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Congenital bilateralperisylviansyndrome (CBPS), described by Kuzniecky et al.  in 1993, consists of developmental delay from childhood, mild to moderate mental retardation, particularly manifest as delays of language acquisition, tonic or atonic seizures and atypical absence seizures beginning in childhood, pseudobulbar palsy, manifest as dysarthria and a poor gag reflex, and bilateralperisylvian malformations on imaging. The most
Background: Congenital bilateralperisylviansyndrome (CBPS) is characterised by bilateralperisylvian polymicrogyria and suprabulbar paresis. Mild tetraparesis, cognitive impairment, and epilepsy are frequently associated. Sensory deficits are surprisingly rare, even though polymicrogyria often extends to auditory and sensorimotor cortex. Objectives: To study the sensorimotor and auditory cortex function and location in CBPS patients. Methods: We mapped the sensory and motor cortex function onto brain magnetic resonance images in six CBPS patients and seven control subjects using sources of somatosensory and auditory evoked magnetic fields, and of rhythmic magnetoencephalographic (MEG) activity phase-locked to surface electromyogram (EMG) during voluntary hand muscle contraction. Results: MEG-EMG coherence in CBPS patients varied from normal (if normal central sulcus anatomy) to absent, and could occur at abnormally low frequency. Coherent MEG activity was generated at the central sulcus or in the polymicrogyric frontoparietal cortex. Somatosensory and auditory evoked responses were preserved and also originated within the polymicrogyric cortex, but the locations of some source components could be grossly shifted. Conclusion: Plastic changes of sensory and motor cortex location suggest disturbed cortex organisation in CBPS patients. Because the polymicrogyric cortex of CBPS patients may embed normal functions in unexpected locations, functional mapping should be considered before brain surgery.
A 9-year-old male with congenital bilateralperisylviansyndrome is described. He had pseudobulbar palsy, mental retardation, and intractable epilepsy. Computed tomography and magnetic resonance images of the brain demonstrated bilateralperisylvian malformations and a diffuse pachygyric appearance. At 8 years of age, he had episodes of excessive drooling, fluctuating impairment of consciousness, unsteady sitting, and frequent head drop that lasted
We describe a rare case of multiple intestinal atresias, congenital bilateralperisylvian polymicrogyria, and chronic pulmonary hypertension in a surviving monochorionic twin with co-twin demise. This constellation of congenital anomalies represents a multiple vascular disruption syndrome due to intrauterine vascular compromise in the setting of possible twin-to-twin transfusion syndrome. PMID:23084212
We present the neuropsychological and linguistic follow-up of a girl with bilateralperisylvian polymicrogyria during 4 years\\u000a of gestural and verbal speech therapy. Some researchers have suggested that children with bilateralperisylvian polymicrogyria\\u000a mentally fail to reach the syntactic phase and do not acquire a productive morphology. This patient achieved a mean length\\u000a of utterance in signs\\/gestures of 3.4, a syntactic
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.
Milanlioglu, Aysel; Ayd?n, Mehmet Nuri; Gokgul, Alper; Hamamc?, Mehmet; Erkuzu, Mehmet Atilla; Tombul, Temel
Congenital lacrimal fistulae are rare in Down syndrome and bilateral presentation is very unusual. It can be associated with nasolacrimal duct obstruction. We report a 3-year-old female with Down syndrome who presented with watering and discharge from both eyes and bilateral fistulous openings present inferonasal to the medial canthus. Upon examination, the lacrimal sac regurgitation test was positive on both sides. Our case report documents a distinctive case of bilateral congenital lacrimal fistulae in association with Down syndrome. It was managed successfully by primary fistulectomy and nasolacrimal duct probing.
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
Miller, Brooke; Khalifa, Yousuf; Feldon, Steven E; Friedman, Deborah I
Early anatomically based models of language consisted of an arcuate tract connecting Broca's speech and Wernicke's comprehension centers; a lesion of the tract resulted in conduction aphasia. However, the heterogeneous clinical pre- sentations of conduction aphasia suggest a greater complexity of perisylvian anatomical connections than allowed for in the classical anatomical model. This article re-explores perisylvian language connectivity using in
Eagle’s syndrome represents a symptomatic styloid process elongation or calcification of stylohyoid or stylomandibular ligament. The symptoms include the throat pain radiating to ipsilateral ear or foreign body sensation in the pharynx causing odynophagia and dysphagia. It is commonly unilateral and bilateral cases are rare. We report a case of bilateral elongation of styloid processes treated surgically by transoral approach.
Baharudin, Abdullah; Rohaida, Ibrahim; Khairudin, Abdullah
A case of congenital nephrotic syndrome with diffuse mesangial sclerosis and bilateral cataract without other ocular anomalies is presented. This association, to our knowledge, has not been reported before. PMID:8971891
Approximately 15% of patients with Sturge-Weber syndrome have bilateral intracranial involvement and the prognosis of these patients is believed to be particularly unfavorable. We reviewed the clinical and neuroimaging features of patients with Sturge-Weber syndrome and bilateral intracranial involvement. Seizure variables, presence of hemiparesis and degree of developmental impairment at last follow-up were compared to imaging abnormalities. Out of 110 Sturge-Weber syndrome patients studied, 14 had bilateral brain involvement, which always showed an asymmetric pattern on glucose metabolism positron emission tomography. Although most patients had frequent seizures initially, associated with frontal hypometabolism on positron emission tomography, six patients (43%) had achieved good seizure-control during follow-up. Bilateral frontal hypometabolism was associated with severe developmental impairment; two children with bitemporal hypometabolism showed autistic features. Hemiparesis was associated with superior frontal (motor cortex) hypometabolism. Three patients had resective surgery, resulting in improved seizure control and/or developmental outcome. The severity of neurological complications and clinical course depend highly on the extent of cortical dysfunction in bilateral Sturge-Weber syndrome; bilateral frontal and temporal hypometabolism is associated with poor developmental outcome. Nevertheless, good seizure control and only mild/moderate developmental impairment can be achieved in about half of bilateral Sturge-Weber syndrome patients, with or without resective surgery.
Alkonyi, Balint; Chugani, Harry T.; Karia, Samir; Behen, Michael E.; Juhasz, Csaba
We report a 14-year-old girl with Alport’s syndrome who developed bilateral exudative retinal detachment in the macula. With the development of chronic renal failure, bilateral serous retinal detachment appeared which had the retinal flecks characteristic of Alport’s syndrome. The serous detachment was resolved and vision recovered following intensive hemodialysis. As far as we know this is the first case with
Anterior sacral meningoceles (ASM) in Marfan syndrome are rare. They may cause constipation, urinary frequency, dysmenorrhea, and low back pain or numbness. This report describes bilateral ASM at the level of S1, S2 and S3 in a woman with Marfan syndrome who was admitted to the gynaecology department for evaluation of left lower abdominal pain. The magnetic resonance appearance of
R. J. Scheck; T. Schramm; K.-P. Gloning; T. Vogl; E. Ostermayer
The autors describe a case of a bilateral shoulder-hand syndrome. A Hashimoto's thyroiditis bound hypothyroidism was retained as promoting factor. Rheumatic manifestations amended slowly with a treatment of corticosteroids associated to thyroid hormones replacement. Eighteen months after the onset of the rheumatic complaints, a colorectal cancer was also diagnosed. The respective role of hypothyroidism and cancer in the emergence of this severe shoulder-hand syndrome is discussed. PMID:15468693
Local vascular and traumatic involvement are well-recognized causes of anterior tibial compartment syndrome (ATCS) terminating as myoneural ischaemia. However, in a large number of patients the cause of ATCS remains unidentified. We document the occurrence of bilateral ATCS in a patient of hypothyroidism, to our knowledge a previously unrecorded association.
We describe an 8-year-old boy with pseudobulbar palsy and speech disturbance associated with fetal distress, mild birth asphyxia and probably intracranial hemorrhage. There is a remarkable dissociation between orofacial voluntary movements and emotional expression. MRI revealed cortical atrophy and subcortical gliosis of both opercula. The clinical and neuroradiological features correspond to bilateral opercular syndrome. We assume that a reduction of
Hasegawa S, Aoyama T, Kakinoki R, Toguchida J, Nakamura T. Bilateral phlegmasia dolens associated with Trousseau's syndrome: a case report.Phlegmasia dolens is a rare disorder caused by massive venous thrombosis. The clinical condition is subclassified as phlegmasia cerulea dolens (PCD) and phlegmasia alba dolens. The 2 forms differ in the venous area affected and remaining blood flow, causing arterial involvement
An autopsy case of amniotic band syndrome with bilateral epibulbar choristoma is described. The left eye reveals a complex choristoma and the right eye a dermis-like choristoma. Both choristomatous lesions included lenticular tissue suggesting that rupture of the amnion, which is the initial event of amniotic band syndrome, might have occurred at about the fourth week of gestation. Since the other systemic manifestations of amniotic band syndrome are considered to be compression deformities of the fetus caused by oligohydramnios or amniotic band, the occurrence of epibulbar choristomas in both eyes in this case suggests that a compression mechanism may play a role in the pathogenesis of epibulbar choristoma. Images
Murata, T; Hashimoto, S; Ishibashi, T; Inomata, H; Sueishi, K
The occurrence of anomalous muscles in the hand is rare, and therefore the clinician frequently does not account for their\\u000a existence when reaching the diagnosis and concluding the cause of carpal tunnel syndrome. This case report describes the highly\\u000a unusual case of the occurrence of bilateral flexor digitorum superficialis muscles in a recreational climber who developed\\u000a CTS following his intensive
Frank Unglaub; Maya Barbara Wolf; Adrian Dragu; Raymund E. Horch
Algodystrophy syndrome is characterized by pain, vasomotor disorders and/or trophic skin changes, without laboratory signs of inflammation. Among the etiological factors of this syndrome (trauma, cerebrovascular accident, diabetes mellitus...), iatrogenic ones including phenobarbital can be observed. We report a case of phenobarbital induced recurrent bilateral shoulder-hand syndrome in a 67 years old women with hypertension, diabetes, and history of epileptic crisis following ischemic cerebrovascular accidents. The patient recovered after barbiturate withdrawal and treatment with calcitonin. It's necessary to diagnose promptly shoulder-hand syndrome of iatrogenic cause in order to withdraw definitively the responsible drug, to start an effective therapy, at this stage, on pain and bone demineralization and to prevent severe sequels. PMID:17722788
Hamza, S Imène; Hassen, Zohra; Loueslati, M Hédi; Toghourti, M Néjib; Belkahia, Chelbi; Hamza, Mohsen
We describe the case of a patient from the emergency ophthalmic clinic who presented with sudden, bilateral visual loss, headache and dizziness. The magnetic resonance imaging (MRI) showed bilateral parieto-occipital vasogenic edema of the white and grey brain matter consistent with the diagnosis of posterior reversible encephalopathy syndrome (PRES). This is a rare cause of sudden bilateral visual loss which describes a condition with bilateral edema of primarily the white but also the grey matter. The edema is usually detectable with MRI but not always with the less sensitive computed tomography (CT). Further clinical signs may be headache, seizure, nausea, character changes and reduced consciousness. Arterial hypertension, drugs inducing hypertension or drug side effects may cause PRES but sometimes the reason remains unknown. In most cases the symptoms resolve simultaneously with the edema but may also lead to severe complications. In suspected cases of PRES the blood pressure should be measured and a MRI performed, followed by intensive care and treatment of the hypertension, other symptoms and complications. PMID:22736265
Bonsel, K S; Oberacher-Velten, I; Hufendiek, K; Jägle, H
We present a 38-year-old woman with bilateral serous retinal detachments and bilateral panuveitis on fundoscopic exam. CT of the orbits and MRI scans revealed bilateral ocular choroidal thickening and bilateral retinal detachments; however, no other CNS or meningeal lesion was detected. This patient met the clinical criteria for Vogt–Koyanagi–Harada (VKH) Syndrome. Review of VKH syndrome as well as the radiographic
Blake E. McGehee; Gregory Chaljub; Rajeev K. Shah; Ayetkin Oto; Alwin C. Camacho; Rosa A. Tang
The Duane syndrome is a strabismus syndrome which is characterized by congenital non-progressive horizontal ophthalmoplegia which primarily affects the abducens nerve. Approximately 70% of the individuals with the Duane syndrome have an isolated disease. We have described here, a case of bilateral Duane syndrome with associated anamolies. PMID:23205369
The Duane syndrome is a strabismus syndrome which is characterized by congenital non-progressive horizontal ophthalmoplegia which primarily affects the abducens nerve. Approximately 70% of the individuals with the Duane syndrome have an isolated disease. We have described here, a case of bilateral Duane syndrome with associated anamolies.
We describe the occurrence of bilateral iridocorneal endothelial (ICE) syndrome with glaucoma in a young girl with Down's syndrome. A 16-year-old girl with Down's syndrome was found to have secondary glaucoma in the right eye with features of progressive iris atrophy in both eyes. She was uncontrolled on maximum tolerable medical therapy for glaucoma. She underwent an uneventful trabeculectomy with mitomycin-C in her right eye. Scanning electron microscopy of the trabecular meshwork obtained in this case is described. PMID:19075416
Background: The outcome of bilateral adrenalectomy (BADx) in patients with Cushing's syndrome (CS) is not well characterized. Methods: A literature search was performed with the search terms "bilateral adrenalectomy" or "total adrenalectomy" and "Cushing's" or "Cushing." Immediate and long-term outcomes after BADx in CS were analyzed using descriptive statistics (median [range]). Results: From 549 screened publications, 37 studies met inclusion criteria (1320 patients, 82% having Cushing's disease [CD], 13% having ectopic CS, and 5% having primary adrenal hyperplasia). Surgical morbidity and mortality of BADx (23 studies, 739 patients) were 18% (6-31) and 3% (0-15), respectively. In patients with CD, surgical mortality was below 1%. Although residual cortisol secretion due to accessory adrenal tissue or adrenal remnants was found in 3-34% (5 studies, 236 patients), less than 2% had a relapse of CS. Symptoms of hypercortisolism (eg, hypertension, obesity, or depression) improved in the majority of the patients after BADx (7 studies, 195 patients). The number of adrenal crises per 100 patient-years was 9.3 (6 studies, 203 patients). Nelson's syndrome occurred in 21% (0-47) of the patients (24 studies, 768 patients). Mortality (23 studies, 690 patients) was 17% (0-88) at a follow-up of 41 months (14-294). Remarkably, 46% of the patients died in the first year after surgery. The median ages at death were 62 years (CD) and 53 years (ectopic CS). Conclusion: BADx is relatively safe and provides adequate success. Excess mortality within the first year after surgery suggests that intensive clinical care for patients after BADx is warranted. PMID:23956347
Ritzel, Katrin; Beuschlein, Felix; Mickisch, Anne; Osswald, Andrea; Schneider, Harald J; Schopohl, Jochen; Reincke, Martin
Background Thoracic Outlet Syndrome (TOS) involves compression of the brachial plexus, subclavius artery and vein. Many studies discuss efficacy of surgery and few discuss conservative treatment. It is unknown what specific forms of conservative treatment are best. Objective Describe conservative management for TOS using unique exercises. Case Description A collegiate football player reported numbness/tingling down his right arm after a right brachial plexus stretch injury. Seven months later, he was diagnosed with recurrent cervical traction neuropraxia. Two months later, he reported bilateral symptoms and was diagnosed with functional TOS. The athlete began shoulder strengthening (deltoid, middle trapezius, rhomboids, pectoralis major, latissimus dorsi, biceps, upper trapezius and rotator cuff) and stretching (pectoralis, scalene and upper trapezius) which failed to resolve his symptoms after four weeks. Surgical resection of bilateral first ribs and quitting football was recommended by four physicians. Unique therapeutic exercises developed by the Postural Restoration Institute™ were used to optimize respiration/posture via muscle activation and inhibition. After six weeks, the athlete was asymptomatic and returned to football but still experienced paresthesia with contact. Additional exercises were prescribed and remaining symptoms were abolished. Outcomes The Northwick Park Neck Pain Questionnaire was 55.5% at initial and 0% at four weeks and discharge. Discussion Athlete did not demonstrate relief of symptoms from shoulder stretching and strengthening. Intervention designed to optimize respiration/posture by repositioning the pelvis/trunk via specific muscle inhibition and activation resulted in abolishing the athlete's symptoms. Management that aims to optimize respiration via muscle inhibition, activation, and repositioning warrants further research.
INTRODUCTION Bilateral acute compartment syndrome of the legs is a rare presentation requiring emergent surgical intervention. PRESENTATION OF CASE We report the case of 41-year-old woman who presented with acute bilateral compartment syndrome of the legs, complicated by rhabdomyolysis and acute renal failure. DISCUSSION There are very few previously reported cases of bilateral compartment syndrome of the legs. In the present case, despite any clear causative factor, we suggest that the aetiology is related to inadvertent posture during sleep. CONCLUSION The diagnosis of acute bilateral compartment syndrome of the legs requires a high index of suspicion, particularly in the absence of obvious aetiology. A successful outcome can be achieved with early diagnosis, prompt surgical intervention and a multidisciplinary approach.
Khan, T.; Lee, G.H.; Alvand, A.; Mahaluxmivala, J.S.
Microadenomectomy via a transsphenoidal approach is today's treatment of choice to achieve normal cortisol blood levels in patients with ACTH-secreting pituitary tumors. Should neurosurgery fail, bilateral adrenalectomy is recommended. Today the endoscopic, transabdominal or retroperitoneal adrenalectomy is regarded as the gold standard for the operation of endocrine-active adrenal tumors. Therefore, in principle, the question of the indication for the endoscopic operation no longer arises but only the question of the technical feasibility in individual cases. We report on a patient with a pituitary-dependent Cushing's syndrome after a twice-repeated unsuccessful transsphenoidal adenomectomy and with additional polycystic kidney and liver disease, who underwent bilateral retroperitoneoscopic adrenalectomy. Despite the massive enlargement of both kidneys, it was possible to safely perform a bilateral retroperitoneoscopic adrenalectomy. In the case of marked bilateral cystic kidneys, a bilateral retroperitoneoscopic adrenalectomy in Cushing's syndrome is technical possible and a safe and effective method of treatment. PMID:15259585
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
Alireza Gharabaghi; Frank Kunath; Michael Erb; Ralf Saur; Stefan Heckl; Marcos Tatagiba; Wolfgang Grodd; Hans-Otto Karnath
The presence of supernumerary teeth, also known as hyperdontia, is the condition of having teeth in addition to the regular number of teeth. The occurrence of multiple supernumerary teeth is often found in association with syndromes such as Gardner syndrome, Anderson-Fabry disease, Ellis-van Creveld syndrome, Ehlers-Danlos syndrome, incontinentia pigmenti, and Tricho-rhino-phalangeal syndrome. Only a few examples of nonsyndromic multiple supernumerary teeth have been reported in literature. In this report, we present the unusual case and 9-year follow up of a non-syndrome female patient with bilateral supernumerary teeth that occurred with an interval of several years.
. Rubinstein-Taybi syndrome is a syndrome of mental retardation associated with digital changes, consisting mainly of broad\\u000a thumbs and large toes. This paper deals with pedal changes in a patient with this syndrome in whom bilateral, large, extra\\u000a tarsal bones occurred between the medial cuneiform and intermediate cuneiform bones. They articulated with these bones, as\\u000a well as with the first
The human HOXA1 mutation syndromes commonly present with abnormalities of the inner ear and ICAs. Previous cases describe varying degrees of hypoplasia or aplasia of the affected structures, often with asymmetric involvement. We present imaging findings documenting complete absence of the ICAs bilaterally with bilateral CLA, which, to our knowledge, has not been previously reported. PMID:20075099
Higley, M J; Walkiewicz, T W; Miller, J H; Curran, J G; Towbin, R B
Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by sudden-onset thunderclap headache and focal neurologic deficits. Once thought to be a rare syndrome, more advanced non-invasive imaging has led to an increase in RCVS diagnosis. Unilateral vertebral artery dissection has been described in fewer than 40% of cases of RCVS. Bilateral vertebral artery dissection has rarely been reported. We describe the case of a patient with RCVS and bilateral vertebral artery dissection presenting with an intramedullary infarct treated successfully with medical management and careful close follow-up. This rare coexistence should be recognized as the treatment differs. PMID:23354867
PURPOSE: To alert ophthalmologists to POEMS syndrome as an unusual cause of optic disk swelling.METHOD: Case report. A 25-year-old white woman developed scotomata and optic disk swelling, and systemic signs and symptoms of POEMS syndrome.RESULTS: Bone marrow biopsy disclosed multiple myeloma. Despite treatment with corticosteroids, radiation, and marrow transplantation, the patient died from multi-organ failure.CONCLUSION: Because POEMS syndrome typically affects
A case of the Joubert Syndrome is reported. This syndrome, of which 11 cases have hitherto been described, is characterized by aplasia of the cerebellar vermis in addition to episodic hyperpnoea, abnormal eye movements, rhythmic protrusion of the tongue, ataxia, and mental retardation.
Balint syndrome after bilateral parietal damage involves a severe disturbance of space representation including impaired oculomotor behaviour, optic ataxia, and simultanagnosia. Binding of object features into a unique spatial representation can also be impaired. We report a patient with bilateral parietal lesions and Balint syndrome, showing severe spatial deficits in several visual tasks predominantly affecting the left hemispace. In particular,
Nathalie Valenza; M. M. Murray; Radek Ptak; Patrik Vuilleumier
Ocular ischaemic syndrome (OIS) is a relatively uncommon condition. Simultaneous bilateral involvement is even less common and has been reported in only 22% of all cases of OIS. It has variable clinical presentations, of which visual loss and ocular pain are the most common. It is believed to occur when there is a 90% or greater carotid artery obstruction. This syndrome is often associated with a number of systemic diseases including diabetes mellitus, hypertension, coronary artery disease, and cerebrovascular disease. Only occasionally has it been described as a complication of rhinocerebral mucormycosis. We report an unusual case of bilateral OIS secondary to bilateral internal carotid artery thrombosis as a complication of invasive rhinocerebral mucormycosis. In addition, a review of clinical presentation, diagnostic work-up and treatment options for OIS is provided.
|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…
Migliaccio, Americo A.; Halmagyi, G. Michael; McGarvie, Leigh A.; Cremer, Phillip D.
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
Mansour, Ahmad M; Jaroudi, Mahmoud O; Medawar, Walid A; Tabbarah, Zuhayr A
Background: Weaver syndrome is a congenital paediatric syndrome characterized by mental, respiratory and musculoskeletal manifestations. The coexisting deformities of the skull, the face, fingers and toes are typical. We report a case of a girl with Weaver syndrome associated with rare bilateral congenital dislocation of the hips associated with congenital hypoplastic talus and subtalar dislocation of her ankle joint. Case Report: A 3-year old girl was admitted in our department with typical manifestations of Weaver syndrome, associated with congenital dislocation of bilateral hips, hypoplastic talus and subtalar dislocation of her right ankle. She was in pain while standing upright and incapable of independent walking. Both hips were treated operatively with open reduction and bilateral iliac osteotomy. Two years afterwards she had an open reduction of her talus and extraarticular arthrodesis of her subtalar joint in her right ankle. Six years postoperatively after the hip operations and four years after the ankle operation the girl is ambulant with a painless independent and unaided walking with a mild limp and full range of movements in all the operated joints. Conclusions: We suggest that children with Weaver syndrome and disabling musculosceletal deformities, particularly affecting their ability to stand up and walk should be treated early, before bone maturity, in order to achieve the best potential musculoskeletal as well as developmental outcome.
Background : Tessier 30 cleft is rare and sparsely reported in the literature. A unique case of an infant with a Tessier 30 cleft, bilateral cleft lip and palate, and other anomalies is presented. In addition to craniofacial anomalies, he had cardiac, gastrointestinal, and genitourinal defects. The constellation of these findings suggests the possibility of a new clinical syndrome. We present these findings and postoperative results following surgical treatment. Patient and Methods : A 37-week gestational male infant with multiple congenital anomalies is presented. Findings on clinical exam were notable for Tessier 30 median mandibular cleft, bilateral cleft of the lip and palate, and bifid tongue. Further workup revealed levocardia, perimembranous moderate-to-large ventricular septal defect, patent foramen ovale, double outlet right ventricle, intestinal malrotation, and bilateral undescended testicles. There were no extremity anomalies, and cytogenetic studies for 22q deletion were negative. Results : The preoperative, intraoperative, and postoperative findings and images are discussed. Conclusion : We present a unique case of a child with a Tessier 30 cleft associated with bilateral cleft lip and palate in the absence of intraoral masses or limb anomalies. Previous reports of median facial clefts have occurred either in the presence of intraoral hamartomas, suggesting the palatal defects are a result of sequence abnormalities, or in association with extremity findings consistent with the spectrum of orofaciodigital syndrome. The case we present is distinct and may represent a new clinical syndrome. PMID:22034959
Adams-Oliver syndrome (AOS) (MIM 100300) was first described in 1945 as a condition of terminal transverse limb defects and aplasia cutis congenita. Since then, its clinical features have been found to be highly variable and include cardiac defects, abdominal wall defects, vascular malformations, brain abnormalities, and ocular anomalies. We report the case of a 3-year-old girl with AOS who was also found to have bilateral anterior polar cataracts and pseudopapilledema secondary to optic disk drusen. To the best of our knowledge, this is the first case of bilateral anterior polar cataracts and pseudopapilledema secondary to optic disk drusen to be reported in association with the AOS. PMID:21641251
Lascaratos, Gerassimos; Lam, Wayne W; Newman, William D; MacRae, Mary
Locked-in syndrome (LIS) usually occurs as a result of pontine lesions and has been classified into various categories on the basis of neurologic conditions, of which transient total mesencephalic LIS is extremely rare. A 53-year-old man presented with bilateral ptosis followed by a total locked-in state. In the clinical course, the patient successfully recovered with only left slight hemiparesis and skew deviation remaining. Magnetic resonance imaging revealed multiple ischemic lesions caused by thrombosis at the top of basilar artery, including the bilateral cerebral peduncles, tegmentum of the midbrain, and the right cerebellar hemisphere. Antecedent bilateral ptosis before the locked-in state may be related to ischemia in the central caudal nucleus of the oculomotor nuclei. We should pay attention to this easily missed condition during the treatment of ischemic stroke involving the basilar artery. PMID:22177934
Tolosa-Hunt syndrome (THS) is a very rare, relapsing, and remitting painful ophthalmoplegia caused by nonspecific granulomatous inflammation in the cavernous sinus. To our knowledge, bilateral complete, simultaneous palsies of all 3 cranial nerves associated with extraocular movement have not been reported. We describe the first such patient with bilateral THS that responded quickly to corticosteroid therapy. A 54-year-old man presented with a periorbital and frontal headache with acute bilateral severe blepharoptosis and fixed eyes, which dramatically responded to corticosteroid therapy. He had diabetes mellitus type II. Brain MRI showed granulomatous inflammation in both cavernous sinuses and thickening of the surrounding dura mater of the cranial base, suggesting the coexistence of focal hypertrophic cranial pachymeningitis. Our experience indicates that steroid therapy with strict control of blood sugar should be considered in patients with THS complicated by diabetes. MRI is a valuable tool for serially monitoring the response of lesions to treatment in THS. PMID:19453827
Background Ocular ischemic syndrome is a devastating eye disease caused by severe carotid artery stenosis. The reduction of blood flow\\u000a produced by bilateral common carotid artery occlusion (BCCAO) of rats for 7 days induces events related to gliosis with no\\u000a evident histological damage. However, retinal degeneration and cellular death occur after 90 days of BCCAO. Our purpose has\\u000a been to investigate
Daniel Lavinsky; Nice Sarmento Arterni; Matilde Achaval; Carlos Alexandre Netto
Bilateral lung transplantation was performed in a 52-year old man with end-stage Kartagener's syndrome. A postimplantation right lower lobectomy was required for volume reduction and dextrocardia. A bronchial fistula developed with an intractable colonized residual pleural cavity. Closure was obtained successfully with multiple-stage procedures including decortication, muscle flap and an open-window thoracostomy without modification of the immunosuppressive protocol. PMID:23575755
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
PHACE syndrome is characterized by posterior fossa malformations (P), large facial hemangiomas (H), arterial anomalies (A), cardiac anomalies or coarctation of aorta (C), and eye anomalies (E) and has striking female predominance. Endocrine abnormalities have recently been described in these patients, involving the thyroid and pituitary glands. We report the case of a 2-year-old girl with the clinical features of PHACE with absence of bilateral internal carotid arteries and isolated growth hormone deficiency. PMID:22010790
Loeys-Dietz syndrome is characterised by vascular aneurysms, hypertelorism, and a bifid uvula. We report on an 11-year-old boy with Loeys-Dietz syndrome who presented with bilateral radial head dislocations and severe osteopaenia with changes of avascular necrosis in both hips causing an out-toeing, wide gait. Considering the poor prognosis for elbow movement and possible radial head dysplasia, surgical reduction of the radial heads was deferred. A subtrochanteric de-rotation osteotomy of the left hip was performed to improve the gait. PMID:24014798
An 18-year-old man presented with severe nephrotic syndrome due to focal segmental glomerulosclerosis. His disease failed to remit with corticosteroids, calcineurin inhibitors, mycophenolate and rituximab. As his disease progressed with time, his anasarca became more resistant to high-dose combination diuretics and he developed multiple life-threatening bacterial infections. He was subjected to bilateral renal artery embolization with 99.8% alcohol to ablate his kidneys. Subsequently, the patient was maintained on hemodialysis and had normal serum albumin and did not have further infections. The procedure itself was simple and well tolerated, with only a minor post-embolization syndrome. PMID:23168861
The authors present an unusual case of bilateral medial foot compartment syndrome in a healthy woman after a low-intensity aerobics exercise class. The majority of compartment syndrome cases have occurred after trauma, such as combat crush injuries and motor vehicle accidents. We wish to call attention to a rare situation in which compartment syndrome occurs in a healthy young adult after low-intensity exercise and highlight the necessity of a high clinical suspicion and a low threshold for fasciotomies to prevent irreversible muscle damage as a result of extremely high pressures. There is a paucity of cases on the clinical management and follow-up of this rare occurrence of compartment syndrome. PMID:22621857
Lam, Suet Kam; McAlister, Jeffrey; Oliver, Noah; Pontell, David
The patient is a 72-year-old man who had tonsillitis and underwent incision on March 6, 1998. He complained headache and nausea from March 10 and double vision from March 12. He was admitted to our hospital on March 14. Neurological examination on March 18 revealed bilateral internuclear ophthalmoplegia with exotropia (WEBINO), bilateral ptosis and disturbance of convergence. Lumbar puncture revealed moderately elevated protein (46 mg/dl) with normal pressure and cells. The T2-weighted images of head MRI showed multiple high intensity lesions in anterior commissure and right pons. After he was treated with methylprednisolone, his headache and ptosis disappeared. The lesion in anterior commissure on MRI disappeared also. But WEBINO remained at the time of discharge. WEBINO syndrome is caused by lesion of pons and midbrain. The most common cause of bilateral internuclear ophthalmoplegia is multiple sclerosis in the European and American countries, but the frequency of vascular origin is higher in Japan than in the European and American countries. All the cases of WEBINO syndrome reported in Japan are vascular origin. This is the first case of WEBINO syndrome not of vascular origin. PMID:10423755
Shikata, E; Uehara, N; Ogawa, K; Oishi, M; Takasu, T
We report a single case study of concordant bilateral Duane's Retraction Syndrome (DRS) (type 1) in female monozygotic (MZ) twins aged 47 years. The twin pair were recruited through the Australian Twin Registry as part of a twin study on myopia. This twin pair were full term and had a similar birth weight: 2.27 kg and 1.81 kg in twin 1 and twin 2, respectively. There was no report of any other childhood medical conditions in either twin. Both twins had an equal amount of restriction in right and left abduction. Narrowing of the palpebral fissures and globe retraction in right and left adduction was also observed in both twins. To our knowledge this is the first case to report concordant bilateral DRS (type 1) in female MZ twins. The concordance for the presence of DRS and associated clinical signs observed in this MZ twin pair supports a genetic origin to DRS. PMID:16872351
Dirani, Mohamed; Chamberlain, Matthew; Garoufalis, Pam; Chen, Christine; Guymer, Robyn H; Baird, Paul N
Supernumerary teeth or hyperdontia are defined as the teeth that exceeds the normal dental formula, regardless of their location and morphology. A supernumerary tooth is a developmental anomaly and it has been argued to arise from multiple aetiologies. Supernumerary teeth with orthodox shapes and sizes, that resemble the normal dentition are called ‘supplemental teeth’. The supplemental teeth are often overlooked because of their normal shapes and sizes. Supplemental lateral incisors are rare and bilateral cases are even rarer, with only two cases being reported in the literature till date. Therefore, we are describing a rare case of non–syndromic, bilateral, supplemental, maxillary, lateral incisors and we have discussed the management of supernumerary teeth.
The aim of the current study was to investigate fine motor control ability and manual dexterity women with fibromyalgia syndrome (FMS) without symptoms in the upper extremity compared to healthy women. Subtests of the Purdue Pegboard Test (one-hand, bilateral and assembly) and of the Jebsen-Taylor hand-function test (writing, turning cards, picking up small, light and large heavy objects, simulated feeding and stacking checkers) were evaluated bilaterally in 20 women with FMS (aged 35-55 years) without symptoms in the upper limb and 20 age- and hand dominance-matched healthy women. Differences between sides and groups were analysed with several analysis of variance (ANOVA). The ANOVA revealed significant differences between groups (P < 0.001) and sides (P = 0.007) for one-hand pin placement subtest: women with FMS showed bilateral worse scores than controls. Patients also exhibited significantly lower scores in bilateral pin placement and assembly subtests when compared to healthy controls (P < 0.001). The ANOVA also revealed significant differences between groups for writing, turning over cards, picking up small objects, stacking checkers, picking up large light objects and picking up large heavy objects (all, P < 0.001): women with FMS needed more time for these subtests than healthy women with both hands. No difference for simulated feeding was found between groups. Our findings revealed bilateral deficits in fine motor control ability and manual dexterity in patients with FMS without symptoms in the upper extremity. These deficits are not related to the clinical features of the symptoms supporting an underlying central mechanism of altered motor control. PMID:23354668
Pérez-de-Heredia-Torres, Marta; Martínez-Piédrola, Rosa M; Cigarán-Méndez, Margarita; Ortega-Santiago, Ricardo; Fernández-de-Las-Peñas, César
Summary Variations in vertebral artery origin and course are well-described in the literature. The origin of right vertebral artery from the right common carotid artery is an extremely rare variant. We describe a unique case of a child with Down syndrome with variant origins of bilateral vertebral artery, an aberrant right subclavian artery and concomitant Moyamoya disease of intracranial circulation. The presence of variations of the origin and course of craniocervical arteries might have profound implications in angiographic and surgical procedures and hence it is of great importance to be aware of such a possibility.
