786060

  3. 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

  4. 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

  5. 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

  6. 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

  7. Akinetic mutism and bilateral anterior cerebral artery occlusion

    PubMed Central

    Freemon, Frank R.

    1971-01-01

    Three cases of bilateral anterior cerebral artery occlusion are presented with akinetic mutism. The anatomical distribution of the infarction in these patients combined with cases in the literature suggests that this syndrome can have a localizing value for the clinician. If increased intraventricular pressure is not present, the clinician can suspect a bilateral lesion of cingulate gyrus, medial nuclei of basal ganglia, and/or anterior and reticular nuclei of the thalamus. Images PMID:5158785

  8. [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

  9. 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

  10. A rare case of complete bilateral ophthalmoplegia and ptosis.

    PubMed

    Hall, Daniel John; Bazaraa, Talal

    2014-01-01

    We describe the case of an 85-year-old gentleman admitted with bilateral ptosis and complete bilateral ocular paralysis. Initial differential diagnoses included myasthenia gravis, diabetic cranial neuropathy, an ischaemic event and possible occult neoplasm. Investigations did not support any of the differentials and Miller Fisher syndrome (MFS) was considered. Anti-GQ1b IgG antibody was positive, supporting the possibility of anti-ganglioside syndrome. This gentleman was treated with intravenous immunoglobulin (IVIG) and made a full recovery. PMID:23999535

  11. 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

  12. Bilateral inferior petrosal sinus sampling using vasopressin

    PubMed Central

    Kotwal, Narendra; Kumar, Yogesh; Upreti, Vimal; Singh, Amandeep; Garg, M. K.

    2016-01-01

    Context: Anatomical localization of pituitary adenoma can be challenging in adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome, and bilateral inferior petrosal sinus sampling (BIPSS) is considered gold standard in this regard. Stimulation using corticotrophin-releasing hormone (CRH) improves the sensitivity of BIPSS, however, same is not easily available in India. Therefore, we undertook this study of BIPPS using vasopressin as agent for stimulation owing to its ability to stimulate V3 receptors present on corticotrophs. Aims: To study the tumor localization and lateralization in difficult to localize cases of ACTH-dependent Cushing's syndrome by bilateral inferior petrosal sinus sampling using vasopressin for corticotroph stimulation. Settings and Design: Prospective observational study. Subjects and Methods: Six patients (5 females) meeting inclusion criteria underwent BIPSS using vasopressin for stimulation. Results: All six patients had nonsuppressible overnight and low dose dexamethasone suppression test with elevated plasma ACTH levels suggestive of ACTH-dependent Cushing's syndrome. High dose dexamethasone suppression test showed suppressible cortisol in two cases, and microadenoma was seen in two patients on magnetic resonance imaging pituitary. Contrast enhanced computed tomography of the abdomen showed left adrenal hyperplasia in one case and anterior mediastinal mass with bilateral adrenal hyperplasia another. Using BIPSS four patients were classified as having Cushing's disease that was confirmed histopathologically following surgery. Of the remaining two, one had primary pigmented nodular adrenocortical disease, and another had thymic carcinoid with ectopic ACTH production as the cause of Cushing's syndrome. No serious adverse events were noted. Conclusions: Vasopressin may be used instead of CRH and desmopressin for stimulation in BIPSS. PMID:27186561

  13. Bilateral dentigerous cysts that involve all four dental quadrants: a case report and literature review

    PubMed Central

    2016-01-01

    Dentigerous cysts are common odontogenic cysts that are associated with the crown of the tooth and typically develop from single lesions. Bilateral and multiple dentigerous cysts are very rare and occur in patients with syndromic conditions. This paper presents a case report of a 15-year-old male patient that experienced non-syndromic bilateral dentigerous cysts that simultaneously occurred in all four dental quadrants around the unerupted third molars. Clinicians should confirm the extent of cystic lesions using a panoramic view and computed tomography, and should keep the possibility of bilateral dentigerous cysts in mind as a potential diagnosis, even in a non-syndromic patient. PMID:27162755

  14. Bilateral dentigerous cysts that involve all four dental quadrants: a case report and literature review.

    PubMed

    Jeon, Jae-Yun; Park, Chang-Joo; Cho, Seok Hyun; Hwang, Kyung-Gyun

    2016-04-01

    Dentigerous cysts are common odontogenic cysts that are associated with the crown of the tooth and typically develop from single lesions. Bilateral and multiple dentigerous cysts are very rare and occur in patients with syndromic conditions. This paper presents a case report of a 15-year-old male patient that experienced non-syndromic bilateral dentigerous cysts that simultaneously occurred in all four dental quadrants around the unerupted third molars. Clinicians should confirm the extent of cystic lesions using a panoramic view and computed tomography, and should keep the possibility of bilateral dentigerous cysts in mind as a potential diagnosis, even in a non-syndromic patient. PMID:27162755

  15. Transient cheiro-oral syndrome due to a ruptured intracranial dermoid cyst.

    PubMed

    Nakamura, M; Mizuguchi, M; Momoi, M Y; Chou, H; Masuzawa, T

    2001-07-01

    We present here a case of episodic, pure cheiro-oral syndrome caused by a ruptured intracranial dermoid cyst. Cranial magnetic resonance imaging (MRI) using the fat-suppression method revealed a fatty mass lesion in the subarachnoid space of the left parasellar region and multiple lipid droplets in the subarachnoid space over the left perisylvian area. Although no evidence for it pathogenesis was obtained, the patient's cheiro-oral syndrome could have resulted from a transient vasospasm around the left ventral posterior thalamic nucleus or postcentral gyrus. PMID:11377009

  16. [Bilateral primary adrenal lymphoma].

    PubMed

    Bakkali, Hanae; el Omari-Alaoui, Hind; Elghazi, El Abbès; Errihani, Hassan; Benjaafar, Nourredine; Elgueddari, Brahim El Khalil

    2002-12-01

    The adrenal gland is a rare site of non-Hodgkin's lymphoma, as only about 70 cases have been reported in the literature, usually with bilateral involvement. Most tumours have a high grade histology, almost always with the B phenotype. Medical imaging is nonspecific and biopsy remains the most reliable diagnostic method. Chemotherapy is the treatment of choice, but the prognosis remains poor in the majority of cases, although long-term survivals have been described. The authors report a case of bilateral high-grade lymphoma of the adrenal glands in a 31-year-old patient presenting with acute adrenal insufficiency. Imaging demonstrated large bilateral adrenal masses, and surgical biopsy of the adrenal gland and staging confirmed the diagnosis of bilateral primary adrenal lymphoma. After corticosteroid replacement therapy, treatment consisted of primary CHOP chemotherapy administered for 9 cycles, followed by external beam radiotherapy delivered at a dose of 40 Gy. After 3 months of follow-up, the patient was still alive, with partial response, with an overall survival of 15 months. PMID:12545639

  17. Bilateral Mandibular Paramolars

    PubMed Central

    Dhull, Rachita Singh; Panda, Swagatika; Acharya, Sonu; Yadav, Shweta; Mohanty, Gatha

    2014-01-01

    ABSTRACT Supernumerary tooth is a developmental anomaly and has been argued to arise from multiple etiologies. These teeth may remain embedded in the alveolar bone or can erupt into the oral cavity. They can cause a variety of complications in the develo­ping dentition. Supernumerary teeth can present in various forms and in any region of the mandible or maxilla, but have a predisposition for the anterior maxilla. Here is the presentation of a case of unusual location of supernumerary teeth located in between mandibular first and second molar region bilaterally. How to cite this article: Dhull KS, Dhull RS, Panda S, Acharya S, Yadav S, Mohanty G. Bilateral Mandibular Paramolars. Int J Clin Pediatr Dent 2014;7(1):40-42. PMID:25206236

  18. Bilateral tuberculate supernumerary teeth.

    PubMed

    Eigbobo, Joycelyn Odegua; Osagbemiro, Babatope Bamidele

    2011-05-16

    Supernumerary teeth are teeth in excess of the normal series occurring in any region of the dental arch. They are located mostly in the anterior maxillary region and are classified according to their location and morphology. The tuberculate type of supernumerary tooth possesses more than one cusp or tubercle (barrel shaped). It is rare to find bilateral tuberculate supernumerary teeth in the premaxillary region and when found they rarely erupt. This report describes a 13-year-old boy with erupted palatally placed bilateral tuberculate supernumerary teeth. The presence of these supernumerary teeth led to the labial displacement and rotations of the anterior maxillary teeth. The treatment involved extraction of the supernumerary teeth and a referral for orthodontic management of the crowding, displacement and rotations. The occurrence of erupted palatally placed tuberculate anterior teeth in this case is a rare experience. However, the associated orthodontic problems are within familiar spectrum. PMID:24765292

  19. Bilateral Nipple Leiomyoma

    PubMed Central

    Kapakli, Mahmut Sertan; Altintoprak, Fatih; Cayırcı, Mine; Manukyan, Manuk Norayk; Kebudi, Abut

    2013-01-01

    Cutaneous leiomyomas are benign smooth muscle neoplasms of the skin. They arise from vascular, arrector pili, genital, and areolar smooth muscles. The most common localizations of cutaneous leiomyomas are the extensor surfaces of the extremities and the trunk. To our knowledge, only few cases of one-sided nipple leiomyomas have been reported, but two-sided nipple leiomyomas have not been presented. For the first time, here, we report a bilateral nipple leiomyoma. PMID:23762734

  20. Confabulations after Bilateral Consecutive Strokes of the Lenticulostriate Arteries

    PubMed Central

    Carota, Antonio; Calabrese, Pasquale

    2012-01-01

    We describe the case of a 75-year-old woman who manifested persistent confabulations after two consecutive strokes encompassing the area of the lenticulostriate arteries territory on both hemispheres. Findings reported on this rare clinical syndrome suggest that fantastic confabulations and delusional thoughts may arise after bilateral damage of subcortical nonthalamic structures. PMID:22649343

  1. 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

  2. Bilateral maculopathy associated with Pierre Robin sequence.

    PubMed

    Witmer, Matthew T; Vasan, Ryan; Levy, Richard; Davis, Jessica; Chan, R V Paul

    2012-08-01

    Pierre Robin sequence has been associated with a number of ocular complications, including myopia, strabismus, Möbius syndrome, nasolacrimal duct obstruction, glaucoma, cataract, microphthalmos, coloboma of choroid, and retinal detachment. We report a 10-day-old boy who presented with micrognathia, glossoptosis, and cleft palate as well as multiple congenital anomalies. Ophthalmic examination was notable for bilateral maculopathy, with focal areas of retinal and retinal pigment epithelial atrophy. The association of Pierre Robin sequence and maculopathy has been reported only twice previously. PMID:22929457

  3. 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

  4. Congenital Bilateral Saccular Cysts and Bifid Epiglottis: Presentation and Management.

    PubMed

    Arif, Rawan; Al-Khatib, Talal; Daghistani, Razan; Shalabi, Maher

    2016-03-01

    Saccular disorders are rare representing only 1.5 % of all laryngeal anomalies. Bifid epiglottis is also an extremely rare congenital anomaly that usually occurs in a syndromic picture in association with other anomalies such as polydactyly, cleft palate and micrognathia, which are seen in Pallister-Hall Syndrome and rarely with other syndromes. We report a case of bilateral saccular cysts and bifid epiglottis in a full term neonate presenting with stridor. The patient's other congenital anomalies included microretrognathia, short neck, polydactyly of four extremities and hypospadias. The patient underwent staged endoscopic microsurgical marsupialization of both cysts and endoscopic repair of the bifid epiglottis. PMID:27066427

  5. Bilateral calcaneal stress fractures: a case report.

    PubMed

    Imerci, Ahmet; Incesu, Mustafa; Bozoglan, Muhammet; Canbek, Umut; Ursavas, Hüseyin Tamer

    2012-01-01

    The majority of plantar heel pain is diagnosed as plantar fasciitis or heel spur syndrome. When history or physical findings are unusual or when routine treatment proves ineffective, one should consider an atypical cause of heel pain. Stress fractures of the calcaneus are a frequently unrecognized source of heel pain. In a normal populatıon, the possibility of calcaneal stress fractures must be borne in mind with patients who have bilateral heel pain. When a stress fracture is considered, clinicans have different imaging options. First of all, x-rays must be used to evaluate for any visible osseous pathology. If plain films are inconclusive, the clinician can proceed with a bone scan or Magnetic Resonance Imaging. In the literature, calcaneal stress fractures are mostly reported in soldiers or athletes, but our case is one of a 44-year-old housewife with bilateral heel pain treated as Achilles tendinitis and plantar faciitis for a long time. Her final diagnosis was bilateral calcaneal fracture by Magnetic Resonance Imaging. PMID:23208939

  6. Melkersson-rosenthal syndrome.

    PubMed

    Sharma, D R; Resident, S; Mohan, C; Minnas, R S; Mohindroo, N K; Sharma, M L

    1999-10-01

    Melkersson - Rosenthal syndrome was described by Melkersson and Rosenthal separately in the year 1928 and 1931 respectively. It is supposed to be a rare syndrome of bilateral alternating recurrent facial paralysis alongwith fissured tongue and oedema of the lips, face and eyelids. A case of Melkersson - Rosenthal syndrome is reported with all the classic findings which is a rarity. In this case there was alternating facial paralysis to begin with followed by bilateral paralysis third time, along with oedema of lips and face, fissured tongue, and dialation of sig-moid colon with absence of haustrations. PMID:23119566

  7. 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

  8. 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

  9. 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

  10. Idiopathic Bilateral Bloody Tearing

    PubMed Central

    Beyazyıldız, Emrullah; Özdamar, Yasemin; Beyazyıldız, 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. Syndrome in question. MAGIC syndrome.

    PubMed

    Nascimento, Ana Cláudia Mendes do; Gaspardo, Daniela Barros Cortez; Cortez, Tatiana Mimura; Miot, Hélio Amante

    2014-01-01

    The authors present a male 40-year-old patient with established diagnosis of Behçet's disease which had evolved to recurrent bilateral auricular polychondritis crises. MAGIC syndrome (mouth and genital ulcers with inflamed cartilage) is rare and groups together patients with this clinical picture without necessarily fulfilling the clinical criteria for Behçet's disease or relapsing polychondritis, demonstrating an independent disorder. PMID:24626673

  12. 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

  13. 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

  14. Acute silicosis with bilateral pneumothorax

    PubMed Central

    Srivastava, G N; Prasad, Rajniti; Meena, Manoj; Hussain, Moosa

    2014-01-01

    We present a case of acute silicosis with bilateral pneumothorax of a 28-year-old man working at a stone crusher factory for 1 year. He presented to the emergency department with cough, respiratory distress and diffuse chest pain. The patient was managed with bilateral intercostal tube drainage under water seal, oxygen inhalation and conservative therapy. On follow-up he showed improvement of resting dyspnoea and was doing well. This case is being reported because of the rare complications of acute silicosis as bilateral pneumothorax. PMID:24862410

  15. Patient with bilateral breast enlargement and hypospadias.

    PubMed

    Ali, A A; Ahmed, T; Rashid, M M

    2009-01-01

    A 20 year old man was admitted in the surgical unit of Dhaka Medical College Hospital on August 2007 presented with Hypospadias & underdeveloped penis from childhood, gradual enlargement of both breasts for the last 10 years, absence of axillary and pubic hair from puberty & painful swelling of both testes for the last 3 years. The patient was regarded as a female upto the age of 10 years. He has got no voice change since puberty. On examination he had average body built, there was bilateral gynaecomastia, hypospadias, rudimentary penis & absence of pubic and axillary hair. His testosterone level, serum prolactin level, serum progesterone level, serum estradiol level was done. Cytology on buccal smear done and ultrasonography revealed no ovary and uterus. Ultimately patient was diagnosed as a case of androgen insensitivity syndrome (AIS). The case is reported for clinical awareness & to share our experience. PMID:19182761

  16. 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

  17. BILATERAL WASTEWATER LAND TREATMENT RESEARCH

    EPA Science Inventory

    Diplomatic relations between the United States and China, established in 1979, opened the door for development of 1985 bilateral environmental research and technology transfer between the USEPA's Robert S. Kerr Environmental Research Laboratory, Ada, Oklahoma, and China's Beijing...

  18. Bilateral internal laryngoceles mimicking asthma.

    PubMed

    Aksoy, Elif A; Elsürer, Cağdaş; Serin, Gediz M; Unal, O Faruk

    2013-05-01

    Laryngocele is an air-filled, abnormal dilation of the laryngeal saccule that extends upward within the false vocal fold, in communication with the laryngeal lumen. A case of 43-year-old male with bilateral internal laryngoceles, who has been treated as asthma for 4 years, is presented. The patient had dyspnea, cough, and excessive phlegm for a month and a late onset stridor. Flexible nasopharyngolaryngoscopy showed bilateral cystic enlargements of the false vocal folds and true vocal folds could not be visualized. Laryngeal CT without contrast enhancement showed bilateral internal laryngoceles. Submucosal total excision of bilateral cystic masses including parts of false vocal folds was performed. The symptoms resolved immediately after surgery. Although the incidence of internal laryngocele is rare, it should be remembered in the differential diagnosis of upper airway problems and diagnostic flexible nasopharnygolaryngoscopy is routinely indicated for airway evaluation in at-risk patients. PMID:24174956

  19. Bilateral Polydactyly in a foal.

    PubMed

    Carstanjen, Bianca; Abitbol, Marie; Desbois, Christophe

    2007-06-01

    The following case report describes the diagnosis and surgery of bilateral polydactyly of unknown origin in a colt. A 7-month-old Berber colt was referred for cosmetic and curative excision of supernumerary digits. Radiographic examination revealed bilateral polydactyly and welldeveloped first carpal bones. Surgery consisted of an osteotomy of both second metacarpal bones combined with an amputation of the supernumerary digits. The follow-up at 18 months after surgery revealed a sound horse with an excellent cosmetic outcome. PMID:17519577

  20. 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...

  1. 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

  2. Hennekam lymphangiectasia syndrome

    PubMed Central

    Lakshminarayana, G.; Mathew, A.; Rajesh, R.; Kurien, G.; Unni, V. N.

    2011-01-01

    Hennekam lymphangiectasia syndrome is a rare disorder comprising of intestinal and renal lymphangiectasia, dysmorphic facial appearance and mental retardation. The facial features include hypertelorism with a wide, flat nasal bridge, epicanthic folds, small mouth and small ears. We describe a case of a multigravida with bad obstetric history and characteristic facial and dental anomalies and bilateral renal lymphangiectasia. To our knowledge this is the first case of Hennekam lymphangiectasia syndrome with anodontia to be reported from India. PMID:22022089

  3. 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. PMID:26982793

  4. 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

  5. Systemic vasculitis with bilateral perirenal haemorrhage in chronic myelomonocytic leukaemia

    PubMed Central

    Aslangul-Castier, E.; Papo, T.; Amoura, Z.; Baud, O.; Leblond, V.; Charlotte, F.; Bricaire, F.; Degos, L.; Piette, J.

    2000-01-01

    The cases of two patients with chronic myelomonocytic leukaemia associated with periarteritis nodosa-like, antineutrophil cytoplasmic antibody negative, systemic vasculitis, are reported.
  A 61 year old man was admitted with fever, diffuse myalgia, and abdominal pain. Blood and bone marrow examination showed chronic myelomonocytic leukaemia. Vasculitis of the gall bladder was responsible for acalculous cholecystitis. A massive spontaneous bilateral perirenal haemorrhage occurred. A 73 year old woman with chronic myelomonocytic leukaemia had been followed up for one year when unexplained fever occurred. Two months after the onset of fever, sudden abdominal pain was ascribed to spontaneous bilateral renal haematoma related to bilateral renal arterial aneurysms. Neuromuscular biopsy showed non-necrotising periarteriolar inflammation.
  To our knowledge, systemic vasculitis has never been reported in chronic myelomonocytic leukaemia. In our two cases a non-random association is suggested because (a) chronic myelomonocytic leukaemia is a rare myelodysplastic syndrome, (b) spontaneous bilateral perirenal haematoma is not a usual feature of periarteritis nodosa.