Mishra, A.; Pendharkar, H.; Jayadaevan, E.R.; Bodhey, N.
Popliteal artery entrapment syndrome (PAES) is the most common cause of lower leg claudication in patients younger than 50 years. The different types of PAES can result in different rates of arterial damage, leading to aneurysmal degeneration or occlusion. We report a rare case of a young patient presenting with asymmetrical bilateral popliteal artery entrapment. Type III PAES on the right resulted in severe limb ischemia and was treated by division of the accessory tendon and replacement of damaged artery with vein graft. On the left, the medial head of gastrocnemius was resected to release a type I PAES. PMID:19628519
Shen, James; Abu-Hamad, Ghassan; Makaroun, Michel S; Chaer, Rabih A
Purpose To report the first case in which Coats’ disease was observed with infantile cataract in a girl with Turner syndrome (TS).\\u000a \\u000a \\u000a \\u000a Materials and methods We examined a 4-year-old female infant with TS who was referred with a diagnosis of leukocoria in the left eye.\\u000a \\u000a \\u000a \\u000a Results and discussion Examination under anaesthesia revealed a bilateral punctate cataract and left eye fundus showed vascular retinal
Galactocele is an uncommon benign breast lesion. Its cause is unknown. Here, we report a male infant with Down syndrome and congenital hypothyroidism during the newborn period. At follow up, when he was 6 months old, bilateral mammillary swelling was detected and diagnosed as galactocele. Although thyroid hormone levels were normal, serum prolactin levels were elevated. Cyst aspiration was performed on the left side and 6 months after the aspiration of the cyst on the left side, both cysts had clinically and sonographically regressed. No recurrence was observed at the end of the 4th year. PMID:24134763
Epidermal nevus syndrome is a rare congenital sporadic neuro-ectodermic disorder, characterized by the presence of epidermal nevi in association with various developmental abnormalities of the skin, eyes, nervous, skeletal, cardiovascular and urogenital systems. We describe a 5-year-old boy with conjunctival lipodermoid, cervical and facial sebaceous nevi who presented at 3 years of age with hypertension due to bilateral renal artery stenosis together with multiple vascular anomalies (aorta, celiac trunk, superior mesenteric artery) as shown by magnetic resonance angiography. Systemic arterial hypertension was difficult to control despite combined anti-hypertensive drugs and the surgical repair of the aortic coarctation. PMID:21720804
A case of bilateral endolymphatic sac tumours is reported. In a patient with von Hippel-Lindau syndrome, tumour growth in the right cerebellopontine angle caused deafness. The tumour was removed and classified as a metastasis from a thyroid carcinoma. However, on thyroidectomy no primary neoplasm could be found. Eight years later a similar tumour was operated on in the left petrosal bone. Histological appearance, immunocytochemical findings, and the clinical context gave evidence that the tumours had to be reclassified as endolymphatic sac tumours--extremely rare entities. The report supports the hypothesis, suggested by the few earlier case reports, that endolymphatic sac tumours could be one of the inherent tumour manifestations in von Hippel-Lindau syndrome. Images
Kempermann, G; Neumann, H P; Scheremet, R; Volk, B; Mann, W; Gilsbach, J; Laszig, R
‘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.
We report the case of a 71-year-old man with progressive metastatic prostate cancer in whom simultaneous occurrence of paraneoplastic Cushing syndrome (CS) and tumor-induced osteomalacia (TIO) initially was suspected. However, the evolution of biochemical markers of phosphate metabolism during disease course and after bilateral adrenalectomy argued against the diagnosis of TIO. Despite the persistence of progressive prostate cancer, CS and hypophosphatemia resolved in parallel after bilateral adrenalectomy. Thus, these data suggest that paraneoplastic CS per se was involved in the pathogenesis of hypophosphatemia. Calcitriol and intact fibroblast growth factor 23 (FGF23) levels were within the reference range at onset, which is inappropriate in the setting of severe hypophosphatemia. All parameters of phosphate metabolism normalized after resolution of hypercortisolism. Based on the known suppressive effect of glucocorticoids (GCs) on bone remodeling and the inverse relationship between bone turnover rate and circulating FGF23 levels, we postulate that GCs interfere indirectly with phosphate homeostasis by inducing inappropriate FGF23 production and release. This mechanism could further aggravate the hypophosphatemia resulting from GC-induced inhibition of intestinal phosphate absorption. Studies directed at the identification of the molecular pathways in bone mediating the interference of GCs with phosphate metabolism are warranted. PMID:21910004
The authors aimed to describe how often the allocation method and the statistical analyses that deal with bilateral involvement are reported in clinical trials for carpal tunnel syndrome and to determine whether reporting has improved over time. Forty-two trials identified from recently published systematic reviews were assessed. Information about allocation method and statistical analyses was obtained from published reports and trialists. Only 15 trialists (36%) reported the method of random sequence generation used, and 6 trialists (14%) reported the method of allocation concealment used. Of 25 trials including participants with bilateral carpal tunnel syndrome, 17 (68%) reported the method used to allocate the wrists, whereas only 1 (4%) reported using a statistical analysis that appropriately dealt with bilateral involvement. There was no clear trend of improved reporting over time. Interventions are needed to improve reporting quality and statistical analyses of these trials so that these can provide more reliable evidence to inform clinical practice. PMID:23552333
Page, Matthew J; O'Connor, Denise A; Pitt, Veronica; Massy-Westropp, Nicola
Background: Coagulation disorders are common in young adults (age less than 55 years) with ischemic stroke. Objectives: To describe a young woman with bilateral strokes of undetermined etiology who had three significant hemostatic disturbances. These were protein S deficiency, Factor V Leiden heterozygosity, and sticky platelet syndrome. The Factor V Leiden mutation was also present in one of her children.
Cerebrovascular accidents are not rare during pregnancy and the postpartum period. Pre-eclampsia is a common condition that is characterized by proteinuria and de novo hypertension that may be complicated by hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome. Spontaneous cervical artery dissection has been rarely reported in the postpartum period but never in association with HELLP syndrome. We describe a case of pre-eclampsia and HELLP syndrome complicated in the postpartum period by bilateral thalamic infarct as result of left vertebral artery dissection. We speculated about the possible common etiopathologic mechanisms involved in this previously unreported association. PMID:22341668
A 32-year-old man with Marfan syndrome presented with enlarging, asymptomatic bilateral subclavian artery aneurysms. He has an extensive surgical history including aortic arch and descending thoracic aorta replacement. The L aneurysm was treated first with an L carotid-vertebral artery vein bypass, aneurysm debranching, and stent-graft repair of aneurysm via the L brachial artery approach. The R aneurysm was treated by placing a stent graft from the proximal R common carotid artery across the R subclavian artery origin and landing in the prosthetic innominate bypass graft via an L common carotid artery conduit. An aneurysm debranching and R carotid-subclavian artery bypass completed the procedure. A proximal type I endoleak was detected in the R aneurysm sac on follow-up computed tomography angiography. This was treated with sternotomy, aorta to L common carotid artery bypass, stent graft removal, and oversewing of the R subclavian artery origin. The patient recovered uneventfully. Subclavian artery aneurysms are rarely diagnosed in patients with Marfan disease. Although durability remains unproven, hybrid repair should be considered in patients with subclavian artery aneurysms, to minimize the morbidity commonly associated with open repair. PMID:19631504
Bowman, Jonathan N; Ellozy, Sharif H; Plestis, Konstadinos; Marin, Michael L; Faries, Peter L
Gynecologically, NBCCS is variously associated (14%-75% cases) to typically bilateral, multinodular, calcified, ovarian fibromas. We report 2 rare cases of unusually recurrent bilateral ovarian fibromas treated with conservative surgery. Preservation of the normal ovarian tissue is always recommended, even though there is the risk of recurrences, given the benign nature of the lesions and the young age of patients. PMID:22381971
Fedele, Luigi; Motta, Francesca; Frontino, Giada; Pallotti, Francesco
We examined the functional organization of cerebral activity in 3-month-old infants when they were listening to their mother language. Short sentences were presented in a slow event-related functional MRI paradigm. We then parsed the infant's network of perisylvian responsive regions into functionally distinct regions based on their speed of activation and sensitivity to sentence repetition. An adult-like structure of functional
We present a case report of acute bilateral excercise-induced compartment syndrome in the adductor longus muscles, which was treated with bilateral medial fasciotomies. Postoperatively, the healing process of the adductor muscles was followed up by repeated MR imagings over six months. Myonecrosis was found in peroperative muscle biopsies. Pain and muscle swelling subsided soon after the fasciotomy, correlating with the early postoperative MR findings. Four months postoperatively, the signal intensity of the adductor muscles was normal in T1- and T2-weighted images, but the normal fibre structure of the adductor muscles could only be seen 6 months postoperatively. At six month's control checkup there was no subjective weakness of the adductors, and hyperesthesia had disappeared and the patient was capable of normal activities. PMID:12439779
A 66-year-old man with Cushing's syndrome due to adrenocorticotropin-independent bilateral macronodular adrenocortical hyperplasia (AIMAH) was treated for 7 years with trilostane, a 3?-hydroxysteroid dehydrogenase inhibitor. Administration of trilostane reduced the serum cortisol level to around the upper limit of normal for 7 years, and symptoms of excessive glucocorticoid production (such as moon face and obesity) were gradually improved. On the other hand, the size of both adrenal glands gradually increased despite treatment with trilostane. Though trilostane therapy could not prevent adrenal growth, it did suppress cortisol secretion over the long term, so it might be a reasonable option for AIMAH in addition to adrenalectomy. PMID:22041369
The management of ectopia lentis in Marfan's syndrome is challenging. Multiple disease-associated factors conspire to deprive these patients of adequate vision. While optical correction with glasses and contact lenses is usually advocated early on, the irregular astigmatism and even partial aphakia that accompanies advanced cases generally warrant surgical intervention. Several surgical strategies have been devised to manage these challenging cases, including the combination of endocapsular or pars plana lensectomy and iris or scleral fixation of the intraocular lens (IOL) or IOL-bag complex. All of the reported cases have been implanted with IOLs that correct for myopia only. With toric lenses, it is now possible to correct for corneal astigmatism in these patients as well, provided that the capsular bag is maintained and can be properly centered. We report the combination of scleral-fixated Cionni endocapsular rings and toric IOLs in a pediatric patient with bilateral ectopia lentis secondary to Marfan's syndrome.
Gimbel, Howard V.; Camoriano, Gerardo D.; Aman-Ullah, Muhammad
Patients suffering from Urbach-Wiethe syndrome (UWS), also known as lipoid proteinosis or hyalinosis cutis et mucosae, may have an ophthalmologist involved in the diagnosis and management of their disease. Along with moniliform blepharosis as a pathognomonic feature of the disease, an ophthalmologist may encounter other manifestations of UWS in any part of the eye such as cornea; conjunctiva; sclera; trabecular meshwork; iris/pupil; lens and zonular fibers; retina; nasolacrimal duct. This paper provides a review on the pathogenesis and the diverse ocular manifestations seen in UWS patients. Uncommon complications are discussed in this paper (glaucoma; dry eye and epiphora; complications of lens, retina, cornea; iris/pupil and conjunctiva). Moreover, a 27-year-old male UWS patient is described with bilateral diffuse anterior stromal iris atrophy, diffuse keratic precipitates; posterior subcapsular cataract; 1 + vitreous cell in anterior vitreous examination. This case was thought to be the first instance of bilateral uveitis associated with UWS. Overall, ophthalmologists may encounter diverse ocular complications accompanying this syndrome. They should be familiar with well-established ophthalmologic manifestations leading them to cooperate with other specialists in diagnosis and management of the disease.
To determine structural brain correlates of naming abilities in older adults, we tested 24 individuals aged 56-79 on two confrontation-naming tests (the Boston Naming Test (BNT) and the Action Naming Test (ANT)), then collected from these individuals structural Magnetic-Resonance Imaging (MRI) and Diffusion Tensor Imaging (DTI) data. Overall, several regions showed that greater gray and white matter volume/integrity measures were associated with better task performance. Left peri-Sylvian language regions and their right-hemisphere counterparts, plus left mid-frontal gyrus correlated with accuracy and/or negatively with response time (RT) on the naming tests. Fractional anisotropy maps derived from DTI showed robust positive correlations with ANT accuracy bilaterally in the temporal lobe and in right middle frontal lobe, as well as negative correlations with BNT RT, bilaterally, in the white matter within middle and inferior temporal lobes. We conclude that those older adults with relatively better naming skills can rely on right-hemisphere peri-Sylvian and mid-frontal regions and pathways, in conjunction with left-hemisphere peri-Sylvian and mid-frontal regions, to achieve their success. PMID:20399492
Obler, Loraine K; Rykhlevskaia, Elena; Schnyer, David; Clark-Cotton, Manuella R; Spiro, Avron; Hyun, JungMoon; Kim, Dae-Shik; Goral, Mira; Albert, Martin L
To determine structural brain correlates of naming abilities in older adults, we tested 24 individuals aged 56 to 79 on two confrontation-naming tests (the Boston Naming Test (BNT) and the Action Naming Test (ANT)), then collected from these individuals structural Magnetic Resonance Imaging (MRI) and Diffusion Tensor Imaging (DTI) data. Overall, several regions showed that greater gray and white matter volume/integrity measures were associated with better task performance. Left peri-Sylvian language regions and their right-hemisphere counterparts, plus left mid-frontal gyrus correlated with accuracy and/or negatively with response time (RT) on the naming tests. Fractional anisotropy maps derived from DTI showed robust positive correlations with ANT accuracy bilaterally in the temporal lobe and in right middle frontal lobe, as well as negative correlations with BNT RT, bilaterally, in the white matter within middle and inferior temporal lobes. We conclude that those older adults with relatively better naming skills can rely on right-hemisphere peri-Sylvian and mid-frontal regions and pathways, in conjunction with left-hemisphere peri-Sylvian and mid-frontal regions, to achieve their success.
Obler, Loraine K.; Rykhlevskaia, Elena; Schnyer, David; Clark-Cotton, Manuella R.; Spiro, Avron; Hyun, JungMoon; Kim, Dae-Shik; Goral, Mira; Albert, Martin L.
Acute febrile neutrophilic dermatosis, or Sweet's syndrome, was first described in 1964 and consists of a triad of erythematous cutaneous plaques infiltrated by neutrophils in association with fever and leukocytosis. Sweet's syndrome has been reported to be associated with conditions ranging from upper respiratory tract infections to inflammatory bowel disease to rheumatoid arthritis. We report a patient with a 2-year
David B. Pharis; Danko Cerenko; S. Wright Caughman
The case is described of a male patient who had a history of acute retinal necrosis (ARN) OS at age 13 years. The eye subsequently had an inoperable retinal detachment with residual light perception visual acuity. After a 34-year disease-free interval, ARN developed OD that responded to medical treatment. This case represents the longest reported interval of ARN quiescence with eventual bilateral involvement and illustrates the importance of long-term patient follow-up. PMID:8304688
The usual polymorphic central nervous system involvement observed in primary Sjögren's syndrome is classified as monofocal, multifocal or diffuse involvement. We present a case of acute rhombencephalitis associated with hearing loss that could correspond to an intermediate form between diffuse and monofocal involvement. The acute onset with fever and not clinical sign of xerostomia or xerophthalmia suggest the possibility of vascular-like or infectious-like forms. We emphasize the importance of obtaining a minor salivary gland biopsy for diagnosis of Sjögren's syndrome and suggest that Sjögren's syndrome could be frequently misdiagnosed. PMID:11965177
Devos, D; Bombois, S; Ferriby, D; Ruzza, I; De Seze, J; Pasquier, F
Consumption of methamphetamine disturbs dopaminergic transmission and sometimes provokes schizophrenia-like-psychosis, named methamphetamine-associated psychosis (MAP). While previous studies have repeatedly reported regional volume reductions in the frontal and temporal areas as neuroanatomical substrates for psychotic symptoms, no study has examined whether such neuroanatomical substrates exist or not in patients with MAP. Magnetic resonance images obtained from twenty patients with MAP and 20 demographically-matched healthy controls (HC) were processed for voxel-based morphometry (VBM) using Diffeomorphic Anatomical Registration using Exponentiated Lie Algebra. An analysis of covariance model was adopted to identify volume differences between subjects with MAP and HC, treating intracranial volume as a confounding covariate. The VBM analyses showed significant gray matter volume reductions in the left perisylvian structures, such as the posterior inferior frontal gyrus and the anterior superior temporal gyrus, and the frontopolar cortices, including its dorsomedial, ventromedial, dorsolateral, and ventrolateral portions, and white matter volume reduction in the orbitofrontal area in the patients with MAP compared with the HC subjects. The smaller regional gray matter volume in the medial portion of the frontopolar cortex was significantly correlated with the severe positive symptoms in the individuals with MAP. The volume reductions in the left perisylvian structure suggest that patients with MAP have a similar pathophysiology to schizophrenia, whereas those in the frontopolar cortices and orbitofrontal area suggest an association with antisocial traits or vulnerability to substance dependence. PMID:23688384
Severe obstructive sleep apnea syndrome (OSAS) threatens patients' lives. To solve ventilation problem, snoring, and avoid another orthognathic surgery for mandibular advancement, bone-anchored rapid maxillary expansion and bilateral interoral mandibular distraction osteogenesis were tried on a 20-year-old Chinese male patient with severe skeletal class II malocclusion and OSAS.The patient had polysomnography (apnea-hypopnea index 54.2), body mass index measurement (19.7 kg/m), and cephalometry before the treatment. Bone-anchored rapid maxillary expansion was performed for the correction of maxillary transverse and minor sagittal deficiency and the improvement of nasal airflow by decreasing nasal resistance. Bilateral interoral mandibular distraction osteogenesis was operated to lengthen the small, retruded mandible by 15 mm. Orthodontic treatment after the maxillary expansion and mandibular distraction osteogenesis can help obtain stable occlusion.The Epworth Sleepiness Scale, a questionnaire for temporomandibular joint, cephalometric analysis, polysomnography, acoustic rhinometry, and multislice spiral computed tomography were performed to evaluate changes from the treatment. All the results showed that the patient had a significantly alleviated OSAS. In addition, an acceptable occlusion was also obtained. PMID:23714918
A 65-year-old man, s/p coronary bypass surgery (CABG) with left internal mammary artery (LIMA) to the left anterior descending (LAD) artery 12 years previously, presented to his local hospital with left upper extremity pain, dizziness, falls, and chest pain. At the outside hospital, a proximal total left subclavian occlusion was found and the patient underwent left subclavian artery to common carotid artery (SCA-CCA) bypass surgery. Shortly thereafter, the patient developed right subclavian thrombosis, and underwent right SCA-CCA bypass surgery. Twenty days later, coronary steal symptoms recurred; troponin levels were elevated and ultrasound exam revealed bilateral SCA-CCA graft occlusion. The patient was then transferred to a tertiary care facility with a diagnosis of non-ST elevation myocardial infarct (NSTEMI). A successful endovascular procedure was performed in the cardiac catheterization laboratory with the use of coronary chronic total occlusion (CTO) devices, to treat the coronary steal syndrome. PMID:23293182
Wisneski, Andrew D; Beyer, Anna T; Shunk, Kendrick A
PURPOSE:Acute retinal necrosis (ARN) syndrome is associated with members of the herpes virus family, but the mechanisms of infection remain unclear. The purpose of this study is to report a unique case of acute retinal necrosis syndrome associated with herpetic encephalitis in order to elucidate possible factors involved in herpetic central nervous system disease.METHOD:Case report.RESULTS:A 64-year-old woman who developed acute
Julian D. Perry; Christopher A. Girkin; Neil R. Miller; Douglas A. Kerr
BACKGROUND: Capsular warning syndrome was first described in 1993, featured with repetitive episodes of motor and\\/or sensory dysfunction without cortical signs. Recently, it has been demonstrated that clinically typical capsular warning syndrome can be associated with pontine infarct and the term â€œpontine warning syndromeâ€? was coined. CASE PRESENTATION: A 54-year-old woman with a history of hypertension was seen with profound
Hai Wei Huang; Shen Wen He; Shuang Quan Tan; Li Li Su
We present the clinical, laboratory, computed tomography (CT), single photon emission computed tomography (SPECT), and histopathological findings of a patient with adrenocorticotropic hormone (ACTH) dependent Cushing’s syndrome with massive bilateral adrenal gland hyperplasia due to a hilar ACTH-producing well-differentiated neuroendocrine carcinoma.
We present the clinical, laboratory, computed tomography (CT), single photon emission computed tomography (SPECT), and histopathological findings of a patient with adrenocorticotropic hormone (ACTH) dependent Cushing's syndrome with massive bilateral adrenal gland hyperplasia due to a hilar ACTH-producing well-differentiated neuroendocrine carcinoma. PMID:22470675
Bilateral congenital diaphragmatic hernia (CDH) is a rare birth defect, with a poor prognosis. We describe a case of bilateral CDH discovered while repairing the right sided CDH. Diaphragmatic defect was repaired and a silo was applied on the abdominal wound to avoid abdominal compartment syndrome. The patient however died postoperatively due to severe pulmonary hypertension. PMID:23061036
Dhua, Anjan Kumar; Aggarwal, Satish K; Mathur, Nb; Sethi, Gr
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…
Gow, David W., Jr.; Keller, Corey J.; Eskandar, Emad; Meng, Nate; Cash, Sydney S.
|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…
Gow, David W., Jr.; Keller, Corey J.; Eskandar, Emad; Meng, Nate; Cash, Sydney S.
Occlusion of the rostral portion of the basilar artery can result in ischaemia of the midbrain and thalami, as well as of the temporal and occipital lobes. The so called “top of the basilar” syndrome manifests clinically as numerous combinations of abnormalities of alertness, sleep–wake cycle, and behaviour and oculomotor or pupillomotor functions. A 67 year old man presented with
K Spengos; J C Wohrle; G Tsivgoulis; G Stouraitis; K Vemmos; V Zis
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
We report a case of post-streptococcal uveitis mainly presenting with bilateral recurrent retinal vasculitis in Korea. A 14-year-old Asian female presented with decreased visual acuity of 20 / 30 in the right eye and 20 / 25 in the left eye. The patient had a history of glomerulonephritis nine months before onset of uveitis. The manifestation of uveitis was predominantly retinal vasculitis. We presumed post-streptococcal uveitis because probable streptococcal infection was confirmed by anti-streptolysin O titer elevation. With topical and oral steroid treatments, the patient experienced complete vision recovery. Post-streptococcal uveitis occurs rarely and mostly involves young patients in the form of non-granulomatous anterior uveitis. However, as this case shows, it may primarily involve the posterior uvea without anterior inflammation and may recur.
A patient with Zellweger syndrome, who manifested marked dilatation of the lateral ventricles, observed at 34 weeks gestation by fetal ultrasonography, is reported. Postnatal magnetic resonance imaging revealed marked colpocephaly and hypogenesis of the posterior part of the corpus callosum. However, pachygyria was limited to the perisylvian regions. Biochemical diagnosis was based on increased serum very-long-chain fatty acids, 2-hydroxysebacic aciduria,
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
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.
Two children are described who suddenly developed an encephalitic illness with intractable bilateral facial seizures. The seizures subsided over several days, but the children were left with the signs of pseudobulbar palsy and are unable to speak or swallow effectively. Bilateral destructive lesions in the opercular regions evolved on computed tomographic scans. Both children were treated with acyclovir relatively early
Padraic J. Grattan-Smith; Ian J. Hopkins; Lloyd K. Shield; David W. Boldt
Multiple supernumerary teeth are very rare, accounting for less than 1% of cases. They are commonly associated with syndromes like Gardner's syndrome and cleidocranial dysostosis and cleft lip and palate. Non-syndromic multiple supernumerary teeth have a predilection to occur in the mandibular premolar region. Orthokeratinized odontogenic cyst (OOC) is a relatively uncommon developmental cyst comprising about 10% of the cases that had been previously implied as odontogenic keratocysts. More than half of the cases of OOC are associated with impacted tooth; but not a single case of OOC associated with supernumerary teeth is reported. Hence, the purpose of this article is to report the first case of multiple supernumerary mandibular premolars associated with OOC in a 35-year-old male and to review the literature associated with multiple bilateral supernumerary mandibular premolars.
Background Tourette syndrome is a neurologic disorder that is characterized by repetitive muscle contractions that produce stereotyped movements or sounds. Approximately 50% of individuals with TS also exhibit obsessive-compulsive behaviors including eye rubbing. We report a case of bilateral self-induced keratoconus in a patient with TS, associated with compulsive eye rubbing. Case presentation A 35-year-old man was first seen in our clinic as an outpatient due to rapid deterioration of vision in his right eye associated with pain and tearing, over a period of one month. Slit lamp biomicroscopy of the right eye showed a central stromal scar due to corneal hydrops. Clinical examination and corneal topography of the left eye were normal. Six months later the patient developed corneal hydrops of his left eye. During the following examinations his vision continued to deteriorate in both eyes, while a central stromal scar was forming in his left cornea. Four years after the initial examination the patient's visual acuity was no light perception in the right eye and counting fingers at 33 cm in the left eye. His right eye was phthisic. Conclusions Our patient developed a rapidly progressing bilateral corneal ectasia and phthisis of his right eye during a time period of 4 years. This unusual pattern suggests that the patient's compulsive behavior compromised both of his corneas and led to bilateral keratoconus.
We evaluated the efficacy of bilateral caudal epidural sacral neuromodulation for the treatment of refractory chronic pelvic pain (CPP), painful bladder syndrome, and interstitial cystitis (IC). Thirty consecutive patients (21 female, 9 male) with severe refractory symptoms underwent bilateral S2-S4 sacral neuromodulation for CPP/IC. Patients were evaluated with the O'Leary IC symptom and problem index (ICSI, ICPI), the short form of the Urogenital Distress Inventory (UDI-6), and the RAND 36-item health survey (SF-36) preoperatively and 6 months postoperatively. The mean and minimum follow-up were 15 and 6 months, respectively. Of the 30 patients, 23 (77%) had a successful trial stimulation and were permanently implanted. Among these patients, the ICSI and ICPI scores improved by 35 (p = 0.005) and 38% (p = 0.007), respectively. The pain score improved by 40% (p = 0.04) and the UDI-6 score by 26% (p = 0.05). On average, patients reported a 42% improvement in their symptoms. SF-36 scores did not improve significantly. In refractory patients, bilateral caudal epidural sacral neuromodulation is another possible mode of treatment, which appears to improve both pelvic pain and voiding symptoms. PMID:17925994
Zabihi, Nasim; Mourtzinos, Arthur; Maher, Mary Grey; Raz, Shlomo; Rodríguez, Larissa V
The clinical, radiologic, and histopathologic findings of a case of bilateral dacryops is reported in a 49-year-old man. The patient presented with cystic masses located in superior lateral orbits and underwent bilateral excisions of these without any postoperative complications. PMID:3178080
A 5-year-old female infant with a extremely rare combination of Pierre Robin syndrome and multiple malformations of the limbs and kidneys is presented. Orthopaedic, hearing, intellectual and speech disabilities and treatment difficulties are discussed.
A case is reported of a 23-year-old male patient who developed, after severe blunt injury of the lumbar region, massive thrombosis of the vena cava inferior (VCI), both renal veins, bilateral pulmonary artery thromboembolism (PATE), nephrotic syndrome (NS). In spite of anticoagulant therapy, the condition of the patient progressively aggravated for 1.5 year: thrombosis involved the ileac and femoral arteries on the right, thrombus floated in the right atrium with PATE recurrent episodes, pulmonary hypertension reached 120 mm Hg with formation of decompensated cor pulmnonale, proteinuria and hypoalbuminemia deteriorated, anasarca edema developed Multigenic thrombophilia was diagnosed (1 homozygous and 5 heterozygous mutations). A radical one-stage operation was successful: thromboectomy from the VCI, right ileac and left renal veins, thrombendarterectomy from the pulmonary arteries, suture of the interatrial septum defect, installation of cava-filter After the operation pulmonary pressure lowered to 40-45 mm Hg, right heart volume normalized, immunosuppressive therapy with prednisolone and cyclosporine led to nephropathy remission. The discussion covers mechanisms and factors (including genetic) of thrombosis progression, correlations between intravascular thrombosis, NS and chronic glomerulonephritis (possible NS development due to bilateral thrombosis of the renal veins and nephropathy role in thrombosis progression), approaches to conservative and surgical treatment of such patients. Global experience in conduction of pulmonary thrombendarterectomy and thrombectomy from VCI is reviewed (one-stage operations were not described earlier). PMID:22616531
Blagova, O V; Dzemeshkevich, S L; Kozlovskaia, N L; Nedostup, A V; Sarkisova, N D; Frolova, Iu V; Raskin, V V; Dzemeshkevich, A S; Abugov, S A; Skipenko, O G; Shilov, E M; Sedov, V P; Gagarina, N V; Sinitsyn, V E; Mershina, E A; Volkova, E Iu
Background Von Hippel-Lindau (VHL) disease is an autosomal dominant inherited disease. It is relatively recent that type 2C was identified as a separate group solely presenting with pheochromocytomas. As an illustration, an interesting case is presented of a pregnant woman with refractory hypertension. It proved to be the first manifestation of bilateral pheochromocytomas. The family history may indicate the diagnosis, but only identification of a germ line mutation in the DNA of a patient will confirm carriership. Case presentation A 27 year pregnant patient with intra uterine growth retardation presented with hypertension and pre-eclampsia. Magnetic resonance imaging revealed bilateral adrenal pheochromocytoma. She underwent laparoscopic adrenelectomy and a missense mutation (Gly93Ser) in exon 1 of the VHL gene on chromosome 3 (p25 – p26) was shown in the patient, her father and her daughter confirming the diagnosis of VHL. Conclusion In almost all VHL families molecular genetic analysis of DNA will demonstrate an inherited mutation. Because of the involvement in several organs, periodic clinical evaluation should take place in a well coordinated, multidisciplinary setting. VHL disease can be classified into several subtypes. VHL type 2C patients present with pheochromocytomas without evidence of haemangioblastomas in the central nervous system and/or retina and a low risk of renal cell carcinoma. Therefore, in such families, periodic clinical screening can be focussed on pheochromocytomas.
Schreinemakers, Jennifer MJ; Zonnenberg, Bernard A; Hoppener, Jo WM; Hes, Frederik J; Rinkes, Inne HM Borel; Lips, Cornelis JM
We report the first case of Susac syndrome in Koreans, in a 23-yr-old female patient who presented with sudden visual loss and associated neurological symptoms. Ophthalmic examination and fluorescein angiography showed multiple areas of branch retinal artery occlusion, which tended to recur in both eyes. Magnetic resonance imaging showed dot-like, diffusion-restricted lesions in the corpus callosum and left fornix, and audiometry showed low-frequency sensory hearing loss, compatible with Susac syndrome. She received immunosuppressive therapy with oral steroid and azathioprine. Three months later all the symptoms disappeared but obstructive vasculitis have been relapsing. This patient demonstrated the entire clinical triad of Susac syndrome, which tends to occur in young females. Although this disorder has rarely been reported in Asian populations, a high index of suspicion is warranted for early diagnosis and timely treatment. PMID:22065912
Joe, Soo Geun; Kim, June-Gone; Kwon, Sun Uck; Lee, Choong Wook; Lim, Hyun Woo; Yoon, Young Hee
We report the first case of Susac syndrome in Koreans, in a 23-yr-old female patient who presented with sudden visual loss and associated neurological symptoms. Ophthalmic examination and fluorescein angiography showed multiple areas of branch retinal artery occlusion, which tended to recur in both eyes. Magnetic resonance imaging showed dot-like, diffusion-restricted lesions in the corpus callosum and left fornix, and audiometry showed low-frequency sensory hearing loss, compatible with Susac syndrome. She received immunosuppressive therapy with oral steroid and azathioprine. Three months later all the symptoms disappeared but obstructive vasculitis have been relapsing. This patient demonstrated the entire clinical triad of Susac syndrome, which tends to occur in young females. Although this disorder has rarely been reported in Asian populations, a high index of suspicion is warranted for early diagnosis and timely treatment.
Joe, Soo Geun; Kim, June-Gone; Kwon, Sun Uck; Lee, Choong Wook; Lim, Hyun Woo
The newly emerged Middle East respiratory syndrome coronavirus (MERS-CoV) infects human bronchial epithelial Calu-3 cells. Unlike severe acute respiratory syndrome (SARS)-CoV, which exclusively infects and releases through the apical route, this virus can do so through either side of polarized Calu-3 cells. Infection results in profound apoptosis within 24 h irrespective of its production of titers that are lower than those of SARS-CoV. Together, our results provide new insights into the dissemination and pathogenesis of MERS-CoV and may indicate that the virus differs markedly from SARS-CoV. PMID:23824802
Tao, Xinrong; Hill, Terence E; Morimoto, Chikao; Peters, Clarence J; Ksiazek, Thomas G; Tseng, Chien-Te K
Acute dacryoadenitis is an uncommon condition that involves inflammation of the lacrimal gland. In rare instances, dacryoadenitis may be bilateral. A delay in proper treatment of an otherwise simple case of dacryoadenitis may lead to significant soft tissue morbidity such as cellulitis, lacrimal gland abscess, or orbital abscess. We report the case of a 24-year-old male who presented to the emergency department with acute bilateral dacryoadenitis. The patient's symptoms did not respond to oral antibiotics and he subsequently required admission for intravenous antibiotics. During his hospitalization the patient had diagnostic testing to try to determine the etiology for his symptoms. The unique aspects of managing a case of bilateral dacryoadenitis as well as treatment recommendations are discussed in this case report.
Ehlers-Danlos syndrome (EDS) is an inherited disorder of connective tissue characterized by hyperextensible skin, hypermobile\\u000a joints, and ab-normalities of the cardiovascular system. Ten types and several subtypes of EDS have so far been recognized\\u000a based on genetic, clinical, and biochemical characteristics. The spectrum of the disorder varies from mild to life-threatening\\u000a vascular complications. EDS type IV is a particularly dangerous
Megalencephaly (MEG), or enlargement of the brain, can either represent a familial variant with normal cerebral structure, or a rare brain malformation associated with developmental delay and neurological problems. MEG has been split into two subtypes: anatomical and metabolic. The latter features a build-up inside the cells owing to metabolic causes. Anatomical MEG has been detected in many different conditions, including many overgrowth syndromes. In 2004 Mirzaa et al. reported five non-consanguineous patients with a new MCA/MR syndrome characterized by severe congenital MEG with polymicrogyria (PMG), postaxial polydactyly (POLY) and hydrocephalus (HYD). The authors argued that these findings identified a new and distinct malformation syndrome, which they named MPPH. We report on a new case of MPPH, the first to be described after the original series (Mirzaa et al., 2004). PMID:18058629
Garavelli, L; Guareschi, E; Errico, S; Simoni, A; Bergonzini, P; Zollino, M; Gurrieri, F; Mancini, G M; Schot, R; Van Der Spek, P J; Frigieri, G; Zonari, P; Albertini, E; Giustina, E Della; Amarri, S; Banchini, G; Dobyns, W B; Neri, G
Ear defects in patients affected by Treacher Collins syndrome necessitate the replacement of the existing anatomic residuals of the ears with custom-made prostheses. This paper describes a multidisciplinary protocol involving both medicine and computer-aided design/computer-aided manufacturing for manufacturing ear prostheses. Using innovative prototyping technologies together with conventional silicone processing procedures, a step-by-step procedure is presented. The complete workflow includes laser scanning of the defective regions of a patient's face, the use of 3D anatomic models from an ear digital library and rapid prototyping of both substructures for bar anchoring and moulds for silicone processing. PMID:19844817
Ciocca, Leonardo; De Crescenzio, Francesca; Fantini, Massimiliano; Scotti, Roberto
We have recently observed a large pedigree with a new rare autosomal dominant spastic paraparesis. In three subsequent generations, 13 affected individuals presented with bilateral cataracts, gastroesophageal reflux with persistent vomiting, and spastic paraparesis with amyotrophy. Bilateral cataracts occurred in all affected individuals, with the exception of one patient who presented with a chorioretinal dystrophy, whereas clinical signs of spastic paraparesis showed a variable expressivity. Using a genomewide mapping approach, we mapped the disorder to the long arm of chromosome 10 on band q23.3-q24.2, in a 12-cM chromosomal region where additional neurologic disorders have been localized. The spectrum of phenotypic manifestations in this family is reminiscent of a smaller pedigree, reported recently, confirming the possibility of a new syndrome. Finally, the anticipation of symptoms suggests that an unstable trinucleotide repeat may be responsible for the condition.
Seri, M; Cusano, R; Forabosco, P; Cinti, R; Caroli, F; Picco, P; Bini, R; Morra, V B; De Michele, G; Lerone, M; Silengo, M; Pela, I; Borrone, C; Romeo, G; Devoto, M
Recent publications have alerted clinicians to a syndrome of uveitic transilluminating iris depigmentation associated with systemic fluoroquinolones and other antibiotics. Bilateral acute iris transillumination, which is associated with loss of the iris pigment epithelium and results in iris transillumination, differs from the previously described bilateral acute depigmentation of the iris, which is associated with atrophy of the iris stroma without transillumination. We present a case of fluoroquinolone-associated uveitis with anterior segment optical coherence tomography imaging to highlight some observations about this syndrome. We interpret pharmacokinetic data to help explain why oral, but not topical, moxifloxacin may cause fluoroquinolone-associated uveitis.
Best evidence protocol was applied and the question addressed, whether there is an outcome advantage of a hybrid approach including bilateral pulmonary banding (BPB) and ductal stenting over Norwood procedure (NP) to maintain systemic circulation and to restrict pulmonary blood flow in neonates with hypoplastic left heart syndrome (HLHS). Out of 80 articles published since the original description of BPB in 2002, eight were selected to answer the clinical question. All studies were retrospective case-series after BLB, only three compared results with NP controls, but not in a randomized fashion. Only three chose high-risk patients. Mean follow-up interval was mentioned only in one study. Reported mean hospital, interstage, and stage 2 mortalities after BPB was 17.3, 12.4, and 25.3%, respectively. Estimated transplant-free survival beyond stage 2 palliation ranged from 7 to 80%, with no significant differences to NP controls in three studies. Reintervention rate was high, ranging from 20 to 42% at different sites. Due to study design or inhomogeneity of patient groups, current literature does not show evidence that bilateral pulmonary banding improves outcome with respect to survival beyond second-stage HLHS palliation. PMID:22215495
Shoulder dislocations are the most common major joint dislocations encountered in the emergency departments. Bilateral shoulder dislocations are rare and of these, bilateral posterior shoulder dislocations are more prevalent than bilateral anterior shoulder dislocations. Bilateral anterior shoulder dislocation is very rare. We present a case of 24-year-old male who sustained bilateral anterior shoulder dislocation following minor trauma, with associated greater tuberosity fracture on one side. Prompt closed reduction followed by immobilization in arm sling and subsequent rehabilitation ensured a good outcome.
Supernumerary teeth are defined as the teeth developed in excess of the number found in a normal dentition. Supernumerary canine is an extremely rare finding particularly in the mandible. This case report presents a 25-year-old female patient with the unique feature of bilateral mandibular supplemental supernumerary canines. The patient was non-syndromic without any other supernumerary teeth.
Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic state accompanied by a unique brain imaging pattern. This cliniconeuroradiological entity usually presents with visual disturbances (cortical blindness, homonymous hemianopia, visual neglect, and blurred vision) along with neurotoxic manifestations. Only a few cases of PRES have previously been reported in patients with advanced HIV disease. The authors describe a case of posterior reversible encephalopathy syndrome (PRES) in a patient with advanced HIV/TBC infection who developed a neurotoxic state following TB and ART therapy initiation. They present a comprehensive review of the literature and discuss the pathogenetic hypotheses. PMID:23243537
Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic state accompanied by a unique brain imaging pattern. This cliniconeuroradiological entity usually presents with visual disturbances (cortical blindness, homonymous hemianopia, visual neglect, and blurred vision) along with neurotoxic manifestations. Only a few cases of PRES have previously been reported in patients with advanced HIV disease. The authors describe a case of posterior reversible encephalopathy syndrome (PRES) in a patient with advanced HIV/TBC infection who developed a neurotoxic state following TB and ART therapy initiation. They present a comprehensive review of the literature and discuss the pathogenetic hypotheses.
Traumatic vertebral artery dissection is not often seen by forensic pathologists, and cases investigated are scarce in the forensic literature. We present the case of a 40-year-old woman cyclist who was struck by a car while wearing a helmet, and was neurologically near normal immediately thereafter at Emergency. She presented 48 h later with acute right hemiparesis, decreasing level of consciousness, and unsteadiness. CT revealed massive cerebellar infarction. CT angiography was normal. The patient died in coma 7 days after injury and autopsy revealed bilateral edematous cerebellar infarction and bilateral vertebral artery dissection. Rotational neck injury and mural tear in the wall of the Atlantic parts of both vertebral arteries is suggested as the possible mechanism of the arterial injury. Head and neck injuries are reported as a precipitating cause of vertebral artery injury. The possible influence of trauma may be further underestimated if longer intervals between vessel dissection and ischemia occur. The current case illustrates that "talk-and-die" syndrome may be due to occult vertebral artery dissection, possibly bilateral. In forensic cases of delayed death after mild trauma to the head and neck, the vertebral arteries should be examined for the cause of death. PMID:21798679
A patient is reported in whom a classic amnesic syndrome developed as a result of repeated episodes of cerebral ischaemia, accompanied by seizures. The amnesia was very severe for both old and newly acquired memories and the critical lesions defined by MRI were circumscribed areas confined to CA1 and CA2 fields of both hippocampi. Images
Bilateral facial paralysis is an unusual clinical entity that occurs in less than 1% of patients with facial paralysis. In children bilateral facial paralysis is even more rare, and establishing its etiology can be challenging. Four pediatric patients ranging in age from 3 to 17 years are presented who developed bilateral facial paralysis as a result of acute otitis media, Lyme disease, recurrent central nervous system leukemia, and acute disseminated encephalomyelitis. The diagnosis and treatment of pediatric bilateral facial paralysis are reviewed, as well as the pertinent literature. PMID:9546263
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
With the increase in incidence of ectopic pregnancy over the decades, bilateral ectopic pregnancy is also increasing. It is usually associated with assisted reproductive techniques (ART) but in recent years few cases of spontaneous bilateral ectopic pregnancy have been reported. Gynaecologists should be aware of this and that ultrasonography has limitations in diagnosis. In cases of ectopic pregnancy where contralateral
Background. The risk of ovarian cancer is increased in the association of ovarian tumor, ascites, and hydrothorax with the significant elevated tumor marker CA-125. However, this association can be observed in a rare clinical and benign pathological entity, that is Demons-Meigs' syndrome. Objective. To describe a rare case of Demons-Meigs' syndrome observed in our department. Methods. A black African woman of 35 years old, seventh gravida and fourth parous, underwent a total abdominal hysterectomy with bilateral salpingoophorectomy for large bilateral ovarian masses associated with significant ascites, bilateral pleural effusion, and particular highly elevated tumor marker CA-125 (1835?UI/mL) in a pronounced general alteration condition. Results. The postoperative course was uneventful characterized by a complete remission of hydrothorax and ascites with normal level of CA-125 three months after tumor excision. Histology of both masses revealed a bilateral ovarian fibrothecoma, a benign tumor of the ovary, thus confirming the diagnosis of Demons-Meigs' syndrome. Conclusion. The Demons-Meigs syndrome, although it strongly mimics the clinical picture of malignant metastatic ovarian cancer, remains a disease with benign prognosis after surgical tumor resection. This is a rare condition that must be known and recognized by practitioners to avoid unnecessary practices.
Incidentally discovered adrenal masses (incidentalomas) are common and present challenges both in diagnosis and management. When incidentally discovered adrenal masses are bilateral, a refined diagnostic approach is warranted since bilateral disease is more likely to be pathologic. We review a case of a 34-year-old man with incidentally discovered bilateral adrenal nodules. A comprehensive diagnostic strategy led to the diagnosis of bilateral pheochromocytoma caused by von Hippel-Lindau syndrome. He was successfully treated with bilateral laparoscopic adrenalectomy and has recovered well. While the initial diagnostic approach is similar to the unilateral incidentaloma, additional testing and/or genetic testing should be considered in the case of the bilateral adrenal mass.
Carlson, Anders L.; Marney, Annis M.; Anderson, Scott R.; Gilbert, Matthew P.
Palmoplantar punctate keratoses may be the main cutaneous sign of various diseases (porokeratosis punctata palmaris et plantaris, keratosis punctata of the palmar creases, familial punctate palmoplantar keratoderma) or represent a secondary feature [Cowden's syndrome (CS) and Darier's disease]. In CS, such keratoses usually appear during the second and third decades of life, together with other mucocutaneous features. We present the case of a 3-year-old girl with palmoplantar punctate keratoses in whom diagnosis of new-onset CS was suspected only after the development of other cutaneous lesions. Genetic analysis confirmed the diagnosis. This case highlights the necessity to consider CS in the differential diagnosis when palmoplantar punctate keratoses are found, even in paediatric patients. A prompt diagnosis is important in order to monitor the development of possible underlying associated neoplasms. PMID:19508470
Ferran, M; Bussaglia, E; Matias-Guiu, X; Pujol, R M
With the increase in incidence of ectopic pregnancy over the decades, bilateral ectopic pregnancy is also increasing. It is usually associated with assisted reproductive techniques (ART) but in recent years few cases of spontaneous bilateral ectopic pregnancy have been reported. Gynaecologists should be aware of this and that ultrasonography has limitations in diagnosis. In cases of ectopic pregnancy where contralateral adnexa is not clearly identified on ultrasound and fertility needs to be conserved, patient should be managed by experts in well equipped centres. A case of spontaneous bilateral tubal pregnancy that remained undiagnosed till laparotomy, is described. PMID:22313653
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.
Bilateral acromial dimples are uncommon in pediatric dermatology. They are usually found as a sporadic finding with limited clinical symptoms but may cause concern for parents. They can occur spontaneously or be inherited. Bilateral acromial dimples may occasionally be present as part of more complex syndromes. This article reports an 18-month-old girl with congenital bilateral acromial dimples and presents a classification of this unusual entity. A literature review of English and non-English publications was performed. We report the second case of bilateral acromial dimples seen in the context of maternal cocaine use during pregnancy. Our case is probably sporadic and nonsyndromal. We suggest that bilateral acromial dimples be classified as syndromal or nonsyndromal and that nonsyndromal cases be subdivided into inherited and sporadic. Although bilateral acromial dimples can be seen in 18q deletion syndrome, Apert syndrome, Say syndrome, and a recently described syndrome in Brazil, our review of the literature does not support the association with trisomy 9 syndrome. Dermatologists need to be aware of this unusual cutaneous finding and potential syndromal associations. The significance of the association with fetal exposure to cocaine during pregnancy has yet to be determined. The biological mother was not available for assessment for bilateral acromial dimples. PMID:23171068
The purpose of this report is to provide two illustrative cases of spontaneous and bilateral perirenal hematomas that occurred during a pregnancy complicated by eclampsia. The sonographic and computed tomographic findings included bilateral perirenal hematoma with no evidence of an underlying malignancy. Since renal hematomas that occur in association with preeclampsia and eclampsia syndrome are extremely rare, but potentially life-threatening complications, prompt laboratory and radiologic evaluations are essential and can reduce the associated morbidity and mortality. PMID:12768075
Oculocerebrocutaneous syndrome (OCCS) is a rare disease (OMIM # 164180) with only around 35 patients reported so far (Hunter 2008, Tambe et al. 2003) characterized by bilateral anophthalmia and orbital cysts, typical skin lesions consisting in skin appendages, focal dermal\\u000a hypoplasia\\/aplasia and punch-like defects, complex brain malformations (mostly of the Dandy-Walker type) associated to mental\\u000a retardation and seizures, and cleft
Temporalis muscle hypertrophy is rare and may present unilaterally or bilaterally. Variable combinations with masseteric hypertrophy are also reported. It may be associated with a parafunctional habit or occur as an idiopathic entity. A host of modalities including CT, MRI, ultrasonography and muscle biopsy may be utilized for diagnosis. While surgical reduction remains an option, medical therapy with boutlinum type A toxin is the treatment of choice. A case of bilateral temporalis hypertrophy in a 38-year-old female is reported. PMID:19836926
Following a hypoxic-ischemic insult, the globus pallidus is selectively spared from ischemic injury in contrast to the caudate and putamen. The known causes for hemorrhagic and necrotic lesions selective for injuring the globus pallidus are varied but few. The most widely known etiology is in fatal cases of carbon monoxide poisoning. Additionally reported associations include fatalities involving 3,4-methylenedioxymethamphetamine; cocaine; opiates; and cyanide poisoning. These bilateral globus palladus necrotic lesions have been reported to occur in 5-10% of opiate addicts. In this article, we present a striking photo of selective symmetrical bilateral globus pallidus necrosis following cocaine and opiate abuse. PMID:22866355
Alquist, Caroline Raasch; McGoey, Robin; Bastian, Frank; Newman, William
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
Endogenous endophthalmitis is a rare and severe intraocular infection which can be vision-threatening. We describe a case of bilateral fungal endogenous endophthalmitis in a 64-year-old male which was successfully treated with systemic administration of fluconazole followed by pars plana vitrectomy with an intravitreous injection of amphotericin B. PMID:23640266
Case reports detailing diagnosis and effective treatment of pisotriquetral loose bodies are scarce. This article describes an even rarer case of bilateral pisotriquetral joint loose bodies, explores the relative diagnostic roles of magnetic resonance imaging versus computed tomography, and outlines effective strategies used for the management of this condition drawn from the literature and our own experience.
Temporalis muscle hypertrophy is rare and may present unilaterally or bilaterally. Variable combinations with masseteric hypertrophy are also reported. It may be associated with a parafunctional habit or occur as an idiopathic entity. A host of modalities including CT, MRI, ultrasonography and muscle biopsy may be utilized for diagnosis. While surgical reduction remains an option, medical therapy with boutlinum type
Bilateral hermaphroditism was diagnosed in a 6-monthold Beagle. Histologically, bilateral ovotestes, epididymides, and fallopian tubes were identified. The dog also had a vagina, phallus, and bicornuate uterus. The dog was genetically a female as determin...
Twenty children with bilateral Wilms' tumor were presented to the Children's Hospital Medical Center and Children's Cancer Research Foundation, Sidney Farber Cancer Institute, and Joint Center for Radiation Therapy (CHMC-CCRF, SFCI, JCRT) from January 1, 1956 to December 31, 1976. Of these 20, 16 had simultaneous and 4 had metachronous disease on presentation. All patients were treated with surgery, radiation and chemotherapy. Of the 16 patients with simultaneous disease, 10 (63%) are alive and free of disease 12+ to 175+ months post diagnosis and treatment, with median follow-up of 121 months. There were no long-term survivors in the metachronous group; all were dead of disease within 21 months from initial presentation of original tumor. With these data we relate prognosis to extent of disease and discuss a general approach to the management of bilateral Wilms' tumor.
\\u000a For some B2C or B2B market places, such as electronic shopping malls, agents representing the buyers could ”go shopping” and\\u000a negotiate with several stores looking for the best deal. To develop agents capable to act in a scenario like this one, it\\u000a is necessary to deal with several issues that go beyond the bilateral negotiation or auction. In the agent
About ten percent of pheochromocytomas are associated with familial syndrome. Hereditary pheochromocytoma has characteristics of early onset, multifocality and bilaterality. We experienced a case of 44-year-old man with bilateral pheochromocytoma without evidence of medullary thyroid cancer. Genetic test detected a L790F germline mutation of RET oncogene. The author found a necessity for genetic tests in cases of young-age, bilateral pheochromocytoma.
The case of a 9 year old male child with Down syndrome and multiple injuries including splenic injury, who was incidentally found to have bilateral Morgagni hernia is presented. Aspects of pre-operative diagnosis and management are discussed. PMID:20169715
The authors present a case of acute mieloblastic leucemia, having as an unique symptom a massive bilateral orbito-palpebral hematoma, but having not other symptoms of the hemoragipar syndroms common in acute leucemiae. After a short period of retreat of the disease, the goeson, leading to death. PMID:155085
Bilateral anterior cingulectomies were performed on 23 patients for the relief of intractable pain. 19 patients suffered from pain due to metastatic disease, 3 from arachnoiditis and 1 from a phantom limb syndrome. Of those patients suffering from pain due to metastatic disease, 10 (52.1%) obtained pain relief. Among the patients suffering from arachnoiditis, 1 obtained pain relief. The case
Lacrimal and/or salivary gland enlargement is a common symptom leading to referral for rheumatological evaluation. Besides glandular enlargement in Sjögren's syndrome, sarcoidosis, lymphoma and other diseases, the symptom can also be found in IgG4-related disease. This is an increasingly recognized syndrome of unknown etiology comprising a collection of disorders that share specific clinical, serological and pathological features. PMID:23685854
Posterior scleritis is an inflammatory process of the posterior part of the sclera. Its prevalence is very low and its diagnosis can be complicated due to the absence of external ocular signs. It is more frequent in women. In young patients it does not usually have other associated pathologies, but in those over 55 years nearly one-third of the cases have a relation with some systemic disease, above all rheumatoid arthritis. The diagnosis of this pathology can require a multidisciplinary approach and the collaboration of ophthalmologists with neurologists, internists or rheumatologists. This article describes a case of idiopathic bilateral posterior scleritis. PMID:21904415
Zurutuza, A; Andonegui, J; Berástegui, L; Arruti, N
The recent increased use of steriods, immunosuppressive agents and cytotoxic drugs has been associated with a rise in the incidence of significant fungal disease. The first case of bilateral renal aspergillosis without disseminated involvement is reported. A multitherapeutic approach, including surgical evacuation of masses of hyphae, parenteral antimycotic chemotherapy and topical instillations of amphotericin B, were necessary to clear the kidneys. Newer systemic agents such as 5-fluorocytosine and rifampicin were also used. Treatment of fungal infections of the urinary tract is discussed. PMID:1113403
Testicular epidermoid cysts are the most common benign tumors of the testes, but account for only 1-2% of all testicular tumors. In a young man presenting with a testicular mass, a high index of suspicion must be maintained for the malignant testicular germ cell tumor, which is 50-times more common than testicular epidermoid cyst. Bilateral testicular epidermoid cysts are a very rare condition, with only a few reports in the literature. It is extremely important in this condition to make a correct pre-operative diagnosis on imaging to enable a testis-sparing surgery.
Loberant, Norman; Bhatt, Shweta; Messing, Edward; Dogra, Vikram S.
Bilateral elbow dislocation is a rare injury, with only a small number of case reports in the literature. The majority of\\u000a these reports describe associated ligamentous injuries only. Simultaneous bilateral radial head fracture in association with\\u000a bilateral elbow dislocation has only been described on one occasion previously. We present the case of a 54-year-old woman\\u000a who dislocated both elbows and
Bilateral renal agenesis or Potter's Syndrome is an extremely rare congenital anomaly associated with oligohydramnios. The infants die off pulmonary hypoplasia. We herewith report five cases of Potter's Syndrome over a span of one year. PMID:16933729
A 52-year-old Mexican man presented with asymptomatic, bilaterally symmetrical lipid infiltrates of the cornea and adjacent limbus. No evidence of previous ocular disease or systemic disorder of lipid metabolism could be detected. Penetrating keratoplasty of the right eye was required. The cornea was rigid and thick, with posterior bulging into the anterior chamber. Light microscopy revealed deep corneal lipid granules, foamy histiocytes, vascularisation, and chronic non-granulomatous inflammation. Transmission electron microscopy showed extracellular lipid spaces and numerous intracytoplasmic lipid vacuoles in histiocytes, keratocytes, conjunctival epithelium, and the endothelium of blood vessels in the corneal stroma and adjacent limbal conjunctiva. Histochemical analysis revealed the presence of neutral fats, free fatty acids, cholesterol, and phospholipids. Images
Alfonso, E.; Arrellanes, L.; Boruchoff, S. A.; Ormerod, L. D.; Albert, D. M.
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
Sharma, D R; Resident, S; Mohan, C; Minnas, R S; Mohindroo, N K; Sharma, M L
Presentation with simultaneous bilateral pneumothorax is uncommon and usually in the context of secondary spontaneous pneumothorax. The association of pneumothorax and silicosis is infrequent and most cases are unilateral. Bilateral pneumothorax in silicosis is very rare with just a few reports in medical literature.
Fotedar, Sanjay; Chaudhary, Dhruva; Singhla, Vikas; Narang, Rajat
This article presents the clinical features of crystal arthropathy after knee replacement. The current literature on pseudogout and gout after both total and partial knee replacement is summarized. A case of bilateral pseudogout 8 years after initial total knee arthroplasty (TKA) is used to highlight the clinical characteristics and treatment options for this underrecognized condition. Presentation mimicked a late septic joint arthroplasty with sudden onset of pain and effusion. The patient was treated successfully with an arthrotomy, debridement, synovectomy, polyethylene insert exchange, oral steroids, and nonsteroidal anti-inflammatories. There are no other reported cases of bilateral pseudogout after bilateral TKA. PMID:23102423
Melkersson - Rosenthal syndrome was described by Melkersson and Rosenthal separately in the year 1928 and 1931 respectively.\\u000a It is supposed to be a rare syndrome of bilateral alternating recurrent facial paralysis alongwith fissured tongue and oedema\\u000a of the lips, face and eyelids. A case of Melkersson - Rosenthal syndrome is reported with all the classic findings which is\\u000a a
Dev Raj Sharma; Sr. Resident; C. Mohan; R. S. Minnas; N. K. Mohindroo; M. L. Sharma
To report a case of bilateral acute angle closure glaucoma (AACG) that occurred after cervical spine surgery with the use of glycopyrolate. A 59-year-old male who presented with severe bilateral bifrontal headache and eye pain that started 12 h postextubation from a cervical spine surgery. Neostigmine 0.05 mg/kg (4.5 mg) and glycopyrrolate 0.01 mg/kg (0.9 mg) were used as muscle relaxant reversals at the end of the surgery. Ophthalmic examination revealed he had bilateral AACG with plateau iris syndrome that was treated medically along with laser iridotomies. Thorough examination of anterior chamber should be performed preoperatively on all patients undergoing surgeries in the prone position and receiving mydriatic agents under general anesthesia.
Jaroudi, Mahmoud; Fadi, Maalouf; Farah, Fadi; El Mollayess, Georges M.
To report a case of bilateral acute angle closure glaucoma (AACG) that occurred after cervical spine surgery with the use of glycopyrolate. A 59-year-old male who presented with severe bilateral bifrontal headache and eye pain that started 12 h postextubation from a cervical spine surgery. Neostigmine 0.05 mg/kg (4.5 mg) and glycopyrrolate 0.01 mg/kg (0.9 mg) were used as muscle relaxant reversals at the end of the surgery. Ophthalmic examination revealed he had bilateral AACG with plateau iris syndrome that was treated medically along with laser iridotomies. Thorough examination of anterior chamber should be performed preoperatively on all patients undergoing surgeries in the prone position and receiving mydriatic agents under general anesthesia. PMID:23741140
Jaroudi, Mahmoud; Fadi, Maalouf; Farah, Fadi; El Mollayess, Georges M
A 56 year-old man presented with vertigo and the right sided weakness. Neurological examination revealed a lethargic man with good orientation to three spheres. His neck was supple. He had anisocoria, the right pupil being larger than the left by 1.5 mm with sluggish light reaction bilaterally. He had exotropia of the right eye in primary gaze. The abduction of both eyes were full with terminal horizontal nystagmus. The adduction of both eyes were quite limited in each eye. He had a limited upward gaze with poor convergence. These were interpreted as the syndrome of the medial longitudinal fasciculus (MLF) bilaterally. He had a depressed gag reflex on the right side with tongue deviation to the right. He had a mild weakness of the right side limb and also had the right sided hemihyperesthesia including his face to pain and temperature. Twenty four hours after the onset, the left brachial angiography revealed a complete occlusion of the rostral portion of the basilar artery without visualization of the posterior cerebral and superior cerebellar arteries bilaterally. CT scans three days after the onset revealed a low density area in the mid pons with extension rostrally up to the mesencephalon. Four days later he became quadriplegic with bilateral horizontal gaze palsy. No more internuclear ophthalmoplegia is noted on both sides. The midline location of the MLF in the pons, and the separate blood supplies by different paramedian branches of the basilar artery, form the anatomical explanation for the frequent unilaterality of vascular and bilaterality of demyelinating lesions. Bilateral MLF syndrome has been considered almost pathognomonic of multiple sclerosis.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:2350928
Kataoka, S; Hirose, G; Tukada, K; Michishita, H; Yoshikawa, H
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.
Aksoy, Elif A.; Elsurer, Cagdas; Serin, Gediz M.; Unal, O. Faruk
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...
The diaphragm is an unusual localization for disseminated erythematous lupus and is generally not recognized. We present the case of a 34-year-old woman who developed bilateral pain in the base of the thorax 15 days before hospitalization without any bronchial signs and NYHA stage II dyspnea. The patient's general health status remained satisfactory and the physical examination was normal except for diffuse inflammatory joint pain and cutaneous photosensitivization. The chest x-ray disclosed ascension of both hemidiaphragms with retracted lungs associated with bilateral basal atalectasia. The diagnosis of lupus with diaphragmatic involvement was retained due to the clinical presentation with diffuse joint pain, photosensitization with facial erythema, pericardial effusion and elevated antinuclear antibody and lymphopenia (1 100/mm3). Lung function tests revealed a restrictive syndrome. Oral corticosteroids 1 mg/kg/d enabled clinical and functional improvement. In light of this observation we discuss the pathogenic mechanisms of this uncommon localization of lupus and the difficulty of establishing a sure diagnosis. PMID:16840998
El Bied, B; Afif, H; Safieddine, S; Hassani, L; Aichane, A; Trombati, N; Bouayad, Z
Extracranial carotid aneurysms caused by Takayasu's arteritis is extremely rare. Their evolutionary risk is dominated by rupture and cerebral ischemia. We report a case of a 23 years old woman presenting bilateral common carotid aneurysm secondary to Takayasu's arteritis. Surgical treatment with the patient under steroid coverage was performed to prevent the risque of rupture. The aneurysm was resected, reconstruction was performed with prothetic aortocarotid bypass. She was discharged without major complications. The graft have remanied patent during the four years follow-up periods. A biologic inflammatory syndrome and a parietal thickening of the aneurysm in a young woman must provoke diagnosis. The surgery must be appropriate every time that the diagnosis is carried considering the evolutionary risk of these aneurysms. PMID:15881852
Sedki, N; El Mahi, O; Bouarhroum, A; Benzirar, A; Lekhel, B; Sefiani, Y; El Mesnaoui, A; Benjelloun, A; Ammar, F; Bensaïd, Y
The clinical syndrome of a 65-year-old patient with a slit-shaped right-sided meso-diencephalic lesion was analysed. A cerebellar syndrome with limb-kinetic ataxia, intention tremor and hypotonicity in all extremities as well as ataxic dysarthria was found. The disruption of the two cerebello-(rubro)-thalamic pathways probably explained the signs of bilateral cerebellar dysfunction. The uncrossed ascending limb of the right, and the crossed one of the left brachium conjunctivum may have been damaged by the unilateral lesion extending between caudal midbrain and dorsal thalamus. Images
Background ACTH-independent macronodular adrenal hyperplasia (AIMAH) is a rare cause of Cushing's syndrome (CS), which is characterized by massive bilateral nodular enlargement of adrenal glands and hypercortisolism in the presence of suppressed ACTH levels. Case A 53 year-old wo- man with a 5-year history of hypertension was referred to our hospital for further evaluation of bilateral adrenal tumors with macronodules,
Eight patients representing the entire spectrum of Cantrell's syndrome are presented. While a combined diaphragmatic and pericardial defect is an essential feature of the syndrome, other manifestations form a continuum ranging from innocent upper abdominal midline defects to the full-blown pentalogy sometimes incompatible with life. In only two cases was the diagnosis established preoperatively. One patient presented uniquely with bilateral
A 5-month-old male infant presented with weak cry, decreased body movements, tightness of whole body since birth, and one episode of generalized seizure on day 4 of life. He was born at term by elective caesarian section performed for breech presentation. The child had failure to thrive, contractures at elbow and knee joints, hypertonia, microcephaly, small mouth, retrognathia, and camptodactyly. There was global developmental delay. Abdominal examination revealed umbilical and bilateral inguinal hernia. Visual evoked response and brainstem evoked response audiometry were abnormal. Nerve conduction velocity was normal. Magnetic resonance imaging of brain revealed paucity of white matter in bilateral cerebral hemispheres with cerebellar and brain stem atrophy. The differential diagnoses considered in the index patient were distal arthrogryposis (DA) syndrome, cerebroculofacioskeletal syndrome, and Pena Shokier syndrome. The index patient most likely represents a variant of DA: Sheldon Hall syndrome. PMID:20300297
This survey addresses the subject of bilateral teleoperation, a research stream with more than 50 years of history and one that continues to be a fertile ground for theoretical exploration and many applications. We focus on the control theoretic approaches that have been developed to address inherent control problems such as delays and information loss. Exposure to several concurrent applications
BACKGROUND: The most common causes of hemotympanum are therapeutic nasal packing, epistaxis, blood disorders and blunt trauma to the head. Hemotympanum is characterized as idiopathic, when it is detected in the presence of chronic otitis media. A rare case of spontaneous bilateral hemotympanum in a patient treated with anticoagulants is presented herein. CASE PRESENTATION: A 72-year-old male presented with acute
Dimitrios G Balatsouras; Panayotis Dimitropoulos; Alexandros Fassolis; Georgios Kloutsos; Nicolas C Economou; Stavros Korres; Antonis Kaberos
Based on a March 1984 address to the Canadian-American Committee, Schlesinger notes that the American natural gas producer has been the main beneficiary of Canadian export policy, which has impeded Canadian exports. He reviews the history of bilateral gas exchanges and the 1954 Phillips decision, which set up conditions that were the inverse of a normal competitive market that no
We report a 57-year-old woman with concurrent tubulointerstitial nephritis and uveitis syndrome (TINU) and Fanconi's syndrome. She presented with sudden onset of bilateral ocular pain, blurred vision, acute renal failure, glucosuria and proteinuria. Slit lamp examination revealed acute bilateral anterior uveitis. Tubulointerstitial nephritis was confirmed by kidney biopsy. Laboratory examination revealed normoglycemic glucosuria, proteinuria, normal anion-gap metabolic acidosis, phosphaturia, urinary uric acid wasting and kaliuresis leading to hypokalemia. Her vision and renal function improved gradually after systemic steroid therapy. There have been rare reports of TINU syndrome which had features of Fanconi's syndrome. The prevalence of TINU syndrome may be underestimated, and its association with Fanconi's syndrome requires further investigation. PMID:21269599
A case of Goldenhar's Syndrome (oculoauriculovertebral dysplasia) in a 3-day-old Nigerian neonate with right anophthalmos and lipodermoid, left limbal dermoid, bilateral preauricular appendages and mandibular hypoplasia is presented. The lipodermoid was attached to the tarsal conjunctiva of the lower lid of the anophthalmic side. The causative factor was presumably maternal drug (traditional medicine) ingestion at three months gestation. No similar case has been reported previously. The literature on Goldenhar's Syndrome is briefly reviewed. PMID:12625694
Progeria is a premature ageing syndrome. Werner Syndrome (WS) is a type of progeria in the adult which includes bilateral juvenile cataracts and cutaneous sclerodermiform changes; it is caused by a mutation if the WRN gene which codes a helicase, a DNA repair enzyme. A case is presented of a patient, a 12 year old girl, with characteristics of WS but with no identifiable mutation in the WRN gene, therefore it was classified as atypical Werner Syndrome (AWS). PMID:20452840
A dominantly inherited syndrome associated with hypopigmentation, heterochromia irides, colobomatous eyes and bilateral hearing loss has been ascertained in Fleckvieh cattle (German White Fleckvieh syndrome). This syndrome has been mapped to bovine chromosome (BTA) 22 using a genome-wide association study with the bovine high density single nucleotide polymorphism array. An R210I missense mutation has been identified within microphthalmia-associated transcription factor (MITF) as responsible for this syndrome. The mutation is located in the highly conserved basic region of the protein and causes a negative-dominant effect. SOX10 and PAX3 promoter binding site mutations in MITF could be ruled out as causative for the German White Fleckvieh syndrome. Molecular characterization of this newly detected bovine syndrome means a large animal model is now available for the Tietz syndrome in humans.