 PMID:10784523

  6. 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

  7. Bilateral extensive persistent pupillary membranes.

    PubMed

    Oner, Ayse; Ilhan, Ozgur; Dogan, Hakki

    2007-01-01

    We examined a 14-year-old girl with bilateral hyperplastic persistent pupillary membranes, present since birth, with poor visual acuity. The membranes were excised surgically; visual acuity improved. Extensive persistent pupillary membranes can be removed safely by surgical methods if they are large enough to obscure the pupillary axis and affect visual acuity. PMID:17274341

  8. 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

  9. Chronic exertional compartment syndrome of the superficial posterior compartment: Soleus syndrome

    PubMed Central

    Gross, Christopher E; Parekh, Bela J; Adams, Samuel B; Parekh, Selene G

    2015-01-01

    Chronic exertional compartment syndrome (CECS) represents the second most-common cause of exertional leg pain with incidence of 27-33%. CECS of the superficial posterior compartment, or soleus syndrome, is rare and has only been discussed briefly in the literature. We discuss the management of two patients with bilateral soleus syndrome or CECS of the superficial posterior compartment. PMID:26538766

  10. 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.

  11. 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 rle 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

  12. Female genital tract tumors in Peutz-Jeghers syndrome.

    PubMed

    Chen, K T

    1986-08-01

    Multiple genital tract tumors, including bilateral ovarian sex cord tumors with annular tubules, adenoma malignum of the cervix, and bilateral ovarian mucinous tumors, developed in a female patient with Peutz-Jeghers syndrome. Although each of these tumor types has been reported to occur in this syndrome, the present case appears to be the first in which the syndrome was complicated by tumors of all three types. PMID:3733073

  13. 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

  14. Bilateral progressive hearing loss and vestibular dysfunction with inner ear antibodies.

    PubMed

    Yukawa, Kumiko; Hagiwara, Akira; Ogawa, Yasuo; Nishiyama, Nobuhiro; Shimizu, Shigetaka; Kawaguchi, Sachie; Nakamura, Mari; Ito, Hiroyuki; Tomiyama, Shunichi; Suzuki, Mamoru

    2010-04-01

    Autoimmune inner ear disease (AIED) is a clinical syndrome of uncertain etiology. We present the neuro-otological findings of 2 cases of bilateral hearing loss, dizziness and the antibody profiles of the inner ears. Case 1 had bilateral progressive hearing loss, vestibular dysfunction and abnormal eye movement as the disease progressed. She had inner ear antibodies against 42 and 58kDa protein antigency on Western blot immune assay, and responded to glycocorticosteroid but not to immunosuppressant treatment. Intratympanic steroid injection temporally eliminated her symptoms. However, she developed idiopathic Cushing's syndrome and underwent labyrinthectomy. Case 2 became deaf as a teenager and experienced dizziness 10 years after becoming deaf. He reacted strongly to 68kDa protein and was a good responder to immunosuppressant with steroid. As we still lack a definitive diagnostic test for AIED, careful observation of the clinical course is critical for differential diagnosis regarding the bilateral progressive hearing loss. PMID:19709829

  15. 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

  16. 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

  17. Bilateral Vocal Cord Paralysis after Anterior Cervical Discectomy Following Cervical Spine Injury: A Case Report

    PubMed Central

    Nikandish, Reza; Zareizadeh, Alireza; Motazedian, Siavash; Zeraatian, Sam; Zakeri, Habib; Ghaffarpasand, Fariborz

    2013-01-01

    Bilateral vocal cord paralysis is a rare and preventable complication of anterior cervical discectomy and fusion. Herein, we report a fatal case of bilateral vocal cord paralysis after anterior cervical discectomy and fusion (ACD/F). A 65-year-old man with cervical spine trauma and anterior cord syndrome, following car overturn presented to our emergency department. The patient had C6-T10 prolapsed discs for which ACD/F was performed. In the recovery room he developed stridor and respiratory distress immediately after extubation, and was reintubated. Otolaryngological evaluation revealed bilateral vocal cord paralysis. He later required a tracheostomy but finally died in a rehabilitation center after an acute coronary event. Awake fibroptic intubation is recommended in patients at high risk for preoperative recurrent laryngeal nerve injury. Intraoperative tracheal tube cuff pressure monitoring and modification of surgical approach to neck are recommended to prevent bilateral nerve damage.

  18. Bilateral suborbital rash: a dermatologic manifestation of neuropsychiatric disease in a pediatric patient.

    PubMed

    Tong, Lana X; Wang, Yen Tun; Beynet, David

    2014-05-01

    A 10-year-old girl presented with a new onset bilateral suborbital rash. Dermatologic examination revealed violaceous, non-tender, well-demarcated patches with an atypical distribution and pigmentation. After further investigation, a diagnosis of Munchausen syndrome was made and the patient was referred to her primary care provider for further management. PMID:24852783

  19. Bilateral cervical ribs in a Dobermann Pinscher.

    PubMed

    Ricciardi, M; De Simone, A; Gernone, F; Giannuzzi, P

    2015-01-01

    An 11-year-old intact female Doberman Pinscher was presented with the complaint of non-ambulatory tetraparesis. Clinical and neurological examination revealed a caudal cervical spinal cord disfunction (C6-T2 spinal cord segments). Magnetic resonance imaging and computed tomographic (CT) findings of the cervical spine were consistent with caudal cervical spondylomyelopathy (CSM). During the diagnostic work-up for the cervical spine, bilateral bone anomalies involving the seventh cervical vertebra and the first ribs were found on radiographs and CT examination. The rib anomalies found in this dog appear similar to cervical ribs widely described in human medicine. In people, cervical ribs are associated with a high rate of stillbirth, early childhood cancer, and can cause the thoracic outlet syndrome, characterized by neurovascular compression at level of superior aperture of the chest. In dogs, only some sporadic anatomopathological descriptions of cervical ribs exist. In this report the radiographic and CT findings of these particular vertebral and rib anomalies along with their relationships with adjacent vasculature and musculature are shown intravitam in a dog. Specific radiographic and CT findings described in this report may help in reaching a presumptive diagnosis of this anomaly. Finally, their clinical and evolutionary significance are discussed. PMID:25650786

  20. Bilateral lower extremity swelling: black pearl.

    PubMed

    Smithson, Clinton C; Ham, Jared C; Juergens, Andrew L

    2015-12-01

    Iatrogenic pneumothorax secondary to thoracentesis is relatively uncommon but does present to the emergency department (ED). Iatrogenic pneumothoraces developing tension physiology are rare. We report a case of an elderly female patient presenting to the ED with an isolated chief complaint of bilateral leg swelling, beginning the day after a thoracentesis, which was performed 3 days prior for pleural effusions secondary to lung cancer. Given that the patient was hemodynamically stable, not hypoxic, and had a history of chronic obstructive pulmonary disease and recent history of pleural effusions with diminished lung sounds throughout, this was a radiologic diagnosis. Immediately upon diagnosis, a 10F intrapleural catheter was inserted at the second intercostal space in the midclavicular line with successful resolution of the tension phenomenon. The patient tolerated the procedure well, and the catheter was removed on hospital day 2 without recurrence of the pneumothorax. She experienced resolution of her lower extremity swelling and was discharged from the hospital 2 days later. Isolated inferior vena cava syndrome secondary to a subacute tension pneumothorax was likely the cause of the patient's symptoms. This presentation is very rare and is undocumented in the literature. A high degree of suspicion for acute chest pathology should exist in every patient presenting to the ED with history of recent pleural violation. PMID:26003746

  1. 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

  2. Macular edema-like change and pseudopapilledema in a case of Scheie syndrome.

    PubMed

    Usui, T; Shirakashi, M; Takagi, M; Abe, H; Iwata, K

    1991-09-01

    We reported a case of Scheie syndrome in which diffuse fine corneal deposits, pigmentary retinal degeneration, pseudopapilledema, and macular edema-like change were observed bilaterally. This is the first report describing macular change in Scheie syndrome. PMID:1836802

  3. Heterogeneity in Waardenburg syndrome.

    PubMed Central

    Hageman, M J; Delleman, J W

    1977-01-01

    Heterogeneity of Waardenburg syndrome is demonstrated in a review of 1,285 patients from the literature and 34 previously unreported patients in five families in the Netherlands. The syndrome seems to consist of two genetically distinct entities that can be differentiated clinically: type I, Waardenburg syndrome with dystopia canthorum; and type II, Waardenburg syndrome without dystopia canthorum. Both types have an autosomal dominant mode of inheritance. The incidence of bilateral deafness in the two types of the syndrome was found in one-fourth with type I and about half of the patients with type II. This difference has important consequences for genetic counseling. Images Fig. 7 Fig. 8 Fig. 9 PMID:331943

  4. 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 ...

  5. [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

  6. Multistep joint bilateral depth upsampling

    NASA Astrophysics Data System (ADS)

    Riemens, A. K.; Gangwal, O. P.; Barenbrug, B.; Berretty, R.-P. M.

    2009-01-01

    Depth maps are used in many applications, e.g. 3D television, stereo matching, segmentation, etc. Often, depth maps are available at a lower resolution compared to the corresponding image data. For these applications, depth maps must be upsampled to the image resolution. Recently, joint bilateral filters are proposed to upsample depth maps in a single step. In this solution, a high-resolution output depth is computed as a weighted average of surrounding low-resolution depth values, where the weight calculation depends on spatial distance function and intensity range function on the related image data. Compared to that, we present two novel ideas. Firstly, we apply anti-alias prefiltering on the high-resolution image to derive an image at the same low resolution as the input depth map. The upsample filter uses samples from both the high-resolution and the low-resolution images in the range term of the bilateral filter. Secondly, we propose to perform the upsampling in multiple stages, refining the resolution by a factor of 2×2 at each stage. We show experimental results on the consequences of the aliasing issue, and we apply our method to two use cases: a high quality ground-truth depth map and a real-time generated depth map of lower quality. For the first use case a relatively small filter footprint is applied; the second use case benefits from a substantially larger footprint. These experiments show that the dual image resolution range function alleviates the aliasing artifacts and therefore improves the temporal stability of the output depth map. On both use cases, we achieved comparable or better image quality with respect to upsampling with the joint bilateral filter in a single step. On the former use case, we feature a reduction of a factor of 5 in computational cost, whereas on the latter use case, the cost saving is a factor of 50.

  7. The management of bilateral Wilms tumor.

    PubMed

    Özyörük, Derya; Emir, Suna

    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

  8. 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

  9. 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

  10. 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

  11. 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

  12. "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

  13. 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

  14. 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

  15. 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

  16. Fast bilateral filtering using recursive moving sum

    NASA Astrophysics Data System (ADS)

    Igarashi, Masaki; Ikebe, Masayuki; Shimoyama, Sohsuke; Motohisa, Junichi

    We propose a constant-time algorithm for a bilateral filter. Bilateral filter can be converted into the operation of three-dimensional (3D) convolution. By using recursive moving sum, we can reduce the number of calculations needed to construct a pseudo-Gaussian filter. Applying one-dimensional Gaussian filter to the 3D convolution, we achieved a constant-time bilateral filter. We used a 3-GHz CPU without SIMD instructions, or multi-thread operations. We confirmed our proposed bilateral filter to be processed in constant time. In practical conditions, high PSNR values over 40 dB are obtained.

  17. Ross' syndrome (tonic pupil plus).

    PubMed Central

    Hedges, T. R.; Gerner, E. W.

    1975-01-01

    Two cases of tonic pupil, hyporeflexia, and segmental hypohidrosis (Ross' syndrome) are reported. The relationship of this syndrome to other autonomic dysfunction is discussed. Those symptoms (emotional instability, loss of sweating, orthostatic hypotensive symptoms, and signs of bilaterality of the tonic pupil) which should alert the clinician to more extensive disease states are noted. It is suggested that these conditions may represent a continuum or spectrum of disorders with a widespread degree of severity and progression. Images PMID:1191625

  18. 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

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

    PubMed

    Lancer, Hannah R; Smitham, Peter; Ray, Pinak

    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

  20. 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

  1. Successful Bilateral Composite Ear Reattachment

    PubMed Central

    2014-01-01

    Summary: A successful bilateral ear composite graft nonmicrosurgical reattachment is presented. In cases where suitable vessels are unavailable for microsurgical revascularization, the reconstructive challenge can be formidable for salvaging the unique anatomic and aesthetic structure of the ear. The case is presented of an 18-year-old woman who was a victim of an assault wherein both of her ears were intentionally amputated by her attacker. She underwent successful surgical reattachment followed by a postoperative regimen of hyperbaric oxygen, cooling, and meticulous wound care. The patient achieved 100% survival of her left ear graft and 95% survival of her right ear graft. Clinical photographs at 18 months are presented, along with a discussion of the possible implications for other reconstructive applications. PMID:25289367

  2. Syndrome In Question*

    PubMed Central

    do Nascimento, Ana Cláudia Mendes; Gaspardo, Daniela Barros Cortez; Cortez, Tatiana Mimura; Miot, Hélio Amante

    2014-01-01

    The authors present a male 40-year-old patient with established diagnosis of Behçet's disease which had evolved to recurrent bilateral auricular polychondritis crises. MAGIC syndrome (mouth and genital ulcers with inflamed cartilage) is rare and groups together patients with this clinical picture without necessarily fulfilling the clinical criteria for Behçet's disease or relapsing polychondritis, demonstrating an independent disorder. PMID:24626673

  3. Usher Syndrome

    MedlinePlus

    ... Rare Diseases Information Center (GARD) Print friendly version Usher syndrome Table of Contents Overview Symptoms Cause Inheritance ... pigmentosa syndrome Dystrophia retinae pigmentosa-dysostosis syndrome Graefe-Usher syndrome Hallgren syndrome Usher's syndrome Related Diseases Usher ...

  4. 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…

  5. Bilateral painful idiopathic ophthalmoplegia: a case report.

    PubMed

    Nieri, Andrea; Bazan, Rodrigo; Almeida, Leila; Rocha, Fernando C; Raffin, César N; Bigal, Marcelo E; Schelp, Arthur O

    2007-06-01

    Around 3% of the individuals with painful ophthalmoplegia have bilateral complaints. In the vast majority of these cases, appropriate investigation demonstrates a secondary etiology, and we are not aware of idiopathic cases reported. Herein we report a case of bilateral ophthalmoplegia where extensive investigation did not suggest a secondary cause. PMID:17578533

  6. Bilateral pulmonary sequestration: computed tomographic appearance

    SciTech Connect

    Wimbish, K.J.; Agha, F.P.; Brady, T.M.

    1983-04-01

    Intralobar pulmonary sequestration is one manifestation of the wide spectrum of congenital bronchopulmonary foregut malformations. Bilateral intralobar pulmonary sequestration is an exceedingly rare anomaly. Only two pathologically proven cases and one possible case have been reported. We report a case presenting as bilateral paraspinal masses, studied by computed tomograpy (CT) and angiography.

  7. 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. PMID:27011881

  8. Bilateral microperc in a severe kyphoscoliosis.

    PubMed

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

    2016-03-01

    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. PMID:27011881

  9. Genetic Syndromes Associated with Craniosynostosis

    PubMed Central

    2016-01-01

    Craniosynostosis is defined as the premature fusion of one or more of the cranial sutures. It leads not only to secondary distortion of skull shape but to various complications including neurologic, ophthalmic and respiratory dysfunction. Craniosynostosis is very heterogeneous in terms of its causes, presentation, and management. Both environmental factors and genetic factors are associated with development of craniosynostosis. Nonsyndromic craniosynostosis accounts for more than 70% of all cases. Syndromic craniosynostosis with a certain genetic cause is more likely to involve multiple sutures or bilateral coronal sutures. FGFR2, FGFR3, FGFR1, TWIST1 and EFNB1 genes are major causative genes of genetic syndromes associated with craniosynostosis. Although most of syndromic craniosynostosis show autosomal dominant inheritance, approximately half of patients are de novo cases. Apert syndrome, Pfeiffer syndrome, Crouzon syndrome, and Antley-Bixler syndrome are related to mutations in FGFR family (especially in FGFR2), and mutations in FGFRs can be overlapped between different syndromes. Saethre-Chotzen syndrome, Muenke syndrome, and craniofrontonasal syndrome are representative disorders showing isolated coronal suture involvement. Compared to the other types of craniosynostosis, single gene mutations can be more frequently detected, in one-third of coronal synostosis patients. Molecular diagnosis can be helpful to provide adequate genetic counseling and guidance for patients with syndromic craniosynostosis. PMID:27226847

  10. Sarcoidosis Presenting as Bilateral Vocal Cord Paralysis due to Bilateral Vagal Nerve Involvement.

    PubMed

    Yamasue, Mari; Nureki, Shin-Ichi; Ushijima, Ryoichi; Mukai, Yutaka; Goto, Akihiko; Kadota, Jun-Ichi

    2016-01-01

    We herein report a rare case of sarcoidosis presenting as bilateral vocal cord paralysis due to bilateral vagal nerve involvement. A 72-year-old woman with uveitis of the left eye complained of hoarseness and aspiration due to bilateral vocal cord paralysis. An endobronchial needle aspiration biopsy specimen of the mediastinal lymph nodes showed non-caseating epithelioid cell granuloma. Total protein and cell concentrations in the cerebrospinal fluid were increased. We diagnosed her to have sarcoidosis with bilateral vagal nerve involvement. Corticosteroid therapy improved her symptoms of hoarseness and aspiration. Sarcoidosis should therefore be taken into consideration as a potential cause of bilateral vocal cord paralysis. PMID:27150886

  11. 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

  12. 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

  13. 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

  14. 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.

  15. Fibromuscular Dysplasia Presenting with Bilateral Renal Infarction

    SciTech Connect

    Doody, O.; Adam, W. R.; Foley, P. T.; Lyon, S. M.

    2009-03-15

    Fibromuscular dysplasia (FMD) describes a group of conditions which cause nonatheromatous arterial stenoses, most commonly of the renal and carotid arteries, typically in young women. We report a rare case of bilateral segmental renal infarction secondary to FMD in a young male patient. His initial presentation with loin pain and pyrexia resulted in a delay in the definitive diagnosis of FMD. He was successfully treated with bilateral balloon angioplasty. The delayed diagnosis in this patient until the condition had progressed to bilateral renal infarcts highlights the need for prompt investigation and diagnosis of suspected cases of FMD.