Background The fracture of clavicle is the most frequently observed bone fracture as birth trauma and it is usually unilateral. It is seen following shoulder dystocia deliveries or breech presentation of macrosomic newborns. Case Presentation We report two macrosomic newborns with bilateral clavicle fracture and brachial plexus palsy due to birth trauma. Chest X-rays confirmed bilateral fracture of clavicles. Both patients were recovered without any sequel. Conclusion Bilateral clavicular fracture should be considered in any neonate with bilateral absent Moro reflexes.
PURPOSE: Bartter syndrome is characterized by hyperplasia of the renal juxtaglomerular apparatus, hyperaldosteronism, and hypokalemic alkalosis. We report a case of Bartter syndrome associated with normal serum calcium levels and posterior choroidal calcification.METHODS: Case report. A 59-year-old man with bilateral cataract and Bartter syndrome underwent a complete ophthalmic examination, including standardized echography before and after cataract surgery.RESULTS: Before cataract surgery,
Giorgio Marchini; Roberto Tosi; Barbara Parolini; Giovanna Castagna; Marco Zarbin
Introduction Hepatologists and internists often encounter patients with unexplained high serum ferritin concentration. After exclusion of hereditary hemochromatosis and hemosiderosis, rare disorders like hereditary hyperferritinemia cataract syndrome should be considered in the differential diagnosis. This autosomal dominant syndrome, that typically presents with juvenile bilateral cataracts, was first described in 1995 and has an increasing number of recognized molecular defects within a regulatory region of the L-ferritin gene (FTL). Case presentation Two patients (32 and 49-year-old Caucasian men) from our ambulatory clinic were suspected as having this syndrome and a genetic analysis was performed. In both patients, sequencing of the FTL 5' region showed previously described mutations within the iron responsive element (FTL c.33 C > A and FTL c.32G > C). Conclusion Hereditary hyperferritinemia cataract syndrome should be considered in all patients with unexplained hyperferritinemia without signs of iron overload, particularly those with juvenile bilateral cataracts. Liver biopsy and phlebotomy should be avoided in this disorder.
We report the clinical findings of a patient with severe bilateral keratectasia 34 years after a penetrating keratoplasty (PK) in both eyes. An otherwise healthy 67-year-old man complained of deterioration of the eyesight in both eyes over the last 6 months. The patient was diagnosed with bilateral keratoconus at the age of 32 years, and he underwent a bilateral PK.
Xavier Valldeperas; Martina Angi; Vito Romano; Mario R. Romano
We report a case of complete bilateral cerebellar infarction diagnosed in utero by routine prenatal ultrasound and magnetic resonance imaging in a 26-week-old fetus. This posterior fossa ischemic stroke with secondary hemorrhage caused transient obstructive hydrocephalus and likely occurred subsequent to vertebrobasilar artery thrombosis. Such posterior fossa ischemic insults diagnosed in utero are rare with scarce clinical reports. The serial imaging characteristics, clinical, and developmental implications of this case are reviewed. PMID:21266324
Ansari, Safdar A; Hunter, Jill V; Nassif, Lisa M; Clark, Gary D; Ramocki, Melissa B
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.
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.
A 54-year-old diabetic, hypertensive man with poorly controlled moderate-severe sleep apnea presented with acute onset of severe anterograde amnesia and well preserved remote memory without additional cognitive impairment. Investigations, including a lumbar puncture, electroencephalogram (EEG) and serology testing ruled out infectious, neoplastic and epilleptogenic causes. MRI taken 10 days after symptom onset, was suggestive of sequential ischemic damage to both hippocampal formations. Neuropyschological evaluation suggested a focal and dense amnestic syndrome with little improvement over time. The bilateral nature of hippocampal ischemia though has been reported, is rare.
Idiopathic orbital inflammation (IOI) is defined as a benign non-infective clinical syndrome characterized by features of non-specific inflammation of the orbit without identifiable local or systemic causes. This can be called orbital myositis if the inflammation is predominantly in the orbital muscles. It is a diagnosis of exclusion based on clinical, radiological, and if necessary, histological findings. The most commons symptoms are swelling, ptosis, proptosis and painful eye movements. To our knowledge, this patient is the first with IOI to demonstrate relapsing flitting bilateral involvement of several individual extra-ocular muscles. PMID:19760410
We present clinical and cytogenetic data on a 2.5 year-old boy with partial monosomy 22p (p11.2-->pter) and distal 10q trisomy (10q24.1-->qter), resulting from maternal t(10;22) reciprocal translocation. The patient had bilateral hydronephrosis and hydroureters but without evidence of vesicoureteral reflux. Our clinical observation suggests that urinary collecting system anomaly may be an integral part of distal 10q trisomy syndrome. We recommend detailed imaging studies of urinary tracts be performed on probands with chromosomal disorders involving a duplication of distal 10q. PMID:14577683
The cognitive deficits are described in a 20-year-old right-handed man with asymmetrical bilateral thalamic lesions and a lesion resulting in the 'locked-in' syndrome. Memory and intellectual assessment, modified due to the physical and communication difficulties, suggested that the patient had little impairment of verbal intelligence and performed normally on memory test involving immediate recall of new material. There was, however, considerable impairment of organization, planning and in the recall of visual and especially verbal memory, over longer periods. Remote memory was relatively intact, except for chronological errors in time-tagged material, and he was disoriented for year, month and day. PMID:11350659
Background. Cogan's syndrome is a rare clinical entity whose etiopathology is still unknown, and the treatment strategies are not clearly defined. Case. A 23-year-old male presented with symptoms of headache, peripheral facial palsy, persistent right hearing loss and bilateral papillitis. Workup excluded all infectious, granulomatous, neoplastic, and immune causes. The diagnosis of atypical Cogan's syndrome was established, and the patient was treated with systemic corticosteroids and later on with cyclophosphamide and methotrexate. There were improvement of visual symptoms and stabilisation of left hearing. Conclusion. Cogan's syndrome is a very rare disease with no specific biological tests for the diagnosis. The diagnostic exams are mostly important to exclude other etiologies. The atypical ocular and audiovestibular manifestations make the diagnosis difficult, delaying the institution of appropriate therapy which may result in profound bilateral deafness.
Bilateral cleft lip nose deformity is a multi-factorial and complex deformity which tends to aggravate with growth of the child, if not attended surgically. The goals of primary bilateral cleft lip nose surgery are, closure of the nasal floor and sill, lengthening of the columella, repositioning of the alar base, achieving nasal tip projection, repositioning of the lower lateral cartilages, and reorienting the nares from horizontal to oblique position. The multiplicity of procedures in the literature for correction of this deformity alludes to the fact that no single procedure is entirely effective. The timing for surgical intervention and its extent varies considerably. Early surgery on cartilage may adversely affect growth and development; at the same time, allowing the cartilage to grow in an abnormal position and contributing to aggravation of deformity. Some surgeons advocate correction of deformity at an early age. However, others like the cartilages to grow and mature before going in for surgery. With peer pressure also becoming an important consideration during the teens, the current trend is towards early intervention. There is no unanimity in the extent of nasal dissection to be done at the time of primary lip repair. While many perform limited nasal dissection for the fear of growth retardation, others opt for full cartilage correction at the time of primary surgery itself. The value of naso-alveolar moulding (NAM) too is not universally accepted and has now more opponents than proponents. Also most centres in the developing world have neither the personnel nor the facilities for the same. The secondary cleft nasal deformity is variable and is affected by the extent of the original abnormality, any prior surgeries performed and alteration due to nasal growth. This article reviews the currently popular methods for correction of nasal deformity associated with bilateral cleft lip, it's management both at the time of cleft lip repair and also secondarily, at a later date. It also discusses the practices followed at our centre.
A 12-year-old patient presented with a severe delay of eruption in permanent maxillary and mandibular incisors. On examination, there was over-retained primary teeth and delayed eruption of permanent teeth. Retained primary teeth showed light yellow discolouration whereas permanent teeth were distinct yellow with thin or little enamel. Subsequent imaging revealed all the premolars except maxillary left first premolar showed signs of intra-alveolar coronal resorption, nephrocalcinosis with bilateral multiple calculi and small papillary tip calcifications, marked increase in alkaline phosphatase. Subsequent dental treatment for restoring the functional and aesthetic requirement followed by appropriate treatment for renal problem was undertaken. PMID:23709541
A 28-year-old woman presented at eight weeks and four days of gestation, according to her menstrual dates, complaining of painless vaginal bleeding for three days. Her urinary pregnancy test was positive. Initial transvaginal ultrasound demonstrated an irregular complex structure with a fluid filled centre in the right adnexum. Despite the diagnosis of a possible underlying unruptured right tubal ectopic pregnancy, she declined surgical intervention and was managed expectantly as an inpatient. When she complained of increasing abdominal pain with haemodynamic instability, an emergency laparotomy was performed and a diagnosis of bilateral tubal ectopic pregnancy was made. PMID:19391453
Marasinghe, Jeevan P; Condous, George; Amarasinghe, W I
Pituitary apoplexy followed by cerebral infarction is rare. We report a 59-year-old male with a known pituitary macroadenoma who was admitted to our emergency department for treatment of an acute myocardial infarction. He underwent coronary angioplasty and was subsequently treated with aspirin, clopidogrel and full-dose enoxaparin. He developed pituitary apoplexy with bilateral compression of both internal carotid arteries, and infarction of both anterior and middle hemispheres; consequently, he died. This patient illustrates the difficulties of administering aggressive anticoagulative and antiplatelet therapy to patients who have a known pituitary adenoma. PMID:19800239
Lill, C M; Hoch, H; Dieste, F-J; Vogel, H-P; Zipp, F; Paul, F
: 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
Introduction Premenstrual symptoms occur in 95% of women of reproductive age. Severe, debilitating symptoms (PMS) occur in about 5% of those women. There is no consensus on how symptom severity should be assessed, which has led to a wide variety of symptoms scales, making it difficult to synthesise data on treatment efficacy. The cyclical nature of the condition also makes it difficult to conduct RCTs. Methods and outcomes We conducted a systematic review and aimed to answer the following clinical questions: What are the effects of drug treatments in women with premenstrual syndrome? What are the effects of hormonal treatments in women with premenstrual syndrome? What are the effects of psychological interventions in women with premenstrual syndrome? What are the effects of physical therapy in women with premenstrual syndrome? What are the effects of dietary supplements in women with premenstrual syndrome? What are the effects of surgical treatments in women with premenstrual syndrome? We searched: Medline, Embase, The Cochrane Library, and other important databases up to July 2009 (Clinical Evidence reviews are updated periodically; please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA). Results We found 56 systematic reviews, RCTs, or observational studies that met our inclusion criteria. We performed a GRADE evaluation of the quality of evidence for interventions. Conclusions In this systematic review, we present information relating to the effectiveness and safety of the following interventions: acupuncture; alprazolam; bright light therapy; buspirone; chiropractic manipulation; clomipramine; cognitive behavioural therapy (CBT); danazol; endometrial ablation; evening primrose oil; exercise; gonadorelin analogues; hysterectomy; laparoscopic bilateral oophorectomy; magnesium supplements; metolazone; non-steroidal anti-inflammatory drugs (NSAIDs); oestrogens; oral contraceptives; progesterone; progestogens; pyridoxine; reflexology; relaxation; selective serotonin reuptake inhibitors (SSRIs); spironolactone; and tibolone.
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.
PURPOSE: To describe a patient who developed bilateral subconjunctival and orbital emphysema after an automobile tire explosion.METHOD: Case report.RESULTS: A 60-year-old man sustained bilateral ocular injury after a tire explosion. Ophthalmic examination disclosed bilateral subconjunctival air, with no visible conjunctival laceration. Computed tomography showed orbital emphysema, with no evidence of orbital fracture. Follow-up examination 2 weeks after the injury disclosed
We report a patient with a diagnosis of systemic lupus erythematosus who concurrently developed a syndrome of thrombotic microangiopathy that resembled thrombotic thrombocytopenic purpura. The patient underwent plasma exchange and immunosuppressive therapy for months before clinical improvement was finally achieved through bilateral nephrectomy. Ultimately, our patient died of disseminated aspergillosis from prolonged immunosuppression. We believe that recognition of bilateral nephrectomy as a potential treatment earlier in her course would have spared her this unfortunate demise. We hope that this review of current literature will help the reader to consider bilateral nephrectomy in patients with refractory systemic lupus erythematosus with clinical overlap of thrombotic microangiopathy resembling thrombotic thrombocytopenic purpura. PMID:22647858
Mai, Tuan Van; Tse, Kevin; Gehr, Marc Kenneth; George, James; Baranski, Joel; Xavier, Marin
Brachial neuritis with bilateral hemidiaphragmatic paralysis has been reported in two previous cases in the literature. We report a patient who experienced severe right shoulder discomfort three weeks prior to hospital admission which evolved to include both shoulders. Two weeks prior to admission he noticed the onset of discomfort in breathing in the supine position and shortness of breath with minor exertion. The admitting diagnoses were myocardial infarction due to significant ECG changes and idiopathic elevated bilateral hemidiaphragms. The patient had findings significant for tachypnea, dyspnea, decreased breath sounds at the bases bilaterally, impaired motion of the bilateral lung bases on inspiration and paradoxical respirations. Comprehensive medical testing and evaluation revealed bilateral elevated hemidiaphragms and vital capacity 40% of normal. Weakness of the proximal shoulder girdle and bicep musculature bilaterally was noted. Electromyography was significant for reduced recruitment pattern in the bilateral shoulder girdle musculature. Nerve conduction studies suggested bilateral phrenic neuropathy. This case is an unusual presentation of brachial neuritis affecting the bilateral shoulder girdle with phrenic nerve involvement. The differential diagnosis of acute shoulder pain associated with respiratory symptomatology should therefore include brachial neuritis. PMID:3800625
Bilateral adrenal hemorrhage is a rare and potentially life threatening situation in adults. The clinical presentation is non-specific, and the diagnosis is based on imaging. The purpose of this report is to provide an illustrative case of spontaneous bilateral adrenal hemorrhage that occurred during pregnancy. The sonographic and computed tomographic findings included large bilateral adrenal hematomas with no evidence of underlying malignancy. Since bilateral adrenal hemorrhage is a rare but potentially life threatening situation, prompt laboratory and imaging evaluation are essential and may reduce both morbidity and mortality. PMID:15205660
Aims To estimate the occurrence of early cataract among patients with Down's syndrome and to evaluate the clinical characteristics of the cases. Methods Cases with Down's syndrome were ascertained from a cohort of all Danish children between 0 and 17?years of age, who were diagnosed with cataract during the period 1977–2001 (n?=?1027). Information on the patients was obtained from the medical records. Results Of the total of 1027 cases with non?traumatic, non?acquired cataract there were 29 cases (13 males, 16 females) with Down's syndrome (2.8%). This corresponds to an occurrence of early cataract among patients with Down's syndrome of 1.4%; 27 had bilateral cataract and two had unilateral cataract. Half of the patients (n?=?14) underwent cataract surgery, of whom two had bilateral primary lens implantation. 10 patients had bilateral cataract observed soon after birth, and five of these underwent cataract surgery within the first 6?months of life. Conclusion The frequency of early cataract among children with Down's syndrome is estimated to be 1.4%, with cataracts requiring surgery during childhood being even rarer. In one third of the 29 cases, bilateral cataract was detected in the neonatal period.
Intravascular migration of bullets and other foreign bodies is a rare but known complication of penetrating trauma. Missile embolization can represent a diagnostic challenge because it may present in various and unexpected ways. We present the case of a 54-year-old female who sustained shotgun pellet emboli to the pulmonary arteries following a left upper extremity gunshot wound and related vascular surgery. The case illustrates bilateral embolization, and the embolic events occurred following surgery. Embolization should be considered in evaluating patients with gunshot wounds, particularly if there are anomalous symptoms or the projectile is not found in the original, or expected, location. Close attention to the location of the foreign bodies on serial radiographs may reveal the diagnosis of intravascular embolization.
We report on an unusual case of laterocervical bilateral metastatatic masses with unknown clinical, radiological or computer tomographicic detected primary site of origin. Cancer of an unknown primary site is a clinical syndrome, accounting for 2%-5% of patients with cancer. The peculiarities of our case are its evolution as fast-growing bilateral tumor masses with involvement of other neck structures and its unexpected origin from the lung, certified by complementary immunohistochemical tests following surgery, in the absence of any other clinical signs or any detectable lung tumor mass by radiological or computer tomographic tests. PMID:23606699
For the first time, Kluver Bucy syndrome (KBS) is described in young children who had no environmental learning of sex. The syndrome has so far been noted only in adults after bilateral temporal lobe affection. A few of its components, especially the hypersexuality and hypermetamorphosis, are likely to manifest differently in very young children. Seven patients in the pre-pubertal age
Two patients with Behçet's syndrome are described. One patient developed spontaneous bilateral popliteal artery aneurysms and the other spontaneous thrombosis of the superior vena cava. These cases emphasize that primary inflammatory lesions of large vessels, with arterial aneurysm formation and thrombosis may be a feature of the syndrome.
B. Anthony Enoch; T. C. L. Khoo; J. L. Castillo-Olivares; R. G. Grainger; L. Henry
A 77-year-old man, with a history of lymphoma, presented with isolated neck extensor weakness and a 2-year history of bilateral carpal tunnel syndrome (CTS). Needle electromyography showed myopathic changes, and biopsy of cervical paraspinal muscles showed amyloid deposition in blood vessels. Amyloidosis should be considered in the differential diagnosis of dropped head syndrome. PMID:21607974
Bilateral serous retinal detachments are rare complications of thrombotic thrombocytopenic purpura (TTP). We report the case of a 28-year-old woman with chronic relapsing TTP with bilateral serous retinal detachments, which resolved after prompt aggressive medical therapy. Unlike serveral previous reports, the retinal detachments in our patient were unrelated to systemic hypertension.
Richard E. Wyszynski; K. Ellen Frank; Hans E. Grossniklaus
Bilateral adrenal hemorrhage with subsequent adrenal insufficiency is a recognized complication of anticoagulant therapy. Because the clinical manifestations are often nonspecific, the antemortem diagnosis of adrenal hemorrhage has been a difficult clinical problem. Computed tomography (CT) provides detailed images of the adrenal glands that are not possible with conventional imaging methods. The CT findings of bilateral adrenal hemorrhage in an anticoagulated patient are reported.
Ling, D.; Korobkin, M.; Silverman, P.M.; Dunnick, N.R.
Women who develop bilateral breast cancer at an early age are likely to harbour germline mutations in breast cancer susceptibility genes. The aim of this study was to test for concordant genetic changes in left and right breast cancer of young women (age <50) with bilateral breast cancer that may suggest an inherited breast cancer predisposition. Microsatellite markers were used
J. Kollias; S. Man; M. Marafie; K. Carpenter; S. Pinder; I. O. Ellis; R. W. Blamey; G. Cross; J. D. Brook
Bilateral parotid swelling is not an uncommon occurrence and may pose a challenge for clinicians and radiologists. Numerous causes of bilateral parotid swellings have been identified. The purpose of this pictorial review is to display this wide array with a focus on multimodality approach.
Gadodia, A; Bhalla, A S; Sharma, R; Thakar, A; Parshad, R
|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…
McLean, Scott P.; Vint, Peter F.; Stember, Amanda J.
Background A 56-year-old male with bilateral flank pain was admitted following diagnosis of bilateral kidney stone disease. He had previously undergone four separate pyelolithotomy sessions (two per kidney), the latest being 14 years prior. At presentation the patient had two stones in the right kidney and seven in the left kidney. The right renal pelvis was dilated, indicating obstruction of
Yahya Murat U?ra?; Ali Güne?; Can Baydinç; Ahmet Soylu
This paper presents a new approach for hyperspectral image visualization. A bilateral filtering-based approach is presented for hyperspectral image fusion to generate an appropriate resultant image. The proposed approach retains even the minor details that exist in individual image bands, by exploiting the edge-preserving characteristics of a bilateral filter. It does not introduce visible artifacts in the fused image. A
Breast cancer in males is rare, accounting for less than 1% of all cases. Bilateral male breast cancer is reported to occur in fewer than 2% of all the diagnosed cases of male breast cancer, and synchronous tumors are exceedingly rare. The authors report a case of male breast cancer that is bilateral and syn- chronous, and review the literature
PETER B. KAHLA; SEBASTIANO CASSARO; FELIX G. VLADIMIR; MICHAEL G. WAYNE; ANGELO CAMMARATA
In this paper, we will consider the current role of simultaneous-bilateral TKA. Based on available evidence, it is our opinion that simultaneous bilateral TKA carries a higher risk of morbidity and mortality and should be reserved for select few. PMID:23118391
OBJECTIVES:: The aim of the study was to investigate whether children with bilateral conductive hearing loss benefit from their second device (i.e., the bilateral bone conduction device [BCD]). DESIGN:: Speech recognition in noise was assessed in 10 children fitted with bilateral BCDs during childhood. Speech recognition was measured in 2 conditions with both BCDs active. Spatial resolution was tested with the Minimum Audible Angle test in the bilateral and monaural listening conditions. RESULTS:: Children demonstrated an improvement in speech recognition when speech was presented from the front and noise was presented from the right-hand side as compared with both speech and noise being presented from the front. The minimum audible angle decreased from 57° in the best monaural condition to 13° in the bilateral condition. CONCLUSIONS:: The audiological outcomes demonstrate the advantage of bilateral BCD fitting in children with bilateral conductive hearing loss. PMID:23698625
Dun, Catharina A J; Agterberg, Martijn J H; Cremers, Cor W R J; Hol, Myrthe K S; Snik, Ad F M
We report acute thromboembolic events in a 14-year-old boy with Down syndrome and repaired atrioventricular septal defect.\\u000a He presented with sudden onset of bilateral lower limb ischemia. Transesophageal echocardiography detected a thrombus in the\\u000a right atrium. An arterial saddle embolus was removed following bilateral iliac embolectomy. Despite anticoagulation, he presented\\u000a again with sudden bilateral loxwer limb ischemia and respiratory distress.
U. R. Mohan; J. S. Mangat; N. Sutaria; R. C. G. Franklin
The fetal consequences of chickenpox complicating pregnancy depends on the period of gestation at which the infection is contracted. The extremely rare classical form of congenital varicella syndrome, resulting from maternal varicella infection in the first trimester of pregnancy, is being reported here. The unusual features in this baby are bilateral hypoplastic lower extremities, fracture of bones, a normal electroencephalogram and phantom hernias of the anterior abdominal wall. PMID:12619963
Lemierre's syndrome is a suppurative thrombophlebitis involving the internal jugular vein, most commonly associated with Fusobacterium necrophorum, usually a complication of oropharyngeal infections. This syndrome is rare and is often overlooked. We present a case of sepsis mimicking initially severe leptospirosis (Weil's disease) due to acute febrile illness with multiorgan failure and hyperbilirubinemia. Finally, blood cultures revealed Fusobacterium necrophorum and computed tomography (CT) demonstrated bilateral pulmonary nodules and a thrombus in the right internal jugular vein. Early clinical suspicion is crucial so that appropriate diagnostic investigation and antibiotic therapy can be initiated to minimize the risk of life-threatening complications.
The Ochoa or urofacial syndrome is a disease characterized by non-neurogenic bladder dysfunction and unusual facial expressions when smiling or crying. It is an extremely rare disorder with over 150 cases reported in the medical literature. This condition has been determined to be inherited by an autosomal recessive pattern. We present radionuclide renogram and renal scan of a boy with a history of incontinence, frequent infections of the urinary tract, and gene mutations consistent with this syndrome. Nuclear medicine images showed extensive bilateral renal scarring and obstructive pattern in diuretic renogram. PMID:23698458
Infante, Jose Rafael; Rayo, Juan I; Serrano, Justo; Domínguez, María L; García, Lucía; Durán, Carmen
Persistent Mullerian duct syndrome (PMDS) is a rare form of internal male pseudohermaphroditism in which Mullerian duct derivatives are seen in a male patient. This syndrome is characterized by the persistence of Mullerian duct derivatives (i.e. uterus, cervix, fallopian tubes and upper two thirds of vagina) in a phenotypically and karyotypically male patient. In this article we present the USG and MRI features of a case of PMDS with bilateral cryptorchidism and left sided inguinal hernia, containing the uterus and fallopian tubes.
Adrenal myelolipomas are rare, usually benign unilateral tumors. Their management has controversially been discussed. The authors here present a 53-year-old African American female Jehovah's witness with postmenopausal uterine bleeding on chronic anticoagulation and episodic right flank pain who was found to have bilateral myelolipomas in addition to primary hyperparathyroidism. In collaboration with gynecology, midline laparotomy was performed to remove uterus and both ovaries in addition to the right adrenal gland for a 62 × 79 mm myelolipoma. An open biopsy of the left adrenal mass measuring 42 × 43 mm revealed myelolipoma and ruled out malignancy. Pathology confirmed bilateral myelolipomas, endometrial polyps, and leiomyomata uteri. After an uneventful recovery, the patient then underwent a right inferior parathyroidectomy for parathyroid adenoma. This case illustrates the challenges in deciding when to surgically intervene for bilateral adrenal myelolipoma and, for the first time, associates various other endocrinopathies, although no known endocrine neoplasia syndrome variant was present. PMID:23426084
Saunders, Rachel N; Koch, Christian A; Brown, Kathryn B; Hairston, Frances J; Daley, William P; Ahmed, Naveed; Vanderlan, Wesley B
In this paper, we propose a simple but effective shadow removal method using a single input image. We first derive a 2-D intrinsic image from a single RGB camera image based solely on colors, particularly chromaticity. We next present a method to recover a 3-D intrinsic image based on bilateral filtering and the 2-D intrinsic image. The luminance contrast in regions with similar surface reflectance due to geometry and illumination variances is effectively reduced in the derived 3-D intrinsic image, while the contrast in regions with different surface reflectance is preserved. However, the intrinsic image contains incorrect luminance values. To obtain the correct luminance, we decompose the input RGB image and the intrinsic image. Each image is decomposed into a base layer and a detail layer. We obtain a shadow-free image by combining the base layer from the input RGB image and the detail layer from the intrinsic image such that the details of the intrinsic image are transferred to the input RGB image from which the correct luminance values can be obtained. Unlike previous methods, the presented technique is fully automatic and does not require shadow detection. PMID:22829402
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.
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
A 55-year-old man with a subacute onset of slurred speech, ataxia, nystagmus, extrapyramidal rigidity, decreased tendon reflexes, vomiting, bilateral optic atrophy, and clonic jerks died of bronchopneumonia and respiratory failure. Neuropathological examination showed lesions characteristic of subacute necrotizing encephalopathy. Clinicopathological observations of reported cases of Leigh's syndrome in the adult are reviewed.
E. Masó; I. Ferrer; J. Herraiz; J. Roquer; S. Serrano
We report here the case of a 17 year-old girl with the classic signs of Goldenhar syndrome in the form of multiple accessory tragi, bilateral ocular dermoids, mandibular hypoplasia (micrognathia) and cervical lordosis. She also had a high arched palate, gingival hypertrophy and malaligned teeth, features which are as yet unreported. PMID:18583796
Oro-facial-digital syndrome type II (OFD-II) is characterized by frenulated tongue, midline cleft lip, high arched or cleft palate, micrognathia, syndactyly and polydactyly, bilateral reduplicated hallux, conductive hearing loss, choroidal coloboma and normal intelligence. There are nine forms of oro-facial-digital syndromes with different modes of inheritance. A young female with features of oro-facio-digital syndrome type-II is being reported. PMID:23777026
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.
Doody, O., E-mail: firstname.lastname@example.org [Alfred Hospital, Department of Radiology (Australia); Adam, W. R. [University of Melbourne and Goulburn Valley Health, School of Rural Health (Australia); Foley, P. T.; Lyon, S. M. [Alfred Hospital, Department of Radiology (Australia)
To describe the clinical course and pathological diagnosis of a 12-year-old female who presented with an acute syndrome of right hemispheric epilepsy and cortical dysfunction and brain MRI demonstrating atrophy of the left cerebral and right cerebellar hemispheres. The patient presented with occasional partial seizures consisting of a left calf sensation followed by left leg clonic jerking. Initial brain MRI showed left cerebral and right cerebellar atrophy with T2 hyperintensity in the left parietal region. After six months, the seizure frequency increased and semiology evolved to include frequent clonic movements of the left side of the face, arm and leg and epilepsia partialis continua (EPC) of the left arm and leg. There was progressive weakness of the left leg and, to a lesser extent, her left arm. MRI at this time demonstrated an additional T2 hyperintensity in the right frontal lobe. An extensive evaluation for paraneoplastic, mitochondrial, and genetic epilepsy syndromes was unrevealing. On biopsy evaluation, chronic T-cell mediated encephalitis was demonstrated within bilateral frontal lobes. Treatment with immunomodulatory therapy resulted in some improvement in her seizure frequency and motor function. Rasmussen's encephalitis can be a challenging diagnosis. The patient's clinical history, including EPC, with bilateral frontal lobe biopsies confirming a T-cell mediated encephalitis supports a diagnosis of bilateral Rasmussen encephalitis. This case highlights the diagnostic challenges and treatment dilemmas that arise in an adolescent presenting with bilateral inflammatory lesions of Rasmussen's encephalitis. [Published with video sequences]. PMID:23917753
Peariso, Katrina; Standridge, Shannon M; Hallinan, Barbara E; Leach, James L; Miles, Lili; Mangano, Francesco T; Greiner, Hansel M
A 30-year-old woman with severe preeclampsia presented at 27 weeks of amenorrhea with left headache, neck pain, blurred vision and numbness of left hemiface that resolved spontaneously within 2 hours. A week later, hypertension remained poorly controlled despite combination of nicardipine and labetalol intravenous therapy; an urgent caesarean section was eventually performed due to onset of HELLP syndrome. At day 5 postpartum, the patient had a Horner syndrome with right ipsilateral disabling tinnitus. A CT-angiography of supra-aortic trunks was performed urgently; it showed a bilateral carotid arterial dissection without stroke, which was subsequently confirmed by MRI angiography. The patient was transferred in neurovascular intensive care unit. Anticoagulant therapy was implemented to prevent cerebral and retinal ischemic lesions. Symptoms resolved quickly and the patient was discharged at day 7 postpartum. MR-angiography performed 4 months later showed a full resolution of the bilateral carotid dissection. Anticoagulant therapy was therefore discontinued. PMID:23498556
Hoffmann, C; Augé, M; Falzone, E; Martel-Jacob, S; Mercier, F J
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
The hippocampus has long been known to be important for memory, with the right hippocampus particularly implicated in nonverbal/visuo-spatial memory and the left in verbal/narrative or episodic memory. Despite this hypothesized lateralized functional difference, there has not been a single task that has been shown to activate both the right and left hippocampi differentially, dissociating the two, using neuroimaging. The transverse patterning (TP) task is a strong candidate for this purpose, as it has been shown in human and nonhuman animal studies to theoretically and empirically depend on the hippocampus. In TP, participants choose between stimuli presented in pairs, with the correct choice being a function of the specific pairing. In this project, TP was used to assess lateralized hippocampal function by varying its dependence on verbal material, with the goal of dissociating the two hippocampi. Magnetoencephalographic (MEG) data were collected while controls performed verbal and nonverbal versions of TP in order to verify and validate lateralized activation within the hippocampi. Schizophrenia patients were evaluated to determine whether they exhibited a lateralized hippocampal deficit. As hypothesized, patients' mean level of behavioral performance was poorer than controls' on both verbal and nonverbal TP. In contrast, patients had no decrement in performance on a verbal and nonverbal non-hippocampal-dependent matched control task. Also, controls but not patients showed more right hippocampal activation during nonverbal TP and more left hippocampal activation during verbal TP. These data demonstrate the capacity to assess lateralized hippocampal function and suggest a bilateral hippocampal behavioral and activation deficit in schizophrenia. PMID:21763438
Hanlon, Faith M; Houck, Jon M; Pyeatt, Clinton J; Lundy, S Laura; Euler, Matthew J; Weisend, Michael P; Thoma, Robert J; Bustillo, Juan R; Miller, Gregory A; Tesche, Claudia D
Paravertebral nerve blocks (PVBs) can provide excellent intraoperative anaesthetic and postoperative analgesic conditions with less adverse effects and fewer contraindications than central neural blocks. Most published data are related to unilateral PVB, but its potential as a bilateral technique has been demonstrated. Bilateral PVB has been used successfully in the thoracic, abdominal, and pelvic regions, sometimes obviating the need for general anaesthesia. We have reviewed the use of bilateral PVB in association with surgery and chronic pain therapy. This covers 12 published studies with a total of 538 patients, and with varied methods and outcome measures. Despite the need for relatively large doses of local anaesthetics, there are no reports of systemic toxicity. The incidence of complications such as pneumothorax and hypotension is low. More studies on the use of bilateral PVB are required. PMID:21233114
Bilateral total renal dysplasia is an extremely rare congenital anomaly associated with Potter's facies. We report an autopsy case of a stillborn male child. Histopathology is showing persistence of structures not seen in normal development. PMID:15471131
Zawar, M P; Kurdukar, M D; Mashal, S N; Inamdar, J S
We report on a unique combination of multiple variations concerning the pectoral muscles and the left external jugular vein. Specifically, a bilateral hypoplasia of the medial clavicular portion of the pectoralis major muscle was noticed along with the coexistence of total right pectoralis minor aplasia, substituted by loose connective and fatty tissue. Simultaneously, a supernumerary anterior-placed external jugular vein was found, which, after its supraclavicular course, pierced the interval between the left clavicular and the sternocostal head, and drained into the left jugular junction. The combination of the above anomalies constitutes an atypical pattern of Poland syndrome. We discuss the related embryological development and the relative literature. Attention was paid to the clinical importance for plastic surgeons, general surgeons, and radiologists, facilitating them with accurate interpretation of anterior thoracic wall findings. PMID:21154292
Acute renal infarction is still an underdiagnosed pathology. Most cases are secondary to arterial embolism in patients with atrial fibrillation or other cardiac illnesses; however, a less known etiology is the vascular affection of systemic lupus erythematosus (SLE). Renal infarction in lupus patients normally appears with positive antiphospholipid antibodies or lupus anticoagulant in the context of an antiphospholipid syndrome (APS). This is characterized by a state of hypercoagulability potentially affecting all segments of the vascular bed with thrombosis. A differential diagnosis with lupus nephritis, a very common pathology in SLE patients, must be carried out. We have to suspect this pathology in patients with SLE and APS who come to the emergency department complaining of abdominal pains or a renal colic. We present the case of a 69-year-old woman who was diagnosed of bilateral segmental renal infarction in the context of recently diagnosed SLE with no other vascular manifestations.