  16. 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

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

    PubMed Central

    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

  18. Management of moyamoya syndrome in patients with Noonan syndrome.

    PubMed

    Gupta, Mihir; Choudhri, Omar A; Feroze, Abdullah H; Do, Huy M; Grant, Gerald A; Steinberg, Gary K

    2016-06-01

    A few isolated reports have described an association between Noonan syndrome and cerebrovascular abnormalities, including moyamoya syndrome. These reports have been limited to pediatric patients presenting with recurrent transient ischemic attacks (TIA) or headaches. Management has primarily been pharmacologic, with only one prior report of surgical revascularization to our knowledge. We report four cases of Noonan syndrome patients presenting with headaches and/or sensorimotor strokes in childhood that caused unilateral sensorimotor impairment. Cerebral angiography and MRI revealed bilateral moyamoya syndrome. All patients underwent successful bilateral extracranial-to-intracranial revascularization. The first patient was a 10-year-old girl who presented following a hemorrhagic stroke and recovered well after indirect bypass. The second patient was an adult with a history of childhood stroke whose symptoms progressed in adulthood. She underwent a direct bypass and improved, but continued to experience TIA at her 4 year follow-up. The third patient was a 7-year-old girl with headaches and a new onset TIA who failed pharmacological therapy and subsequently underwent bilateral indirect bypass. The fourth patient was a 24-year-old woman with worsening headaches and an occluded left middle cerebral artery from unilateral moyamoya syndrome. A left sided direct bypass was completed given delayed MRI perfusion with poor augmentation. To our knowledge these are the first reported surgical cases of combined Noonan and moyamoya syndrome. These cases highlight the need to recognize moyamoya syndrome in patients with Noonan syndrome. Early surgical revascularization should be pursued in order to prevent symptom progression. PMID:26778511

  19. 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

  20. Bilateral internuclear ophthalmoplegia associated with pediatric brain tumor progression: a case series and review of the literature.

    PubMed

    Rismanchi, Neggy; Crawford, John R

    2013-12-01

    Internuclear ophthalmoplegia (INO) is a rare disorder of conjugate lateral gaze that has been described in a number of neurologic conditions including multiple sclerosis, stroke and less commonly brain tumors. We describe a series of 3 boys (11, 12, 15 years) diagnosed with primary central nervous system tumors (pilomyxoid variant astrocytoma, anaplastic oligoastrocytoma, gliomatosis cerebri) who developed bilateral INO as a manifestation of progressive disease. Time from diagnosis to development of bilateral INO ranged from 13-36 months. All children died of their disease 1-9 months following diagnosis of bilateral INO and had significant dorsal pontine invasion on magnetic resonance imaging at progression. Only one child had brainstem involvement at diagnosis. Our case series highlights this rare ophthalmologic syndrome of bilateral INO in association with tumor progression and provides a literature review of brain tumor associations with INO. PMID:24048548

  1. 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

  2. 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

  3. 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

  4. 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

  5. Scheie syndrome

    MedlinePlus

    ... as MPS I S. See also: MPS I H (Hurler syndrome) MPS II (Hunter syndrome) MPS IV (Morquio syndrome) ... individuals with Scheie syndrome, and also Hurler and Hurler-Scheie syndromes. Early detection and treatment of spinal cord compression ...

  6. 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.

  7. BILATERAL ANTERIOR GLENOHUMERAL DISLOCATION: CLINICAL CASE.

    PubMed

    Silva, Luís Pires; Sousa, Cristina Varino; Rodrigues, Elisa; Alpoim, Bruno; Leal, Miguel

    2011-01-01

    Bilateral anterior glenohumeral dislocation is a rare occurrence. We present a case of bilateral anterior glenohumeral dislocation caused by a fall. The interest in publishing this case is that this is a clinical rarity with few cases reported in the literature. An 89-year-old female patient was brought to the emergency department after a fall, complaining of intense pain in both shoulders and inability to move them. Objective examination showed clinical signs giving the suspicion of bilateral anterior glenohumeral dislocation, which was confirmed by x-ray imaging. Both dislocations were successfully reduced in the emergency department using the modified Milch technique. When a synchronous and symmetrical force has acted on both shoulders and these are painful with significant functional limitation, the suspicion of bilateral glenohumeral dislocation is a differential diagnosis to be considered, even though it is rare. PMID:27047826

  8. Bilateral persistent pupillary membranes associated with cataract.

    PubMed

    Ahmad, Syed Shoeb; Binson, Caroline; Lung, Chong Ka; Ghani, Shuaibah Abdul

    2011-01-01

    Exuberant persistent pupillary membranes (PPM) are rare in adult eyes. We report the case of a 53-year-old man diagnosed with bilateral, profuse, persistent pupillary membranes and unilateral cataract. PMID:23362401

  9. Bilateral persistent pupillary membranes associated with cataract

    PubMed Central

    Ahmad, Syed Shoeb; Binson, Caroline; Lung, Chong Ka; Ghani, Shuaibah Abdul

    2011-01-01

    Summary Exuberant persistent pupillary membranes (PPM) are rare in adult eyes. We report the case of a 53-year-old man diagnosed with bilateral, profuse, persistent pupillary membranes and unilateral cataract. PMID:23362401

  10. BILATERAL ANTERIOR GLENOHUMERAL DISLOCATION: CLINICAL CASE

    PubMed Central

    Silva, Luís Pires; Sousa, Cristina Varino; Rodrigues, Elisa; Alpoim, Bruno; Leal, Miguel

    2015-01-01

    Bilateral anterior glenohumeral dislocation is a rare occurrence. We present a case of bilateral anterior glenohumeral dislocation caused by a fall. The interest in publishing this case is that this is a clinical rarity with few cases reported in the literature. An 89-year-old female patient was brought to the emergency department after a fall, complaining of intense pain in both shoulders and inability to move them. Objective examination showed clinical signs giving the suspicion of bilateral anterior glenohumeral dislocation, which was confirmed by x-ray imaging. Both dislocations were successfully reduced in the emergency department using the modified Milch technique. When a synchronous and symmetrical force has acted on both shoulders and these are painful with significant functional limitation, the suspicion of bilateral glenohumeral dislocation is a differential diagnosis to be considered, even though it is rare. PMID:27047826

  11. 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

  12. 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

  13. 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

  14. 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. PMID:27034568

  15. Bilateral multicystic kidneys--an unusual case.

    PubMed

    Agrawal, Lila; Millard, Marie-Louise; Fairhurst, Joanna; Gilbert, Rodney D

    2002-11-01

    Multicystic dysplasia of the kidneys is a condition whose prognosis is good as it usually presents unilaterally. Bilateral cases are usually fatal in utero. We report a case of bilateral multicystic dysplasia of the kidneys where the lower moiety of the right kidney was spared cystic change. The patient had normal renal function and, following conservative management, remains alive and well 6 months later. PMID:12432443

  16. 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

  17. 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

  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. Laparoscopic simultaneous bilateral adrenalectomy for testosterone-secreting bilateral adrenal tumors.

    PubMed

    Suzuki, Takahisa; Furuse, Hiroshi; Kurita, Yutaka; Ushiyama, Tomomi; Mugiya, Soichi; Ozono, Seiichiro; Oki, Yutaka

    2014-01-01

    Laparoscopic adrenalectomy is widely accepted as a safe and minimally-invasive procedure. Although it is a standard procedure for the surgical treatment of adrenal tumors, its simultaneous use with bilateral adrenalectomy is relatively rare. A 21-year-old woman was referred to Hamamatsu University School of Medicine University Hospital complaining of a deepening voice, hirsutism and secondary amenorrhea. Abdominal computed tomography showed bilateral adrenal tumors, and hormonal examinations showed that the tumors secreted excessive testosterone, resulting in virilizing symptoms. Laparoscopic simultaneous bilateral adrenalectomy was carried out. Postoperatively, serum testosterone levels immediately recovered to within the normal range. Menstruation began the month after the operation, and the hirsutism gradually regressed. This is the third reported case of bilateral virilizing adrenal tumors, and the first to be successfully treated with laparoscopic simultaneous bilateral adrenalectomy. PMID:23601096

  20. Sarcoidosis (Heerfordt syndrome): a case report.

    PubMed

    Tamme, Tiia; Leibur, Edvitar; Kulla, Andres

    2007-01-01

    We report the case of a 22-year-old woman who is suspected of having primary Sjögren s syndrome. She complaining of bilateral swelling of eyelids and the parotid glands of three weeks duration. Physical examination revealed a bilateral enlargement of both parotid glands, which were solid and painful. Sjögren s syndrome was suspected at that stage, and the serologic and specific analysis were done. All these tests didn t find any autoimmune or visceral features typical of Sjögren s syndrome and autoantibodies were negative. During follow-up time the right facial nerve palsy developed. Pulmonary radiography revealed bihilar lymphadenopathy and labial salivary gland biopsy revealed non-caseating granuloma. The patient was classified as having stage I sarcoidosis. This case demonstrates the importance of being aware of the leading clinical signs and symptoms in case of Heerfordt syndrome. PMID:17637529

  1. 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

  2. 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

  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. 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

  5. Auriculotemporal Syndrome (Frey Syndrome).

    PubMed

    Motz, Kevin M; Kim, Young J

    2016-04-01

    Frey syndrome is a common sequela of parotidectomy, and although it is not frequently manifested clinically, it can cause significant morbidity for those affected. Frey syndrome results from synkinetic autonomic reinnervation by transected postganglionic parasympathetic nerve fiber within the parotid gland to the overlying sweat glands of the skin. Many surgical techniques have been proposed to prevent the development of Frey syndrome. For those who develop clinical symptoms of Frey syndrome, objective testing can be performed with a Minor starch-iodine test. Some of the current methods to prevent and treat symptomatic Frey syndrome are reviewed. PMID:26902982

  6. 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

  7. Sanfilippo syndrome

    MedlinePlus

    ... as MPS III. See also: MPS I H (Hurler syndrome) MPS II (Hunter syndrome) MPS IV (Morquio syndrome) ... unlike the cloudy corneas seen in persons with Hurler syndrome (MPS I H). Neurological testing will reveal signs ...

  8. Rett Syndrome

    MedlinePlus

    ... Awards Enhancing Diversity Find People About NINDS NINDS Rett Syndrome Information Page Condensed from Rett Syndrome Fact Sheet ... Clinical Trials Organizations Publicaciones en Español What is Rett Syndrome? Rett syndrome is a childhood neurodevelopmental disorder that ...

  9. Tourette Syndrome

    MedlinePlus

    ... Sledding, Skiing, Snowboarding, Skating Crushes What's a Booger? Tourette Syndrome KidsHealth > For Kids > Tourette Syndrome Print A A ... Act Around Someone Who Has It? What Is Tourette Syndrome? Tourette syndrome is a condition that affects a ...

  10. 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 ...

  11. Bilateral Abducens Nerve Palsy due to Idiopathic Intracranial Hypertension as an Initial Manifestation of Systemic Lupus Erythematosus.

    PubMed

    Katsuyama, Eri; Sada, Ken-Ei; Tatebe, Noriko; Watanabe, Haruki; Katsuyama, Takayuki; Narazaki, Mariko; Sugiyama, Koichi; Watanabe, Katsue S; Wakabayashi, Hiroshi; Kawabata, Tomoko; Wada, Jun; Makino, Hirofumi

    2016-01-01

    Idiopathic intracranial hypertension (IIH) is a syndrome of increased intracranial pressure and presents as an intractable headache, vomiting, and ophthalmologic manifestations. We herein report the case of a young girl who presented with bilateral abducens nerve palsy due to IIH as the onset of systemic lupus erythematosus (SLE). The patient was successfully treated with corticosteroid therapy. Our case lacked the typical symptoms of IIH, such as headache or nausea; therefore, it is necessary to carefully determine the cause of bilateral abducens nerve palsies. The development of IIH in SLE patients is a rare occurrence, but this manifestation should not be overlooked. PMID:27086818

  12. 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

  13. A newborn with complex skeletal abnormalities, joint contractures, and bilateral corneal clouding with sclerocornea.

    PubMed

    Sellars, Elizabeth A; Bosanko, Katherine A; Lepard, Tiffany; Garnica, Adolfo; Schaefer, Gerald Bradley

    2014-06-01

    A newborn presented to genetics with complex skeletal abnormalities, joint contractures, and bilateral corneal clouding with sclerocornea. The patient survived for 8 months before succumbing to respiratory failure. Exome sequencing revealed a compound heterozygous mutation in theB3GALT6gene. Mutations in this gene have been associated with both Ehlers- Danlos syndrome, progeroid type 2 and spondyloepimetaphyseal dysplasia with joint laxity type 1. These diagnoses encompass the skeletal and joint findings. Our patient expands the phenotype of these diagnoses, as anterior segment eye anomalies have not been described with either syndrome, and he is much more profoundly affected. Interestingly, our patient fits the description of a rare genetic disease referred to as Al-Gazali syndrome, for which the genetic cause is unknown. PMID:25149931

  14. [Mohr's syndrome : type II orofaciodigital syndrome (author's transl)].

    PubMed

    Ajacques, J C

    1981-01-01

    Mohr's syndrome consists of an association of three types of malformation : oral, facial, and digital. It is very similar, therefore, to the syndrome of Papillon-Léage and Psaume. Apart from this, precise, constant characteristics can be recognized which identify it as a separate entity. Firstly, the genetic mode of transmission differs. Secondly, clinical signs are dominated by the frequency and severity of the distal lesions, such that bilateral syndactyly of the big toe is a key element in the differential diagnosis. PMID:6269173

  15. 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

  16. 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

  17. 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

  18. Syphilitic aortitis causing bilateral coronary ostial stenosis.

    PubMed

    Hosoba, Soh; Suzuki, Tomoaki; Koizumi, Yusuke; Asai, Tohru

    2011-02-01

    Coronary ostial stenosis in otherwise normal coronary vessels is a rare complication of syphilitic aortitis. A 47-year-old man with no coronary risk factors developed severe isolated ostial stenosis in the left main coronary artery and right coronary artery. He underwent coronary artery bypass grafting using the bilateral internal thoracic arteries and gastroepiploic artery and recovered uneventfully. PMID:21345777

  19. Bilateral giant cyst of the shoulder.

    PubMed

    Agarwal, A; Ferrante, J; Schmidt, R; Eisenbeis, C H

    1987-01-01

    The case of a 61 year old white female with a rapidly progressive rheumatoid arthritis who developed bilateral giant cyst of the shoulder is described here. Arthrographic investigation indicated that these giant cysts were true synovial cysts rather than "pseudocysts". PMID:3427842

  20. Pott's Spine with Bilateral Psoas Abscesses

    PubMed Central

    Masavkar, Sanjeevani; Shanbag, Preeti; Inamdar, Prithi

    2012-01-01

    A high degree of suspicion and appropriate imaging studies are required for the early diagnosis of Pott's spine. We describe a 4-year-old boy with Pott's disease of the lumbar spine with bilateral psoas abscesses. The child responded to conservative treatment with antituberculous treatment and ultrasonographically guided percutaneous drainage of the abscesses. PMID:23259114

  1. Subcutaneous bilateral sporotrichosis: a rare presentation.

    PubMed

    Alves, Sydney Hartz; Aurélio, Pedro Lucas; Tecchio, Mateus Z; Zuchetto, Augusto; Schirmer, Régis; Santurio, Janio M

    2004-10-01

    Sporotrichosis is the most common subcutaneous mycosis observed in Brazil as well as in different regions of the world. We report a rare case of sporotrichosis in a Caucasian male agricultural worker whose lesions occurred bilaterally and simultaneously on the upper limbs. PMID:15645169

  2. Management of Bilateral Carotid Occlusive Disease

    PubMed Central

    Jadhav, Ashutosh P.; Ducruet, Andrew F.; Jankowitz, Brian T.; Jovin, Tudor G.

    2016-01-01

    Background Symptomatic bilateral internal carotid occlusive disease is a rare but potentially devastating entity. Medical therapy alone is associated with high rates of mortality and recurrent stroke. The optimal management of this disease remains poorly understood. Methods A retrospective review of a prospectively maintained database was conducted for patients who presented with an acute stroke in the setting of bilateral carotid occlusive disease between May and October 2013. Results We identified 3 patients. The admission National Institutes of Health Stroke Scale score ranged from 4 to 7. All patients had small- to moderate-sized infarcts in the anterior circulation on presentation. Angiography confirmed bilateral internal carotid occlusions with collateral filling via the posterior communicating artery and retrograde filling via external carotid artery supply to the ophthalmic artery. All patients were initially managed with permissive hypertension and anticoagulation followed by carotid angioplasty and stenting. At 1-year follow-up, all patients demonstrated a modified Rankin scale score of 0-1. Conclusions Carotid stenting may be a safe and effective therapy for patients presenting with symptomatic bilateral carotid occlusions. PMID:27051405

  3. 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

  4. Fast and Provably Accurate Bilateral Filtering.

    PubMed

    Chaudhury, Kunal N; Dabhade, Swapnil D

    2016-06-01

    The bilateral filter is a non-linear filter that uses a range filter along with a spatial filter to perform edge-preserving smoothing of images. A direct computation of the bilateral filter requires O(S) operations per pixel, where S is the size of the support of the spatial filter. In this paper, we present a fast and provably accurate algorithm for approximating the bilateral filter when the range kernel is Gaussian. In particular, for box and Gaussian spatial filters, the proposed algorithm can cut down the complexity to O(1) per pixel for any arbitrary S . The algorithm has a simple implementation involving N+1 spatial filterings, where N is the approximation order. We give a detailed analysis of the filtering accuracy that can be achieved by the proposed approximation in relation to the target bilateral filter. This allows us to estimate the order N required to obtain a given accuracy. We also present comprehensive numerical results to demonstrate that the proposed algorithm is competitive with the state-of-the-art methods in terms of speed and accuracy. PMID:27093722

  5. Bilateral spontaneous hyphema in juvenile xanthogranuloma.

    PubMed

    Vijayalakshmi, P; Shetty, Shashikant; Jethani, Jitendra; Devi, T B Uma

    2006-03-01

    This report describes a rare occurrence of bilateral, spontaneous, nontraumatic hyphema in a 6 weeks old infant, associated with a small, multiple skin lesions. The diagnosis of juvenile xanthogranuloma was confirmed by histopathological examination of the cutaneous lesions. The hyphaema cleared gradually in 2 weeks time with conservative management. PMID:16531672

  6. A Bilateral Traumatic Hip Obturator Dislocation.

    PubMed

    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

  7. Cowden Syndrome Presenting as Breast Cancer: Imaging and Clinical Features

    PubMed Central

    Seo, Mirinae; Ahn, Hye Shin; Moon, Hyeong-Gon

    2014-01-01

    Cowden syndrome is an uncommon, autosomal dominant disease which is characterized by multiple hamartomas of the skin, mucous membrane, brain, breast, thyroid, and gastrointestinal tract. The diagnosis of Cowden syndrome implicates an increased risk of developing breast cancer. We report a case of a 22-year-old woman with Cowden syndrome that presented as breast cancer with concomitant bilateral exuberant benign masses in both breasts. PMID:25246819

  8. Uveodermatologic syndrome in a rat terrier.

    PubMed

    Blackwood, Sarah E; Barrie, Kathleen P; Plummer, Caryn E; Taylor, David; Nunnery, Catherine M; Seltzer, Judith D; Ben-Shlomo, Gil; Brooks, Dennis E

    2011-01-01

    A 4 yr old intact male rat terrier presented with severe bilateral nonresponsive panuveitis. Bilateral uveitis, blepharospasm, conjunctival hyperemia, diffuse corneal edema, peripheral bullous retinal detachment, and secondary ocular hypertension were noted. Ocular lesions progressed despite aggressive medical treatment and were followed by cutaneous depigmentation and crusting along the nasal planum. Intensive oral and topical anti-inflammatory and topical antiglaucoma medications were administered, but the ocular disease progressed. A bilateral enucleation was performed. Uveodermatologic syndrome was diagnosed from histopathologic examination of a skin biopsy as well as histopathology of both globes after bilateral enucleation. To maintain control of the dermatologic lesions, oral azathioprine was initiated, but it was not well tolerated by this patient. Immunosuppressive doses of oral cyclosporine and anti-inflammatory doses of oral prednisone were used to control the depigmentation and crusting skin lesions. PMID:21730094

  9. Opercular syndrome: A case report and review.

    PubMed

    Desai, Soaham Dilip; Patel, Dipen; Bharani, Sheela; Kharod, Nikhil

    2013-05-01

    We present a 9-year-old boy with history of perinatal asphyxia and neonatal seizures; who presented with delayed development of speech, with predominant dysarthria, dysphagia, and drooling of saliva and unable to protrude tongue along with delayed motor and mental milestones. He had complex partial seizures since last 3 years requiring multiple anti-epileptic drugs. He had dysarthria, nasal twang, and drooling of saliva with difficulty in chewing and swallowing. Hearing and understanding were normal. Bilateral trigemino-facio-linguo-pharyngeal palsy was noticed on voluntary movements with normal jaw jerk with preserved automatic and emotional motor movements. Electroencephalography revealed focal left fronto-temporal epileptiform discharges and brain imaging was suggestive of bilateral cortical and subcortical region encephalomalacia, predominantly involving bilateral opercular region. The clinical and neuroimaging features correspond to bilateral opercular syndrome which could have resulted from the perinatal insult in this case. PMID:24082930

  10. 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

  11. 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

  12. 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

  13. 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

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

    PubMed

    Kubinec, V; Polakovičová, 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

  15. Bilateral stereotactic radiofrequency amygdalohippocampectomy for a patient with bilateral temporal lobe epilepsy.

    PubMed

    Luo, Huimin; Zhao, Quanjun; Tian, Zengmin; Wu, Zhaohui; Wang, Fuli; Lin, Hong; Yin, Feng; Zhao, Hulin; Xiao, Xia; Yu, Xue; Wang, Haiying; Zhao, Hongxi; Cai, Changlan

    2013-11-01

    To search for a method for treatment of bilateral temporal lobe epilepsy (BTLE), we report one patient with BTLE experienced bilateral stereotactic radiofrequency amygdalohippocampectomy (SAHE). Neuropsychological examinations were performed before and 5 days, and 6, 18, and 48 months after operation. No seizure occurred in the follow-up time, and no long-term memory and intelligence deficits were found except for a transient decline of the scores immediately after operation. Because severe damage of memory could be caused by bilateral resection surgery, bilateral SAHE should be considered as a possible approach for the treatment of BTLE. However, further studies with accumulation of cases are needed, especially in the detailed assessment of neuropsychological function. PMID:24116395

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

    PubMed Central

    Eliçora, Sultan Şevik; Dinç, Aykut Erdem; Bişkin, 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

  17. Sporadic bilateral synchronous multicentric papillary renal cell carcinoma masquerading as bilateral multifocal pyelonephritis

    PubMed Central

    Karthikeyan, VS; Kumar, S; Vijayakumar, AR; Ramesh, A; Ganesh Rajesh, N; Halanaik, D; Gupta, A

    2014-01-01

    Pyelonephritis is defined as an inflammation of the kidney and renal pelvis. The diagnosis is usually clinical. Acute multifocal bacterial nephritis is a rare form of pyelonephritis that is more severe and sepsis is more common. We report a patient who presented with fever and right-sided abdominal pain associated with right flank tenderness, suggesting right acute pyelonephritis. Bilateral multifocal pyelonephritis was diagnosed on ultrasonography, radionuclide renal scintigraphy and computed tomography. However, owing to non-resolution of symptoms, a biopsy was performed, which showed bilateral papillary renal cell carcinoma (PRCC). PRCC is known to exhibit multicentricity. To our knowledge, a case of bilateral multicentric PRCC masquerading as bilateral multifocal pyelonephritis has not been reported in the English literature. This case highlights the need to be vigilant while treating patients with focal lesions of the kidney as an inflammatory condition lest a malignancy should be missed. PMID:24992402

  18. Laparoscopic total extraperitoneal repair of preoperatively diagnosed bilateral obturator and incidental bilateral femoral herniae.

    PubMed

    Malik, Muhammad Usman; Connelly, Tara M; Hamid, Mustafa; Pretorius, Frederik

    2016-01-01

    Obturator hernia (OH), a rare type of hernia, is associated with high morbidity and mortality. Diagnosis is often delayed as clinical symptoms are typically non-specific. OH is frequently associated with other occult inguinopelvic herniae. Early diagnosis is vital to decrease morbidity and mortality. We report the case of a 75-year-old woman who presented to the surgical outpatients' department with non-specific bilateral groin pain radiating to the thighs. CT of the pelvis demonstrated bilateral OH with no radiological evidence of bowel obstruction. Semiurgent elective laparoscopic total extraperitoneal mesh repair was performed. Intraoperative findings confirmed bilateral obturator herniae as well as incidental bilateral femoral herniae. This case highlights the need for a high index of suspicion for such concomitant hernias that, in the presence of OH, may only be identified intraoperatively. PMID:27113790

  19. Bilateral Chest X-Ray Shadowing and Bilateral leg lesions - A case of Pulmonary Kaposi Sarcoma

    PubMed Central

    Khattak, M

    2016-01-01

    We report a case of a 30 year old gentleman seen on the respiratory ward with no significant past medical history presenting with a three week history of worsening dyspnoea, productive cough, fever, bilateral leg swelling and bilateral leg swelling. Initial differential diagnosis included community-acquired pneumonia, cellulitis and deep vein thrombosis. After much investigation a diagnosis of AIDS-related kaposi's sarcoma with visceral manifestations was made.

  20. Leriche Syndrome Presenting as Depression with Erectile Dysfunction.

    PubMed

    Bhatia, M S; Gautam, Priyanka; Saha, Rashmita

    2016-03-01

    Leriche syndrome results from thrombotic occlusion of the abdominal aorta immediately above the site of its bifurcation. Impotence in leriche syndrome is caused due to proximal obstruction, commonly involving isolated common iliac, internal iliac, internal pudendal or dorsalis penis artery. The symptoms of Leriche syndrome include intermittent and bilateral claudication, pallor, coldness and fatigue in lower extremities. Data regarding psychiatric morbidity in Leriche syndrome is unavailable. We hereby report the case of Leriche syndrome, presenting to psychiatry outpatient department with depressive disorder and erectile dysfunction (ED) with focus on dilemmas faced in the diagnosis and management in psychiatry. PMID:27134979

  1. Leriche Syndrome Presenting as Depression with Erectile Dysfunction

    PubMed Central

    Gautam, Priyanka; Saha, Rashmita

    2016-01-01

    Leriche syndrome results from thrombotic occlusion of the abdominal aorta immediately above the site of its bifurcation. Impotence in leriche syndrome is caused due to proximal obstruction, commonly involving isolated common iliac, internal iliac, internal pudendal or dorsalis penis artery. The symptoms of Leriche syndrome include intermittent and bilateral claudication, pallor, coldness and fatigue in lower extremities. Data regarding psychiatric morbidity in Leriche syndrome is unavailable. We hereby report the case of Leriche syndrome, presenting to psychiatry outpatient department with depressive disorder and erectile dysfunction (ED) with focus on dilemmas faced in the diagnosis and management in psychiatry. PMID:27134979

  2. Bilateral multicystic renal dysplasia with potter sequence. A case with penile agenesis.

    PubMed

    Dursun, Ahmet; Ermis, Bahri; Numanoglu, Varim; Bahadir, Burak; Seckiner, Ilker

    2006-11-01

    Hereditary renal adysplasia (HRA) is a rare autosomal dominant condition. Patients have several other anomalies including Potter facies, thoracic, cardiac, and extremity deformities. The case present dysmorphic facial features such as hypertelorism, prominent epicanthic folds, a flat and broad nose, choanal stenosis, low-set ears, and a receding chin. He had femoral bowing, hypoplastic right tibia and agenesis of the right foot. He had rich and thick skin. He had also a dysplastic empty scrotum, penile agenesis, and anal atresia. The autopsy revealed pulmonary hypoplasia, ventricular septal defect, bilateral multicystic renal dysplasia, agenesis of both ureter and bladder, intraabdominal testicles, and a single umbilical artery. The penile agenesis was first reported, and including the consanguinity in the parents might further delineate the bilateral multicystic HRA. Vater/caudal regression anomalies, Mullerian duct/aplasia, unilateral renal agenesis, and cervicothoracic somite anomalies association, and Coloboma, heart anomaly, choanal atresia, retardation, genital and ear anomalies syndrome has been considered in differential diagnosis. PMID:17106555

  3. 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.

  4. Cryptogenic organising pneumonia presenting with bilateral hilar and mediastinal lymphadenopathy

    PubMed Central

    Kahraman, Hasan; Tokur, Mahmut; Sayar, Hamide; Inci, Mehmet Fatih

    2013-01-01

    Cryptogenic organising pneumonia is not considered in the differential diagnosis of bilateral hilar and mediastinal lymphadenopathy. We submitted a patient presenting with bilateral hilar and mediastinal lymphadenopathy. We suspected diagnosis of sarcoidosis, but the patient was diagnosed as cryptogenic organising pneumonia with the histological result. This is the second case report of cryptogenic organising pneumonia presenting with bilateral hilar and mediastinal lymphadenopathy. PMID:23761506

  5. Nongranulomatous anterior uveitis in a patient with Usher syndrome

    PubMed Central

    Alzuhairy, Sultan Abdulaziz S.; Alfawaz, Abdullah

    2013-01-01

    A 34-year-old female with Usher syndrome, but no family history of similar illness, presented with complaints of vision reduction, redness, and photophobia. Biomicroscopic examination showed mildly injected conjunctivae bilateral, small, round keratic precipitates; bilateral +2 cells with no flare reaction in the anterior chamber; and bilateral posterior subcapsular cataracts. No associated posterior synechiae, angle neovascularization, or iris changes were detected; normal intraocular pressures were obtained. Fundus examination demonstrated waxy pallor of both optic nerves, marked vasoconstriction in retinal vessels, and retinal bone spicule pigment formation, with a normal macula. Electroretinography confirmed the diagnosis of retinitis pigmentosa, optical coherent tomography was normal and otolaryngology consultation was conducted. To our knowledge, an association between Usher syndrome and bilateral nongranulomatous anterior uveitis has not been previously reported, and our purpose is to report this association. PMID:24371428

  6. 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

  7. 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

  8. Bilateral granulomatous mastitis with a different etiology.

    PubMed

    Choi, Sung Hyouk; Jang, Ki Seok; Chung, Min Sung

    2015-01-01

    Idiopathic granulomatous mastitis (IGM) and Tuberculosis mastitis (TM) are rare inflammatory diseases of the breast that can clinically mimic malignancy causing misdiagnosis as breast cancer. We present a rare case of bilateral granulomatous mastitis with a different etiology. An initial lesion developed in the right breast was diagnosed as IGM, which was treated with antibiotics and surgery. A subsequent lesion developed in the contralateral breast 5 months later and was diagnosed as TM, which also completely responded to antituberculosis medication without surgical excision. Differential diagnosis was made using the results of the polymerase chain reaction for tuberculosis (TBC-PCR) of both of the breast lesions in addition to typical pathologic findings of IGM in the right breast and an antituberculosis medication response in the left breast. To the best of our knowledge, this is the first case of bilateral granulomatous mastitis with a different etiology. PMID:25519013

  9. Simultaneous, Isolated Traumatic Bilateral Patella Fractures

    PubMed Central

    Madi, Sandesh; Naik, Monappa; Rao, Sharath; Vijayan, Sandeep

    2016-01-01

    Introduction Isolated traumatic fractures of both Patellae, occurring at the same time, in an otherwise healthy person, are very rare. The Patella, as a subcutaneous and a cancellous bone, is vulnerable to injury. However, simultaneous injury to both Patellae without the involvement of any other bony injuries occurs infrequently, and only a few cases have been reported in the literature. Case Presentation We report a rare case of isolated, traumatic bilateral Patella fracture with unusual fracture patterns and briefly review the literature. Conclusions In simultaneous bilateral Patella fractures, good functional outcome can be expected following a stable surgical fixation and a structured rehabilitation regime. However, personality of the fracture determines salvage versus sacrifice of the Patella. PMID:27218047

  10. Amyloidosis in bilateral external auditory canals

    PubMed Central

    Hosoi, Takahiro; Dhaliwal, Gurpreet; Tokuda, Yasuharu

    2014-01-01

    We report a 76-year-old man, a retired farmer, presenting with bilateral external auditory canal obstruction. Skin examination revealed multiple pruritic nodules and periorbital purpura. Ear canal opening surgery was performed. Skin and ear canal biopsy revealed extensive amyloid depositions. Immunoelectrophoresis for urinary Bence-Jones protein was positive, and bone marrow examination showed plasma cell dyscrasia. He received chemotherapy for amyloid light-chain amyloidosis secondary to light-chain multiple myeloma but died from myeloma progression. This case demonstrated how infiltrative conditions such as amyloidosis can lead to bilateral auditory canal obstruction and that the diagnosis of amyloidosis can be missed if a clinician focuses solely on a single organ. PMID:24825551

  11. BILATERAL LUXATIO ERECTA, A CASE REPORT

    PubMed Central

    Acosta, César Augusto Xavier; da Silva Resch, Elemar; Rodrigues, Rafael

    2015-01-01

    Inferior shoulder dislocation (luxactio erecta) is a rare lesion affecting approximately 0.5% of dislocations of this joint. The vast majority of these cases occur unilaterally. In September 2004, a 43 year old man was brought to the emergency room of the University Hospital of Santa Maria (HUSM) complaining of bilateral shoulder pain and inability to lower the arms, as a result of a fall with the arms abducted, while carrying out work activities. After physical examination and radiological exams, it was found that the patient had a bilateral inferior shoulder dislocation. He was referred to the surgical ward and after intravenous sedation, both shoulders were reduced by closed reduction using the traction-countertraction maneuver. The patient was discharged the day after the reduction. Both arms were immobilized with a velpeau sling in total adduction and intrarotation, with instructions to maintain immobilization for three weeks. The patient was also advised to receive physiotherapy.

  12. 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

  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 superior keratoconus: two case reports

    PubMed Central

    Rogers, G J; Attenborough, M

    2014-01-01

    Purpose To present two cases of the rare entity of bilateral superior keratoconus and to review the literature regarding presentation and clinical findings. Methods Case report based on chart review. Results Two patients presented to our corneal service with clinical and topographical features of superior keratoconus. Conclusion The unusual clinical and topographical findings of superior keratoconus are presented and treatment options are considered. PMID:24993320

  15. 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

  16. Bilateral caudate infarct--a case report.

    PubMed

    Lim, J K; Yap, K B

    1999-07-01

    Caudate strokes comprise only a small proportion of all subtypes of strokes. Bilateral caudate infarcts are even rarer and only a few cases have been reported in the literature. We report an 86-year-old woman with bilateral caudate infarcts. She had no past medical history of note. She presented with headache for several days and drowsiness on day of admission. Clinical examination revealed abulia, inability to comprehend or verbalize (acute mutism), right-sided neglect and right-sided hemiparesis. Computed tomographic (CT) scan brain revealed decreased attenuation in both heads of both caudate nuclei with extension across the anterior limb of both internal capsules to involve the lentiform nuclei. Echocardiography showed aortic valve sclerosis, mild mitral and aortic regurgitation and normal left ventricular function. Carotid ultrasound revealed mild stenosis of proximal right internal carotid and left distal common carotid and adjacent proximal internal carotid arteries. She showed initial improvement in the first week, but subsequently had a progressive downward course despite rehabilitation and died 44 days after her stroke. A patient with bilateral caudate infarcts is likely to have poor prognosis for rehabilitation and survival. PMID:10561773

  17. 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.

  18. 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.

  19. Brain Perfusion in Corticobasal Syndrome with Progressive Aphasia

    PubMed Central

    Abe, Yoshitake; Kimura, Noriyuki; Goto, Megumi; Aso, Yasuhiro; Matsubara, Etsuro

    2016-01-01

    Background Brain perfusion may differ between patients with corticobasal syndrome (CBS) with and without aphasia. Methods Twenty-six (9 males and 17 females; mean age 76 ± 5.3 years) patients with CBS were enrolled in the study. Brain MRI and single-photon emission computed tomography were performed in all subjects. Language was evaluated using the Standard Language Test of Aphasia. The patients were divided into two subgroups according to the presence or absence of progressive aphasia. Differences in the regional cerebral blood flow (rCBF) between the two groups were detected based on voxel-by-voxel group analysis using Statistical Parametric Mapping 8. Results All patients exhibited asymmetric motor symptoms and signs, including limb apraxia, bradykinesia, and akinetic rigidity. Of 26 patients, 9 had a clinically obvious language disturbance, characterized as nonfluent aphasia. Almost all CBS patients with aphasia exhibited cortical atrophy predominantly in the left frontal and temporal lobes with widening of the Sylvian fissure on MRI. The rCBF in the left middle frontal gyrus differed significantly between CBS patients with and without aphasia. Conclusion CBS patients with aphasia exhibit motor symptoms predominantly on the right side and cortical atrophy mainly in the left perisylvian cortices. In particular, left frontal dysfunction might be related to nonfluent aphasia in CBS. PMID:27195001

  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. Paraneoplastic Syndromes

    MedlinePlus

    ... fine motor coordination, slurred speech, memory loss, vision problems, sleep disturbances, dementia, seizures, sensory loss in the limbs, and vertigo or dizziness. Paraneoplastic syndromes include Lambert-Eaton myasthenic syndrome, stiff-person syndrome, encephalomyelitis, myasthenia ...

  2. Malabsorption Syndromes

    MedlinePlus

    ... If you have a malabsorption syndrome, your small intestine cannot absorb nutrients from foods. Causes of malabsorption syndromes include Celiac disease Lactose intolerance Short bowel syndrome. This happens after surgery to remove ...

  3. Brown Syndrome

    MedlinePlus

    ... Does Brown syndrome cause eye problems besides abnormal eye movements? Some children with Brown syndrome have poor binocular ... In the congenital form of Brown syndrome, the eye movement problem is usually constant and unlikely to resolve ...

  4. Clinical Characteristics of Bilateral versus Unilateral Chronic Subdural Hematoma

    PubMed Central

    Lee, Jungjun

    2014-01-01

    Objective Chronic subdural hematoma (CSDH) is a common intracranial hemorrhage that is associated with significant morbidity. Bilateral lesions are occasionally found in neurosurgical practice. The purpose of this study is to analyze clinical characteristics of bilateral CSDH compared with unilateral CSDH. Methods Between January 2005 and January 2013, the authors treated 114 surgical patients with CSDH. Clinical presentations, precipitating factors, computed tomography (CT) findings, postoperative complications, and outcomes of patients were retrospectively analyzed in the bilateral and unilateral CSDH groups. Results Bilateral CSDH was identified in 28 (24.6%) of the 114 CSDH patients. The mean age was 77.85 years in the bilateral CSDH group. The frequency of altered consciousness as a presenting symptom was significantly higher in the bilateral CSDH, and that of hemiparesis was significantly higher in the unilateral CSDH (p=0.015). Diabetes mellitus was more common in the bilateral CSDH (p=0.001). CT scans revealed significant differences in the degree of midline shift (p=0.001). The mean modified Rankin scale at discharge was 1.5 in the bilateral CSDH group and 0.6 in the unilateral group (p=0.019). Conclusion Bilateral CSDH showed different clinical characteristics from unilateral CSDH. Bilateral CSDH is prone to occurrence in the patient of old and diabetics. The patients of bilateral CSDH seem to reveal worse mental status and neurologic sign than unilateral CSDH in both baseline and postoperative state. PMID:27169033

  5. 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

  6. Simultaneous bilateral spontaneous hydropneumothorax: a rare presentation of bilateral malignant pleural mesothelioma

    PubMed Central

    Fayed, Hossam E; Woodcock, Victoria K; Grayez, Jamal

    2013-01-01

    This is a case of a 69-year-old man with a history of asbestos exposure who presented with acute shortness of breath. His chest x-ray showed bilateral hydropneumothorax. Further investigations including CT chest and video-assisted thoracoscopic surgery revealed bilateral pleural thickening and histology confirmed epithelioid mesothelioma. This case highlights the need for clinicians to be aware of atypical presentations of malignant pleural mesothelioma as well as the importance of considering underlying secondary causes such as malignancy in the older patient presenting with spontaneous pneumo/hydropneumothorax. PMID:23682089

  7. 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.