Padilla-Fernandez, Barbara; Garcia-Casado, Diana; Martin-Izquierdo, Manuela; Manzano-Rodriguez, Carmen; Garcia-Garcia, Javier; Lorenzo-Gomez, Maria F.
The acquisition of selected motor skills was studied in a 40-year-old man with a severe amnestic syndrome resulting from a bilateral medial temporal-lobe resection carried out 13 years before. On Rotary Pursuit, Bimanual Tracking, and Tapping, his scores improved from session to session, and on the one task where it was feasible to retest several days after the end of
Spontaneous intracranial hypotension (SIH) is a well-documented syndrome characterized typically by a benign, self-limited\\u000a course. Patients typically present with postural or exertional headaches that can be temporarily relieved by lying in a supine\\u000a or recumbent position. A 35-year-old Caucasian male suffered orthostatic headache that developed to a bilateral abducens palsy.\\u000a We ordered relative rest and the patient improved and completely
A bilateral nuclear-weapons freeze to stop the arms race is, next to the economy, the major public issue. The author feels that a freeze would lessen the risk of launch-on-warning errors that could lead to nuclear war. Further, a bilateral freeze would preserve the parity of existing US-Soviet arms, but prevent the destabilization of a new counterforce capability. It must
Choriocarcinoma is the most malignant tumor of gestational trophoblastic neoplasia. It grows rapidly and metastasizes to the lung, liver, and less frequently, the brain. Metastases to the kidney are rare in the literature, and bilateral involvement is even more scarce. Renal involvement of choriocarcinoma is highly exceptional and may mimic renal cell carcinoma. Here we report a case of bilateral renal choriocarcinoma presenting 5 years after a history of a total anterior hysterectomy because of a hydatidiform mole.
During routine dissection of a 60 years male cadaver, it was observed that the two divisions of sciatic nerve were separate in the gluteal region on both the sides with the tibial nerve passing below the piriformis and the common peroneal nerve piercing the piriformis muscle. The abnormal passage of the sciatic nerve (SN), the common peroneal nerve (CPN), and the tibial nerve (TN), either through the piriformis or below the superior gemellus may facilitate compression of these nerves. Knowledge of such patterns is also important for surgeons dealing with piriformis syndrome which affects 5-6% of patients referred for the treatment of back and leg pain. A high division may also account for frequent failures reported with the popliteal block. PMID:22049898
We report the clinical findings of a patient with severe bilateral keratectasia 34 years after a penetrating keratoplasty (PK) in both eyes. An otherwise healthy 67-year-old man complained of deterioration of the eyesight in both eyes over the last 6 months. The patient was diagnosed with bilateral keratoconus at the age of 32 years, and he underwent a bilateral PK. At presentation, visual acuity was 20/200 in the right eye and light perception in the left eye. A Pentacam pachymetric map revealed a central pachymetry of 720 mum in the right eye and of 710 mum in the left eye, as well as an average paracentral pachymetry of 436 and 270 mum in the 9-mm zone in the right and the left eye, respectively. Corneal topography revealed bilateral irregular and asymmetric bowing with generalized steepening and high corneal power. We describe a case of bilateral keratectasia 34 years after PK in a patient who was originally diagnosed with bilateral keratoconus. PMID:20737056
Valldeperas, Xavier; Angi, Martina; Romano, Vito; Romano, Mario R
We report the clinical findings of a patient with severe bilateral keratectasia 34 years after a penetrating keratoplasty (PK) in both eyes. An otherwise healthy 67-year-old man complained of deterioration of the eyesight in both eyes over the last 6 months. The patient was diagnosed with bilateral keratoconus at the age of 32 years, and he underwent a bilateral PK. At presentation, visual acuity was 20/200 in the right eye and light perception in the left eye. A Pentacam pachymetric map revealed a central pachymetry of 720 ?m in the right eye and of 710 ?m in the left eye, as well as an average paracentral pachymetry of 436 and 270 ?m in the 9-mm zone in the right and the left eye, respectively. Corneal topography revealed bilateral irregular and asymmetric bowing with generalized steepening and high corneal power. We describe a case of bilateral keratectasia 34 years after PK in a patient who was originally diagnosed with bilateral keratoconus.
Valldeperas, Xavier; Angi, Martina; Romano, Vito; Romano, Mario R.
In studies aimed at local treatment of experimental osteoarthritis (OA) it is optimal to have an internal (untreated) OA control. Such an approach excludes interanimal variation, and allows paired statistical evaluation of treatment efficacy. For this purpose, we developed and characterized a bilateral version of the canine Groove model. We hypothesized that the bilateral version of the canine Groove model would show consistent and clear development of features of OA similar to those found in the unilateral version. In six Beagle dogs, grooves were surgically made in the articular cartilage of the femoral condyles of both knee joints. Six additional dogs underwent bilateral sham surgery. The degree of OA was quantified 20 weeks after surgery and was compared in retrospect to 23 animals that undergone the same procedure in a single knee joint with the contralateral knee serving as a non-OA control. Bilateral groove surgery resulted in OA. This was based on the observed ineffective repair response in which an increase in proteoglycan synthesis, a diminished retention of these newly formed proteoglycans, and an enhanced loss of resident proteoglycans resulted in a decreased cartilage proteoglycan content. These biochemical effects were corroborated by clear histological features of OA. All these effects were found in femor as well as in the (surgically untouched) tibia. Interestingly, features of OA were slightly more severe in the bilateral model than in the unilateral variant. The bilateral canine Groove model showed consistent and clear development of features of OA, comparable to the unilateral model. PMID:18473386
Intema, Femke; DeGroot, Jeroen; Elshof, Bram; Vianen, Marieke E; Yocum, Sue; Zuurmond, Annemarie; Mastbergen, Simon C; Lafeber, Floris P
In bilateral thumb amputations, the functional impairment is serious and every attempt should be made to reconstruct the thumb. We report a case of bilateral post traumatic thumb amputation, reconstructed with bilateral second toe transfer. Only two such cases have been reported in literature so far. Though there are various modalities for the reconstruction of thumb, microvascular toe transfer has its own merits. The convalescent period is minimal with excellent function. It is bilaterally symmetric and aesthetically superior to the osteoplastic reconstruction. The technical details are discussed, and the long term functional and aesthetic results are presented.
Two brothers of normal not consanguineous parents, with bilateral intrabdominal cryptorchidism, were admitted to our Institution. Both children had short stature, limb and hand malformations and craniofacial patterns of Robinow syndrome. During the orchidopexy, bilateral epididymal and vasal abnormalities were found in both of them. This anomaly associated with Robinow syndrome has never been reported before. These two cases provides the Authors with the opportunity of reviewing clinical features, genetics and radiological patterns of this rare syndrome. PMID:9312747
Fabbro, M A; D'Agostino, S; Costa, L; Musi, L; Cappellari, F
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.
The advantages of sequential bilateral cochlear implantation were assessed in 29 children with a severe to profound hearing loss. The effect of age at second implantation and the effect of duration of bilateral implant use on the outcomes in speech perception and directional hearing were investigated. The children received their second cochlear implant at an age ranging from 2.8 to 8.5 years. Measurements were carried out preoperatively and postoperatively after 6, 12 and 24 months of bilateral implant use. A matched control group of 9 children with a unilateral implant were also measured over time and were compared with the study group after 12 and 24 months. Speech reception in both quiet and in noise and lateralization were measured. After 24 months, a minimum audible angle task was carried out. Bilateral advantages with regard to speech reception in quiet and in noise were already present after 6 months of bilateral implant use and improved thereafter. After 24 months, speech reception in noise had significantly improved with bilateral implants compared to that of children with a unilateral implant. The percentage of children that could accurately lateralize increased from 57% after 6 months to 83% after 24 months. With regard to the minimum audible angle task, loudspeakers were placed on average at ±42°. Age at second implantation did not have an influence on all outcomes. From the results it can be concluded that the advantages of bilateral hearing occur after sequential bilateral implantation and that age at second implantation does not influence the amount of bilateral advantage. Furthermore, it can be concluded that longer periods of bilateral implant use lead to greater bilateral advantages. PMID:20980740
Sparreboom, Marloes; Snik, Ad F M; Mylanus, Emmanuel A M
To assess the association between unilateral carpal tunnel syndrome and space-occupying lesions, 128 patients have been reviewed. They were divided into bilateral, subclinical (unilateral signs and symptoms, and bilateral slowing in the median nerve conduction) and unilateral carpal tunnel syndrome. Space-occupying lesions were investigated on the basis of physical examination and wrist imaging using plain radiographs and ultrasonograms. Of 20 patients with unilateral carpal tunnel syndrome, space-occupying lesions were found in seven (occult ganglion in five and occult calcified mass in two). In contrast, none of 89 patients with bilateral carpal tunnel syndrome and 19 with subclinical carpal tunnel syndrome had space-occupying lesions. We conclude that careful examination and wrist imaging on suspicion of local pathology, especially a space-occupying lesion, are needed when the condition is unilateral and the aetiology is not clear from the history and on physical examination. PMID:8308434
We describe a case of nevus sebaceus syndrome characterized by a linear sebaceous nevus, extending on the left half of the face from forehead to lower neck, including the auricle; alopecia on the temporoparietal region; generalized hypotonia; insufficiency of eyes in following moving objects and insufficiency in controlling the head; lipodermoid cysts in the left and right eyes; bilateral conjunctival masses; corneal vascularization in the right eye; coloboma of the left eyelid and right optic disc. This is a rare case of a sebaceous nevus occurring concomitantly with optical and central nervous system abnormalities. PMID:8519188
Küçüködük, S; Ozsan, H; Turanli, A Y; Dinç, H; Selçuk, M
Twenty-four flexor carpi radialis (FCR) tendon interposition arthroplasties of the trapezium for bilateral trapeziometacarpal osteoarthritis were reviewed. Pain was reduced in all cases. Function was improved in all right hands and in 92% of the left hands. FCR tendon interposition arthroplasty for bilateral trapeziometacarpal osteoarthritis yields satisfactory long-term results on both sides.
... also: MPS I H (Hurler syndrome) MPS II, Hunter syndrome MPS IV Morquio syndrome MPS III (Sanfilippo ... syndrome is the mildest form of mucopolysaccharidosis type 1. The syndrome is transmitted as an autosomal recessive ...
A recent trend has been the implantation of bilateral cochlear implants (CIs) for children with severe to profound hearing loss. A review of available research on bilateral CIs was conducted to determine the support for this trend. A replicable review was undertaken to evaluate published research studies that examined the effectiveness of bilateral paediatric cochlear implantation. Databases, reference lists, and journals were searched for relevant documents using a pre-determined search protocol. Twenty-nine articles met the review's inclusion criteria and were retrieved and reviewed. This review adds to the previously published reviews on the topic by identifying additional paediatric studies. Sound localization and speech recognition in noise appear to be improved with bilateral compared to unilateral cochlear implants. Similarly, evoked potential measures suggest improved morphology when the second CI is implanted early. Well-designed and controlled studies that explore a variety of outcomes including cost-effectiveness, quality of life, speech, language, and psycho-educational measures should be further explored in order to provide additional support for parents and clinicians confronted with the bilateral cochlear implant decision. PMID:19382017
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
A 39-year-old male was admitted to our clinic with symptoms of headache, dizziness, nausea, otalgia, otorrhea, tinnitus, and hearing loss in both ears for 3 weeks. Physical examination revealed edema in the tympanic membrane and external ear canal, and pain by palpation in the mastoid area bilaterally. There was no nystagmus, and the rest of the physical examination was otherwise normal. Temporal bone high resolution computed tomography (CT) showed a lesion causing erosion in the mastoid cortex, tegmen tympani, ossicles, and in the bone covering the sigmoid sinus bilaterally. There was also erosion in the superior semicircular canal and petrous bone on the left side. Cortical mastoidectomy was performed under general anesthesia. Histopathologic examination of the tissue revealed Langerhans cell histiocytosis (LCH). In this paper a case with LCH, presenting with bilateral mastoid involvement which has been rarely reported in the literature, is discussed with the existing literature. PMID:23841005
Fatal bronchial asthma is classically characterized in pathology textbooks and journal articles as associated with lung hyperinflation at autopsy. The following is a report of a case of fatal bronchial asthma associated instead with bilateral lung collapse. This manifestation of asthma at autopsy has not been previously reported, to my knowledge. A 31-year-old man with a history of recurrent asthma was found in an unresponsive state in his automobile after a low-speed collision. Medical personnel during the resuscitation noted a decrease in breath sounds bilaterally and some wheezing. There was no escaping air with needle thoracostomies at the outset. Cardiac monitoring demonstrated pulseless electrical activity during most of the 30-minute resuscitation attempt. An autopsy disclosed collapsed lungs bilaterally. Microscopic examination of the lungs disclosed the characteristic histopathologic features of bronchial asthma. PMID:21030852
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
A 58-year-old-man presented with painful rapidly progressive bilateral proptosis with restricted ocular movements of 15 days duration. There was history of significant weight loss in the recent past. Computed tomography scan of the head and orbit revealed bilateral multiple, well-defined, round, soft tissue masses, isointense with muscles in intraconal and extraconal space. Fine needle aspiration cytology and incision biopsy from the lesion, urine for Bence-Jones proteins and immunofixation clinched the diagnosis of multiple myeloma. Skeletal survey did not reveal any bony involvement. The diagnosis of multiple myeloma should be kept in mind in cases of bilateral proptosis. Bony involvement is not universal in cases of orbital myeloma. Early diagnosis can be established with extensive biochemical and histopathological investigations and timely treatment is life saving for these patients.
Hypertrophic olivary degeneration resulting from lesions of the dento-rubro-olivary pathway, also called Guillain-Mollaret-triangle, has been described previously in a number of cases. Reports about bilateral hypertrophic olivary degeneration of the inferior olivary nuclei are very limited, and the magnetic resonance imaging findings of hypertrophic olivary degeneration in Wilson disease have not yet been described to the best of our knowledge. Herein, we present the first report of bilateral hypertrophic olivary degeneration diagnosed by magnetic resonance imaging in a patient suffering from Wilson disease.
Renal cell carcinoma (RCC) is a malignant lethal tumour with high potential of metastasis. However, metastasis from RCC to the skin is much less common. It is virtually a sign of poor prognosis. We represent a 42 years old man with bilateral RCC of clear cell type followed by metastasis to the scalp one month later. In this case the relatively young age of the patient, bilaterality of RCC and occurance of skin metastasis in the absence of recurrent kidney tumour are interesting. PMID:23865145
Dissection of a renal artery is rare and is usually associated with underlying arterial disease. Bilateral renal artery dissection following extreme exertion is exceptionally uncommon, and thus presents a diagnostic challenge. We report a case of a middle-aged, otherwise healthy man who presented to the hospital with left flank pain after a long bicycling trip. Initial laboratory tests and urinalysis were normal. Careful review of a contrast-enhanced computed tomography angiogram (CTA) with 3D reconstruction revealed bilateral segmental renal artery dissection and thrombosis with corresponding renal infarcts. He was treated medically and rapidly recovered. PMID:21955870
Baroudi, S; Bastani, B; Balci, N; Bieneman, B K; Sobti, P; Kudva, G C
A strong tendency to symmetrical development of the organ systems manifests itself in the syndrome of Polysplenia. These symmetrical organs show the anatomy of leftsided structures. For this reason the syndrome is also referred to as the "syndrome of bilateral leftsidedness". It encompasses complex abnormalities of situs, typical cardiac and vascular deformities as well as extracardiac malformations. Polysplenia is the leading symptom. Diagnosis is rarely made intra vitam. Certain typical deformities can give hints to this diagnosis, which is finally proved by szintigraphic and arteriographic visualization of multiple spleens. A typical case is reported and subsequently discussed according to the picture of this syndrome in the literature. PMID:142049
A pleural window communicating between bilateral pleural cavities is a serious condition in patients with pneumothorax, allowing air to leak from the affected lung into the contralateral pleural cavity and resulting in bilateral spontaneous pneumothorax. We treated a patient with a history of right-sided bullectomy for simultaneous bilateral spontaneous pneumothorax that subsequently recurred. A pleural window (1 cm long) was detected in the mid-mediastinum, and direct suture closure with localized pleural abrasion using argon beam coagulation on the circumference of the lesion was performed at video-assisted thoracoscopic surgery. PMID:18355546
Agenesis and hypoplasia of the internal carotid artery (ICA) are rare congenital anomalies, occurring in less than 0.01% of the population. We report a rare case of bilateral hypoplasia of the ICA in a patient with post-traumatic subarachnoid hemorrhage. We describe the embryological development of the cerebral vasculature and present a review of literature. PMID:22223934
Bhat, Dhananjaya I; Somanna, Sampath; Kovoor, Jerry Me
On November 2006, a bilateral hand allotransplantation was performed for a 47-year-old female who had suffered radiocarpal amputations 28 years before. Technical aspects of the operation are detailed. Alemtuzumab induction, and triple therapy of tacrolimus, mycophenolate mofetil and prednisone were used to control rejection. The evolution of the result and functioning at 20 months are presented in detail. Two acute
In this technical note, the use of desmopressin to stimulate pituitary adrenocorticotropic hormone secretion in place of the commercially available corticotropin releasing hormone (CRH) in bilateral inferior petrosal sinus sampling is described. Although the use of CRH is the standard of practice, it is currently unavailable in the USA and desmopressin provides reliable results with no additional observed side effects. PMID:22735860
Botulinum toxin is a new and innovative method of treating bilateral masseteric hypertrophy which offers many advantages over conventional surgical treatment. Experience gained through the successful use of this drug when given as an intramuscular injection is reported. No significant side-effects have occurred and this technique is recommended for the routine treatment of masseteric hypertrophy. PMID:8136335
For most of the past half-century, Western Pacific countries largely eschewed preferential trade agreements. Their preferred form of trade liberalisation was unilateral action on a non-discriminatory basis. In the past four years, however, more than 20 preferential schemes involving two or more Western Pacific countries have been put forward. The new interest in bilateralism is explained by: an increasing awareness
Vascular anomalies and variants are common in patients undergoing imaging studies, and patients with these anomalies are generally asymptomatic. Remnants of fetal carotid-basilar circulation are rarely identified. We report a rare case of persistent type 2 bilateral proatlantal arteries, in which the patient presented with dizziness.
The computed tomographic (CT) features of bilateral adrenal tuberculosis are reported in two cases that demonstrate two typical different clinical and morphological manifestations of the disease. The incidence and CT appearance of adrenal tuberculosis are discussed, with emphasis on differential diagnosis.
We present a patient with bilateral scalp necrosis caused by giant cell arteritis (temporal arteritis). A 67-year-old woman, who had been treated with 5 mg of oral prednisolone every other day for polymyalgia rheumatica, developed painful egg-sized regions of necrosis on both of her temples. Doppler pulsemetory revealed bilateral obstruction of the temporal arteries. Biopsy revealed ischemic necrosis of the skin and necrotic angiitis of the temporal arteries with giant cell infiltration. Bilateral stenosis of the internal carotid arteries and moderate retinal bleeding were revealed by angiography. Daily administration of prednisolone (20 mg/day) with intravenous and topical limaprost alphadex markedly improved her condition. The ulcers reepithelized without surgical treatment. There are few reports of bilateral scalp necrosis. Rapid and complete obstruction of the temporal artery may result in this condition. Simultaneous development of two ulcerative lesions in the ventro-parietal cranial regions is thought to correspond to systemic arterial involvement, including involvement of the internal carotid arteries. PMID:12692357
Sialoadenitis is a non-inflammatory condition that affects mainly the parotid, and is characterised by bilateral, painless swelling, which is an appreciable problem in young women. A 28-year-old woman presented with parotid sialoadenitis with masseteric hypertrophy. She was treated by superficial parotidectomy and ostectomy of the mandibular angle and made an uncomplicated recovery. PMID:14649690
Adrenal infarction is an uncommon cause of adrenal insufficiency. We herein present unique occurrence of bilateral adrenal infarction detected on imaging in a young female with known history of Crohn's disease. The patient responded well to steroids and is on follow up. To our knowledge, this is the first case reported in English literature of adrenal infarct associated with Crohn's disease as extraintestinal manifestation.
Khandelwal, Ashish; Krishna, J. Sateesh; Khandelwal, Kanika; Virmani, Vivek; Ryan, John
In human decision making behaviors, people rely on random selection processes, such as flipping a coin, to handle a situation that involves so much uncertainty that it subsequently becomes difficult for them to rationally judge a decision. For bilateral multi-issue negotiations, given even bargaining power and two-sided uncertainty, negotiators are often faced with difficult decisions that cannot be handled through
A 64-year-old patient experienced palsy of the right spinal accessory nerve after rhytidectomy. She was evaluated 6 months after surgery for unexplained shoulder pain and weakness since the procedure. Electrophysiological testing showed bilateral spinal nerve palsy without abnormalities in the other shoulder nerves. The electrophysiological findings supported entrapment as the mechanism, rather than nerve section or pure axonal disease due to
Thoracic bilateral paravertebral block is a technique commonly used in the ambulatory setting for numerous plastic surgery procedures. Paravertebral block has not been reported with abdominoplasty surgery. This case series explores this anesthetic technique in the inpatient and day patient setting. PMID:18346612
Rudkin, Glenda E; Gardiner, Sarah E; Cooter, Rodney D
We report here a case study of a rare neurological patient with bilateral brain damage encompassing a substantial portion of the so-called “limbic system.” The patient, Roger, has been studied in our laboratory for over 14 years, and the current article presents his complete neuroanatomical and neuropsychological profiles. The brain damage occurred in 1980 following an episode of herpes simplex
Justin S. Feinstein; David Rudrauf; Sahib S. Khalsa; Martin D. Cassell; Joel Bruss; Thomas J. Grabowski; Daniel Tranel
Occipital horn syndrome, formerly known as Ehlers-Danlos syndrome type IX or X-linked cutis laxa, is an allelic variant of Menkes' syndrome. Although the clinical symptomatology and systemic pathology findings have been well described in occipital horn syndrome, the neuropathology has not previously been reported. A kindred affected by the X-linked occipital horn syndrome is followed at the University of Alabama at Birmingham. A severely mentally retarded dysmorphic man, who died at the age of 26 years, never gained the ability to walk or talk. Among other findings at autopsy, the patient had the skeletal anomalies previously described with occipital horn syndrome. Neuropathologic findings included neovascularization and extreme reduplication of the cerebral arteries, in conjunction with cystic medial degeneration; bilateral cerebellar hypoplasia; focal cortical dysplasia, and cerebellar heterotopias. These findings are similar to those seen in the brains of patients with Menkes' syndrome, which is not surprising, given the known phenotypic overlap and the proven allelism of occipital horn syndrome with classic Menkes' syndrome. PMID:11669352
Ectopic secretion of adrenocorticotropic hormone (ACTH) is an infrequent cause of Cushing's syndrome. We report a case of ectopic ACTH syndrome caused by a pheochromocytoma. A 53-year-old female with clinical features of Cushing s syndrome presented with serious recurrent hypertensive crisis. Endocrinological investigation confirmed the diagnosis of ectopic ACTH production and revealed markedly elevated urinary catecholamines leading to the diagnosis of pheochromocytoma. Abdominal computerized tomography (CT) scan showed a 3.5 cm left adrenal mass and a nodular hypertrophic right adrenal gland. Bilateral selective adrenal vein catheterization suggested bilateral pheochromocytoma. After treatment with phenoxybenzamine, bilateral adrenalectomy was performed and resulted in remission of Cushing's syndrome and hypertensive crisis. In addition, this article provides a short guideline for endocrine testing if Cushing's disease or pheochromocytoma is suspected. However, the most important message of this article is to think of them. PMID:18304406
In this case report, we describe a case of adult-onset bulbar ptosis in a patient with Joubert syndrome. Joubert syndrome is a rare neurodevelopmental disorder with malformations in cerebellum and brainstem. Many ocular abnormalities have been noted in Joubert syndrome, but the association of this syndrome with adult-onset ptosis has not been described to date. This 24-year-old Joubert patient developed a cerebrospinal fluid cyst in her midbrain. She had signs of bilateral third nerve palsy and abducens palsy in the left eye. The bilateral central third nerve palsy causing functional blindness secondary to severe bilateral levator palsy was treated successfully with silicone sling frontalis suspension, as the seventh nerve nucleus was not involved. PMID:22291457
Polycystic ovarian syndrome (PCOS) is defined by ultrasound appearance of polycystic ovaries (PCO), enlarged ovaries with\\u000a at least 10 peripherally located follicles measuring between 2 and 10 mm, associated with symptoms of oligo-amenorrhoea, obesity\\u000a and hyperandrogenism (acne and hirsutism) . Stein–Leventhal syndrome was described in 1935 as a condition of obese women\\u000a with amenorrhoea, signs of excess androgen production and bilateral
The authors describe the case of a 16-month-old girl who presented with bilateral hydronephrosis and renal failure secondary to bilateral renal pelvic botryoid Wilms' tumour (nephroblastoma). The term 'botryoid' describes an intrapelvic polypoid renal Wilms tumour, either extending from the renal parenchyma or primarily pelvis-based tumour. Both tumours filled the renal pelvis and extended down the proximal ureter, with no intraparenchymal tumour seen. Bilateral intralobar nephrogenic rests were present. Histological examination demonstrated triphasic nephroblastoma, with focal rhabdomyomatous differentiation of the stromal element bilaterally. Postchemotherapy, the patient underwent bilateral nephrectomy. Post complete resection of her bilateral disease, this patient has had an excellent outcome. This is only the third reported case of bilateral botryoid Wilms' tumour. While the condition is extremely rare, it should be included in the differential diagnosis of a young child with bilateral renal pelvic masses. PMID:22962373
There is concern about safety of bilateral total hip arthroplasty (THA).This study aims to compare in-hospital complication rates between unilateral, simultaneous and staged bilateral THAs. The Nationwide Inpatient Sample from 2002-2010 was used. Patients and complications were identified using ICD-9-CM codes. In multivariate analysis, bilateral THA had higher risk of systemic complications (Odds ratio (OR): 2.1, P<0.001) compared to unilateral procedure, whereas no significant difference existed between simultaneous and staged bilateral THAs. The rate of local complications was higher in bilateral versus unilateral (4.96% versus 4.54%, P=0.009) and in staged versus simultaneous bilateral THAs (OR: 1.75, P=0.05). Bilateral THA increases risk of systemic complications compared to unilateral surgery and simultaneous bilateral THA appears to be safer than staging during one hospitalization. PMID:23664280
Rasouli, Mohammad R; Maltenfort, Mitchell G; Ross, David; Hozack, William J; Memtsoudis, Stavros G; Parvizi, Javad
This case study describes a 45 year old female with bilateral, profound sensorineural hearing loss due to Meniere’s disease. She received her first cochlear implant in the right ear in 2008 and the second cochlear implant in the left ear in 2010. The case study examines the enhancement to speech recognition, particularly in noise, provided by bilateral cochlear implants. Speech recognition tests were administered prior to obtaining the second implant and at a number of test intervals following activation of the second device. Speech recognition in quiet and noise as well as localization abilities were assessed in several conditions to determine bilateral benefit and performance differences between ears. The results of the speech recognition testing indicated a substantial improvement in the patient’s ability to understand speech in noise and her ability to localize sound when using bilateral cochlear implants compared to using a unilateral implant or an implant and a hearing aid. In addition, the patient reported considerable improvement in her ability to communicate in daily life when using bilateral implants versus a unilateral implant. This case suggests that cochlear implantation is a viable option for patients who have lost their hearing to Meniere’s disease even when a number of medical treatments and surgical interventions have been performed to control vertigo. In the case presented, bilateral cochlear implantation was necessary for this patient to communicate successfully at home and at work.
Holden, Laura K.; Neely, J. Gail; Gotter, Brenda D.; Mispagel, Karen M.; Firszt, Jill B.
Five patients aged 55 to 73 years (mean 63 years) underwent one-stage clipping for unruptured aneurysms in the bilateral middle cerebral arteries (mean size 4.5 mm, range 2 to 7 mm) via the bilateral pterional keyhole approach in our institute. Important points are as follows: the head is affixed with no rotation; one side manipulation is started 5 minutes after the other side to avoid conflict of surgical instruments; a 5-cm curvilinear skin incision is made inside the hairline and pterional keyhole craniotomy is made bilaterally using 2 burr holes; the whole operating table is rotated 15 degrees to one side to facilitate the microsurgical trans-sylvian approach and aneurysm clipping; the operating table is rotated to the other side for the contralateral procedure; and particular care is taken to avoid bilateral brain injury. This approach provided minimum but sufficient working space required for trans-sylvian dissection. Aneurysm neck clipping was safely performed in a mean operation time of 5 hours 17 minutes. No complications occurred and satisfactory cosmetic results were obtained in all patients. Postoperative neuroimaging studies exhibited bilateral complete clipping with minimal intracranial air content and minimum consequences of brain retraction. One-stage clipping via the pterional keyhole approach is a safe and effective therapeutic option for small bilateral aneurysms. PMID:23524497
Only one case of wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) has been described in central nervous system cryptococcosis. The disorder was initially unilateral, then became bilateral with skew deviation and vertical upgaze deficit. We report a case of WEBINO in central nervous system cryptococcosis in a patient with acquired immune deficiency syndrome. Magnetic resonance imaging revealed high signal on T2 images in the right midbrain, left frontal vertex, left splenium, and cerebellum. With treatment, the internuclear ophthalmoplegia improved; however, the convergence insufficiency remained. Disruption of input from cortical supranuclear locations or the region of the rostral interstitial nucleus of the medial longitudinal fasciculus has been proposed as a mechanism in the absence of convergence. This correlates in our patient with the lesions seen on magnetic resonance images. PMID:10380136
Background Linear scleroderma "en coup de sabre" (LSCS) usually affects one side of the face and head in the frontoparietal area with band-like indurated skin lesions. The disease may be associated with facial hemiatrophy. Various ophthalmological and neurological abnormalities have been observed in patients with LSCS. We describe an unusual case of LSC. Case presentation A 23 year old woman presented bilateral LSCS and facial atrophy. The patient had epileptic seizures as well as oculomotor and facial nerve palsy on the left side which also had pronounced skin involvement. Clinical features of different stages of the disease are presented. Conclusions The findings of the presented patient with bilateral LSCS and facial atrophy provide further evidence for a neurological etiology of the disease and may also indicate that classic progressive facial hemiatrophy (Parry-Romberg syndrome) and LSCS actually represent different spectra of the same disease.
Congenital long QT syndrome (LQTS) is a disorder of prolonged cardiac repolarisation, manifest by a prolonged QT interval and characterised by recurrent presyncope/syncope, polymorphic ventricular tachycardia (PMVT), or sudden cardiac death. A 46 year old woman with no family history of sudden death or deafness presented with recurrent syncope. Physical examination and electrolytes were normal and a 12 lead ECG showed a corrected QT interval of 458 ms. A monitored syncopal episode documented PMVT. Despite potassium and magnesium supplements, beta blockade, implantation of a single then dual chamber implantable cardioverter defibrillator (ICD), amiodarone, nicorandil, and mexiletine, the patient continued to experience arrhythmia storms, receiving more than 700 ICD discharges over seven months. She was ultimately treated successfully with bilateral thoracoscopic cervicothoracic sympathectomies. This is the first reported bilateral thoracoscopic treatment of a patient with LQTS and symptomatic life threatening ventricular tachyarrhythmias refractory to current pharmacological and pacing techniques. PMID:15604323
Congenital long QT syndrome (LQTS) is a disorder of prolonged cardiac repolarisation, manifest by a prolonged QT interval and characterised by recurrent presyncope/syncope, polymorphic ventricular tachycardia (PMVT), or sudden cardiac death. A 46 year old woman with no family history of sudden death or deafness presented with recurrent syncope. Physical examination and electrolytes were normal and a 12 lead ECG showed a corrected QT interval of 458 ms. A monitored syncopal episode documented PMVT. Despite potassium and magnesium supplements, ? blockade, implantation of a single then dual chamber implantable cardioverter defibrillator (ICD), amiodarone, nicorandil, and mexiletine, the patient continued to experience arrhythmia storms, receiving more than 700 ICD discharges over seven months. She was ultimately treated successfully with bilateral thoracoscopic cervicothoracic sympathectomies. This is the first reported bilateral thoracoscopic treatment of a patient with LQTS and symptomatic life threatening ventricular tachyarrhythmias refractory to current pharmacological and pacing techniques.