  8. 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

  9. Chronic Subdural Hematomas: Comparison between Unilateral and Bilateral Involvement

    PubMed Central

    Park, Hyun Seok; Park, Jun Bum; Kwon, Soon Chan; Lyo, In Uk; Kim, Min-ho; Sim, Hong Bo

    2014-01-01

    Objective Chronic subdural hematoma (CSDH) is a common intracranial hemorrhage, encountered in neurosurgical practice. Most CSDHs are unilateral, but some show bilateral involvement. However, the clinical characteristics of bilateral CSDH remain unclear. In this study, we investigated the clinical differences between bilateral and unilateral CSDH. Methods A retrospective study was performed on 120 patients with CSDH surgically treated at our institute from January 2008 to December 2012. Patients were divided into two groups: the bilateral CSDH and the unilateral CSDH groups. Clinical presentations, precipitating factors, computed tomography (CT) findings, postoperative complications, and outcomes of patients were analyzed. Results Bilateral CSDH was identified in 11 of 120 (10.9%) patients with CSDH. Patients with bilateral CSDH tended to have a lower rate of head injury compared to patients with unilateral CSDH (36.4% vs. 59.6%), but it had no statistical significance (p=0.201). The frequency of marked midline shift on CT scans was significantly greater in unilateral CSDH than in bilateral CSDH (p=0.010). Presenting symptoms, coexisting systemic diseases, postoperative complications, and clinical outcomes were not significantly different between the two groups. Conclusion Bilateral CSDH has comparatively similar clinical features and precipitating factors as unilateral CSDH. Patients with bilateral CSDH have significantly lower incidences of midline shift on CT scans, and most patients with either bilateral or unilateral CSDH have good postoperative outcomes. PMID:27169034

  10. Familial extensive idiopathic bilateral pleural fibrosis.

    PubMed

    Azoulay, E; Paugam, B; Heymann, M F; Kambouchner, M; Haloun, A; Valeyre, D; Battesti, J P; Tazi, A

    1999-10-01

    The authors report three sisters with bilateral isolated apical pleural fibrosis of unknown origin, which did not respond to empirical antituberculosis therapy and oral corticosteroids. The disease evolved in an unrelenting fashion producing pleural fibrosis at the lung bases and leading to the death of two sisters and to lung transplantation in the other one. There was no history of other familial disease or consanguinity. The particular features of these cases and the differences from other reports of apparently cryptogenic pleural fibrosis are outlined. PMID:10573252

  11. Bilateral Symmetry in Morphogenesis of Embryos

    PubMed Central

    Jehle, Herbert

    1970-01-01

    It is suggested that differentiated embryonic cells have a high specificity of molecular constitution as regards the surface layers surrounding their cellular membranes. Correspondingly, specific interface energies may characterize the early contacts between different cell types. The question is raised whether the morphology of the developing embryo may be understood in terms of cellular arrangements which minimize the total interface energy. Bilateral symmetry prevalent in early embryonic development of higher animals might be understood on the basis of the adoption of such a minimum energy principle if, in addition, one assumes that embryonic development is uniquely determined for a particular species. PMID:5272310

  12. 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

  13. 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.

  14. Bilateral sequential Propionibacterium acnes exogenous endophthalmitis.

    PubMed

    Saffra, Norman; Moriarty, Emily; Milman, Tatyana

    2016-12-01

    A 68-year-old man underwent uncomplicated sequential cataract extractions performed more than a year apart. He presented 6 months after the second surgery with persistent intraocular inflammation in both eyes. Cultures from both eyes grew Propionibacterium acnes and he responded well to treatment. Suspicion for delayed-onset post-operative endophthalmitis must remain high in uveitis cases that fail to resolve with anti-inflammatory treatments. The authors believe this is the first reported case of bilateral sequential P. acnes exogenous endophthalmitis. PMID:27220771

  15. Bilateral Ossiculoplasty in 1 Case of Achondroplasia

    PubMed Central

    Jung, Jongyoon; Yang, Chulwon; Lee, Sunkyu

    2013-01-01

    Achondroplasia is the most common skeletal dysplasia and it combines various complications with normal longevity. Hearing disturbance due to otitis media or an ossicular anomaly is one of the most common complications. Conductive hearing loss is suggested as the most common form of hearing loss. Temporal bone and middle ear structures are distorted in achondroplasia because of rotational change of the skull base. Authors experienced a case of an achondroplastic patient with bilateral hearing disturbance. We faced making a potential mistake during the previous operation but a favorable postoperative result occurred. Our experience could be helpful to other clinicians who face achondroplastic patients. PMID:24653923

  16. Idiopathic yellow nail syndrome successfully treated with OK-432.

    PubMed

    Yamagishi, Toru; Hatanaka, Nobuyuki; Kamemura, Hirotaka; Nakazawa, Ichiro; Hirano, Yusuke; Kodaka, Norio; Miura, Atsuo; Kitahara, Asako; Sawata, Tetsuro; Hosaka, Kimio; Sanno, Ken

    2007-01-01

    A 70-year-old female presented with yellow discoloration of the nail beds of all fingers and toes, as well as bilateral pleural effusions. The patient was diagnosed as having the yellow nail syndrome based on the triad of yellow nails, lymphedema, and pleural effusions. The patient's intractable bilateral pleural effusion was treated with pleurodesis using OK-432. The treatment prevented the accumulation of pleural fluid for a long period of time. Pleural effusion associated with yellow nail syndrome is thought to be difficult to treat; however, this patient's excellent clinical course suggests that pleurodesis with OK-432 could be used to treat the disease in the future. PMID:17634713

  17. Masquerade syndrome of multicentre primary central nervous system lymphoma.

    PubMed

    Guerriero, Silvana; Giancipoli, Ermete; Ciracì, Lorenza; Ingravallo, Giuseppe; Prete, Marcella; Di Leo, Elisabetta; Cimmino, Antonietta; Cardascia, Nicola

    2011-01-01

    Purpose. In Italy we say that the most unlucky things can happen to physicians when they get sick, despite the attention of colleagues. To confirm this rumor, we report the sad story of a surgeon with bilateral vitreitis and glaucoma unresponsive to traditional therapies. Methods/Design. Case report. Results. After one year of steroidal and immunosuppressive therapy, a vitrectomy, and a trabeculectomy for unresponsive bilateral vitreitis and glaucoma, MRI showed a multicentre primary central nervous system lymphoma, which was the underlying cause of the masquerade syndrome. Conclusions. All ophthalmologists and clinicians must be aware of masquerade syndromes, in order to avoid delays in diagnosis. PMID:22611509

  18. An Unusual Case of Neuralgic Amyotrophy Presenting with Bilateral Phrenic Nerve and Vocal Cord Paresis

    PubMed Central

    Holtbernd, F.; Zehnhoff-Dinnesen, A. am; Duning, T.; Kemmling, A.; Ringelstein, E.B.

    2011-01-01

    Background Neuralgic amyotrophy (brachial plexus neuropathy, brachial plexus neuritis, or Parsonage-Turner syndrome) is an uncommon inflammatory condition typically characterized by acute and severe shoulder pain followed by paresis with muscle weakness and atrophy of the upper limb or shoulder girdle. We report an unusual clinical manifestation of neuralgic amyotrophy, namely bilateral phrenic nerve palsy with concomitant laryngeal paresis. Case Report A 55-year-old male presented with orthopnea and aphonia after an episode of bilateral shoulder pain preceded by an upper respiratory tract infection. Spirometry, chest X-ray and videolaryngoscopy revealed bilateral and simultaneous paresis of the diaphragm and the vocal cords. Clinical examination at admission and at the 2-month follow-up did not show upper limb weakness or atrophy, except for a mild atrophy of the right supraspinatus muscle. An electromyography of the upper limb muscles and nerve conduction studies did not reveal signs of denervation. Analysis of the cerebrospinal fluid and an MRI of the neuraxis were unremarkable. After treatment with prednisolone, vocal cord function markedly improved within 8 weeks, whereas paresis of the diaphragm persisted. Conclusion Shoulder pain followed by diaphragmatic paralysis with dyspnea and hoarseness may be a manifestation of neuralgic amyotrophy even if upper limb or shoulder girdle palsies are absent. PMID:21490716

  19. Bilateral symmetrical supernumerary heads of biceps brachii with rare pectoralis major insertion.

    PubMed

    Fraser, Patrick Richard; Howard, Levi William; Rosales, Armando Aviles; Guttmann, Geoffrey David

    2015-04-01

    During a routine dissection of a 51-year-old Caucasian male cadaver, bilateral symmetrical supernumerary heads (SH) of the biceps brachii muscles with insertion into the pectoralis major (PM) muscles were discovered. Multiple case reports have documented supernumerary heads for the biceps brachii; however, none have shown bilateral insertion into the pectoralis major. This study describes a previously undocumented variation of the SH that has potential for clinical impact. Healthcare providers could be confounded by patients presenting with shoulder pain or muscle tears as a result of the anomaly. Furthermore, MRI studies on patients with possible shoulder muscle tears could reveal unexpected results. Such cases would warrant consideration of SH anomaly and treatment should be adjusted accordingly. The significant bulk and angle of the SH insertion on the PM we observed changes force vectors which would have an unknown effect in performance, surgical interventions and pain syndromes. A second biceps brachii anomaly was observed on the left brachium in addition to the bilateral SH. We postulate that these variants provide the potential for clinical complications regarding muscular injury to these aforementioned muscle groups. PMID:24817560

  20. 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

  1. [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

  2. Heart and Down Syndrome

    MedlinePlus

    ... Series Videos Webinar Series Health Care Associated Conditions ADHD & Down Syndrome Alzheimer's Disease & Down Syndrome Anesthesia & Down Syndrome Atlantoaxial Instability & Down Syndrome Blood Diseases & Down Syndrome Dental Issues & Down Syndrome Dual Diagnosis of Down Syndrome & Autism Ear, Nose & Throat Issues & ...

  3. Dental Issues & Down Syndrome

    MedlinePlus

    ... Series Videos Webinar Series Health Care Associated Conditions ADHD & Down Syndrome Alzheimer's Disease & Down Syndrome Anesthesia & Down Syndrome Atlantoaxial Instability & Down Syndrome Blood Diseases & Down Syndrome Dental Issues & Down Syndrome Dual Diagnosis of Down Syndrome & Autism Ear, Nose & Throat Issues & ...

  4. 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

  5. Urgent Bilateral Endoscopic Marsupialization for Respiratory Distress due to Bilateral Dacryocystitis in a Newborn

    PubMed Central

    Kim, Hochang; Park, Jongyeop; Jang, Jaeho; Chun, Junwoo

    2014-01-01

    Abstract We describe an infant with respiratory distress due to bilateral dacryocystoceles and dacryocystitis who was successfully treated with urgent bilateral endoscopic marsupialization. A male infant was brought to our outpatient department 7 days after birth, with red, acutely inflamed swellings near the medial canthal area of both eyes. From birth, there had been bluish swelling near the medial canthal area, and redness and swellings developed within 3 days. On physical examination, the child was afebrile but showed respiratory distress with coarse breathing sound. That day, the infant was admitted and treated with intravenous cefotaxime 150 mg. After withholding oral intake for appropriate preoperative fasting, urgent bilateral probing with endoscopy was done. On endoscopy, huge bilateral congenital dacryocystoceles were found. Because of its huge size, the inferior surface of the cyst was touching the nasal floor, which made probe unable to perforate the wall of dacryocystocele. Therefore, an endoscopy-assisted marsupialization of dacryocystoceles and bicanalicular silicone intubation were performed. Both swellings and erythema subsided within 48 hours postoperatively, and the patient was discharged after 72 hours from treatment. PMID:24799096

  6. A case of bilateral aldosterone-producing adenomas differentiated by segmental adrenal venous sampling for bilateral adrenal sparing surgery.

    PubMed

    Morimoto, R; Satani, N; Iwakura, Y; Ono, Y; Kudo, M; Nezu, M; Omata, K; Tezuka, Y; Seiji, K; Ota, H; Kawasaki, Y; Ishidoya, S; Nakamura, Y; Arai, Y; Takase, K; Sasano, H; Ito, S; Satoh, F

    2016-06-01

    Primary aldosteronism due to unilateral aldosterone-producing adenoma (APA) is a surgically curable form of hypertension. Bilateral APA can also be surgically curable in theory but few successful cases can be found in the literature. It has been reported that even using successful adrenal venous sampling (AVS) via bilateral adrenal central veins, it is extremely difficult to differentiate bilateral APA from bilateral idiopathic hyperaldosteronism (IHA) harbouring computed tomography (CT)-detectable bilateral adrenocortical nodules. We report a case of bilateral APA diagnosed by segmental AVS (S-AVS) and blood sampling via intra-adrenal first-degree tributary veins to localize the sites of intra-adrenal hormone production. A 36-year-old man with marked long-standing hypertension was referred to us with a clinical diagnosis of bilateral APA. He had typical clinical and laboratory profiles of marked hypertension, hypokalaemia, elevated plasma aldosterone concentration (PAC) of 45.1 ng dl(-1) and aldosterone renin activity ratio of 90.2 (ng dl(-1) per ng ml(-1 )h(-1)), which was still high after 50 mg-captopril loading. CT revealed bilateral adrenocortical tumours of 10 and 12 mm in diameter on the right and left sides, respectively. S-AVS confirmed excess aldosterone secretion from a tumour segment vein and suppressed secretion from a non-tumour segment vein bilaterally, leading to the diagnosis of bilateral APA. The patient underwent simultaneous bilateral sparing adrenalectomy. Histopathological analysis of the resected adrenals together with decreased blood pressure and PAC of 5.2 ng dl(-1) confirmed the removal of bilateral APA. S-AVS was reliable to differentiate bilateral APA from IHA by direct evaluation of intra-adrenal hormone production. PMID:26538381

  7. A case of bilateral aldosterone-producing adenomas differentiated by segmental adrenal venous sampling for bilateral adrenal sparing surgery

    PubMed Central

    Morimoto, R; Satani, N; Iwakura, Y; Ono, Y; Kudo, M; Nezu, M; Omata, K; Tezuka, Y; Seiji, K; Ota, H; Kawasaki, Y; Ishidoya, S; Nakamura, Y; Arai, Y; Takase, K; Sasano, H; Ito, S; Satoh, F

    2016-01-01

    Primary aldosteronism due to unilateral aldosterone-producing adenoma (APA) is a surgically curable form of hypertension. Bilateral APA can also be surgically curable in theory but few successful cases can be found in the literature. It has been reported that even using successful adrenal venous sampling (AVS) via bilateral adrenal central veins, it is extremely difficult to differentiate bilateral APA from bilateral idiopathic hyperaldosteronism (IHA) harbouring computed tomography (CT)-detectable bilateral adrenocortical nodules. We report a case of bilateral APA diagnosed by segmental AVS (S-AVS) and blood sampling via intra-adrenal first-degree tributary veins to localize the sites of intra-adrenal hormone production. A 36-year-old man with marked long-standing hypertension was referred to us with a clinical diagnosis of bilateral APA. He had typical clinical and laboratory profiles of marked hypertension, hypokalaemia, elevated plasma aldosterone concentration (PAC) of 45.1 ng dl−1 and aldosterone renin activity ratio of 90.2 (ng dl−1 per ng ml−1 h−1), which was still high after 50 mg-captopril loading. CT revealed bilateral adrenocortical tumours of 10 and 12 mm in diameter on the right and left sides, respectively. S-AVS confirmed excess aldosterone secretion from a tumour segment vein and suppressed secretion from a non-tumour segment vein bilaterally, leading to the diagnosis of bilateral APA. The patient underwent simultaneous bilateral sparing adrenalectomy. Histopathological analysis of the resected adrenals together with decreased blood pressure and PAC of 5.2 ng dl−1 confirmed the removal of bilateral APA. S-AVS was reliable to differentiate bilateral APA from IHA by direct evaluation of intra-adrenal hormone production. PMID:26538381

  8. Multifocal bilateral metatarsal tuberculosis: a rare presentation.

    PubMed

    Vijay, Vipul; Sud, Alok; Mehtani, Anil

    2015-01-01

    Tuberculosis, or phthisis (consumption) as it was popularly known in the Greek era, has been endemic in Southeast Asia and Sub-Saharan Africa; however, the human immunodeficiency virus epidemic has seen the re-emergence of this disease in the areas in which it was not very commonly reported. With this, the need for understanding and treatment of rare presentations of tuberculosis has become of paramount importance to achieve the World Health Organization millennium goal of a "reversal of incidence by 2015." Foot involvement has been reported in 0.1% to 0.3% of extrapulmonary cases. Multifocal lesions have an incidence of <10% in osteoarticular tuberculosis. Bilateral feet involvement in multifocal tuberculosis has not yet been reported in either children or adults in published studies. We report a case of tuberculosis with lesions in the bilateral metatarsals, the occurrence of which is very rare. The diagnosis was mainly histopathologic owing to the paucibacillary nature of the disease. Early identification and treatment with antitubercular drugs will normally result in a good cosmetic and functional result. PMID:25441279

  9. Bilateral tension pneumothorax following equipment improvisation.

    PubMed

    Zambricki, Christine; Schmidt, Carol; Vos, Karen

    2014-02-01

    This case report describes an unexpected event that took place as a result of using improvised equipment. The patient, a 16-year-old female undergoing complex oral surgery, suffered bilateral pneumothorax following the improper use of an airway support device. During the immediate postoperative period with the patient still intubated, oxygen tubing was attached to a right angle elbow connector with the port closed and 10 L/minute oxygen flow was administered to the patient in a manner that did not allow the patient to exhale. Within seconds, pneumothorax was apparent as the patient's vital signs deteriorated, visible swelling was noted in the shoulders and neck, and there was an absence of breath sounds on auscultation. This case study has application beyond the immediate discussion of bilateral pneumothorax, serving as a caution about the unintended consequences of equipment improvisation. In addition to highlighting the hazards of providing patient care with a non-standard device, this study also provides a powerful example of the human factors that can contribute to medical errors in the healthcare setting. PMID:24654348

  10. 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.

  11. 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

  12. 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.

  13. 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.

  14. 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

  15. 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

  16. 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

  17. 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

  18. 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

  19. Etiology of bilateral abductor vocal cord paralysis: a review of 389 cases.

    PubMed

    Holinger, L D; Holinger, P C; Holinger, P H

    1976-01-01

    The etiology of 389 cases of partial or complete bilateral abductor vocal cord paralysis has been determined and classified. One hundred and forty-nine were infants and children 12 years of age and under; 240 were adults, age 13 and older. In the infants and children the paralyses were congenital in 82 cases, of which 43 were associated with other congenital anomalies, and 39 were without associated anomalies. Fifty-nine cases were considered acquired, most being secondary to underlying congenital anomalies, particularly the associated findings of meningomyelocele, Arnold-Chiari malformation, and hydrocephalus. Eight cases of paralysis in this age group were of undetermined etiology. Of the 240 adult cases of bilateral vocal cord paralysis. 138 followed thyroidectomy. Fifty-two cases were associated with various neurologic disorders, including poliomyelitis, Parkinson's disease, cerebrovascular accident, Guillain-Barré syndrome, multiple sclerosis, neoplasms and other miscellaneous neurologic conditions. Sixteen cases were due to malignant neoplasms of the neck and mediastinum. The remaining 34 cases constitute a miscellaneous group which includes foreign bodies, bilateral neck dissection, infection, congenital lesions, trauma, and idiopathic paralyses. The characteristic symptoms of bilateral abductor vocal cord paralysis include normal or near normal phonation with inspiratory stridor which may progress to complete respiratory obstruction. These symptoms are due to the stationary but flaccid midline position of the vocal cords which places them in a phonating position, where they both obstruct the airway and produce a faily clear voice or cry. This paradoxical combination of symptoms was frequently found to be responsible for a failure or delay in diagnosis. PMID:949150

  20. Bilateral optic nerve head drusen with chorioretinal coloboma in the right eye.

    PubMed

    Dehghani, Ali Reza; Rezaei, Leila; Ghanbari, Heshmatollah

    2016-01-01

    Chorioretinal coloboma is a congenital defect of the eye caused by improper closure of the embryonic fissure. Optic nerve head drusen (ONHD) are white calcareous deposits that are generally asymptomatic. We report a very rare association of both in a healthy patient with no any systemic syndrome. A 16-year-old man was referred to our clinic from suffering blurred vision. Best corrected visual acuity of the right eye was 6/10 and 10/10 in the left one. External ocular and slit lamp examination were normal. Dilated ophthalmoscopy showed marked swelling in both optic nerves and chorioretinal coloboma in the right eye inferiorly. Ultrasonography showed an echodense structure with acoustic shadowing in both eyes consistent with buried ONHD. Visual field testing showed normal field in the left eye and moderate superior field depression in the right eye corresponding to inferior coloboma in funduscopy. Results of general medical and neurologic, cardiologic, and other examinations were normal. To the best our knowledge combination of bilateral ONHD and unilateral chorioretinal coloboma in a healthy patient with no any systemic syndrome has not been published in the literature. We reported this very rare association and recommended examine eyes and other body organs. In such cases that coloboma is associated with ONHD, we should keep in mind Noonan syndrome. The diagnosis of Noonan syndrome is clinical and confirm by the consultant pediatricians and clinical geneticists. PMID:27099847

  1. Bilateral optic nerve head drusen with chorioretinal coloboma in the right eye

    PubMed Central

    Dehghani, Ali Reza; Rezaei, Leila; Ghanbari, Heshmatollah

    2016-01-01

    Chorioretinal coloboma is a congenital defect of the eye caused by improper closure of the embryonic fissure. Optic nerve head drusen (ONHD) are white calcareous deposits that are generally asymptomatic. We report a very rare association of both in a healthy patient with no any systemic syndrome. A 16-year-old man was referred to our clinic from suffering blurred vision. Best corrected visual acuity of the right eye was 6/10 and 10/10 in the left one. External ocular and slit lamp examination were normal. Dilated ophthalmoscopy showed marked swelling in both optic nerves and chorioretinal coloboma in the right eye inferiorly. Ultrasonography showed an echodense structure with acoustic shadowing in both eyes consistent with buried ONHD. Visual field testing showed normal field in the left eye and moderate superior field depression in the right eye corresponding to inferior coloboma in funduscopy. Results of general medical and neurologic, cardiologic, and other examinations were normal. To the best our knowledge combination of bilateral ONHD and unilateral chorioretinal coloboma in a healthy patient with no any systemic syndrome has not been published in the literature. We reported this very rare association and recommended examine eyes and other body organs. In such cases that coloboma is associated with ONHD, we should keep in mind Noonan syndrome. The diagnosis of Noonan syndrome is clinical and confirm by the consultant pediatricians and clinical geneticists. PMID:27099847

  2. [Cushing syndrome in children].

    PubMed

    Raux-Demay, M C; Girard, F

    1993-09-01

    In pediatric patients, endogenous Cushing syndrome is an infrequent condition almost always due to one of two conditions. 1) Adrenal gland tumors account for 70% of Cushing syndromes in young pediatric patients. They cause rapidly progressive hypercorticism not due to increased ACTH production (elevated plasma and urine cortisol levels, very low ACTH and LPH levels unchanged by dexamethasone, metyrapone or CRH). Imaging techniques determine the side and spread of the tumor and look for metastases. Following surgical removal, patients with indicators of malignant disease (tumor weight above 30 g, extracapsular spread or metastases, independently from pathological data) are given op'DDD. 2) Cushing disease occurs in peripubertal patients and causes overweight with delayed statural gain. ACTH production is increased (positive dexamethasone suppression test and provocative metopirone and CRH tests) as a result of a pituitary adenoma which should be looked for by magnetic resonance imaging and whose removal ensures recovery in 50% of cases. Other therapeutic tools include op'DDD, radiation to the pituitary, and bilateral adrenalectomy as the last resort given the high risk of post-adrenalectomy pituitary tumor (50% of pediatric patients). Other causes are exceedingly rare: primary nodular hyperplasia of the adrenal glands and production of ACTH by a nonpituitary tumor. Corticosteroid treatment is the most common cause of Cushing syndrome in children. PMID:8239397

  3. 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

  4. 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.

  5. Incidence of bilateral Marcus Gunn jaw-wink.

    PubMed

    Sobel, Rachel K; Allen, Richard C

    2014-01-01

    This case report describes the unusual finding of bilateral Marcus Gunn jaw-wink. Only a few bilateral jaw-wink patients have been published as case reports. The authors dispute this notion as portrayed in the literature that a bilateral wink is a rare entity. A thorough review of prior case series on congenital ptosis and jaw-wink demonstrates that bilateral involvement is more common than previously thought. A supplemental video shows the classic action of lateral pterygoid contraction causing ipsilateral eyelid retraction. PMID:23880977

  6. 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

  7. 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

  8. A rare case of bilateral nasolabial cysts.

    PubMed

    Sethukumar, Priya; Taghi, Ali; Kuchai, Romana

    2015-01-01

    Nasolabial cysts are rare non-odontogenic cystic lesions representing around 0.7% of all maxillofacial cysts. They usually present as unilateral painless swellings, sometimes with epiphora and dacryocystitis as well as pain in cases of rapid growth or infection. We have reviewed the literature and present an extremely rare case of bilateral nasolabial cysts in a young Afro-Caribbean man presenting with chronic nasal blockage, epiphora and rhinorrhoea. We describe our successful surgical management using a sublabial approach for complete excision, leading to a disease-free outcome at 6 months follow-up. Other modalities have been described, from endoscopic marsupialisation to simple aspiration. However, with the exception of complete surgical excision, all other surgical techniques are associated with a high recurrence rate. We therefore advocate complete surgical excision as described below for optimal results. PMID:25795742

  9. Objective Bayesian Inference for Bilateral Data

    PubMed Central

    M’lan, Cyr Emile; Chen, Ming-Hui

    2015-01-01

    This paper presents three objective Bayesian methods for analyzing bilateral data under Dallal’s model and the saturated model. Three parameters are of interest, namely, the risk difference, the risk ratio, and the odds ratio. We derive Jeffreys’ prior and Bernardo’s reference prior associated with the three parameters that characterize Dallal’s model. We derive the functional forms of the posterior distributions of the risk difference and the risk ratio and discuss how to sample from their posterior distributions. We demonstrate the use of the proposed methodology with two real data examples. We also investigate small, moderate, and large sample properties of the proposed methodology and the frequentist counterpart via simulations.

  10. 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

  11. Bilateral Trade Flows and Income Distribution Similarity

    PubMed Central

    2016-01-01

    Current models of bilateral trade neglect the effects of income distribution. This paper addresses the issue by accounting for non-homothetic consumer preferences and hence investigating the role of income distribution in the context of the gravity model of trade. A theoretically justified gravity model is estimated for disaggregated trade data (Dollar volume is used as dependent variable) using a sample of 104 exporters and 108 importers for 1980–2003 to achieve two main goals. We define and calculate new measures of income distribution similarity and empirically confirm that greater similarity of income distribution between countries implies more trade. Using distribution-based measures as a proxy for demand similarities in gravity models, we find consistent and robust support for the hypothesis that countries with more similar income-distributions trade more with each other. The hypothesis is also confirmed at disaggregated level for differentiated product categories. PMID:27137462

  12. 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

  13. Unusual bilateral traumatic maculopathy following whiplash injury.

    PubMed

    Chronopoulos, Argyrios; Lipski, Andreas; Jonescu-Cuypers, Christian-Paul; Thumann, Gabriele

    2014-01-01

    An unusual extensive bilateral macular oedema (MO) with spontaneous resolution occurred following a car crash accident. Qualitative and quantitative analysis of the macular region using spectral domain optical coherence tomography (SD-OCT) and multifocal electroretinogram (mfERG) was performed daily during the first 7 days, as well as at 3 and 6 months following the accident. SD-OCT examination demonstrated extensive MO accompanied by neurosensory detachment and subretinal fluid. During the 7 days following the accident there was gradual resolution of the oedema accompanied by visual recovery. One year later no anatomical changes were observed, the mfERG showed complete recovery and visual acuity returned to normal level. Although whiplash is a common injury in motor vehicle accidents, whiplash maculopathy (WMP) is rarely reported, most likely due to underdiagnosis. Here we describe the spontaneous resolution of a severe MO after whiplash injury in a car crash accident. PMID:25414232

  14. 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

  15. Bilateral Trade Flows and Income Distribution Similarity.

    PubMed

    Martínez-Zarzoso, Inmaculada; Vollmer, Sebastian

    2016-01-01

    Current models of bilateral trade neglect the effects of income distribution. This paper addresses the issue by accounting for non-homothetic consumer preferences and hence investigating the role of income distribution in the context of the gravity model of trade. A theoretically justified gravity model is estimated for disaggregated trade data (Dollar volume is used as dependent variable) using a sample of 104 exporters and 108 importers for 1980-2003 to achieve two main goals. We define and calculate new measures of income distribution similarity and empirically confirm that greater similarity of income distribution between countries implies more trade. Using distribution-based measures as a proxy for demand similarities in gravity models, we find consistent and robust support for the hypothesis that countries with more similar income-distributions trade more with each other. The hypothesis is also confirmed at disaggregated level for differentiated product categories. PMID:27137462

  16. [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

  17. Bilateral mandibular fracture related to osteoradionecrosis

    PubMed Central

    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

  18. 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

  19. 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

  20. Cushing's syndrome associated with recurrent endometrioid adenocarcinoma of the ovary.

    PubMed Central

    Crawford, S M; Pyrah, R D; Ismail, S M

    1994-01-01

    Ectopic production of adrenocorticotrophic hormone (ACTH) by malignant neoplasms is a well recognised cause of Cushing's syndrome but is extremely rare in ovarian carcinoma. A patient who underwent surgery for ovarian carcinoma followed by a course of chemotherapy is reported. The tumour was a bilateral moderately differentiated endometrioid adenocarcinoma and contained numerous chromogranin immunoreactive endocrine cells as well as small foci of ACTH immunoreactivity. She subsequently presented with Cushing's syndrome in association with extensive pelvic recurrence of the tumour. Images PMID:7962637

  1. 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

  2. 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

  3. Jugular foraminal stenosis in Crouzon syndrome.

    PubMed

    Martinez-Perez, D; Vander Woude, D L; Barnes, P D; Scott, R M; Mulliken, J B

    1996-11-01

    We describe a 4-year-old boy with Crouzon syndrome, with associated acanthosis nigricans and Chiari-I malformation, who developed increased intracranial pressure necessitating posterior cranial expansion. Postoperatively, an arteriovenous fistula appeared over the mastoid region. Cerebral angiography demonstrated bilateral atresia of the jugular veins and occlusion of the left sigmoid and right transverse sinuses. We propose that increased intracranial venous pressure, secondary to bilateral jugular foraminal stenosis, caused hydrocephaly and venous dilation in the scalp vasculature. The latter set the stage for a traumatic arteriovenous fistula of the scalp, probably resulting from laceration of an adjacent artery and vein. Jugular atresia is a basilar malformation common to achondroplasia and certain eponymous syndromic craniosynostoses. Our patient has a mutation in fibroblast growth factor receptor 3, a different locus in the same gene mutated in achondroplasia. PMID:9309789

  4. A prospective study of bilateral inguinal hernia repair.

    PubMed Central

    Serpell, J. W.; Johnson, C. D.; Jarrett, P. E.

    1990-01-01

    A prospective study of outcome after inguinal hernia repair in patients undergoing simultaneous repair of bilateral hernias (n = 31), sequential repair of bilateral hernias (n = 5), and unilateral hernia repair (n = 75) is reported. There were no differences in wound complications, post-operative respiratory complications, or other adverse effects in the three groups. Operating time was similar in the unilateral and bilateral simultaneous repairs (median 55 min), but was longer (100 min) for the combination of two sequential repairs. Hospital stay was shortest for patients undergoing unilateral repair (2 days) but was less with bilateral simultaneous repair (4 days) than after two sequential repairs (total of 6 days). There were 12 (11%) wound complications of which five (5%) were infections. There was no difference in complication rate between unilateral and bilateral hernia repair. Postoperative recovery was assessed prospectively and was recorded at 1 month. There was no difference between unilateral and bilateral simultaneous repairs in the number of days before the patient was able to climb stairs easily, drive a car or return to work. The duration of the requirement for analgesia was similar in each group. We conclude that bilateral simultaneous hernia repair can be carried out with no greater morbidity than a unilateral repair, and the return to normal activity is as rapid. Bilateral hernias should be repaired simultaneously rather than sequentially. PMID:2221764

  5. Simultaneous bilateral quadriceps tendon rupture while playing basketball.

    PubMed

    Shah, M; Jooma, N

    2002-04-01

    Simultaneous bilateral quadriceps tendon rupture is an uncommon injury in healthy people and only a few cases have been reported in athletes. This is the first report of a patient with simultaneous bilateral quadriceps tendon rupture incurred while playing basketball. The injury was surgically repaired and the patient had a good functional outcome. PMID:11916903

  6. 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

  7. 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

  8. 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

  9. 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…

  10. 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...

  11. 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

  12. Bilateral Thoracic Ganglion Cyst : A Rare Case Report

    PubMed Central

    Kazanci, Burak; Tehli, Ozkan; Guclu, Bulent

    2013-01-01

    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

  13. Osteomyelitis of Bilateral Femoral Heads After Childbirth: A Case Report

    PubMed Central

    Lee, Kyung Soo; Kong, Sangwon; Kim, Junho; Kim, Taikon; Choi, Chan Beom; Kim, Yee-Suk

    2015-01-01

    Hip and pelvic pain during pregnancy or after delivery is a common problem in young females, and in most cases this problem has a self-limiting course. The patient described in this case suffered from severe hip pain after childbirth. MR imaging study was performed and it showed arthritis of bilateral hip joints and osteomyelitis of femoral heads with an abscess in the surrounding muscle. Infection, such as septic arthritis or osteomyelitis, is an extremely rare cause of peripartum joint pain. The patient's clinical symptoms and laboratory findings improved with antibiotic therapy. However, limitation of motion of the bilateral hip joints persisted although the patient continued rehabilitative therapy for 15 months, and the patient had to undergo bilateral total hip replacement. Hereby, we present a case of severe osteomyelitis and pyogenic arthritis of bilateral femoral heads and hip joints after delivery, which eventually required bilateral total hip replacement. PMID:26161359

  14. 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

  15. 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

  16. Usher Syndrome

    MedlinePlus

    Usher syndrome is an inherited disease that causes serious hearing loss and retinitis pigmentosa, an eye disorder ... hearing and vision. There are three types of Usher syndrome: People with type I are deaf from ...

  17. [Gardner's syndrome].

    PubMed

    Femiano, F; Cozzolino, S; Malzone, A

    1990-01-01

    The Gardner syndrome is characterized by polyposis coli and multiple hard and soft tissue tumors. This work show crucial dentist's role in the early diagnosis important for the highly malignant potential of this syndrome. PMID:2097961

  18. Hurler syndrome

    MedlinePlus

    Hurler syndrome is a rare disease of metabolism in which a person cannot break down long chains of sugar molecules called glycosaminoglycans (formerly called mucopolysaccharides). Hurler syndrome belongs to a group of diseases called mucopolysaccharidosis, ...

  19. 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 disorder ...

  20. Proteus Syndrome

    MedlinePlus

    ... Gift Stock Gift Sunshine Society Contact Privacy Policy Proteus Syndrome Definition Common Signs Diagnostic Criteria (I have ... NIH to go with this criteria) Glossary Videos Proteus Syndrome is a condition which involves atypical growth ...

  1. 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 ...

  2. Rett syndrome

    MedlinePlus

    Rett syndrome is a disorder of the nervous system that leads to developmental problems in children, especially in ... Rett syndrome occurs almost always in girls. It may be diagnosed as autism or cerebral palsy. Most Rett ...

  3. 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. ...

  4. Serotonin syndrome

    MedlinePlus

    ... medicines that caused the syndrome In life-threatening cases, medicines that keep your muscles still (paralyze them) and ... Syndrome. Am J Psychiatry . 1991: 148:705. US Food and Drug Administration. FDA Public Health Advisory: Combined Use of ...

  5. 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 ...

  6. Turner Syndrome

    MedlinePlus

    Turner syndrome is a genetic disorder that affects a girl's development. The cause is a missing or incomplete ... t work properly. Other physical features typical of Turner syndrome are Short, "webbed" neck with folds of skin ...

  7. Branchiootorenal Syndrome

    MedlinePlus

    ... Rare Disease Day More Search for News on Rare Diseases Search Go Advanced News Search About GARD About ... GARD Home Diseases Branchiootorenal syndrome Diseases Genetic and Rare Diseases Information Center (GARD) Print friendly version Branchiootorenal syndrome ...

  8. Zellweger Syndrome

    MedlinePlus

    ... cure disorders such as Zellweger syndrome. NIH Patient Recruitment for Zellweger Syndrome Clinical Trials At NIH Clinical Center Throughout the U.S. and Worldwide NINDS Clinical Trials Organizations Column1 Column2 National Institute of Child Health and ...

  9. Warburg micro syndrome in siblings from India

    PubMed Central

    Sekhon, Prabhjot Kaur; Premalatha, R.; Sabapathy, Sarala

    2016-01-01

    Warburg syndrome is a rare disorder characterized by microcephaly, microcornea, congenital cataract, developmental delay, and hypogonadism. Here, we report two siblings from India who presented with developmental delay, microcornea, microphthalmia, and bilateral congenital cataracts, born to the third-degree consanguineously married couple. Both children had hypoplasia of corpus callosum. In this report, we aim to highlight and compare clinical features of these two cases with previously reported cases. PMID:27195044

  10. A new case of TEMPI syndrome

    PubMed Central

    Mohammadi, Farnaz; Wolverson, Michael K.; Bastani, Bahar

    2012-01-01

    We present an interesting case of a woman with new onset hypertension and abdominal fullness who was found to have huge bilateral perinephric fluid collections. Extensive workup revealed that she had secondary polycythemia, extensive truncal and proximal extremities telangiectasia and IgA-lambda monoclonal gammopathy of underdetermined significance. We believe that this is one of the rare cases consistent with the recently described TEMPI syndrome. PMID:26069800

  11. Imaging findings of Gorlin-Goltz syndrome

    PubMed Central

    Hajalioghli, Parisa; Ghadirpour, Ali; Ataie-Oskuie, Reza; Kontzialis, Marinos

    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

  12. [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

  13. Novel neuropathologic findings in the Haddad syndrome

    PubMed Central

    Tomycz, Nestor D.; Haynes, Robin L.; Schmidt, Edith F.; Ackerson, Kate; Kinney, Hannah C.