The majority of paediatric surgeons will encounter a patient with prune belly syndrome (PBS) only a few times in their clinical practice. There have been many opposing views in the literature regarding the pathogenesis and management of this complex condition. A detailed review was conducted using PubMed to identify key publications involving PBS. This article discusses the evolution of our understanding of the pathogenesis and diagnosis of PBS, including its typical characteristics. We describe the management options available for bilateral intra-abdominal testes, the deficient abdominal wall, the dilated urinary system and examine the evidence base used to support the current approaches employed. PMID:22198807
Bilateral synchronous breast cancer appears to have a worse prognosis than comparable unilateral breast cancer. HER-2\\/neu expression in bilateral breast cancer has not been reported. The purpose of this study was to review the characteristics\\u000a of patients with bilateral synchronous breast cancer and to report the incidence of HER-2\\/neu overexpression. Between 1984 and 1998, 58 patients were diagnosed with bilateral
Malek Safa; Elyse E. Lower; P. O. Hasselgren; Eric S. Hungness; Paula Gazder; Bernard Aron; Elizabeth A. Shaughnessy; Rawia Yassin
Background Hydatid cyst disease is still a problem in many countries. Surgical removal is currently the generally accepted choice of treatment for lung hydatidosis. However, operating on bilateral widespread lung hydatidosis is still controversial. The aim of this retrospective study was to evaluate the results of surgical treatment in bilateral multiple hydatid disease of the lung. Methods In this study, we reviewed our experience in the surgical treatment of 17 (3.7%) patients with bilateral, and at least three, lung hydatid cysts. These 17 patients (8 male, 9 female), with an average age of 34.6 years (range 12–58 years), underwent bilateral staged thoracotomy. Results In total 105 lung cysts were removed from 17 patients who underwent staged thoracotomies. The mean count of cysts was 6.7 (range 3–20 cysts). Most of the cysts (38.2%) were located in the right lower lobe. The mean interval between thoracotomies was 4.2 (range 3–5) days. Two patients (11.7%) had cysts associated with hepatic hydatidosis and one (5.8%) had cysts associated with the spleen; they were treated via phrenotomy during thoracotomies. All cysts were removed without lung resection. We observed some complications such as prolonged air leaks (n?=?2), atelectasis (n?=?3) and empyema (n?=?2). No further surgery was required for management of complications. The mean hospital stay was 9.3 days. (range 7–23 days). Oral albendazole was started on the 2nd post operative day after the first thoracotomy in the dose of 10–20 mg/kg and was continued for 3 months with a gap of 1 week after each 21 days. No recurrences or deaths occured during the follow-up period. Conclusions Although staged thoracotomy applied in 3–5 days after the initial thoracotomy increases the total hospital stay, it decreases the chance of possible complications can occur in cysts in the other lung when long intervals are preferred between the first and the second thoracotomy. In our experience, bilateral staged thoracotomy is an appropriate surgical option because morbidity rates are minimal and the hospital stay is acceptable for the treatment of bilateral widespread lung hydatidosis, even in patients who had a total of 20 hydatid cysts.
Polysplenia, or left isomerism, is a rare heterotaxy syndrome characterized by bilateral bi-lobed lungs, bilateral pulmonary atria, a symmetrical midline liver, and multiple aberrant splenic nodules. We report a case of polysplenia associated with congenital lobar emphysema apart from other typical anomalies. Such an association has not been previously reported. The patient was a young male with progressive exertional breathlessness referred for high resolution CT of the lungs. CT, MRI and echocardiography revealed (in addition to congenital lobar emphysema of right lung) a hemiazygos continuation of the inferior vena cava, a persistent left superior vena cava, multiple splenunculi in the right hypochondrium, midline liver, bilateral bilobed lungs, a large pulmonary artery (suggestive of severe pulmonary artery hypertension) and a large VSD—a typical constellation of findings described in polysplenia syndrome.
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
Blackwood, Sarah E; Barrie, Kathleen P; Plummer, Caryn E; Taylor, David; Nunnery, Catherine M; Seltzer, Judith D; Ben-Shlomo, Gil; Brooks, Dennis E
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.
Purpose and background: Bilateral internal carotid artery occlusion is an extremely rare entity, therefore, studies of the natural history of this disease are lacking in the English literature. The purpose of this study is to analyze the natural history and surgical alternatives for patients with bilateral internal carotid artery occlusion. Patient population and methods: Twenty-one patients with bilateral internal carotid
A bilateral export demand function is used to estimate the effects on the Chinese bilateral exports of three real exchange rates: (1) bilateral real exchange rate capturing the price-competitiveness of the Chinese products in the market of the considered import country (traditional effect); (2) real effective exchange rate capturing the price-competitiveness of the goods of Chinese competitors in the same
Idiopathic bilateral vestibulopathy (IBV) is an acquired bilateral peripheral vestibular disorder of unknown cause. Three patients diagnosed as IBV by neuro-otological examination were reported. They underwent vestibular evoked myogenic potential (VEMP) testing which reflects the functionality of the sacculo-collic pathway. As a result, 2 of the 3 patients showed bilateral absence of VEMPs and one showed unilateral absence. The VEMPs
BackgroundVertebral artery dissecting aneurysm is now increasingly recognized as a cause of posterior circulation stroke in young adults. Here, we report a case of bilateral VADA with SAH, treated by bilateral coil occlusion using GDCs.
Hereditary breast cancer (HBC) shows extant clinical and genetic heterogeneity. Clinically one finds the onset of breast cancer at an early age, an excess of bilaterality, and patterns of multiple primary cancer such as combinations of breast and ovarian carcinoma in the hereditary breast-ovarian cancer (HBOC) syndrome. In addition to HBOC, one sees a variety of putative breast cancer-prone genotypes
Henry T. Lynch; Jane Lynch; Theresa Conway; Patrice Watson; Jean Feunteum; Gilbert Lenoir; Steven Narod; Robert Fitzgibbons
The neuropathological changes found at autopsy in a case of Kearns-Sayre syndrome are described. We have previously analyzed the respiratory chain function in isolated muscle mitochondria and also described a large deletion of muscle mitochondrial DNA (mtDNA) in this case. The neuropathological examination revealed prominent neuronal degeneration and gliosis of the basal ganglia and there were bilateral areas of softening
A. Oldfors; I.-M. Fyhr; E. Holme; N.-G. Larsson; M. Tulinius
We report here 3 cases with the classic signs of Goldenhar syndrome in the form of multiple accessory tragi, bilateral ocular dermoids, mandibular hypoplasia (micrognathia), and facial microsomia. One of the patients also had vitiligo, which is yet to be reported as an association.
Background Morning glory syndrome (MGS) is a congenital optic disc dysplasia often associated with craniofacial anomalies, especially basal encephalocele. Clinical presentations are varied and often occult. Case report We describe a case of bilateral MGS associated with basal encephalocele that was detected by chance when treatment was sought for respiratory distress. Conclusions MGS and basal encephalocele should always be suspected
Severe bilateral cataract was found in a 7 year-old naïve female cynomolgus monkey (Macaca fascicularis) 3 months before necropsy. During macroscopic examination, severe opacity and thinning of the lens were observed in both eyes. Histopathology revealed that the lens nuclei and majority of cortex lens fibers had disappeared and become excavated, while the lens fibers in the subcapsular area were swollen and distorted. Other observations included atrophy and vacuolation in the lens epithelial cells and proliferation of spindle cells and collagen fiber beneath the anterior capsule of the right eye. Immunohistochemical staining of these spindle cells revealed the presence of vimentin, cytokeratin and ?-smooth muscle actin (?-SMA), which were considered to be derived from lens epithelial cells. This is a rare case of spontaneous, bilateral, hypermature cataract in a cynomolgus monkey.
Breast masses have a variety of benign and malignant etiologies. We present the case of a 28-year-old woman with bilateral large painful breast masses that developed rapidly in the three weeks before first presentation. Further investigation revealed bilateral ovarian masses. Biopsies of both ovarian masses were taken, and the pathology reported Burkitt's lymphoma. Additional staging with a PET scan was suggestive of bone marrow involvement, but bone marrow biopsy was negative. Examination of the cerebrospinal fluid did not identify malignant cells. The patient underwent CODOX-M/IVAC chemotherapy, and a complete response was demonstrated after one cycle of treatment. Six months after finishing chemotherapy the patient remained in complete remission. To our knowledge this is the first case reporting simultaneous involvement of breast, ovaries, and bones in Burkitt's lymphoma. Gynecologists and oncologists should be aware of this pattern. Polychemotherapy treatment must be initiated rapidly with curative intent.
Pulmonary sequestration (PS) is a rare malformation consisting of aberrant lung tissue which is not affiliated with the normal bronchial system and is fed by an aberrant artery that derives from systemic arteries. However, PS is usually seen unilaterally but, only rarely, it is bilateral. Most patients with PS are diagnosed because of symptoms due to pulmonary infection or cardiac disease, while a small portion of patients are asymptomatic and diagnosed incidentally. In this report, we present an extremely rare case of asymptomatic bilateral PS which was diagnosed at advanced age. To our knowledge, this case represents the oldest patient in the literature, and the second case that was diagnosed in a patient over the age of 50.
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
Breast masses have a variety of benign and malignant etiologies. We present the case of a 28-year-old woman with bilateral large painful breast masses that developed rapidly in the three weeks before first presentation. Further investigation revealed bilateral ovarian masses. Biopsies of both ovarian masses were taken, and the pathology reported Burkitt's lymphoma. Additional staging with a PET scan was suggestive of bone marrow involvement, but bone marrow biopsy was negative. Examination of the cerebrospinal fluid did not identify malignant cells. The patient underwent CODOX-M/IVAC chemotherapy, and a complete response was demonstrated after one cycle of treatment. Six months after finishing chemotherapy the patient remained in complete remission. To our knowledge this is the first case reporting simultaneous involvement of breast, ovaries, and bones in Burkitt's lymphoma. Gynecologists and oncologists should be aware of this pattern. Polychemotherapy treatment must be initiated rapidly with curative intent. PMID:24066248
Thieringer, Friederike; Sartorius, Gideon; Kalf, Katrin; Heinzelmann, Viola; Vetter, Marcus
Unilateral and bilateral sternoclavicular joint (SCJ) dislocations are rare injuries. The difficulty in assessing this condition often leads to delay in diagnosis and treatment. We report a rare case of bilateral asymmetrical traumatic SCJ dislocations in a 45-year-old male. The right anterior SCJ dislocation was reduced in the emergency room (ER) and resulted in residual instability. The left posterior SCJ dislocation was asymptomatic and unnoticed for six months. It is important for ER physicians and orthopaedic surgeons to be able identify and treat this condition. All suspected SCJ dislocations should be evaluated by computed tomography (CT) scan for confirmation of the diagnosis and evaluation of both SCJs. Posterior SCJ dislocation is a potentially fatal injury and should not be overlooked due to the presence of other injuries. Surgical intervention is often necessary in acute and old cases.
Although single-incision laparoscopic surgery made an appearance on the surgical scene only recently, it is being increasingly applied in the treatment of a variety of disorders. We report single-incision bilateral laparoscopic oophorectomy and salpingooophorectomy performed in two patients who had previously undergone breast conservation surgery for early breast cancer. Each procedure was undertaken using two 5-mm and one 3-mm ports inserted through a 2-cm transverse supraumbilical incision and standard laparoscopic instruments. The operative time was 50 and 65 min respectively and the blood loss negligible. The patients were discharged 36 and 24 h after surgery, required minimal postoperative analgesia and remain well at a follow up of 19 and 17 months, respectively. With the benefit of improved cosmesis, the single-incision approach holds the potential to replace the traditional bilateral laparoscopic oophorectomy.
Three patients developed bilateral breast cancer at 10 to 24 years after mantle irradiation for locally or systemically advanced Hodgkin's disease (HD). Four of the six cancers in the three patients were detected only by mammography. Pathologically, five of the cancers were intraductal carcinomas (four with an invasive component) with one being a lobular carcinoma. Five of the six lesions were Stage I pathologically without evidence of axillary nodal involvement. It is recommended that female patients with Hodgkin's disease who have received mantle irradiation as part of the therapy for their Hodgkin's disease and who are observed for 10 or more years after completion of mantle irradiation be considered at risk for the development of breast cancer. Such patients should be monitored appropriately by routine bilateral mammograms to increase the early detection of early stage lesions.
Anderson, N.; Lokich, J. (New England Baptist Hospital, Boston, MA (USA))
Hard metal pneumoconiosis, first described in 1964, is a diffuse disease caused by the inhalation of cobalt particles. The disease can manifest as occupational asthma, interstitial disease or allergic alveolitis. We report the case of a young male, working as a tool sharpener, who presented with dry cough and progressive dyspnea for one year, as well as with spontaneous bilateral pneumothorax at admission. The diagnosis was confirmed by open lung biopsy. PMID:20209319
Moreira, Maria Auxiliadora Carmo; Cardoso, Amanda da Rocha Oliveira; Silva, Daniela Graner Schuwartz Tannus; Queiroz, Maria Conceição de Castro Antonelli Monteiro de; Oliveira, Albino Alegro; Noleto, Tiago Marinho Almeida
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
A 32-year-old male who had infective endocarditis complained of photophobia and blurred vision in both eyes. Biomicroscopic examination and fundus examination revealed anterior chamber reaction, vitritis, optic disc swelling, and Roth spots. He was diagnosed with bilateral nongranulomatous uveitis and treated with topical steroid eye drops and posterior sub-Tenon injection of triamcinolone. His visual symptoms were resolved within 1 week, and inflammation resolved within 4 weeks after treatment.
Morgagni hernia is a rare type of congenital diaphragmatic hernia found in the anterior aspect of the diaphragm. It typically\\u000a presents in the pediatric population and rarely is diagnosed in adults. Only 3% of diaphragmatic hernias are the Morgagni\\u000a type, and only 4% of these are found to present bilaterally. Surgical repair of Morgagni hernia has been performed through\\u000a various
Odontodysplasia is a rare clinicopathologic condition that has a variety of expressions, including a range of tooth coloration, formation of hypoplastic enamel affecting one or multiple teeth, bizarre radiographic appearance, and delayed eruption of teeth. An unusual case is presented of bilateral regional odontodysplasia associated with a vascular nevus of the face and neck. A review of the etiology with emphasis on the "local circulatory disease" theory is presented. PMID:1667684
The Saethre–Chotzen syndrome (SCS) is an autosomal dominant craniosynostosis syndrome with uni- or bilateral coronal synostosis and mild limb deformities. It is caused by loss-of-function mutations of the TWIST 1 gene. In an attempt to delineate functional features separating SCS from Muenke's syndrome, we screened patients presenting with coronal suture synostosis for mutations in the TWIST 1 gene, and for
Wolfram Kress; Christian Schropp; Gabriele Lieb; Birgit Petersen; Maria Büsse-Ratzka; Jürgen Kunz; Edeltraut Reinhart; Wolf-Dieter Schäfer; Johanna Sold; Florian Hoppe; Jan Pahnke; Andreas Trusen; Niels Sörensen; Jürgen Krauss; Hartmut Collmann
Noonan syndrome describes a rare multisystem condition that manifests with Turner syndrome phenotype combined with numerous systemic and facial characteristics. The most common systemic findings include cardiac defects, short stature, chest deformity, hearing loss, and bleeding diatheses. Patients with Noonan syndrome are also at a greater risk of developing various types of malignancies. Facial characteristics of Noonan syndrome consist of broad forehead, low-set ears, short and webbed neck, and low hairline. External ocular findings include hypertelorism, ptosis, epicanthal folds, antimongoloid slant, downward-sloping palpebral fissures, and malar flattening. In this case series and review of the literature, the authors present 2 cases of Noonan syndrome that embody the diverse spectrum of orbital manifestations associated with this rare disorder. The first case demonstrates more profound orbital pathology, including bilateral orbital giant cell reparative granulomas, proptosis, hypertelorism, shallow orbits, upper eyelid ptosis, and lower eyelid retraction. The second case displays classic Turner-syndrome-like facial characteristics including a broad forehead with low hairline, low-set ears, and short and webbed neck. Orbital findings were subtle and consisted of bilateral lower eyelid retraction and shallow orbits. In conclusion, Noonan syndrome is a rare congenital disorder with a wide spectrum of clinical findings. Various intraorbital and extraorbital tumors may occur in patients with Noonan syndrome, with giant cell reparative granuloma being the most commonly encountered. In patients with orbital tumors and eyelid retraction, the authors describe successful treatment through decompression, tumor extraction, and lower eyelid retractor release. Patients who present with ocular irritation and exposure due to less severe lower eyelid malposition may be successfully treated with lower eyelid retraction repair combined with lateral internal tarsoconjunctival tarsorrhaphy. PMID:21464791
Randolph, John C; Sokol, Jason A; Lee, Hui Bae H; Nunery, William R
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 , 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.
We report here a case study of a rare neurological patient with bilateral brain damage encompassing a substantial portion of the so-called “limbic system.” The patient, Roger, has been studied in our laboratory for over 14 years and the current article presents his complete neuroanatomical and neuropsychological profiles. The brain damage occurred in 1980 following an episode of herpes simplex encephalitis. The amount of destroyed neural tissue is extensive and includes bilateral damage to core limbic and paralimbic regions, including the hippocampus, amygdala, parahippocampal gyrus, temporal poles, orbitofrontal cortex, basal forebrain, anterior cingulate cortex, and insular cortex. The right hemisphere is more extensively affected than the left, although the lesions are largely bilateral. Despite the magnitude of his brain damage, Roger has a normal IQ, average to above average attention, working memory, and executive functioning skills, and very good speech and language abilities. In fact, his only obvious presenting deficits are a dense global amnesia and a severe anosmia and ageusia. Roger's case presents a rare opportunity to advance our understanding of the critical functions underlying the human limbic system, and the neuropsychological and neuroanatomical data presented here provide a critical foundation for such investigations.
Feinstein, Justin S.; Rudrauf, David; Khalsa, Sahib S.; Cassell, Martin D.; Bruss, Joel; Grabowski, Thomas J.; Tranel, Daniel
We report here a case study of a rare neurological patient with bilateral brain damage encompassing a substantial portion of the so-called "limbic system." The patient, Roger, has been studied in our laboratory for over 14 years, and the current article presents his complete neuroanatomical and neuropsychological profiles. The brain damage occurred in 1980 following an episode of herpes simplex encephalitis. The amount of destroyed neural tissue is extensive and includes bilateral damage to core limbic and paralimbic regions, including the hippocampus, amygdala, parahippocampal gyrus, temporal poles, orbitofrontal cortex, basal forebrain, anterior cingulate cortex, and insular cortex. The right hemisphere is more extensively affected than the left, although the lesions are largely bilateral. Despite the magnitude of his brain damage, Roger has a normal IQ, average to above-average attention, working memory, and executive functioning skills, and very good speech and language abilities. In fact, his only obvious presenting deficits are a dense global amnesia and a severe anosmia and ageusia. Roger's case presents a rare opportunity to advance our understanding of the critical functions underlying the human limbic system, and the neuropsychological and neuroanatomical data presented here provide a critical foundation for such investigations. PMID:19763994
Feinstein, Justin S; Rudrauf, David; Khalsa, Sahib S; Cassell, Martin D; Bruss, Joel; Grabowski, Thomas J; Tranel, Daniel
Bilateral cochlear implants (CIs) may offer deaf children a range of advantages compared to unilateral CIs. However, speech perception in noise is mainly facilitated by better-ear effects and much less by interaural comparisons or true 'binaural' hearing. Little is known about the development of the binaural auditory system with CIs provided at a young age. It is possible that, as with adults, binaural sensitivity exists but is not accessed due to technical limitations in electrical stimulation methods. In this paper, we present results on binaural hearing in children with bilateral CIs. Binaural masking level differences (BMLDs) were measured for a 180-degree phase shift in a 125-Hz sinusoid, presented in a 50-Hz-wide noise band and modulating a 1000-pps carrier pulse train. Stimuli were presented to a single electrode in the middle of the electrode array at both ears. Eight children between 6 and 15 years of age participated in this study. Six children had a significantly better detection threshold when the signal was out of phase (dichotic) between two ears than when it was in phase (diotic), with a mean difference (BMLD) of 6.4 dB. The present results show that children with bilateral CIs are sensitive to binaural cues in electrical stimuli, similar to adults, even when implants are provided at a later age and with a longer delay between implantations. PMID:19141992
Van Deun, Lieselot; van Wieringen, Astrid; Francart, Tom; Scherf, Fanny; Dhooge, Ingeborg J; Deggouj, Naïma; Desloovere, Christian; Van de Heyning, Paul H; Offeciers, F Erwin; De Raeve, Leo; Wouters, Jan
A 27-year-old man presented with decreased vision bilaterally. Ophthalmologic examination revealed panuveitis with bilateral serous retinal detachments (figure 1) characteristic of Vogt-Koyanagi-Harada (VKH) syndrome. Analysis of CSF revealed mild pleocytosis. No oligoclonal bands were detected. On MRI, there was bilateral abnormal thickening of the choroid with retinal detachment (figure 2, A and B), with hyperintense lesion on fluid-attenuated inversion recovery (FLAIR) image in the periventricular deep white matter (figure 2C). VKH syndrome is one of the causes of uveo-meningeal syndromes. Differential diagnosis of VKH syndrome includes Wegener granulomatosis, sarcoidosis, and Behçet disease.(1) The main disorders showing association with hyperintense plaques on deep white matter FLAIR images are inflammatory, metabolic, vascular, degenerative, autoimmune, and demyelinating diseases. MRI is a helpful tool in diagnosing VKH syndrome; in addition to the typical bilateral ocular findings, scattered periventricular white matter lesions on FLAIR are also described.(2.) PMID:24019391
We describe a 28-year-old man with presumed VKH syndrome, whose presenting symptoms were bilateral impaired vision and headaches. Orbital MR imaging findings included bilateral choroidal and retrobulbar contrast enhancement, while brain findings included white matter abnormalities on FLAIR and leptomeningeal enhancement. Pachymeningeal enhancement has been described previously; herein, we report a patient with VKH syndrome presenting solely with leptomeningeal enhancement. Thus, MR imaging may detect early CNS involvement by VKH disease before the onset of neurologic symptoms. PMID:21051514
Lohman, B D; Gustafson, C A; McKinney, A M; Sarikaya, B; Silbert, S C
Introduction. Interferon is a glycoprotein produced by assigned cells of immune system. It has been used in many different diseases. Although flu-like syndrome, myalgia, rash, hypotension, thrombocytopenia and peripheral neuropathy due to interferon use are encountered frequently, ocular side effects are rare, generally mild and transient. Case Report. 47-year-old female patient, presented with a mass lesion in right renal pelvis. Right radical nephrectomy was applied and the histopathological examination was consistent with papillary renal cell carcinoma. Interferon alpha treatment was started subcutaneously at the dose of 5?MIU/3 times in a week. Four weeks after the interferon therapy, suddenly bilateral visual loss developed. We discussed the diagnosis, followup, and treatment of the patient who developed irreversible ischemic optic neuropathy and had no previous known primary systemic disease to cause this condition. Conclusion. We suggest that patients should be screened for risk factors causing optic ischemic neuropathy, before interferon therapy. Although there was no adequate information in the literature for the followup, patients should be monitorized before, 1 month after, and 2 months after the treatment. And if there is no complication, we suggest that they should be followed up at 3-month intervals. PMID:23119208
Introduction. Interferon is a glycoprotein produced by assigned cells of immune system. It has been used in many different diseases. Although flu-like syndrome, myalgia, rash, hypotension, thrombocytopenia and peripheral neuropathy due to interferon use are encountered frequently, ocular side effects are rare, generally mild and transient. Case Report. 47-year-old female patient, presented with a mass lesion in right renal pelvis. Right radical nephrectomy was applied and the histopathological examination was consistent with papillary renal cell carcinoma. Interferon alpha treatment was started subcutaneously at the dose of 5?MIU/3 times in a week. Four weeks after the interferon therapy, suddenly bilateral visual loss developed. We discussed the diagnosis, followup, and treatment of the patient who developed irreversible ischemic optic neuropathy and had no previous known primary systemic disease to cause this condition. Conclusion. We suggest that patients should be screened for risk factors causing optic ischemic neuropathy, before interferon therapy. Although there was no adequate information in the literature for the followup, patients should be monitorized before, 1 month after, and 2 months after the treatment. And if there is no complication, we suggest that they should be followed up at 3-month intervals.
We observed a rare, bilateral congenital deficiency of the pectoralis major muscle in a 72-year-old female cadaver in our gross anatomy dissection laboratory. The outward appearance of the anterior thoracic wall, which included well-developed breasts, revealed no obvious abnormalities. Upon dissection, the following features were observed: 1) on the left side, the sternal portion of the sternocostal head of the pectoralis major muscle was absent, the costal portion of the sternocostal head and the clavicular head were both well developed, a normal pectoralis minor was present, and the deltoid and subclavius muscles were not hypertrophied as is often the case when the pectoralis major muscle is deficient; 2) on the right side, the entire pectoralis major muscle was absent and the pectoralis minor, deltoid, and coracobrachialis muscles were infiltrated with connective tissue and fat; and 3) on both sides, the lateral pectoral nerves were absent and the medial pectoral nerves were present. The absence of the lateral pectoral nerves suggests that the deficiencies in the pectoralis major muscles are congenital malformations resulting from a developmental failure of the embryonic muscles rather than a sequel to polio or Poland's syndrome. PMID:12794921
During the past two decades, several new hereditary renal cancers have been discovered but are not yet widely known. Hereditary renal cancer syndromes can lead to multiple bilateral kidney tumors that occur at a younger age than that at which the nonhereditary renal cancers occur. The aim of our work is to review the features of hereditary renal cancers, the basic principles of genetic relevant to these syndromes, and the various histopathologic features of renal cancer. In addition, we will describe the known familial syndromes associated with small renal masses.
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.
Various studies have demonstrated enhanced visual processing when information is presented across both visual hemifields rather than in a single hemifield (the bilateral advantage). For example, reported that observers were able to track twice as many moving visual stimuli when the tracked items were presented bilaterally rather than unilaterally, suggesting that independent resources enable tracking in the two visual fields. Motivated by similarities in the apparent capacity and neural substrates that mediate tracking and visual working memory (WM), the present work examined whether or not a bilateral advantage also arises during storage in visual WM. Using a recall procedure to assess working memory for orientation information, we found a reliable bilateral advantage; recall error was smaller with bilateral sample displays than with unilateral displays. To demonstrate that the bilateral advantage influenced storage per se rather than just encoding efficiency, we replicated the observed bilateral advantage using sequentially presented stimuli. Finally, to further characterize how bilateral presentations enhanced storage in working memory, we measured both the number and the resolution of the stored items and found that bilateral presentations lead to an increased probability of storage, rather than enhanced mnemonic resolution. Thus, the bilateral advantage extends beyond the initial selection and encoding of visual information to influence online maintenance in visual working memory.
Umemoto, Akina; Drew, Trafton; Ester, Edward F.; Awh, Edward
Gas-forming infection of the kidneys can affect either the parenchyma or the collecting system. They are known as emphysematous pyelonephritis (EPN) and emphysematous pyelitis (EP) respectively. Bilateral EPN is a fairly established entity and numerous articles about this condition have been published. However, much less is known about bilateral EP. We report a rare case of bilateral EP and a literature review of this disease. A 66-year-old woman with long-standing bilateral staghorn calculi presented with bilateral EP and severe sepsis. She was treated with antibiotics and bilateral double-J stents to drain the upper urinary tracts. She recovered after 1 month of intensive care and medical therapy. Percutaneous nephrolithotomy (PCNL) had been scheduled to treat her staghorn calculi. A literature search on MEDLINE and Google Scholar with the terms "bilateral emphysematous pyelitis" only found 1 case report in English and another 2 reports in the Korean language that discussed bilateral EP. The collective experience of these few cases, including the present case, suggests that bilateral EP runs a more benign course than bilateral EPN. It should be diagnosed as soon as possible with computed tomography (CT) scans of the renal system. Current evidence shows that can be treated successfully with timely antibiotics. Drainage of the collecting system either percutaneously or with placement of double-J stents might facilitate recovery. PMID:24045515
Tan C C K Ho, G H; Bahadzor, B; Praveen, S; Goh, E H; Syahril, A S; Zulkifli, M Z
The theory and numerical results are presented to the effective dielectric constant and characteristic impedance of bilateral and unilateral finlines with metallization thickness. The full wave analysis of the transverse transmission line — TTL method is used to determine the electromagnetic fields of the structure in Fourier transform domain — FTD. Applying the suitable boundary conditions and the moment method, a homogeneous matrix system is obtained and the effective dielectric constant is extracted. The characteristic impedance is obtained using the relation between the voltage in slot and the transmitted power. Computational programs are developed to obtain numerical results to the effective dielectric constant and characteristic impedance.
Fernandes, Humberto César Chaves; Martins de Souza, Eduardo Amorim; de Souza Queiroz Júnior, Idalmir
Bilateral simultaneous rupture of the quadriceps tendons is very rare and occurred in an 82-year-old man. This may be the fifth case to have been reported in the English literature. Only minor stress on extensor mechanisms preceded the ruptures. Interrupted mattress sutures restored muscle continuity and function. A "pull-out" wire system was not used for protection of healing muscles because adequate amount of tissue mass was available for approximation. Excellent results require intensive postoperative physical therapy. Old age of the patient should not be a deterrent to surgical treatment in carefully selected individuals. PMID:657630
The etiology of congenital idiopathic talipes equinovarus deformity is unclear. Studies on populations, families, and twins have suggested a genetic component. However, the mode of inheritance does not fit classic patterns. The intrauterine posture and environmental and developmental causative factors have also been associated with the deformity. Neurologic, muscular, bony, connective tissue, and vascular structures can be affected. We present the case of monochorionic triplets with bilateral congenital idiopathic talipes equinovarus deformities. To the best of our knowledge, such a presentation has not been previously described and supports a genetic etiology of congenital idiopathic talipes equinovarus deformity. PMID:21616690
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.
Many variations in the anatomy of the carotid artery are described in the literature. There are few case reports of hypoplasia or the retropharyngeal course of the internal carotid artery. We describe the unique case of a 72-year-old woman with bilateral complete retroesophageal common carotid arteries and concomitant hypoplasia of the right carotid system. Anatomical detection of such a variation is critical because the misidentification of a retroesophageal carotid artery carries the risk of potential injury during orotracheal intubation and oropharyngeal procedures. PMID:23601592
Patient. We report the case of a 20-year-old caucasian male with an Ewing's sarcoma in the left upper femur. After induction chemotherapy, he underwent resection of the left upper femur and insertion of a cemented proximal femoral replacement. Four years later, he presented with a solitary bone metastasis in the right upper femur. He underwent further chemotherapy followed by resection and endoprosthetic replacement of the other proximal femur. He remains disease free with excellent function almost a year after the second operation. Discussion. We believe this is the first reported case of bilateral proximal femoral endoprostheses.
Total, bilateral ophthalmoplegia is very rare. More than 50% of cases are Miller Fisher (MFS) and Guillain-Barré (GBS) syndromes. There is a correlation of MFS with anti-GQ1b antibodies. High levels of GQ1b gangliosides are found in myelin sheathes of cranial nerves supplying the extraocular muscles. This may explain the association of anti-GQ1b antibodies with ophthalmoplegia. Anti-GQ1b were also found in cases of GBS accompanied by ophthalmoplegia, atypical MFS (MFS without ataxia), MFS/GBS overlap syndromes and Bickerstaff brainstem encephalitis. This has led some authors to classify them as 'anti-GQ1b syndromes'. In this article we describe a diagnostically difficult case of a patient with a very rare, total bilateral paralysis of all ocular muscles, accompanied by bilateral ptosis, diminished tendon reflexes of upper extremities, paresis and hypoesthesia of the left upper extremity. PMID:21866487
Rajska, Kinga; Ro?niecki, Jacek; Loba, Piotr; Zieli?ska, Ma?gorzata; Broniarczyk-Loba, Anna
Management of retinoblastoma (Rb), the most common intraocular malignant tumor in childhood, is tailored to each individual case and based on the overall situation. We present a case of bilateral Rbs in a 4-month-old girl, referred to our center for bilateral leukocoria. In the right eye, the optic disc was partially visible, and three large foci of retinoblastoma were noted adjective in the vitreous cavity with satellite retinal detachment. The macula was obscured by the tumors. The tumor was therefore classified as group D (International classification of retinoblastoma), and thus underwent intravenous chemotherapy with the standard three-agent protocol of ifosfamide, carboplatin and etoposide (ICE protocol) delivered monthly for six cycles, regressing with a type I calcified pattern. The left eye presented three multifocal yellow-white retinal masses, with a total retinal detachment, and secondary glaucoma. The lesions were classified as group E and, therefore, taking the results obtained by Shields et al. in group E tumors into consideration, underwent enucleation. This case clearly shows that this cancer is curable if detected at a stage in which it is still contained within the retina, subretinal space or vitreous, and that the management of Rb relies on an experienced team of ocular oncologists, pediatricians and pediatric ophthalmologists working together for the single goal of saving the child's life. PMID:23277210
Pichi, Francesco; Lembo, Andrea; De Luca, Mariacarla; Hadjistilianou, Theodora; Nucci, Paolo
Purpose. To report two unusual cases of idiopathic lipid keratopathy with symmetrical bilateral annular corneal lipid infiltration and describe confocal microscopy findings. Methods. Case reports. Results. We report two patients with bilateral peripheral deep stromal lipid deposits beginning in an arcuate pattern and progressing to a complete annular shape. Cholesterol crystals were observed in the paracentral area in both cases with characteristic crystalline-like structures in the confocal microscopy. Deep thin corneal blood vessels were observed in one patient, but no cause for then was established, despite decades of followup. This patient had an idiopathic limbitis as well, occurring in episodes. No previous ocular trauma, systemic disease or family history was reported for both cases. Conclusion. These two cases of idiopathic annular lipid keratopathy were observed for more than a decade with documented slow and insidious progression of the infiltrates, in spite of the use of topical steroids in one case. In the majority of other reported cases, a penetrating keratoplasty was made necessary. Differently, we showed that the visual acuity can remain quite good for years with very slow deterioration.
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.
We experienced a patient with a combination of multiple osteochondroses: Blount's disease, bipartite patella, and Sinding-Larsen-Johansson disease in the left knee, and a combination of bipartite patella and Osgood-Schlatter disease in the right knee. The patient was a healthy, active 12-year-old boy with bilateral knee pain. He had been diagnosed with Blount's disease of the left tibia at 2 years of age, and had been treated with open wedge osteotomy. He was diagnosed with bilateral bipartite patellae at the age of 9 years, and was diagnosed with Osgood-Schlatter disease in the right knee and Sinding-Larsen-Johansson disease in the left knee at 10 years of age. The second growth spurt was observed during this period. At 11 years of age, he was diagnosed with an osteochondral fracture of the left lateral femoral condyle and was observed without surgery. This patient showed the sequential appearance of an ossification disorder, probably due to the abnormal response of enchondral ossification to mechanical stress. Overuse in this growth period may have played a role in the development of these osteochondroses. The osteochondral fracture was probably caused by a disruption at one of the weakest parts of the developing skeleton, between the ossification center and the overlying cartilage in the background of an ossification disorder. PMID:11484125
Simultaneous bilateral spontaneous pneumothoraces (SBSP) are uncommon. This report presents the case of a previously well 19-year-old man with a diagnosis of SBSP and symptoms suggestive of occupational asthma. Despite bilateral bullectomy and pleurodesis using a video-assisted thoracoscopic surgical technique, the pneumothorax reoccurred unilaterally and open surgery was performed. This case illustrates a rare condition of bilateral pneumothoraces presenting as a first presentation of occupational asthma and the issues surrounding its management. PMID:24027249
Khan, Waseem Asrar; Curl-Roper, Thomas; Helm, Jenifer; Houghton, Catherine
When patients present with bilateral anterior cruciate ligament (ACL) deficiency and require reconstruction in both knees,\\u000a a single setting or staged approach can be adopted. Although single-setting reconstruction has been described, there are no\\u000a published case series that describe simultaneous bilateral ACL reconstruction. We report a case series of eight patients who\\u000a underwent simultaneous bilateral ACL reconstruction. We used two-camera
Adnan Saithna; Jamie Arbuthnot; Richard Carey-Smith; Tim Spalding
. The aim of this article is to report the MRI features of bilateral parotid haemangiomas of infancy and highlight the diagnostic\\u000a and prognostic value of MRI especially with regard to those lesions that may cause respiratory compromise. Retrospective review\\u000a of MRI scans in six infants with bilateral parotid haemangiomas. The bilateral haemangiomas were symmetrical in all but one\\u000a patient.