    2012-01-01

    Haddad syndrome (congenital central hypoventilation syndrome and Hirschsprung's disease) is a rare disorder for which in-depth neuropathologic analysis is lacking. We report the brain findings in a full-term male infant with Haddad syndrome who died at 27 days of life. Bilateral hypoplasia of the superior temporal lobe and gyral anomalies in the frontal cortex were present. Immunohistochemistry with an antibody to tyrosine hydroxylase (noradrenaline synthesis) demonstrated hypoplasia of the locus coeruleus (implicated in chemoreception) and A5 region. Other findings included delayed maturation of the arcuate nucleus (putative human homologue of ventral medullary neurons in animals critical for chemoreception) and aberrant fascicles in the nucleus of the solitary tract. Efforts to determine the putative gene mutation were unsuccessful. This study implicates novel brain findings in Haddad syndrome mimicking those in murine Phox2b null mutants. This case suggests that abnormalities occur in CCHS in a network of sites critical to chemoreception. PMID:19844731

  14. Ascher's syndrome: A rare case report

    PubMed Central

    Chandravanshi, Shivcharan Lal; Mishra, Vinay

    2015-01-01

    An 18-year-old Indian girl with upper lip deformity presented with on and off painless swelling of her both upper eyelids for 3 years. Clinical evaluation revealed bilateral blepharochalasis, narrowing of horizontal palpebral fissure, decreased outer intercanthal distance, iris coloboma, cleft soft palate, bifid uvula, sensorineural deafness and double upper lip. Clinical examination of the thyroid, thyroid hormone assay and ultrasonography revealed normal thyroid gland structure and function. Ascher's syndrome was diagnosed. To our knowledge, this is the first reported case of Ascher's syndrome associated with iris coloboma, heterochromia iridum, and narrowing of horizontal palpebral fissure and decreased outer intercanthal distance secondary to lengthening of lateral canthal ligament. PMID:25971175

  15. A male with type I orofaciodigital syndrome.

    PubMed Central

    Goodship, J; Platt, J; Smith, R; Burn, J

    1991-01-01

    We describe a three generation family with three females showing minor features of orofaciodigital syndrome type I and a severely affected male in the third generation. In addition to the classical features of OFD I, the male had bilateral duplication of the halluces, a feature diagnostic of OFD II, and an atrioventricular septal defect. Heart defects have not previously been reported in OFD I but have been reported in OFD II. It is important to examine the mothers of all male neonates with orofaciodigital syndrome with care before making a diagnosis of OFD II. Images PMID:1941964

  16. Learning about Down Syndrome

    MedlinePlus

    ... genetic terms used on this page Learning About Down Syndrome What is Down syndrome? What are the symptoms ... syndrome Additional Resources for Down Syndrome What is Down syndrome? Down syndrome is a chromosomal condition related to ...

  17. Craniofacial Syndrome Descriptions

    MedlinePlus

    ... Dysplasia Goldenhar/Hemifacial Moebius syndrome Pfeiffer syndrome Pierre Robin Sequence Treacher Collins syndrome Other syndromes Wonder News & ... absence of the radial limb. Pfeiffer syndrome Pierre Robin Sequence Saethre-Chotzen Saethre-Chotzen syndrome is a ...

  18. [Pseudomeigs syndrome in a patient with Krukenberg's tumor].

    PubMed

    Bayod, M J Herráiz; Carlón, M Elorz; Idoate, M A

    2007-01-01

    We report the case of a fiftyone-year-old woman with a past medical history of Linfoma no Hodking and a gastric adenocarcinoma with signet ring cells. She came to our institution with a twenty month history of dysnea secondary to pleural effussion, bilateral lower extremity edema and probably had ascitis. On CT and US two bilateral pelvic masses were found and biopsied. The anatomopathological analysis showed bilateral ovarian implants from signet ring cell adenocarcinoma (Krukenberg tumor). This patient developed a PseudoMeigs syndrome consisting on malignant ovarian tumor asociated with ascitis and pleural effusion without malignant cells. Oncological patients who present with ascitis and benign pleural effusion, the diagnosis of PseudoMeigs syndrome should be considered. PMID:18183782

  19. Fulminant crural compartment syndrome preceded by psychogenic polydipsia.

    PubMed

    Ulstrup, Anton; Ugleholdt, Randi; Rasmussen, Jeppe Vejlgaard

    2015-01-01

    We report a case of bilateral anterolateral crural compartment syndrome elicited by hyponatraemia and psychogenic polydipsia. The unusual constellation of clinical findings and diminished pain expression made initial diagnostic procedures challenging. The possible pathogenesis and treatment options are discussed. Impairment of lower extremity function at follow-up was serious and permanent. PMID:25976194

  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. 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…

  2. Cushing Syndrome

    MedlinePlus

    ... links Share this: Page Content What is Cushing’s syndrome? Cushing’s syndrome is a condition that occurs when the body’s ... medication or as a result of a tumor, Cushing’s syndrome can develop. Many factors influence whether this happens, ...

  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. 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

  5. Spatial Benefit of Bilateral Hearing Aids

    PubMed Central

    Ahlstrom, Jayne B.; Horwitz, Amy R.; Dubno, Judy R.

    2013-01-01

    Objectives To assess the extent to which hearing aids improve spatial benefit by restoring the availability of interaural difference cues, the benefit attributable to spatial separation of speech and babble with and without bilateral hearing aids was measured as a function of low-pass cutoff frequency. Design Twenty-one older adults with sloping high-frequency hearing loss were provided commercially available bilateral hearing aids. After a 3 to 6 month acclimatization period, speech levels corresponding to 50% correct recognition of sentences from the Hearing in Noise Test (HINT) were measured in a 65-dB SPL babble, with speech and babble low-pass filtered at 1.8, 3.6, and 5.6 kHz. Sentences were always at 0° azimuth, and babble was at either 0° or 90°. Speech and babble spectra for all conditions were digitally recorded using a probe microphone placed in each ear canal of each subject. Spectra and levels of speech and babble and unaided thresholds for narrowband noises were used to calculate the aided audibility index and provide predictions of unaided and aided thresholds for HINT sentences, hearing aid benefit, and spatial benefit for each cutoff frequency. In addition, subjects’ willingness to tolerate background noise with and without amplification was measured in the spatially coincident and spatially separated conditions using the Acceptable Noise Level (ANL) procedure. Results Thresholds for HINT sentences in babble and ANL improved significantly when aided and when speech and babble were spatially separated. Specifically, hearing aid benefit improved significantly as cutoff frequency increased from 1.8 to 3.6 kHz but only when speech and babble were spatially separated; likewise, spatial benefit improved significantly from 1.8 to 3.6 kHz but only in the aided condition. No further improvement in hearing aid or spatial benefit was observed when cutoff frequency was increased from 3.6 to 5.6 kHz, although improvement in hearing aid benefit was predicted. Conclusions Hearing aid benefit, although significant, was poorer than predicted, suggesting that these older adults with high-frequency hearing loss did not take full advantage of the increase in audible speech information provided by amplification. Hearing aid benefit was also limited because hearing aids for some subjects did not restore speech audibility across the full bandwidth of speech. Unaided and aided spatial benefit was significantly greater than predicted, and aided spatial benefit was greater than unaided. This suggests that these older adults were able to take advantage of interaural level and time difference cues to improve speech recognition in babble and that they benefited from these cues to a greater extent with than without bilateral hearing aids. Finally, in contrast to results of previous studies, ANL may vary for an individual depending on the listening condition. PMID:19194292

  6. Novel brain MRI abnormalities in Gitelman syndrome.

    PubMed

    El Beltagi, Ahmed; Norbash, Alexander; Vattoth, Surjith

    2015-10-01

    Gitelman syndrome is an autosomal recessive renal tubular disorder characterized by hypokalemic metabolic alkalosis, hypomagnesemia and hypocalciuria. The syndrome is caused by a defective thiazide-sensitive sodium chloride co-transporter in the distal convoluted tubules of the kidneys. Gitelman syndrome could be confused with Bartter syndrome; the main differentiating feature is the presence of low urinary calcium excretion in the former. Descriptions of neuroradiological imaging findings associated with Gitelman syndrome are very scarce in the literature and include basal ganglia calcification, idiopathic intracranial hypertension and sclerochoroidal calcification. Cauda equina syndrome-like presentation has been reported, but without any corresponding imaging findings on lumbar spine MRI. We report a 13-year-old male with Gitelman syndrome who presented with altered mental status following a fall and scalp laceration and unremarkable brain CT, followed during hospitalization by somnolence and seizures. Metabolically the patient demonstrated hypokalemia and hypomagnesemia. MRI demonstrated features of encephalopathy including predominantly right-sided cerebral hemispheric signal abnormality and cytotoxic edema, with bilateral symmetric involvement of the thalami, midbrain tegmentum and tectum and cerebellar dentate nuclei. MRI after five months obtained during a later episode of encephalopathy showed resolution of the signal abnormalities with setting in of brain atrophy and also areas of newly developed cytotoxic edema in the left thalamus, bilateral dorsal midbrain and right greater than left dentate nuclei. The described abnormalities, either recurrent or in isolation, have not previously been published in patients with Gitelman syndrome. We believe that the findings are due to alteration of respiratory chain function secondary to the metabolic derangement and hence have a similar imaging appearance as encephalopathy related to mitochondrial cytopathy or metabolic encephalopathy. PMID:26443301

  7. 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

  8. 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.

  9. 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.

  10. [Accidental bilateral asymmetric ruby laser maculopathy].

    PubMed

    Lang, G K; Lang, G; Naumann, G O

    1985-05-01

    A 19-year-old man sustained a bilateral coagulation of the macula caused accidentally by a ruby laser rangefinder mounted on a tank. Five days later vision was OD 0.7, OS 0.05; there was a distinct disk of coagulation of the retinal pigment epithelium with clumping of melanin granula, more in the left eye than in the right, and subretinal hemorrhage on the left. Visual improvement corresponded to the asymmetric morphologic findings: Within two months visual acuity in the right eye recovered to 1.0. In the left eye there was a temporary increase to 0.3, then permanent deterioration of vision to 0.05 OS. The macula of the left eye showed massive lipofuscein aggregation in the subretinal space. Fluorescein angiography after two months showed some hyperfluorescence, more in the left than in the right eye, but no fluorescein extravasation. The vision in the left eye is still 0.05 one year after the accident. PMID:4021426

  11. 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

  12. 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.

  13. 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

  14. 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

  15. 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

  16. Bilateral cochlear implantation: current concepts, indications, and results.

    PubMed

    Basura, Gregory J; Eapen, Rose; Buchman, Craig A

    2009-12-01

    The optimal treatment for bilateral hearing loss continues to evolve as cochlear implant (CI) and hearing aid technologies advance, as does our understanding of the central auditory system. Ongoing discussions continue on the validity and feasibility of bilateral CI in terms of performance, justification of need, medical/surgical safety concerns, and economics. The purpose of this review article is to provide an update on the advantages and disadvantages of bilateral CI and to provide a discussion on timing (simultaneous vs. sequential), technology (bimodal vs. binaural) and feasibility. Binaural advantages are found in both adult and pediatric bilateral CI recipients, the greatest being the head shadow effect and improvements in localization and loudness summation. This theoretically offers an advantage over their unilateral implanted counterparts in terms of improved sound localization and enhanced speech perception under noisy conditions. Most investigators agree that bilateral stimulation during critical periods of development is paramount for optimizing auditory functioning in children. Currently, bilateral CI is widely accepted as a safe and effective means of bilateral auditory stimulation. PMID:19894280

  17. 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

  18. 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.

  19. 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.

  20. Reduced 123I Ioflupane Binding in Bilateral Diabetic Chorea

    PubMed Central

    Sato, Kenichiro; Hida, Ayumi; Kameyama, Masashi; Morooka, Miyako; Takeuchi, Sousuke

    2016-01-01

    Abstract We report a 64-year-old man with diabetic chorea whom we investigated with dopamine transporter SPECT, 18F FDG PET, 99mTc ethylcysteinate dimer (ECD) SPECT, and 123I metaiodobenzylguanidine (MIBG) scintigraphy. Dopamine transporter SPECT revealed reduced 123I ioflupane binding in the bilateral striatum. 18F FDG PET showed metabolic dysfunction in the bilateral striatum, as shown in earlier studies. 99mTc ECD SPECT revealed reduced brain perfusion in the bilateral caudate nucleus and putamen. 123I MIBG scintigraphy revealed no cardiac sympathetic nerve dysfunction. Our case suggests a possible nigrostriatal presynaptic dopaminergic involvement in diabetic chorea. PMID:26975011

  1. Parkinsonism and transient bilateral ptosis in systemic lupus erythematosus

    PubMed Central

    Teoh, P. C.; Richard, A. T. Ng; Wong, P. K.

    1974-01-01

    Many neurological abnormalities have been described in systemic lupus erythematosus (SLE), but transient bilateral ptosis and parkinsonism are rarely encountered. This paper describes a young Malay girl with SLE who develops psychosis, bilateral ptosis and parkinsonism during an exacerbation of her illness. These neurological features disappeared after adequate treatment with cyclophosphamide. Though the pathogenesis of these neurological abnormalities is not clearly known, it is likely that transient bilateral ptosis is due to myoneural dysfunction not unlike that of myasthenia gravis. As for parkinsonism, it can probably be explained on the basis of ‘vasculitis’ of the basal ganglia leading to microinfarcts and encephalomalacia. ImagesFig. 1Fig. 2

  2. 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

  3. Spheniodal mucocele causing bilateral optic neuropathy and ophthalmoplegia

    PubMed Central

    Selvakumar, Ambika; Mahalaxmi, Balasubramanyam; Ananth, V; Gautam, Cugati

    2014-01-01

    Sphenoid sinus mucocele comprises only 2% of all paranasal sinus mucoceles. In literature, there is a case report on sphenoidal mucocele causing bilateral optic neuropathy, with unilateral partial recovery and cranial nerve palsy, but we did not come across any literature with bilateral optic neuropathy and ophthalmoplegia together caused by spheno-ethmoidal mucocele. We present such a rare case of spheno-ethmoidal mucocele causing bilateral optic neuropathy and unilateral sixth nerve palsy who had postsurgery, unilateral good vision recovery, and complete resolution of sixth nerve palsy. PMID:23571243

  4. Bilateral activity and callosal connections in the somatosensory cortex.

    PubMed

    Iwamura, Y; Taoka, M; Iriki, A

    2001-10-01

    Earlier studies recording single neuronal activity in the postcentral somatosensory cortex of monkeys converged in suggesting that the bilateral receptive fields were related exclusively to the body midline including the trunk, perioral face, and oral cavity. These neurons were recorded mostly in the rostral part of the gyrus, areas 3b and 1. However, the authors recently found a substantial number of neurons with bilateral receptive fields on extremities, hand/digits, shoulders/arms, or legs/feet in the caudalmost part (areas 2 and 5) of the postcentral gyrus. The authors review these results and discuss functional implications of the bilateral representation in the postcentral somatosensory cortex. PMID:11597101

  5. Bilateral ureteric stones: an unusual cause of acute kidney injury.

    PubMed

    Sumner, Daniel; Rehnberg, Lucas; Kler, Aaron

    2016-01-01

    A 49-year-old man presented to the accident and emergency department, with a short history of vague abdominal pain, abdominal distension and two episodes of frank haematuria. A plain chest film showed dilated loops of large bowel and blood results on admission showed an acute kidney injury (stage 3). A diagnosis of bowel obstruction was made initially but a CT scan of the abdomen showed bilateral obstructing calculi. After initial resuscitation, the patient had bilateral ultrasound-guided nephrostomies and haemofiltration. He later underwent bilateral antegrade ureteric stenting. A decision will later be made on whether or not he is fit enough to undergo ureteroscopy and laser stone fragmentation. PMID:27030462

  6. Geleophysic dysplasia associated with bilateral angle closure glaucoma

    PubMed Central

    Saricaoglu, Murat Sinan; Güven, Dilek; Karakurt, Ahmet; Hasiripi, Hikmet

    2013-01-01

    In this case report, we present occurrence of bilateral angle closure glaucoma in a 9-year-old girl with geleophysic dysplasia. Bilateral YAG laser iridotomy was applied, but intraocular pressure (IOP) remained at high levels, necessitating bilateral trabeculectomy with mitomycin C. On her follow-up examinations for 3 years, IOP remained in the mid-20s with no need for further intervention or antiglaucoma medication. There are few reports describing the ocular findings of geleophysic dysplasia in literature. To our knowledge, this is the first case report describing an application of glaucoma surgery and its results at geleophysic dysplasia. PMID:23514648

  7. Geleophysic dysplasia associated with bilateral angle closure glaucoma.

    PubMed

    Saricaoglu, Murat Sinan; Güven, Dilek; Karakurt, Ahmet; Hasiripi, Hikmet

    2013-03-01

    In this case report, we present occurrence of bilateral angle closure glaucoma in a 9-year-old girl with geleophysic dysplasia. Bilateral YAG laser iridotomy was applied, but intraocular pressure (IOP) remained at high levels, necessitating bilateral trabeculectomy with mitomycin C. On her follow-up examinations for 3 years, IOP remained in the mid-20s with no need for further intervention or antiglaucoma medication. There are few reports describing the ocular findings of geleophysic dysplasia in literature. To our knowledge, this is the first case report describing an application of glaucoma surgery and its results at geleophysic dysplasia. PMID:23514648

  8. Pycnodysostosis associated with bilateral congenital pseudarthrosis of the clavicle.

    PubMed

    Karakurt, Lokman; Yilmaz, Erhan; Belhan, Oktay; Serin, Erhan

    2003-04-01

    We report the case of a 22-year-old woman with pycnodysostosis associated with bilateral congenital pseudarthrosis of the clavicle. The patient was first seen at the age of 19 years and had no symptoms during the 3-year follow-up period. The diagnosis of pycnodysostosis was made by typical clinical and radiological findings. Bilateral painless mid-clavicular mass and plain radiograms confirmed the diagnosis of bilateral congenital pseudarthrosis of the clavicle. Surgery was not performed for pseudarthrosis of the clavicles, just observation was preferred. PMID:12721693

  9. Bilateral adrenocortical carcinoma in a patient with multiple endocrine neoplasia type 1 (MEN1) and a novel mutation in the MEN1 gene

    PubMed Central

    2011-01-01

    The incidence of adrenal involvement in MEN1 syndrome has been reported between 9 and 45%, while the incidence of adrenocortical carcinoma (ACC) in MEN1 patients has been reported between 2.6 and 6%. In the literature data only unilateral development of ACCs in MEN1 patients has been reported. We report a 31 years-old female MEN1-patient, in whom hyperplasia of the parathyroid glands, prolactinoma, non functioning pancreatic endocrine carcinoma and functioning bilateral adrenal carcinomas were diagnosed. Interestingly, a not previously described in the literature data, novel germline mutation (p.E45V) in exon 2 of MEN1 gene, was detected. The association of exon 2 mutation of the MEN1 gene with bilateral adrenal carcinomas in MEN1 syndrome, should be further investigated. PMID:21266030

  10. Connectivity-based parcellation of the thalamus explains specific cognitive and behavioural symptoms in patients with bilateral thalamic infarct.

    PubMed

    Serra, Laura; Cercignani, Mara; Carlesimo, Giovanni A; Fadda, Lucia; Tini, Nadia; Giulietti, Giovanni; Caltagirone, Carlo; Bozzali, Marco

    2014-01-01

    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

  11. Laparoscopic Adrenalectomy in a Patient of Von Hippel Lindau Syndrome with Ventriculo-Peritoneal Shunt-Anaesthetic management.

    PubMed

    Mungasuvalli, Nagaraj Channappa; Seenappa Pujari, Vinayak; Chinnappa, Jithendra; Iyer, Sadasivan Shanker; Thammanna, Prathima Padavarahalli

    2014-09-01

    Von Hippel Lindau (VHL) syndrome has a predilection to manifest multiple haemangioblastomas in the retina and central nervous system. We report a rare case of raised intracranial pressure during bilateral laparoscopic adrenalectomy for pheochromocytoma in a patient of VHL syndrome who had a ventriculoperitoneal shunt. PMID:25386452

  12. Laparoscopic Adrenalectomy in a Patient of Von Hippel Lindau Syndrome with Ventriculo-Peritoneal Shunt-Anaesthetic management

    PubMed Central

    Mungasuvalli, Nagaraj Channappa; Chinnappa, Jithendra; Iyer, Sadasivan Shanker; Thammanna, Prathima Padavarahalli

    2014-01-01

    Von Hippel Lindau (VHL) syndrome has a predilection to manifest multiple haemangioblastomas in the retina and central nervous system. We report a rare case of raised intracranial pressure during bilateral laparoscopic adrenalectomy for pheochromocytoma in a patient of VHL syndrome who had a ventriculoperitoneal shunt. PMID:25386452

  13. 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

  14. Role of bilateral inferior petrosal sinus sampling (BIPSS) in the diagnosis of Cushing's disease in a patient with double superior vena cava.

    PubMed

    Tashi, Sonam; Ng, Keng Sin

    2015-08-01

    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

  15. 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

  16. Bilateral atraumatic medial meniscal tears in a 17-year-old rower.

    PubMed

    Taylor, Taryn Lise; Frankovich, Renata; Rumball, Jane

    2009-01-01

    Meniscal injury produces disability in a large portion of the population, and sports injuries are a common cause. Atraumatic meniscal tears may occur after repetitive low-energy loading. Rowing is a highly technical sport and very demanding on an athlete's body. There are numerous reports on patellofemoral and iliotibial band friction syndrome in rowers but there is an extremely low incidence of meniscal tears reported in these athletes. This is a unique case report of a young adolescent athlete who suffered bilateral medical meniscal tears during sporting activity. Rowing is a low impact sport making this an unusual occurrence, especially in a young individual. This case report highlights the importance of considering all training activities when trying to isolate the mechanism of injury in an athlete. PMID:21686387

  17. 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

  18. 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

  19. Changes in bilateral bispectral index VISTA monitoring system during Wada test.

    PubMed

    Pacreu, S; Vila, E; Rodríguez, C; Arroyo, R; Fernández, S; Fernández, J L

    2014-12-01

    The Wada test is a procedure used in the preoperative assessment before epilepsy surgery in order to determine language lateralization, to assess the post-operative risk of an amnesia syndrome, and to evaluate the risk of material-specific memory deficits, in particular verbal memory deficits. This test involves inserting a cannula into the internal carotid artery via the femoral artery, and then to inject amobarbital to shut down brain function, usually in one of the brain hemispheres. The bilateral bispectral index (BIS) VISTA™ monitoring system (BVMS) was used to detect changes in EEG, and in the power spectrum distribution using the density spectral array (DSA) of both hemispheres. We describe a patient with an agenesis of the A1 segment of the right anterior cerebral artery, scheduled for a Wada test, in whom the BVMS demonstrated its potential value. PMID:24657004

  20. 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

  1. 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

  2. Bilateral carpal tunnel release at one operation: report of 228 patients.

    PubMed

    Pagnanelli, D M; Barrer, S J

    1992-12-01

    The division of the transverse carpal ligament for relief of carpal tunnel syndrome has been a standard operative procedure since the early 1950s. The surgical technique, however, is controversial, and the literature is rife with individual preferences and biases. This report describes our results in 228 tensor fasciae patients who underwent a bilateral carpal ligament release in one procedure that used a small transverse incision in the wrist crease. When the patients' responses to a questionnaire, records of postoperative visits, and follow-up phone calls were analyzed, 216 patients (94.7%) had a satisfactory outcome. One hundred fifty-five patients were working before surgery, with 88.3% back to work in 6 weeks or less. There were no major complications. PMID:1335136

  3. Diagnosis of osteopetrosis in bilateral congenital aural atresia: Turning point in treatment strategy

    PubMed Central

    Verma, Ritu; Jana, Manisha; Bhalla, Ashu Seith; Kumar, Arvind; Kumar, Rakesh

    2016-01-01

    Aural atresia is a rare congenital malformation of the external and middle ear. There are several syndromic associations of this anomaly with those involving the first and second branchial arches. Occurrence of aural atresia with sclerosing skeletal dysplasia is unknown and has never been reported. The co-existence of a sclerosing dysplasia can make the surgical treatment in aural atresia difficult and risky; and the auditory improvement may not be as expected. Moreover, internal auditory canal narrowing and hence sensorineural hearing loss in sclerosing dysplasia might add to the already existing conductive hearing loss in such patients. In this case report we have described an unknown association of bilateral microtia with sclerosing skeletal dysplasia (autosomal dominant osteopetrosis) and clinical implications of these two conditions occurring together leading to a change in the management plan. PMID:27170934

  4. Compression therapy and liposuction of lower legs for bilateral hereditary primary lymphedema praecox.

    PubMed

    Espinosa-de-Los-Monteros, Antonio; Hinojosa, Carlos A; Abarca, Leonardo; Iglesias, Martín

    2009-01-01

    In this report, we describe a case of bilateral non-syndromic hereditary lymphedema praecox of lower legs. The patient was diagnosed at age 16. Ten years later, he was unable to ambulate due to increased bilateral lower leg volume, continuous pain, and recurrent episodes of cellulitis. He was treated at our tertiary-care center with compression therapy and circumferential liposuction of lower legs, ankles, and dorsum of feet in order to remove hypertrophic fat deposits, facilitate conservative therapy, and decrease further risk of cellulitis. No complications were seen and compression therapy was continued. Fourteen month follow-up reveals no increase in leg volume over time, absence of pain, and no further episodes of cellulitis with complete ability to ambulate and return to normal activities. Even when it does not eliminate the underlying cause of primary lymphedema, combined therapy consisting of compression and liposuction is safe and is able to achieve control, at least on a short term, of clinically disabling conditions associated with advanced stages. PMID:19174259

  5. Zellweger syndrome with severe malnutrition, immunocompromised state and opportunistic infections.

    PubMed

    Cardoso, Patrícia; Amaral, Maria Emanuel; Lemos, Sónia; Garcia, Paula

    2016-01-01

    Peroxisome biogenesis disorders are related to a spectrum of genetic diseases that range from severe Zellweger syndrome to milder infantile Refsum disease. Zellweger syndrome is characterised by dysmorphic features, severe hypotonia, seizures, failure to thrive, liver dysfunction and skeletal defects. Increased levels of very long chain fatty acids are the biochemical hallmark and the most common mutations found in the PEX1 gene. We report an unusual presentation of Zellweger syndrome in a 2-month-old female infant with severe malnutrition, opportunistic infections, lymphopaenia and a small thymic shadow on chest radiography. With this clinical picture, an initial hypothesis of primary immunodeficiency was considered. It was later confirmed to not be the case. On follow-up, global developmental delay, bilateral optic nerve atrophy and moderate bilateral sensorineural deafness grade II were documented. There were no further infectious complications and we concluded malnutrition was the cause of the infant's immunocompromised state. PMID:27090541

  6. Epilepsy in Muenke syndrome: FGFR3-related craniosynostosis.

    PubMed

    Agochukwu, Nneamaka B; Solomon, Benjamin D; Gropman, Andrea L; Muenke, Maximilian

    2012-11-01

    Epilepsy, a neurologic disorder characterized by the predisposition to recurrent unprovoked seizures, is reported in more than 300 genetic syndromes. Muenke syndrome is an autosomal-dominant craniosynostosis syndrome characterized by unilateral or bilateral coronal craniosynostosis, hearing loss, intellectual disability, and relatively subtle limb findings such as carpal bone fusion and tarsal bone fusion. Muenke syndrome is caused by a single defining point mutation in the fibroblast growth factor receptor 3 (FGFR3) gene. Epilepsy rarely occurs in individuals with Muenke syndrome, and little detail is reported on types of epilepsy, patient characteristics, and long-term outcomes. We present seven patients with Muenke syndrome and seizures. A review of 789 published cases of Muenke syndrome, with a focus on epilepsy and intracranial anomalies in Muenke syndrome, revealed epilepsy in six patients, with intracranial anomalies in five. The occurrence of epilepsy in Muenke syndrome within our cohort of 58 patients, of whom seven manifested epilepsy, and the intracranial anomalies and epilepsy reported in the literature, suggest that patients with Muenke syndrome may be at risk for epilepsy and intracranial anomalies. Furthermore, the impact of Muenke syndrome on the central nervous system may be greater than previously thought. PMID:23044018

  7. Epilepsy in Muenke Syndrome: FGFR3-Related Craniosynostosis

    PubMed Central

    Agochukwu, Nneamaka B.; Solomon, Benjamin D.; Gropman, Andrea L.; Muenke, Maximilian

    2014-01-01

    Epilepsy, a neurologic disorder characterized by the predisposition to recurrent unprovoked seizures, is reported in more than 300 genetic syndromes. Muenke syndrome is an autosomal-dominant craniosynostosis syndrome characterized by unilateral or bilateral coronal craniosynostosis, hearing loss, intellectual disability, and relatively subtle limb findings such as carpal bone fusion and tarsal bone fusion. Muenke syndrome is caused by a single defining point mutation in the fibroblast growth factor receptor 3 (FGFR3) gene. Epilepsy rarely occurs in individuals with Muenke syndrome, and little detail is reported on types of epilepsy, patient characteristics, and long-term outcomes. We present seven patients with Muenke syndrome and seizures. A review of 789 published cases of Muenke syndrome, with a focus on epilepsy and intracranial anomalies in Muenke syndrome, revealed epilepsy in six patients, with intracranial anomalies in five. The occurrence of epilepsy in Muenke syndrome within our cohort of 58 patients, of whom seven manifested epilepsy, and the intracranial anomalies and epilepsy reported in the literature, suggest that patients with Muenke syndrome may be at risk for epilepsy and intracranial anomalies. Furthermore, the impact of Muenke syndrome on the central nervous system may be greater than previously thought. PMID:23044018

  8. 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

  9. 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.

  10. Synchronous bilateral male breast cancer: a case report.

    PubMed

    Sun, Woo-Young; Lee, Ki-Hyeong; Lee, Ho-Chang; Ryu, Dong-Hee; Park, Jin-Woo; Yun, Hyo-Young; Song, Young-Jin

    2012-06-01

    Synchronous bilateral breast cancer is extremely rare in men and has not, up to date, been reported in Korea. A 54-year-old man presented with a palpable mass in the right breast. The right nipple was retracted and bilateral axillary accessory breasts and nipples were present. On physical examination, a 2 cm-sized mass was palpated directly under the right nipple, and, with squeezing, bloody discharge developed in a single duct of the left nipple. There was no palpable mass in the left breast, and axillary lymph nodes were not palpable. Physical examination of external genitalia revealed a unilateral undescended testis on the left side. Synchronous bilateral breast cancer was diagnosed using mammography, ultrasonography, and core-needle biopsy. Histopathological examination revealed invasive ductal carcinoma in the right breast and ductal carcinoma in situ in the left breast. Bilateral total mastectomy, sentinel lymph node biopsy, and excision of accessory breasts in the axilla were performed. PMID:22807945

  11. Recurrent Bilateral Breast Abscess Due to Nontuberculous Mycobacterial Infection

    PubMed Central

    Yoo, Hyunkyung; Choi, Sang Hyun; Kim, Sei Joong; Cho, Young Up; Choi, Suk Jin

    2014-01-01

    Since recurrent bilateral breast infection due to nontuberculous mycobacterium (NTM) is rare, its diagnosis is easily overlooked; in addition, complete recovery is often difficult to achieve. We report a case of recurrent bilateral infection in a 35-year-old woman who had completed treatment for NTM. Although various infectious diseases show similar clinical conditions and imaging findings, recurrences should raise suspicion of NTM infection, and this possibility should be considered in differential diagnoses. PMID:25320630

  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. Surgical approach to isolated bilateral orbital floor fractures.

    PubMed

    Giarda, M; Tavolaccini, A; Arcuri, F; Brucoli, M; Benech, A

    2015-10-01

    Isolated bilateral orbital floor fractures are uncommon and are rarely described in the scientific literature. They are usually seen in association with naso-ethmoidal fractures, zygomatic fractures, or fractures of the middle third. We report our experience in the management of a patient presenting bilateral isolated orbital floor fracture. The difficulties in management of these fractures are due to the lack of an uninjured contralateral side for intraoperative comparison. PMID:26824920

  14. Bilateral eyelid agenesis repair in a captive Texas cougar.

    PubMed

    Cutler, Tim J

    2002-09-01

    Bilateral eyelid agenesis was presented with multiple ocular anomalies in a captive Texas cougar (Felis concolor). Corneal exposure resulted in substantial keratoconjunctivitis and blepharospasm. Bilateral inferopapillary fundic colobomas, persistent pupillary membranes, and an atypical iris coloboma OD were present. Surgical repair with a rotational pedicle flap resulted in functionally effective eyelids. Trichiasis was later treated with cryotherapy. Eyelid agenesis is reported infrequently in domestic cats and among large felids has only been reported in the snow leopard. PMID:12236863

  15. 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

  16. 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

  17. Bilateral Traumatic Globe Luxation with Optic Nerve Transection

    PubMed Central

    Tok, Levent; Tok, Ozlem Yalcin; Argun, Tugba Cakmak; Yilmaz, Omer; Gunes, Alime; Unlu, Elif Nisa; Sezer, Sezgin; Ibisoglu, Seda; Argun, Mehmet

    2014-01-01

    Purpose The purpose of this study was to document clinical findings and management of a patient with bilateral globe luxation and optic nerve transection. Materials and Methods A 25-year-old female patient was admitted to the emergency department with bilateral traumatic globe luxation following a motor vehicle accident. Results Visual acuity testing showed no light perception. The right pupil was dilated and bilaterally did not react to light. The globes were bilaterally intact. A computed tomography scan revealed Le Fort type II fractures, bilateral optic nerve transection and disruption of all extraocular muscles. The globes of the patient were bilaterally reduced into the orbit. However, the patient developed phthisis bulbi in the right eye at month 3. Conclusion Globe luxation presents a dramatic clinical picture, and may lead to the development of severe complications due to the concomitance of complete optic nerve dissection and multiple traumas. Even if the luxated globe is repositioned into the orbit, there is still an increased risk of the development of phthisis due to ischemia. PMID:25606034

  18. 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

  19. 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

  20. Klinefelter Syndrome

    MedlinePlus

    ... have varying degrees of cognitive, social, behavioral, and learning difficulties. Adults with Klinefelter syndrome may also experience primary hypogonadism (decreased testosterone production), small testes, enlarged ...

  1. Proteus Syndrome Foundation

    MedlinePlus

    ... Gift Stock Gift Sunshine Society Contact Privacy Policy Proteus Syndrome Foundation The Proteus Syndrome Foundation , a 501c3 ... 1 Trial with ARQ 092 in Proteus Syndrome Proteus Syndrome Patient Registry The Proteus Syndrome Foundation Contact ...

  2. 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

  3. 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

  4. Laparoscopic removal of Müllerian structures and orchiopexy for persistent Müllerian duct syndrome.

    PubMed

    Shirasaki, Yoshinori; Nagai, Atsushi; Nasu, Yasutomo; Iguchi, Hiroki; Kumon, Hiromi

    2003-12-01

    A 1-year-old boy presented for evaluation of bilateral undescended testes. Diagnostic laparoscopy was conducted, and uterine tissue with bilateral gonads was detected in the rectovesical fossa. Chromosomal analysis revealed a 46,XY karyotype. Persistent müllerian duct syndrome was diagnosed, and laparoscopic surgery was performed to treat this condition. The uterus was incised at a distal site and withdrawn through the port. The bilateral testes were fixed in the scrotum. The patient was discharged 3 days later. We believe that the laparoscopic approach is a valid, alternative choice to traditional surgery for resolution of this condition, permitting minimally invasive surgery. PMID:14665371

  5. 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

  6. 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

  7. 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

  8. 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

  9. 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

  10. An unusual case of sub-condylar bilateral fracture and bilateral post-traumatic temporomandibular ankylosis.

    PubMed

    Matteini, C; Belli, E

    2001-01-01

    A case of bilateral sub-condylar fracture with wide stump dislocation associated with a central facial trauma, fracture-intrusion of the rhino-orbital-maxillary complex and a parasymphyseal mandibular fracture, is reported. After surgery and inter-maxillary fixation an unusual temporo-mandibular ankylosis developed. Maximum mouth opening, lateral and protrusive movements were severely limited. Surgical treatment of ankylosis was requested and performed. The originality of this case lies in the atypical lateral dislocation of condylar neck fractured stumps to the zygomatic arches and in the later appearance of ankylosis between the glenoid fossa, zygomatic arch, condylar neck stump, and the condylar process displaced anteromedially. The ankylosed blocks were resected, displaced condyles were also removed due to the strong adhesion with the ankylotic tissue and the lack of any anatomical continuity or connection with the glenoid fossa. Functional therapy allowed the resolution of the functional limitation. PMID:11723434

  11. 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

  12. 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

  13. Alagille Syndrome

    MedlinePlus

    ... person's discomfort. Scientists have not yet found a way to prevent Alagille syndrome. Caregivers and parents of children with Alagille syndrome should try to maximize their children's potential for growth through good eating, diet, and nutrition. [ Top ] Clinical Trials The National ...

  14. Tourette Syndrome

    MedlinePlus

    If you have Tourette syndrome, you make unusual movements or sounds, called tics. You have little or no control over them. Common tics are ... words, spin, or, rarely, blurt out swear words. Tourette syndrome is a disorder of the nervous system. It ...

  15. Tourette Syndrome.

    ERIC Educational Resources Information Center

    Look, Kathy

    Tourette Syndrome has a history of being misdiagnosed or undiagnosed due to its unusual and complex symptoms. This paper describes: the symptoms of Tourette Syndrome; its etiology; age of onset; therapeutic methods, such as drug therapy, psychotherapy, diet control, and hypnosis; educational implications; and employment prospects. Several…

  16. 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

  17. Marfan Syndrome

    MedlinePlus

    ... thin, and loose jointed. Most people with Marfan syndrome have heart and blood vessel problems, such as a weakness in the aorta or heart valves that leak. They may also have problems with ... diagnose Marfan syndrome. Your doctor may use your medical history, family ...

  18. 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 ...

  19. Postthrombotic Syndrome

    MedlinePlus

    ... in the prevention and management of postthrombotic syndrome. Ann Pharmacother . 2009;43:1824–1835. Reproduced from Vazquez ... in the prevention and management of postthrombotic syndrome. Ann Pharmacother . 2009 ; 43 : 1824 –1835. OpenURL CrossRef Medline ↵ ...

  20. 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.