Savvas Andronikou; Kieran McHugh; Saaleeha Jadwat; Jane Linward
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
Hosgood, G; Salisbury, K; Blevins, W E; Widmer, W R
Breast cancer is a common manifestation of an underlying genetic susceptibility to cancer, and 5% to 10% of all breast cancers are associated with a germline mutation in a known risk allele. Detection of mutations has implications for targeted screening and prevention strategies for probands, and for genetic counseling and testing of their family members. This report presents a case involving a 35-year-old woman with no family history of breast or ovarian cancer who presented with a palpable right breast lump. Imaging revealed multiple bilateral breast masses and right axillary adenopathy, and core needle biopsies showed invasive ductal carcinoma in both the right and left breast. This report discusses the appropriate genetics evaluation for a patient with bilateral breast cancer at a young age, including testing for mutations in BRCA1 and BRCA2, followed, if negative, by consideration of testing for mutations in TP53 (Li-Fraumeni syndrome). Given the specialized counseling and testing needs of patients with Li-Fraumeni syndrome, and the implications for targeted screening strategies if a mutation is found, referral to a cancer genetics expert is strongly recommended. PMID:23667202
de Bruin, Monique A; Ford, James M; Kurian, Allison W
Ectrodactyly or split hand and foot malformations (SHFMs) are rare malformations of the limbs, characterized by median clefts of the hands and feet, syndactyly, and aplasia and/or hypoplasia of the phalanges. They represent a clinically and genetically heterogeneous disorder, with both sporadic and familial cases. Most of the genomic rearrangements identified to date in some forms of SHFM are autosomal dominant traits, involving various chromosome regions. Bilateral radial ray defects comprise also a large heterogenous group of disorders, including trisomy 18, Fanconi anemia, and thrombocytopenia-absent-radius syndrome, not commonly associated with ectrodactyly. The present paper describes a case of ectrodactyly associated with bilateral radial ray defects, diagnosed in the first trimester of pregnancy, in a fetus affected by trisomy 10. Only four cases of sporadic and isolated ectrodactyly, diagnosed by ultrasonography between 14 and 22 weeks' gestation, have been reported. To our knowledge, the present case is the first report of mosaic trisomy 10 associated with SHFM and radial aplasia. Trisomy 10 is a rare lethal chromosomal abnormality, most frequently found in abortion products. Only six liveborn mosaic trisomy 10 infants, with severe malformations, dead in early infancy, have been reported. A severe clinical syndrome can be defined, comprising ear abnormalities, cleft lip/palate, malformations of eyes, heart, and kidneys, and deformity of hands and feet and most often associated with death neonatally or in early infancy. PMID:23401811
Background: Moebius syndrome (MBS) is an infrequent disease, having an incidence of 1 in 10,000 births, mainly characterized by a congenital bilateral facial paralysis due to an agenesia of the sixth and seventh cranial nerves. In addition, orofacial and limb anomalies are frequently found in these patients. The diagnosis is fundamentally based on different clinical manifestations of the disorder. Clinical case: a female newborn with the clinical picture of Moebius syndrome is presented, and genetic or environmental aspects are discussed. Since the use of misoprostol for abortion and inducing uterine activity in combination with NSAIDs, the number of newborns with MBS associated with this drug has increased. Nowadays, either genetic or environmental factors are associated with MBS. Conclusions: it is necessary that the general and medical community be aware of the risk of teratogenic effects of misoprostol, and the usefulness of genetic counseling whenever there is a newborn with Moebius syndrome. PMID:24144154
Weismann-Netter-Stuhl (WNS) syndrome is a rare skeletal anomaly that affects the diaphyseal part of both the tibiae and fibulae with posterior cortical thickening and anteroposterior bowing. This anomaly is usually bilateral and symmetrical. The patients are generally of short stature. In some cases, a family history suggesting genetic transmission of a mutation with an unknown locus has been reported. In this paper we present an infant with WNS syndrome with bilateral involvement of the femur. Similar clinical findings were defined in three other family members. Conflict of interest:None declared.
A 5-day-old Angus x Hereford calf presented for bilateral haired masses on the eyes and nasolabial planum since birth. The masses were surgically excised from the inferonasal cornea and bulbar conjunctiva of both eyes and the left and right side of the dorsomedial nasolabial planum. Histopathology of the excised tissue confirmed bilateral corneoconjunctival dermoids with ectopic lacrimal glands, and bilateral nasal choristomas and ectopic nasal glandular tissue. Surgery was curative and healing was uneventful. Bilateral ocular dermoids in combination with nasal choristomas and ectopic glandular tissue have not been documented previously in cattle. PMID:18435664
Brudenall, Denise K; Ward, Daniel A; Kerr, Larry A; Newman, Shelley J
We report an 8-month-old boy with Emanuel syndrome who also had the clinical features of Goldenhar syndrome. At birth, he was observed to have bilateral microtia with multiple auricular pits, retrognathia, and a unilateral lipodermoid. Further testing revealed cardiac defects. The finding of a lipodermoid in Emanuel syndrome demonstrates phenotypic overlap between Goldenhar and Emanuel syndromes and suggests a role for genetic analysis in all patients with clinical features that include ear anomalies and lipodermoids. Correct identification of patients with Emanuel syndrome is important for determining whether there is risk of long-term neurodevelopmental disability, and genetic testing can determine parental carrier status to aid in family planning. PMID:23528375
Glaser, Tanya S; Rauen, Katherine A; Jeng, Linda J B; de Alba Campomanes, Alejandra G
Objective To determine the efficacy of “simultaneous” bilateral cochlear implantation (both implants placed during a single surgical procedure) by comparing bilateral and unilateral implant use in a large number of adult subjects tested at multiple sites. Design Prospective study of 37 adults with postlinguistic onset of bilateral, severe to profound sensorineural hearing loss. Performance with the bilateral cochlear implants, using the same speech processor type and speech processing strategy, was compared with performance using the left implant alone and the right implant alone. Speech understanding in quiet (CNCs and HINT sentences) and in noise (BKB-SIN Test) were evaluated at several postactivation time intervals, with speech presented at 0° azimuth, and noise at either 0°, 90° right, or 90° left in the horizontal plane. APHAB questionnaire data were collected after each subject underwent a 3-wk “bilateral deprivation” period, during which they wore only the speech processor that produced the best score during unilateral testing, and also after a period of listening again with the bilateral implants. Results By 6-mo postactivation, a significant advantage for speech understanding in quiet was found in the bilateral listening mode compared with either unilateral listening modes. For speech understanding in noise, the largest and most robust bilateral benefit was when the subject was able to take advantage of the head shadow effect; i.e., results were significantly better for bilateral listening compared with the unilateral condition when the ear opposite to the side of the noise was added to create the bilateral condition. This bilateral benefit was seen on at least one of the two unilateral ear comparisons for nearly all (32/34) subjects. Bilateral benefit was also found for a few subjects in spatial configurations that evaluated binaural redundancy and binaural squelch effects. A subgroup of subjects who had asymmetrical unilateral implant performances were, overall, similar in performance to subjects with symmetrical hearing. The questionnaire data indicated that bilateral users perceive their own performance to be better with bilateral cochlear implants than when using a single device. Conclusions Findings with a large patient group are in agreement with previous reports on smaller groups, showing that, overall, bilateral implantation offers the majority of patients advantages when listening in simulated adverse conditions.
Litovsky, Ruth; Parkinson, Aaron; Arcaroli, Jennifer; Sammeth, Carol
Across bilateral cochlear implants, contralateral threshold shift has been investigated as a function of electrode difference between the masking and probe electrodes. For contralateral electric masking, maximum threshold elevations occurred when the position of the masker and probe electrode was approximately place-matched across ears. The amount of masking diminished with increasing masker-probe electrode separation. Place-dependent masking occurred in both sequentially implanted ears, and was not affected by the masker intensity or the time delay from the masker onset. When compared to previous contralateral masking results in normal hearing, the similarities between place-dependent central masking patterns suggest comparable mechanisms of overlapping excitation in the central auditory nervous system. PMID:23363113
A 57-year-old otherwise healthy woman presented with painless binocular vision loss 1 week after direct application of radiofrequency energy to her orbits. She had no light perception bilaterally. Pupils were dilated and not reactive to light. Fundoscopic exam initially showed optic disc swelling in the right eye and a normal-appearing disc in the left eye. Magnetic resonance imaging of the brain and orbits showed gadolinium enhancement of both intraorbital optic nerves. She underwent a course of high-dose steroid treatment without recovery of vision. Optic discs were pale 11 weeks after injury. With exclusion of other possible causes, this represents a unique case of irreversible binocular optic nerve damage and blindness secondary to radiofrequency exposure.
Liu, Dianna; Cruz, Franz Marie; Subramanian, Prem S
Rotational vertebral artery occlusion, or bow hunter's stroke, is reversible, positional symptomatic vertebrobasilar ischemia. The typical mechanism of action is obstruction of a dominant vertebral artery with contralateral head rotation in the setting of baseline ipsilateral vertebral artery stenosis or occlusion. Here we present a rare case of mechanical occlusion of bilateral patent vertebral arteries manifesting as near syncope with rightward head rotation. Diagnostic cerebral angiography showed dynamic right C5 vertebral occlusion and left C2 vertebral occlusion. The patient underwent right C4/5 transverse process decompression. Postoperative angiogram showed patent flow through the right vertebral artery in neutral position and with head turn with resultant resolution of symptoms. PMID:23465174
Dargon, Phong T; Liang, Conrad W; Kohal, Anmol; Dogan, Aclan; Barnwell, Stanley L; Landry, Gregory J
Chikungunya fever is a relatively rare from of vector-borne viral fever caused by chikungunya virus and spread by bites of the Aedes aegypti and Aedes albopictus mosquito. Epidemics of chikungunya fever have been reported in the past from different parts of the world. Although the virus had been passive for quite some time, recent reports of outbreaks of chikungunya fever in several parts of Southern India have confirmed the re-emergence of this virus. Symptoms of this infection include abrupt onset of fever, chills, and headache, rash, severe joint pain, conjunctival injection and photophobia. Ocular manifestations have been recently reported with this infection. We report a case of a 48-year-old female patient, who presented with defective vision two weeks after a serology proven chikungunya infection. There was bilateral neuroretinitis with peripapillary cotton wool spots. These findings should be kept in mind as an ocular manifestation of chikungunya virus infection.
A case of bilateral congenital pseudarthrosis of the olecranon is reported in a 15-month-old boy. This abnormality was noted at birth because of a fixed flexion deformity of both elbows. The left side was operated on at the age of 16 months and the right side at 21 months. Surgical exploration showed a mobile pseudarthrosis with a 2-cm gap between the tip of the olecranon and the nucleus, each one covered with cartilage. This cartilage was excised, the triceps tendon was lengthened to allow the lowering of the nucleus, and both fragments were fixed with nylon threads. The elbow was kept extended for 30 days in a plaster cast. Fusion was obtained shortly. At the 2-year follow-up, the function was nearly normal as was the radiological appearance. This abnormality is very rarely reported in the literature, and we emphasize the necessity of an early operation. PMID:9260655
Pouliquen, J C; Pauthier, F; Kassis, B; Glorion, C
The variable origin of muscles is not uncommon though the complete or partial absence of the clavicular head of pectoralis major muscle is rare. This paper deals with bilateral partial absence of clavicular head of pectoralis major muscle. The clavicular head was arising only in the middle of medial half of the clavicle and was separated from sternocostal head by a gap of 2.5cm and laterally from the origin of deltoid from clavicle by a gap of 2.5cm. Nerve supply of this muscle was from lateral and medial pectoral nerves as usual. The partial absence of the clavicular head of pectoralis major muscle becomes important in cases of reconstructive surgery as this head is required to cover the lateral acromioclavicular defect and in cases of established facial paralysis. PMID:16827097
Masseter muscle hypertrophy is characterized by unilateral or bilateral enlargement of the masseter muscles affecting both females and males after puberty. Limitations on mouth opening, swollen cheek, and also tension in the region of the hypertrophied muscle are symptoms reported. Also, masseter hypertrophy can cause aesthetic and functional problems. A 40-year old woman was referred to our clinic with the chief complaint of facial appearance with square-face type. To eliminate undesirable facial appearance, surgical intraoral approach compromising reduction of deep masseter muscle with monocortical and bicortical ostectomy of the angle of the mandible was performed. The patient was satisfied with both functional outcomes and aesthetic outcomes on both facial profile and frontal view. No complication was seen intraoperatively and postoperatively after a 12-month follow-up period. This treatment modality would be suggested to gain optimal aesthetic results especially in a square face from the lateral profile. PMID:22337448
Idiopathic granulomatous mastitis (IGM) is an uncommon benign disorder of the breast. At clinical examination, IGM is characterized by an inflammatory process of the breast, usually unilateral. Possible clinical findings are palpable mass with erythematous skin, pain, sterile abscesses, fistula and nipple retraction. Mammography and ultrasound findings are not specific for IGM. Magnetic resonance imaging (MRI) is a useful tool for the differential diagnosis; it is also necessary to delineate the exact extension of the disease and to plan the correct treatment. Final diagnosis is histological. We described an unusual case of IGM with bilateral involvement in a patient with history of pacemaker implantation and IGM typical clinical symptoms. Mammography, ultrasound, and MRI examinations were performed to identify the inflammatory disorder and to plan the correct therapy. Imaging features were correlated with final histological diagnosis of IGM. PMID:23781373
Pistolese, C A; Di Trapano, R; Girardi, V; Costanzo, E; Di Poce, I; Simonetti, G
Alagille syndrome (OMIM 118450) is an autosomal dominant disorder associated with abnormalities of the liver, heart, eye, skeleton, and a characteristic facial appearance. Also referred to as the Alagille-Watson syndrome, syndromic bile duct paucity, and arteriohepatic dysplasia, it is a significant cause of neonatal jaundice and cholestasis in older children. In the fully expressed syndrome, affected subjects have intrahepatic bile
... Pendred syndrome never develop a goiter. Top How common is Pendred syndrome? The SLC26A4 gene, which causes Pendred syndrome, accounts for about five to ten percent of hereditary hearing loss. As researchers gain more knowledge about the syndrome and its features, they hope ...
Acute pyelonephritis complicates 1-2% of pregnancies and causes significant maternal and fetal morbidity and mortality. The diagnosis of renal tuberculosis (TB) is often delayed and commonly presents with sterile pyuria or along with other pyogenic organisms. We report a case where the diagnosis of renal TB was missed in a pregnant woman when she presented with acute pyelonephritis, septic shock, and acute renal failure. There was clinical recovery with antibiotics, but bilateral psoas and perinephric abscesses (TB, Enterococcus sp., and E. coli) were diagnosed when she presented with loin pain and palpable left renal angle swelling. Bilateral psoas abscess due to TB in the absence of skeletal TB and human immunodeficiency virus infection is rare. The presentation of renal TB in pregnancy, its complications, and its management are discussed. PMID:23580809
Veerappan, I; Shanmugam, A; Kumar, S; Velayutham, P
Acute pyelonephritis complicates 1-2% of pregnancies and causes significant maternal and fetal morbidity and mortality. The diagnosis of renal tuberculosis (TB) is often delayed and commonly presents with sterile pyuria or along with other pyogenic organisms. We report a case where the diagnosis of renal TB was missed in a pregnant woman when she presented with acute pyelonephritis, septic shock, and acute renal failure. There was clinical recovery with antibiotics, but bilateral psoas and perinephric abscesses (TB, Enterococcus sp., and E. coli) were diagnosed when she presented with loin pain and palpable left renal angle swelling. Bilateral psoas abscess due to TB in the absence of skeletal TB and human immunodeficiency virus infection is rare. The presentation of renal TB in pregnancy, its complications, and its management are discussed.
Veerappan, I.; Shanmugam, A.; Kumar, S.; Velayutham, P.
Meckel-Gruber syndrome is a rare autosomal recessive lethal malformation characterized by typical manifestations of occipital encephalocele, bilateral polycystic kidneys and post axial polydactyly. The worldwide incidence varies from 1 in 13,250 to 1 in 140,000 live births. Highest incidence was reported in Gujarati Indians. We report a rare case of Meckel-Gruber syndrome and review of literature.
Fahr's disease refers to a rare syndrome characterized by symmetrical and bilateral intracranial calcifications. The basal ganglia and dentate nucleus are the most common site of involvement and most cases present extrapyramidal symptoms. This disease is mostly associated with a phosphocalcic metabolism disorder, especially to hypoparathyroidism. The authors report a case of Fahr syndrome (FS) discovered when a young patient
H. Sbai; L. Smail; S. Hamdani; Y. Essatara; M. Harrandou; M. Khatouf; N. Kanjaa
This report describes a patient who had bilateral facial nerve paralysis, external ophthalmoplegia, absence of pectoralis major muscle at right side, ipsilateral hand and foot, and controlateral hand anomalies. To our knowledge, this is the first patient with Poland syndrome reported in combination with Moebius syndrome, presenting with controlateral hand and ipsilateral foot anomalies.
This report describes a patient who had bilateral facial nerve paralysis, external ophthalmoplegia, absence of pectoralis major muscle at right side, ipsilateral hand and foot, and controlateral hand anomalies. To our knowledge, this is the first patient with Poland syndrome reported in combination with Moebius syndrome, presenting with controlateral hand and ipsilateral foot anomalies.
PURPOSE: Silicone punctal plugs are widely used to conserve moisture in tear-deficient eyes. We studied the efficacy and tolerability of punctal plugs in symptom reduction and improvement of ocular surface disease parameters in dry eye syndrome.METHODS: We reviewed the records of 50 consecutive patients with dry eye syndrome who were symptomatic despite maximally tolerable lubricant therapy and had bilateral lower
Stimulation of cortisol secretion by food intake has been implicated in the pathogenesis of some cases of ACTH-independent Cushing's syndrome, via an aberrant response of the adrenal glands to gastric inhibitory polypeptide (GIP). We report here a novel case of food- dependent Cushing's syndrome in a patient with bilateral ma- cronodular adrenal hyperplasia. In this patient we were able to
FRANCOIS P. PRALONG; FULGENCIO GOMEZ; LOUIS GUILLOU; FRANCOIS MOSIMANN; SEBASTIANO FRANSCELLA; ROLF C. GAILLARD
Mutations in FLCN cause Birt-Hogg-Dubé syndrome, an autosomal dominant disorder notable for development of cutaneous fibrofolliculomas or trichodiscomas, a variety of renal tumors, and spontaneous pneumothorax due to cystic lung changes. We present a woman referred for genetic evaluation due to bilateral parotid gland tumors, who was subsequently diagnosed with Birt-Hogg-Dubé syndrome.
The standard surgery for exertional anterior compartment syndrome is fasciotomy of the anterior and lateral compartments of the leg. We prospectively studied the necessity of lateral compartment release, which can add morbidity and extend recovery. We performed 30 anterior compartment releases in 20 patients (10 bilateral operations) with exertional anterior compartment syndrome but no lateral compartment involvement. We alternately performed
Anthony A. Schepsis; Sanjitpal S. Gill; Timothy A. Foster
A patient who sustained closed head injury demonstrated brachial diplegia and intact leg strength, a case of man-in-the-barrel syndrome. Extensive hemorrhagic contusion was seen in the inferior frontal lobes bilaterally. Although aptly descriptive, the syndrome is neither predictive of an exact anatomical substrate nor representative of a specific etiology. PMID:8186530
This report describes a patient who had bilateral facial nerve paralysis, external ophthalmoplegia, absence of pectoralis major muscle at right side, ipsilateral hand and foot, and contralateral hand anomalies. To our knowledge, this is the first patient with Poland syndrome reported in combination with Moebius syndrome, presenting with contralateral hand and ipsilateral foot anomalies. PMID:16053910
Cetin, Ibrahim Ilker; Akta?, Dilek; Tunçbilek, Ergül
Pheochromocytomas can be a part of familial neoplastic syndromes, in which case they tend to be multiple and involve both adrenal glands. Therefore, sparing adrenocortical function represents a major concern while dealing with these hereditary lesions. Herein, we describe the clinical characteristics and the management strategy of a patient with von Hippel-Lindau (VHL) disease who had multiple, bilateral pheochromocytomas as well as bilateral renal masses, pancreatic masses, and a paracaval mass. Only a portion of the left adrenal gland has remained in situ after two consecutive open surgeries and a percutaneous radiofrequency ablation which have been performed to treat the various components of this syndrome. No adrenal or extra-adrenal pheochromocytoma recurrences have been detected during a follow-up period of more than 2 years. Pancreatic and adrenal functions were normal throughout the postoperative period and never necessitated any replacement therapy. Adrenal cortex-sparing surgery is a valid option for VHL disease patients who present with synchronous bilateral adrenal pheochromocytomas.
Purpose To retrospectively investigate the effectiveness of tube drainage in the treatment of bilateral pneumothorax. Methods We retrospectively examined 40 patients with bilateral pneumothorax treated at the Thoracic and Cardiovascular Surgery Departments of Selcuk University between January 1994 and December 2000. There were 37 male and 3 female patients, aged 5–80 years (mean age, 42 years). The definite diagnosis of
Background The current treatment intervention study determined the effect of coupled bilateral training (i.e., bilateral movements and EMG-triggered neuromuscular stimulation) and resistive load (mass) on upper extremity motor recovery in chronic stroke. Methods Thirty chronic stroke subjects were randomly assigned to one of three behavioral treatment groups and completed 6 hours of rehabilitation in 4 days: (1) coupled bilateral training with a load on the unimpaired hand, (2) coupled bilateral training with no load on the unimpaired hand, and (3) control (no stimulation assistance or load). Results Separate mixed design ANOVAs revealed improved motor capabilities by the coupled bilateral groups. From the pretest to the posttest, both the coupled bilateral no load and load groups moved a higher number of blocks and demonstrated more regularity in the sustained contraction task. Faster motor reaction times across test sessions for the coupled bilateral load group provided additional evidence for improved motor capabilities. Conclusions Together these behavioral findings lend support to the contribution of coupled bilateral training with a load on the unimpaired arm to improved motor capabilities on the impaired arm. This evidence supports a neural explanation in that simultaneously moving both limbs during stroke rehabilitation training appears to activate balanced interhemispheric interactions while an extra load on the unimpaired limb provides stability to the system.
Cauraugh, James H.; Coombes, Stephen A.; Lodha, Neha; Naik, Sagar K.; Summers, Jeffery J.
An unusual combination of downgaze palsy and bilateral ptosis occurred in a patient with central nervous system lymphoma involving bilateral thalamus and midbrain tegmentum. Following treatment with corticosteroids, the transition from total paralysis of downgaze to the supranuclear form was noted, along with alleviation of ptosis, followed by complete resolution of both. The results of serial magnetic resonance images were
Ken Johkura; Atsushi Komiyama; Osamu Hasegawa; Yoshiyuki Kuroiwa
Kerosene ingested, intentionally or accidentally, is toxic. Data is scarce on complications and outcomes of hydrocarbon poisoning following kerosene aspiration in adults and there has been no known case of bilateral hemorrhagic effusion occurring due to it in literature. We, hereby, report a case of a bilateral hemorrhagic pleural effusion secondary to hydrocarbon aspiration in a 40-year old adult.
|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…
Bohnert, Andrea; Spitzlei, Vera; Lippert, Karl L.; Keilmann, Annerose
Bilateral deafness is a rare but possible symptom for ischemia of the vertebrobasilar system, primarily derived from occlusion of anterior inferior cerebellar arteries or their branches. Patients 1 and 2 developed sudden bilateral deafness, soon followed by coma. The proximal segment of the basilar artery was occluded due to atherothrombosis in Patient 1 and arterial dissection in Patient 2. Thrombolytic
This paper proposes separated master system to decrease operational force of bilateral control in free motion. Operational force in bilateral control is not desirable because it prevent accurate reproduction of remote environmental force. Thus to decrease operational force is important. The master system consists of a grip part and an actuator part. A human operator manipulates the grip part. In
Pleomorphic adenoma is the most common benign neoplasm of the parotid gland. However, bilateral synchronous pleomorphic adenomas occur infrequently. We report a case of bilateral synchronous pleomorphic adenoma involving the parotid gland in a 50-yr-old man diagnosed by fine-needle aspiration biopsy. PMID:15108237
Regional Trade Agreements have proliferated in the past two decades. Most recently the United States has spearheaded a drive for comprehensive bilateral free trade agreements with a number of countries including with those of Latin America. The bilateral free trade agreements are WTO plus, very comprehensive and extremely similar in their structure and content. It can be easily argued that
Bilateral facial nerve palsy is a rare but recognised manifestation of HIV seroconversion illness. The pathophysiology of this clinical presentation is thought to be associated with the immune response of the dissemination of virus throughout the body. We describe a case of bilateral facial nerve palsy, subsequently diagnosed with HIV. Related medical literature is also reviewed. The case highlights the
We present a case of bilateral Morgagni hernia in a 68-year-old male with an intermittent history of progressive onset of breath shortness and occasional cardiac arrhythmias. Diagnosis was made by clinical examination and the findings in a plain chest radiograph and was confirmed by computed tomography scan. The patient was operated electively and subjected to a transabdominal approach. A bilateral
Bilateral control has been widely used in many industries including medical surgery. Slave manipulator usually works in a remote site or in a hazardous environment. Master operator is sensing the environment through the slave manipulator. Usually doctors are very sensitive to the tactile sensation they feel through medical tools. If bilateral control is used in surgery, ideally doctor should feel
Traumatic Basal ganglia hemorrhage is relatively uncommon. Bilateral basal ganglia hematoma after trauma is extremely rare and is limited to case reports. We report two cases of traumatic bilateral basal ganglia hemorrhage, and review the literature in brief. Both cases were managed conservatively. PMID:23293672
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.
Monaural Cochlear Implantation is a widely accepted and effective way to enable deaf people to achieve excellent speech understanding in quiet. Recently, it has been started to implant bilaterally to provide patients with the advantages of binaural information. We have conducted a series of basic experiments to study the availability of binaural cues to a bilateral CI-user of interaurally unsynchronized
Bilateral facial paralysis or paresis of peripheral origin is a rare condition and therefore represents a diagnostic challenge. We here present a case of a previously healthy woman who was hospitalized for symptoms of meningitis. On the second day of her hospital stay, she developed bilateral facial paresis. Later, the patient developed also tachycardia and dysrhythmias. A thorough diagnostic procedure including lumbar puncture, routine blood investigation with serological tests, MRI of the brain, Holter monitoring and transoesophageal echocardiographia, revealed meningitis with radiculitis, facial paresis and myocarditis. The clinical triad of meningitis, radiculitis and facial palsy is known as the Bannwarth Syndrome (Lyme disease). The patient was treated with ceftriaxone and recovered well. Despite repeatedly taken serological tests, Borrelia burgdorferi immunoglobulins were not detected. Acquired bilateral facial paralysis can occur in several diseases of infectious, neurological, idiopathic, iatrogenic, toxic, neoplastic or traumatic origin. In this article, we review the differential diagnoses and treatment options of bilateral facial paresis and present a scheme that is helpful in the diagnostic evaluation of this condition. PMID:12836471
Although unilateral common carotid artery (CCA) occlusion and bilateral internal carotid artery (ICA) occlusion have been reported in the past, bilateral CCA occlusion is rare. The management and mechanism of unilateral CCA occlusion and bilateral ICA occlusion are debatable, but those of bilateral CCA occlusion are largely unknown. Herein, we present a case of bilateral CCA occlusion that had an
Case. A 33-year-old male nonsmoker presented with sudden onset of dyspnoea and thoracic pain. Chest radiograph showed a left-sided pneumothorax. Few days later he developed a right-sided pneumothorax. He had a positive family history of pneumothorax. High-resolution computed tomography of the chest showed multiple pulmonary cysts predominantly located in the lower lung regions. We suspected Birt-Hogg-Dubé syndrome (BHD). Conclusion. Birt-Hogg-Dubé syndrome is a rare autosomal dominant inherited genodermatosis and characterised by clinical manifestations including hamartomas of the skin, renal tumors, and pulmonary cysts with spontaneous pneumothorax. BHD is probably underdiagnosed because of the wide variability in its clinical expression. It is important to recognize these patients because of the possibility of developing renal cancer.
We present a report on 16 patients with Goldenhar's syndrome. The criteria we required for the diagnosis of Goldenhar's syndrome consisted of an eye abnormality (lipoma, lipodermoid, epibulbar dermoid, or upper eyelid coloboma) associated with ear, mandibular, or vertebral anomalies (two of the three). Although Treacher Collins' syndrome can be easily differentiated from Goldenhar's syndrome, the differences between Goldenhar's syndrome and hemifacial microsomia are more difficult to delineate. PMID:626178
Bilateral periventricular nodular heterotopia is a neuronal migration disorder characterized by gray matter cellular rests in the periventricular regions. Megalencephaly has not been reported in children with bilateral periventricular nodular heterotopia. No other disorder with a similar phenotype has been reported. Here we report the case of a 5-year-old Japanese boy with bilateral periventricular nodular heterotopia and megalencephaly. Relative macrocephaly was evident at birth, and bilateral periventricular nodular heterotopia and megalencephaly were noted on magnetic resonance imaging (MRI). However, no hydrocephalus or indication of cerebral cortical dysplasia was seen. A mild intellectual disability was present, but the patient had no history of seizures. Genetic analysis revealed no mutation on the capillary sequences for FLNA, and no pathogenic abnormalities were evident on array comparative genomic hybridization. This case could represent a new disease entity: bilateral periventricular nodular heterotopia with megalencephaly. PMID:23439715
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.
The bilateral filter is a nonlinear filter that does spatial averaging without smoothing edges; it has shown to be an effective image denoising technique in addition to some other applications. There are two main contributions of this paper. First, we provide an empirical study of the optimal parameter selection for the bilateral filter in image denoising applications. Second, we present an extension of the bilateral filter: multi-resolution bilateral filter, where bilateral filtering is applied to low-frequency subbands of a signal decomposed using an orthogonal wavelet transform. Combined with wavelet thresholding, this new image denoising framework turns out to be very effective in eliminating noise in real noisy images. We provide experimental results with both simulated data and real data.
A 67-year-old woman and a 22-year-old man, great aunt and great nephew, were examined because of hyperferritinaemia; both had been operated during adolescence for bilateral cataract. The clinical diagnosis 'hereditary hyperferritinaemia-cataract syndrome' (HHCS) was confirmed after DNA-analysis, which showed a point mutation in the L-ferritin gene on chromosome 19 (32G > A, the previously reported Pavia-1 mutation). The further supervision of the patients consisted of reassurance, providing an explanation about the background of HHCS and how it differs from HFE-gene related haemochromatosis, and informing other family members. Both patients were referred back to their general practitioners. HHCS is an autosomal dominant disorder that is characterised by elevated serum ferritin in the absence of iron overload. The dysregulation of ferritin production is caused by heterogenous mutations in the iron responsive element of the L-ferritin gene on chromosome 19, which reduce the binding of iron-regulatory proteins. This results in exaggerated L-ferritin mRNA translation, which is normally tightly controlled by the intracellular iron availability. The only relevant clinical symptom is early-onset, bilateral cataract, which is due to L-ferritin deposits in the stroma of the lens. Internists and haematologists should differentiate this syndrome from haemochromatosis in order to avoid invasive diagnostics and mistreatment. Ophthalmologists should consider this syndrome in patients with congenitial or juvenile cataract. PMID:14560693
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.
Ahlstrom, Jayne B.; Horwitz, Amy R.; Dubno, Judy R.
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.
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
Persistent müllerian duct syndrome (PMDS) is a rare form of male pseudohermaphroditism. Two anatomic forms are described in this syndrome, the male and the female one; the second one is usually associated with bilateral cryptorchidism. About 150 cases are reported in literature; most of them are focused on anatomical, genetic, and surgical aspects. Radiological imaging features, important for diagnosis and therapy planification, are rarely reported. We describe a case of a female type of PMDS studied with multidetector computed tomography. PMID:18603188
The TAR syndrome is an inherited disorder characterized by limb abnormalities, especially absent radii, and hypomegakaryocytic\\u000a thrombocytopenia. Previous reports have included two infants with genitourinary abnormalities. We report a newborn with bilaterally\\u000a absent radii and foreshortened ulnae, hypoplastic humeri, a left clubfoot, a ventricular septum defect, and persistent thrombocytopenia.\\u000a This constellation of abnormalities is consistent with the TAR syndrome. In
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The plausibility and stability of alternative states of the world with large bilateral reductions in nuclear weapons are examined. The current state, with approximately 50,000 nuclear weapons, is compared with two alternative states with arms control agreements with significant verification provisions. The first, minimum deterrence, reduces each superpower's nuclear weapons to a few hundred each and prohibits strategic defense. The second, zero-nuclear-weapons deterrence, abolishes deployed nuclear weapons, but, the superpowers maintain the capability to assemble and deploy a few hundred nuclear weapons on short notice. Strategic defense is encouraged to decrease the incentive to violate the arms control agreements. A conceptual framework is described that captures the fundamental arms procurement and arms control structure in economic terms. The three states are considered the initial conditions and four analyses are performed: potential attack (either a false alarm, an accidental attack, or an intentional attack) stability, major superpower crisis stability, arms procurement stability (the incentives to procure weapons which, although not in violation of the arms control agreements, may be destabilizing), and arms control stability (the incentives to violate the agreements). The analysis focuses on arms procurement and arms control stability.
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.
Stapanian, Martin A.; Stapanian, Adrienne M. P.; Staley, Keith E.
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. PMID:21218683
Stapanian, Martin A; Stapanian, Adrienne M P; Staley, Keith E
We present a case of 11-year-old girl with a history of prominent superficial veins over abdomen and thorax since birth. A superficial vein extending from either inguinal region joined in umbilical region and extended up to right supraclavicular region. Other features of Klippel-Trenaunay syndrome like nevus, limb edema were absent. On radiological investigations both external iliac veins could not be visualized and venous return from lower limbs was draining into the right subclavian vein via these superficial veins. Both external iliac veins could not be identified during surgery. PMID:22729029
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
We report a 29-year-old white female with conjunctival pigmentation after a Stevens–Johnson syndrome (SJS) episode triggered\\u000a by sulfasalazine. The patient developed bilateral tarsal and forniceal conjunctiva and black pigmentation. Diagnostic biopsy\\u000a showed stromal monocyte infiltration consistent with chronic phase SJS and conjunctival pigment of melanic origin and not\\u000a due to drug deposition. Treatment with topical steroids and unpreserved artificial tears
Graciana Fuentes-Páez; Maria C. Mendez; Javier Montañez; Jose M. Herreras; Maria A. Saornil; Margarita Calonge
The case of a 5-month-old black female child with a linear sebaceous naevus syndrome and multiple congenital anomalies is presented. Ocular malformations consisted of colobomatous changes of the lid and retina, dermoid of the conjunctiva, chorioretinal changes, and peripapillary atrophy of the optic nerve. Systemic findings included midline cleft of the secondary palate with involvement by the naevus, bilateral hearing loss, asymmetrical skull bones, ventricular septal defect, epidermal inclusion cyst, and developmental delay without seizures. Images
Classic Lemierre syndrome is a septic internal jugular venous thrombophlebitis secondary to oropharyngeal anaerobic infection in adolescents and young adults. Upper respiratory tract infection is the most common antecedent. We report a case of Lemierre syndrome as a rare infectious disease. A 20-year-old man complained of high fever, right neck discomfort and chest pain. Chest X-ray revealed infiltrative shadows, suggesting bacterial pneumonia. Although cefcapene pivoxil hydrochloride hydrate (CFPN-PI) was given in a local clinic, his symptoms did not improve. Then he was referred to our hospital. Chest CT findings showed bilateral multiple nodular shadows with small cavities, suggesting septic embolization. Fusobacterium necrophorum was cultured from specimen of the blood, and an enhanced neck CT scan showed thrombosis in the right internal jugular vein. These findings led us to a diagnosis of Lemierre syndrome. Four weeks of antibiotics and anticoagulants ameliorated inflammatory findings in blood, but internal jugular vein thrombosis remained. Currently, there is no consensus opinion on the use of anticoagulation in patients with Lemierre syndrome complicated by septic internal jugular thrombosis and embolism. Early and effective antibiotics therapy may prevent the development of the syndrome and its associated complication, although it is unclear whether the outcome will be favorable. PMID:21735747
XX 'pure' gonadal dysgenesis is a disease related to Turner's syndrome. Patients of this disease are characterized by normal female external genitalia, bilateral streak gonads, amenorrhea and sexual infantilism. Recently, it has been reported that point mutations of the FSH receptor gene may be one of cause of this disease. The relationship between criminal behavior and XYY syndrome is still controversial. Increased incidence of disomic sperm in 47,XYY males has been reported by fluorescent in situ hybridization(FISH). Genetic counseling should be done when they undergo intracytoplasmic sperm injection. PMID:14968536
We report the case of an Olympic taekwondo athlete with an atypical bilateral intermittent claudication that represented a handicap in her performance during competition fight. Diagnosis of chronic compartment syndrome was established by confirmation of compartment hypertension and the patient was submitted to fasciectomy. Recurrence of symptoms like numbness or tingling after 6 months raised the suspicion of deep compartmental hypertension that, once confirmed, was treated by a repeat deep fasciectomy. New symptoms appeared 4 months after surgery but no hypertension in the compartment was detected. Functional popliteal artery entrapment syndrome (FPAES) was suspected, an unusual form of this syndrome, in which a hypertrophic plantaris tendon as the cause of the entrapment was observed. Resection of the band was performed in a third operation. The patient evolved favourably, maintaining high competition level. Key pointsWe need a thorough clinical examination to reach a satisfactory clinical diagnosis.FPAES is an uncommon disease that is frequently misdiagnosed and overlooked.Dynamic tests are essential for diagnosis de FPAES. PMID:24149572
Vilás, Ramón Olivé; Rodriguez, Lorenzo Álvarez; Campos, Montserrat Yeste; Moran, Antonio De la Torre; Mas, Ferran Latorre
We report the case of an Olympic taekwondo athlete with an atypical bilateral intermittent claudication that represented a handicap in her performance during competition fight. Diagnosis of chronic compartment syndrome was established by confirmation of compartment hypertension and the patient was submitted to fasciectomy. Recurrence of symptoms like numbness or tingling after 6 months raised the suspicion of deep compartmental hypertension that, once confirmed, was treated by a repeat deep fasciectomy. New symptoms appeared 4 months after surgery but no hypertension in the compartment was detected. Functional popliteal artery entrapment syndrome (FPAES) was suspected, an unusual form of this syndrome, in which a hypertrophic plantaris tendon as the cause of the entrapment was observed. Resection of the band was performed in a third operation. The patient evolved favourably, maintaining high competition level. Key points We need a thorough clinical examination to reach a satisfactory clinical diagnosis. FPAES is an uncommon disease that is frequently misdiagnosed and overlooked. Dynamic tests are essential for diagnosis de FPAES.
Vilas, Ramon Olive; Rodriguez, Lorenzo Alvarez; Campos, Montserrat Yeste; Moran, Antonio De la Torre; Mas, Ferran Latorre
Patella aplasia-hypoplasia is a rare condition characterized by the congenital absence or marked reduction of the patellar bone. It is well known that patella aplasia-hypoplasia occurs in nail-patella syndrome, small patella syndrome, and several other syndromes. However, isolated patella aplasia-hypoplasia without associated clinical or radiologic anomalies, first described by Kutz in 1949, is an extremely rare condition. This report describes 12 years' follow-up after medial patellofemoral ligament reconstruction surgery performed for bilateral recurrent patellar dislocation in a 29-year-old patient with isolated patella aplasia-hypoplasia. The patient had no radiographic findings of elbow abnormalities, exostoses of the ilia, or abnormal ossification of the ischiopubic junction. The right patella was severely subluxated, and the left patella was mildly subluxated. The preoperative Kujala score was 28 points for the right patella and 23 points for the left. Medial patellofemoral ligament reconstruction, wide lateral release, and patellaplasty with multiple drilling were done in July 1993 for the left knee and in August 1993 for the right. At 12 years' follow-up, the Kujala score was 68 points for the right patella and 73 points for the left, and the patient was satisfied with the result. Magnetic resonance images showed both patellae without subluxation and bulky regenerated ligaments. PMID:17916489
Exophthalmos is typically associated with Graves' ophthalmopathy. Although originally described by Harvey Cushing, exophthalmos is an underappreciated sign of Cushing's syndrome. We present a case of a 38-year-old female who presented with severe bilateral proptosis and was subsequently diagnosed with Cushings disease. We discuss the possible mechanisms causing exophthalmos in patients with either endogenous or exogenous hypercortisolemia.
|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…
We report a 6-year-old boy who presented with status epilepticus, who had facial dysmorphism, growth and mental retardation. On investigation, he had hypocalcaemia, hypoparathyroidism and bilateral calcification of basal ganglia in cranial tomographs; features consistent with Sanjad Sakati syndrome. He was treated with intravenous calcium gluconate initially followed by oral calcium and calcitriol and recovered completely. PMID:23378547
Prasad, Rajniti; Kumari, Chhaya; Mishra, Om Prakash; Singh, Utpal Kant
Two unrelated girls with craniosynostosis and bilateral cleft lip and palate who also had developmental delay and umbilical herniae are presented. We propose that these patients have the same condition, and that their combination of features may constitute a new syndrome. Management of the patients is discussed. PMID:21187764
We report a boy with a nasal deformity, choanal atresia, bifid uvula, severe bilateral microphthalmia and a facial cleft who showed regression of development at the age of 2 years with subsequent improvement. We suggest he represents a further case of the rare Fryns "Anophthalmia-Plus" syndrome. PMID:12514364
We observed the Joubert syndrome (JS) associated with bilateral morning glory disk anomaly and cystic dysplastic kidneys in three patients from a consanguineous kindred. Homozygosity mapping excluded three JS candidate loci as sites harboring the disease gene. We thus delineate an autosomal recessive disorder, distinct from JS and related conditions.
Andreas R Janecke; Thomas Müller; Ingmar Gassner; Alfons Kreczy; Eduard Schmid; Florian Kronenberg; Barbara Utermann; Gerd Utermann
Acute respiratory distress syndrome (ARDS) is a life threatening respiratory failure due to lung injury from a variety of precipitants. Pathologically ARDS is characterised by diffuse alveolar damage, alveolar capillary leakage, and protein rich pulmonary oedema leading to the clinical manifestation of poor lung compliance, severe hypoxaemia, and bilateral infiltrates on chest radiograph. Several aetiological factors associated with the development
A Dushianthan; M P W Grocott; A D Postle; R Cusack
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
Thoracotomic, trans-sternal or thoracoscopic approaches through transmediastinal access for contralateral lung are operative alternatives for bilateral pulmonary lesions. Video-assisted thoracoscopic surgery (VATS) for spontaneous pneumothorax (PTX) is now considered as a standard approach. Herein, we report a novel method of apico-posterior transmediastinal ipsilateral approach using VATS to perform simultaneous bilateral bullectomy in two young men with simultaneous bilateral spontaneous PTX. This new VATS access is technically feasible and may mitigate postoperative pain and avoid a secondary thoracic incision. PMID:18187456
Cho, Deog Gon; Do Cho, Kyu; Kang, Chul Ung; Seop Jo, Min
A single pleural space can lead to serious simultaneous bilateral pneumothorax in cases of congenital or acquired pleuro-pleural communication. Here we report a 35-year-old man with bilateral pneumothorax. Chest computed tomography scans revealed a small air space between the esophagus and aorta, suggesting pleuro-pleural communication. Bilateral thoracoscopic bullectomy was performed. Repeated inspection revealed a 2-cm-long pleural window between the aorta and esophagus, which was closed with a collagen patch. Although congenital pleuro-pleural communication has been regarded as rare, as many as nine patients have been reported (if suspected cases are included). The lower middle mediastinum should be carefully inspected.
A single pleural space can lead to serious simultaneous bilateral pneumothorax in cases of congenital or acquired pleuro-pleural communication. Here we report a 35-year-old man with bilateral pneumothorax. Chest computed tomography scans revealed a small air space between the esophagus and aorta, suggesting pleuro-pleural communication. Bilateral thoracoscopic bullectomy was performed. Repeated inspection revealed a 2-cm-long pleural window between the aorta and esophagus, which was closed with a collagen patch. Although congenital pleuro-pleural communication has been regarded as rare, as many as nine patients have been reported (if suspected cases are included). The lower middle mediastinum should be carefully inspected. PMID:23372954
A 75-year-old female visited our hospital with bilateral adrenal masses that were detected incidentally during lumbar spine magnetic resonance imaging (MRI) for the evaluation of radiating flank pain. Consecutive computed tomography and MRI revealed bilateral adrenal masses with no evidence of lymph node enlargement or local invasion; 2[(18)F]fluoro-2-deoxyglucose (FDG)-positron emission tomography showed an intense FDG accumulation in both adrenal glands without abnormal FDG uptake in extra-adrenal regions. The laboratory test results were within normal ranges. We performed a bilateral adrenalectomy. The pathologic diagnosis of both adrenal masses was consistent with adrenocortical carcinoma. The patient recovered well with no complications.
Kim, Dong Gon; Kim, Sang Deuk; Cha, Jai Seong; Pak, Chul-Ho
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
Elective bilateral exposure of iliac arteries during endovascular or laparoscopic aneurysm repair is commonly performed through two retroperitoneal incisions in the iliac fossa. Larger incisions are necessary when simultaneous external and common iliac exposures are needed. We describe a new technique using a single incision for bilateral approach of the iliac arteries. Exposure of iliac arteries through this bilateral anterior paramedian retroperitoneal approach allows the introduction of endografts, crossover ilioiliac bypass, implantation of graft limbs for bifurcated bypass grafting, reconstruction of internal iliac arteries, and ligature of iliac arteries. PMID:19450948
Colacchio, Giovanni; Tomescot, Andre; de Loubresse, Christian Garreau; Coggia, Marc
Tibial tubercle sleeve fracture is a rare injury. In concept, it is similar to the patellar sleeve fracture in a skeletally immature patient. We describe a unique case of simultaneous bilateral tibial tubercle sleeve fractures in a 12-year-old boy. Radiographs and MRI confirmed the injury. The patient underwent open surgical repair of bilateral sleeve fractures with suture anchor fixation. At the final followup, 3 years after his initial injury, the patient demonstrated full knee function bilaterally without radiographic evidence of growth disturbances.
Turner syndrome (TS) is the human being's most frequent sex chromosome abnormality. Progressive sensorineural hearing loss is documented in more than 50% of the women affected by this syndrome. Although Mondini defect is the cochlear congenital malformation most frequently identified in other polymalformative syndromes, it has rarely been reported in TS. We describe the case of a 32-year-old woman with TS who presented progressive sensorineural hearing loss. The computed tomography of the ears showed bilateral Mondini deformity. PMID:21529720
Exercise-induced acute compartment syndrome of the thigh is an uncommon entity. We present a rare case of bilateral exercise-induced three-compartment syndrome of the thighs that required fasciotomies. The objective of this study was to understand the history, physical examination, signs, symptoms, pathophysiology, diagnosis, and treatment of compartment syndrome and rhabdomyolysis. A 42-year-old man presented to the Emergency Department (ED) complaining
Timothy W. King; Oren Z. Lerman; Joseph J. Carter; Stephen M. Warren
We report a full-term male infant born to nonconsanguinous parents who had clinical features of Goldenhar syndrome and cri du chat syndrome. At birth, the infant was noted to have dysmorphic features with bilateral preauricular tags, rotated ears, bilateral epicanthic folds, a left epibulbar lipodermoid, and an accessory left nipple. After he was assessed for feeding difficulty and tachypnea, he was found to have esophageal atresia with tracheoesophageal fistula. In addition, he had a high-pitched, cat-like cry, characteristic of cri-du-chat syndrome. He also failed a hearing test. Chromosomal analysis and fluorescence in situ hybridisation studies showed an unbalanced karyotype with a terminal deletion of the segment p14 on the short arm of chromosome 5, which is consistent with the cri-du-chat locus. The association of Goldenhar syndrome and cri-du-chat syndrome in this patient suggests that the chromosome 5p14 locus may harbor a gene implicated with Goldenhar syndrome. PMID:12825068
... Fanconi syndrome is unknown. Common causes of Fanconi syndrome in children are genetic defects that affect the body's ability to break down certain compounds such as: Cystine (cystinosis) Fructose ( fructose ...
... children with Moebius syndrome have some degree of autism. There are four recognized categories of Moebius syndrome: Group I, characterized by small or absent brain stem nuclei that control the cranial nerves; Group II, characterized by loss and ...
Duane's syndrome is an unusual congenital form of strabismus where there is paradoxical anomalous lateral rectus innervation of the affected eye due to misdirection of axons destined for the medial rectus. Three types of Duane's syndrome are recognized. Most cases of Duane's syndrome are sporadic but up to 10% are familial, usually with autosomal dominant inheritance. Several autosomal dominant syndromes with dysmorphic features are associated with Duane's syndrome. Chromosomal loci for genes contributing to Duane's syndrome have been suggested at 4q, 8q and 22q. Duane's syndrome is heterogeneous at multiple levels with variations in its ocular manifestations, accompanying systemic manifestations and in the chromosomal loci with which it may be associated. The definition clinically and genetically of the various subgroups of Duane's syndrome will provide a valuable insight into brainstem axonal guidance to the extraocular muscles during human development. PMID:10809934
... called mucopolysaccharidoses, or MPS. See also: MPS II (Hunter syndrome) MPS IV (Morquio syndrome) MPS III (Sanfilippo ... For more information and support, contact one of the following ... Society for MPS and Related Diseases -- www.mpssociety.ca Society ...
Asherman syndrome is the formation of intrauterine adhesions (scar tissue), which typically develop after uterine surgery. ... Asherman syndrome is a rare condition. In most cases, it occurs in women who have had several dilatation ...
... in different stages of the disease over a lifetime. The NICHD has supported research on Rett syndrome ... Rett disorder RTT Medical or Scientific Names 1 Autism-dementia-ataxia-loss of purposeful hand use syndrome ...
... can cause many complications. Sometimes the complications are life threatening. Overview Marfan syndrome is a genetic disorder. ... live longer and enjoy a good quality of life. Many people who have Marfan syndrome and are ...
... with Turcot syndrome have multiple adenomatous colon polyps (polyps in the colon made up of cells that form mucous), an ... Turcot syndrome diagnosed? A person with multiple adenomatous colon polyps and/or colorectal cancer , along with either glioblastoma ...
NINDS Sotos Syndrome Information Page Synonym(s): Cerebral Gigantism Table of Contents (click to jump to sections) What is Sotos Syndrome? Is there any treatment? What is the prognosis? What research is ...
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We report on a 16-week fetus, in which detection of increased nuchal translucency thickness and bilateral intracardiac echogenic foci led to the prenatal diagnosis of truncus arteriosus communis, interruption of the aortic arch and aplastic thymus. Cytogenetic examination confirmed a 22q11.2 microdeletion consistent with the suspected CATCH 22 syndrome. Subsequently hydrops fetalis developed and the fetus died in utero at 18 weeks. This case report supports the hypothesis that both cardiac failure and left ventricular outflow tract obstruction may cause increased nuchal translucency thickness. The association between increased nuchal translucency thickness and CATCH 22 syndrome should be considered in diagnostic procedures. The sonographic diagnosis of both increased nuchal translucency thickness and intracardiac echogenic foci requires specialist ultrasonography and echocardiography. In particular, identification of right-sided or bilateral echogenic foci should prompt further detailed examination. PMID:11982988
Machlitt, A; Tennstedt, C; Körner, H; Bommer, C; Chaoui, R
Cowden syndrome, or the multiple hamartoma syndrome, is a familial cancer syndrome with involvement of various organ systems. Inheritance is autosomal dominant with variable expression. Progressive macrocephaly, scrotal tongue, and mild to moderate mental retardation are important signs indicating the syndrome in young children. Other mucocutaneous symptoms, for example, trichilemmomas in the nasolabial folds and palmar and plantar hyperkeratotic pits, usually become evident later in childhood. They are often accompanied by the appearance of subcutaneous lipomas and cutaneous haemangiomas. Images
In this article, we present two cases of bilateral retinal detachment in toxemia of pregnancy. The first case describes 31-year old patient admitted to hospital due to toxemia of pregnancy in the 38th week of the second pregnancy. Medical examination following admission showed blood pressure 180/130 mm Hg oedema of face and legs. Accessory investigations displayed proteinuria, leukocyturia, bacteriuria. At the fundus of eye the features of initial retinopatia hypertonica were discovered. On the fifth day of the patient's hospitalization because of the worsening of general health condition and sudden blood pressure increase, the pregnancy was delivered by cesarean section. Some hours after awakening the patient reported the loss of vision of both eyes. Ophthalmological examination showed the occurrence of retinal detachment. Tearing wasn't found. When the protein deficiency was supplemented and antiinflammatory and oedema--reducing treatment was administered the blood pleasure stabilized retina attached itself and visual functions returned. In the second case 22-year old patient was admitted to hospital at term of labour with blood pressure 150/90 mm Hg and a little proteinuria. On the second day of hospitalization the pregnancy was delivered through natural passages. A few hours after the delivery the patient reported indistinct vision and blurring of the image. Through ophthalmological examination retinal detachment was stated. Tearing was not found. Thanks to bed regime and resorbing treatment total retinal attachment was gained and visual functions returned. This case suggests that retinal detachment of pregnant woman does not have to be proceeded by symptoms of toxemia of pregnancy and the period of delivery may accelerate and release mechanisms damaging choriocapillaries, which causes the flow of liquid from vessels of chorioidea to subretinal space. PMID:10526447
BACKGROUND Steroidogenic factor 1 (SF1/AdBP4/FTZF1, NR5A1) is a nuclear receptor transcription factor that plays a key role in regulating adrenal and gonadal development, steroidogenesis and reproduction. Recently, haploin-sufficiency of SF1 has been described in several 46,XY individuals with mild gonadal dysgenesis and impaired androgenization, but normal adrenal function, suggesting that dosage-sensitive or domain-specific effects of SF1 action are important in human testicular development and function. Our objective was to investigate whether partial defects in SF1 function might be associated with milder male reproductive phenotypes, such as bilateral anorchia (‘vanishing testis syndrome’) and micropenis. METHODS This study involved mutational analysis of NR5A1 in 24 individuals with bilateral anorchia and micropenis from the French Collaborative Anorchia study, as well as in vitro functional studies of SF1-dependent transcriptional activation and computer modeling. RESULTS A novel heterozygous missense mutation (V355M) in SF1 was found in one boy with a micropenis and testicular regression syndrome. This non-synonymous change was found to affect a highly conserved amino acid within helix 7 of the ligand-binding domain of SF1. This V355M mutation did not affect stability or nuclear localization, but did result in an ~50% reduction in SF1 activity in several different assay systems. CONCLUSIONS In conclusion, heterozygous partial loss of function mutations in SF1 may be associated with bilateral anorchia (‘vanishing testis syndrome’) and micropenis in humans.
Philibert, Pascal; Zenaty, Delphine; Lin, Lin; Soskin, Sylvie; Audran, Francoise; Leger, Juliane; Achermann, John C.; Sultan, Charles
What is Klinefelter syndrome? Klinefelter syndrome is a group of conditions that affects the health of males who are born with at least one extra ... Y chromosome (XY) is male. Most males with Klinefelter syndrome, also called XXY males, have two X ...
Describe since the beginning of the fifties, the mannequin syndrome consists of anorexia, amenorrhea and swelling of the parotid glands. This variety of symptoms is usually found in young female patients who want to remain thin. In fact, this syndrome has been several times diagnosed in fashion models or airhostesses;hence the term "mannequin syndrome". PMID:3799184
Cosins, J M; Frederickx, Y; Yousif, A; Hamoir, M; Van den Eeckhaut, J
In recent years, a number of comprehensive reviews have been written on inherited intestinal polyposis syndromes (1–7), but none has dealt specifically with Gardner's syndrome and none has focused on basic research being carried out in an attempt to under-stand this syndrome and to improve the medical management of affected patients. A better understanding of this rare genetic disorder is
The authors examine a family, in which two brothers were affected by a severe expression of arteriohepatic dysplasia (ADH, Alagille syndrome), an autosomal dominant disorder associated with intrahepatic cholestasis, characteristic facial appearance, congenital embryotoxon. One of these two cases presented a keratoconus and both had retinal pigmentary degeneration with pigment clumping. The father showed a benign expression of ADH, including the characteristic facial appearance and posterior embryotoxon. Another brother presented only retinal pigmentary abnormalities and a bilateral arcus senilis-like corneal opacity, without any other clinical sign of ADH. The presence of posterior embryotoxon in all the cases of Alagille syndrome confirms that this sign is a hallmark of ADH, also in its benign expression. PMID:1779119
Ricci, B; Lepore, D; Iossa, M; Santo, A; Chiaretti, A
BACKGROUND: Temporomandibular joint (TMJ) ankylosis is an extremely disabling affliction that causes problems in mastication, digestion, speech, appearance, and hygiene. The disease also has an impact on the psychological development of the patients. CASE REPORT: A 49-year-old male patient came to our unit with complaint of restriction in his alimentation and of mouth opening. In the anamnesis, the patient reported that this limitation began 11 years ago, soon after a serious car accident that caused trauma on the face. In the clinical examination, the patient's mouth opening was 1 mm, and there was satisfactory occlusion. He was diagnosed with bilateral temporomandibular joint (TMJ) ankylosis after an imaging examination. DISCUSSION: In order to confirm the post-traumatic etiology of the anquilose, we got the medical records from the hospital where the patient received the first treatment. The records, at that time, reported facial trauma with bilateral condylar fracture and comminuted symphyseal fracture which was reduced and fixed followed by maxillomandibular fixation that remained for 45 days. After collecting all the information needed for diagnosis, surgical treatment was proposed through arthroplasty. The patient was placed on immediate post-operative jaw physiotherapy, and 12 months after surgery, his mouth opening improved to 35 mm. Thus, in our case, ankylosis may have been developed as a sequel of the mandibular fractures. PMID:23306946
Benaglia, Matheus B; Gaetti-Jardim, Ellen Cristina; Oliveira, Janayna G Paiva; Mendonça, Jose Carlos Garcia
Supernumerary paramolars are a rare anomaly of the maxillofacial complex. They are more common in the maxilla than the mandible. This article reports a rare case of bilateral maxillary paramolars, their complications and management.
The differential diagnosis of bilateral parotid gland enlargement in children includes infectious, inflammatory, and neoplastic disorders. We present the case of a 13-year-old male who presented with a 5-week history of bilateral parotid swelling. On exam, both parotid glands were nontender, smooth, and diffusely enlarged. He had slightly elevated inflammatory markers, but other lab results were normal. A neck CT revealed symmetric enlargement of the parotid, submandibular, and sublingual glands. A chest CT revealed scattered peripheral pulmonary nodules and bilateral hilar adenopathy. A parotid gland biopsy showed multiple noncaseating granulomas with multinucleated giant cells surrounded by lymphocytes, consistent with the diagnosis of sarcoidosis. Special stains for acid-fast and fungal organisms were negative. Using this illustrative case, we discuss the differential diagnosis of bilateral salivary gland enlargement in children and review the etiology, diagnosis, clinical manifestations, and treatment of pediatric sarcoidosis. PMID:23102965
Banks, Gretchen C; Kirse, Daniel J; Anthony, Evelyn; Bergman, Simon; Shetty, Avinash K
The hypothesis that bilaterally redundant, and mutually coupled circadian pacemakers are located near the lobula of the optic lobes of the cockroach,Leucophaea maderae, was investigated in a series of lesion experiments.
The bilateral system was designed to study limb stiffness in different movement tasks. The mechatronics consists of two angular voice coil actuators (+/- 40 Nm) with embedded rotary (+/- 20 degrees) and torque sensors driven by voltage controlled current ...
Congenital absence of tibia is a rare anomaly, and may be total or partial, unilateral or bilateral. Total absence is more frequent than partial, unilateral absence occurs more often than bilateral, with right limb more commonly affected than the left. In partial defect, almost always the distal end of the bone is affected, and of the bilateral cases, there may be total absence on both sides, or total on one side and partial on the other. Males are slightly more commonly affected than the females. Though, the family history is usually negative for congenital abnormalities and other diseases, there is a considerable chance of occurrence of congenital defect of the tibia or of other abnormalities, in near or remote relatives. We report a case of newborn having bilateral tibial hemimelia type VIIa.
Sternal wound complications after cardiac surgery are associated with increased morbidity and mortality. Wire migrations associated with sternal dehiscence can lead to catastrophic haemorrhage unless intervened in time. We present a case of sternal wire migration causing bilateral pneumothoraces.
A simple inexpensive scheme of an electronic amplifier for Wheatstone bridge application is presented. The bridge is excited from a bilateral constant current square wave source. The advantage of constant current drive is presented.
A simple inexpensive scheme of an electronic amplifier for Wheatstone bridge application is presented. The bridge is excited from a bilateral constant current square wave source. The advantage of constant current drive is presented. PMID:18647085
A simple inexpensive scheme of an electronic amplifier for Wheatstone bridge application is presented. The bridge is excited from a bilateral constant current square wave source. The advantage of constant current drive is presented.
We present a patient with a Rathke's cleft cyst who presented with rapidly progressive bilateral 6th nerve palsy. A 20-year-old woman with a history of cleft palate, hypertension, and hydronephrotic kidneys presented with a one month history of headache, associated with dizziness and diplopia on horizontal gaze. Examination was significant for profound bilateral 6th nerve palsies. Magnetic resonance imaging showed a hypodense mass that filled the sella and compressed the right cavernous sinus without contacting the optic chiasm. Pituitary function was normal. An endoscopic, transnasal transsphenoidal resection of the lesion was performed; microscopic examination revealed a Rathke's cleft cyst. Surgical excision resulted in near complete resolution of the bilateral 6th nerve palsy. Rathke's cleft cysts are an unusual cause of bilateral sixth nerve palsy and represent a potential cause of cranial neuropathy. PMID:19621262
Grover, Vinni; Hamrahian, Amir H; Prayson, Richard A; Weil, Robert J
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.
Taylor, Taryn Lise; Frankovich, Renata; Rumball, Jane
A 46-year-old woman was admitted to our hospital because of behavioral changes. Her mentality was fluctuating vigorously and neurological examination revealed disorientation and word finding difficulty. MRI demonstrated bilateral frontal and right temporal lesions. Cerebrospinal fluid examination showed predominantly lymphocytic pleocytosis. Brain biopsy disclosed inflammation but not neoplasm. Repeated steroid therapy gave her a recovery in neurological manifestations and MRI findings. As we got a positive result of anti-Hu antibody after her complete recovery, we did screening for tumors and found small cell lung cancer. She got a chemotherapy and remains free of relapse of any symptoms. There have been few reports in that anti-Hu associated paraneolastic syndrome showed steroid responsive frontal lesions. We suggest that anti-Hu associated paraneoplastic encephalitis should be considered for steroid responsive encephalitis with brain lesions other than limbic system, because early detection of paraneoplastic encephalitis and timely antitumor treatment are important for patient's prognosis. PMID:23603540
The purpose of this study was to evaluate the functional outcomes in patients affected by bilateral temporomandibular joint (TMJ) ankylosis treated with TMJ total alloplastic reconstruction with stock prosthesis. As a matter of fact, ankylosis of the TMJ may produce the narrowing of the oropharyngeal airway space resulting in the obstructive sleep apnea syndrome, a pathological condition characterized by repetitive upper airway obstructions during sleep, resulting in arousal from sleep, sleep fragmentation, arterial oxygen desaturation. and daytime hypersomnolence.Clinical results demonstrate that total TMJ reconstruction with prosthesis is the surgical modality of choice to obtain the counterclockwise rotation and the advancement of the maxillomandibular complex, which significantly increase the volume of the oropharyngeal airway space in this kind of patients. PMID:23524813
Introduction The congenital constriction ring of lower extremity is very uncommon and rare condition. The actual incidence in general population is not known. In English literature, very few cases are reported time to time as congenital constriction band syndrome associated with musculoskeletal disorder like congenital talipes equino varus. The lesion can involve skin only or goes to deeper structure up to bone, which can lead to gangrene of foot or auto amputation. Case presentation We are presenting a case of bilateral congenital constriction ring in lower limb who presented at age of 4 year without any other associated congenital anomaly, simply managed by Z-plasty, which improves quality of life after physiotherapy. Conclusion Congenital constriction ring of lower limb is extremely rare condition in children. Early diagnosis and management is mandatory, either in single stage or by stage procedure, to prevent auto-amputation of limb and to improve quality of life on feet.
We identified a Canadian-Mennonite family in which a brother and sister have hydrocephalus due to obstruction at the foramen of Monro and profound bilateral sensorineural deafness. This appears to be a unique combination of anomalies and, to our knowledge, has not been reported previously. Both parents and a brother are phenotypically normal. The parents are second cousins. Thus, on the basis of consanguinity, affected sibs of both sexes, and in the absence of evidence for intrauterine infections or other adverse perinatal events, this syndrome is likely inherited in an autosomal recessive fashion. 37 refs., 5 figs.
A 22-year-old man was referred to our hospital because of facial edema and increasing body weight. Under the diagnosis of Cushing syndrome due to an adrenocorticotropic (ACTH)-producing thyroid tumor, thyroidectomy with regional lymph node dissection was performed. Histopathological diagnosis was thyroid carcinoid. In spite of the operation, serum ACTH and cortisol concentrations increased again due to mediastinal lymph node metastasis. His hyper-cortisolemia was resistant to drug therapy. Then, laparoscopic bilateral adrenalectomy was performed. After the operation, hyper-cortisolemia and clinical symptoms markedly improved. An additional chemotherapy is implemented because of new metastasis in the mediastinum lymph nodes. PMID:23945320
A patient is described who developed co-existent Reiter's disease and bilateral non-traumatic retinal detachment. Although numerous ophthalmological disorders are associated with Reiter's disease and HLA-B27, to our knowledge this is the first report of co-existent retinal detachment. Although this relationship may be fortuitous, there are a number of immunological mechanisms common to both entities. Retinal detachment, unlike bilateral conjunctivitis and
We report the case of a 21-year-old male patient admitted emergently with progressive bilateral severe visual loss for 1month. Posterior segment examination revealed bilateral stellate neuroretinitis. Infectious serologies were negative, and brain CT was normal. Physical examination was remarkable for malignant hypertension of 220/150mmHg. Diagnostic work-up revealed a pheochromocytoma documented by histopathological exam upon adrenalectomy. The disc edema and macular exudates resolved once the hypertension was controlled. PMID:23790444
El Haddad, S; Benchekroun, N; Rhafour, A; Ahbeddou, S; Rhafour, I; Imdary, I; Idrissi Alami, S; Soufi, G; Berraho, A
A 48-year-old woman noted progressive, painless visual loss in her left eye. She was diagnosed with bilateral optic nerve sheath meningiomas (ONSMs), extending across the planum sphenoidale. Radiation was offered, but the patient declined. She has been followed for more than 27 years with stable visual function and neuroimaging findings. Bilateral ONSMs, although usually described as aggressive in nature, may follow a stable clinical course. PMID:23001155
Kothari, Nikisha A; Kulkarni, Kaushal M; Lam, Byron L
We report a case of bilateral apical lung bullae that collapsed following an episode of community-acquired pneumonia with bilateral air fluid levels. With standard treatment for community-acquired pneumonia, management of a patient that may have qualified for bullectomy, (as in our case) showed complete resolution of all pathology without surgical intervention. Conservative management took precedence in alleviating pathology over surgical intervention. PMID:23843717
Shariff, Masood A; Singh, Vijay A; Daniele, Edward D; Goyal, Nikhil; Peykova, Deliana; Nabagiez, John P; Rosell, Frank M