Cases reported "Nervous System Diseases"

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1/165. Parenchymatous cerebral neurocysticercosis in a quadriplegic patient.

    OBJECTIVE: To present and discuss a case of cerebral neurocysticercosis in a quadriplegic patient. DESIGN: Case report of a case of neurocysticercosis in a high level spinal cord injury (SCI) patient who developed episodes of autonomic dysreflexia and orthostatic hypotension associated with transient neurologic deficits and seizures. SETTING: spinal cord Unit of the University Hospital of Geneva, switzerland. SUBJECT: Single patient case report. MAIN OUTCOME MEASURE: Clinical and radiological magnetic resonance imaging follow-up of the patient between July 1995 and October 1997. RESULTS: Treatment of cysticercosis with praziquantel relieved the patient from autonomic dysreflexia, symptomatic orthostatic hypotension, transitory neurological deficits and seizures. CONCLUSION: diagnosis of neurocysticercosis in a quadriplegic patient might be difficult because of frequent overlaps with some usual symptoms occurring in high level SCI, mostly autonomic dysreflexia and orthostatic hypotension. neurocysticercosis should be kept in mind when a SCI patient living in, or coming from endemic zones presents with new neurological abnormalities and seizures. magnetic resonance imaging appears to be more sensitive than computerised tomography to confirm the diagnosis of active cysticercosis. Treatment with praziquantel associated with cimetidine to increase the drug bioavailability and prednisone to reduce the inflammatory reaction gives good results.
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2/165. 18Fluoro-2-deoxyglucose (18FDG) PET scan of the brain in type IV 3-methylglutaconic aciduria: clinical and MRI correlations.

    The clinical, 18fluorodeoxyglucose positron emission tomography (18FDG PET) and the magnetic resonance imaging (MRI) brain scan characteristics of four patients diagnosed to have 3-methylglutaconic aciduria were reviewed retrospectively. The disease has a characteristic clinical pattern. The initial presentations were developmental delay, hypotonia, and severe failure to thrive. Later, progressive encephalopathy with rigidity and quadriparesis were observed, followed by severe dystonia and choreoathetosis. Finally, the patients became severely demented and bedridden. The 18FDG PET scans showed progressive disease, explaining the neurological status. It could be classified into three stages. Stage I: absent 18FDG uptake in the heads of the caudate, mild decreased thalamic and cerebellar metabolism. Stage II: absent uptake in the anterior half and posterior quarter of the putamina, mild-moderate decreased uptake in the cerebral cortex more prominently in the parieto-temporal lobes. Progressive decreased thalamic and cerebellar uptake. Stage III: absent uptake in the putamina and severe decreased cortical uptake consistent with brain atrophy and further decrease uptake in the cerebellum. The presence of both structural and functional changes in the brain, demonstrated by the combined use of MRI and 18FDG PET scan, with good clinical correlation, make the two techniques complementary in the imaging evaluation of 3-methylglutaconic aciduria.
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3/165. Neurologic complications in immune-mediated heparin-induced thrombocytopenia.

    OBJECTIVE: To evaluate neurologic complications in patients with immune-mediated heparin-induced thrombocytopenia (HIT) with respect to incidence, clinical characteristics, outcome, and therapy. methods: One hundred and twenty consecutive patients with immune-mediated HIT were recruited over a period of 11 years and studied retrospectively for the occurrence of neurologic complications. diagnosis of HIT was based on established clinical criteria and confirmed by detection of heparin-induced antibodies using functional and immunologic tests. RESULTS: Eleven of the 120 patients (9.2%) presented with neurologic complications; 7 suffered from ischemic cerebrovascular events, 3 from cerebral venous thrombosis, and 1 had a transient confusional state during high-dose heparin administration. Primary intracerebral hemorrhage was not observed. The relative mortality was much higher (Chi-square test, p < 0.01) in HIT patients with neurologic complications (55%) as compared to patients without neurologic complications (11%). The mean platelet count nadir in neurologic patients was 38 /- 25 x 10(9)/l on average, and was lower in patients with fatal outcome compared to those who survived (21 /- 13 x 10(9)/l versus 58 /- 21 x 10(9)/l; p < 0.05, Wilcoxon test). In three patients neurologic complications preceded thrombocytopenia. There was a high coincidence of HIT-associated neurologic complications with other HIT-associated arterial or venous thrombotic manifestations. CONCLUSION: Neurologic complications in HIT are relatively rare, but associated with a high comorbidity and mortality. HIT-associated neurologic complications include cerebrovascular ischemia and cerebral venous thrombosis. They may occur at a normal platelet count.
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4/165. A case of Perlman syndrome: fetal gigantism, renal dysplasia, and severe neurological deficits.

    We report on a neonate presenting with polyhydramnios; macrosomia; macrocephaly; visceromegaly including bilateral nephromegaly, hepatomegaly, cardiomegaly; thymus hyperplasia; cryptorchidism; generalized muscle hypotonia; and a distinctive facial appearance. The clinical course was marked by severe neurodevelopmental deficits combined with progressive respiratory decompensation leading to death at the age 6 months. magnetic resonance imaging (MRI) disclosed a generalized cerebral atrophy with a marked deficit of the white matter. Renal ultrasound and MRI showed markedly enlarged kidneys with multiple small cystic lesions, a pattern indistinguishable from polycystic kidney disease. The postmortem kidney biopsy revealed dysplastic changes, microcysts, and a focal nephrogenic rest, characteristic features of the Perlman syndrome. In children with fetal gigantism, renal abnormalities, and neurological deficits, Perlman syndrome should be considered and may be confirmed by kidney biopsy.
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5/165. Neurological events associated with the infusion of cryopreserved bone marrow and/or peripheral blood progenitor cells.

    Reports of neurological toxicity of cryopreserved stem cell infusion are infrequent. Three of 179 consecutive patients experienced significant neurological events in this context. Transient global amnesia developed following infusion in one patient and in the other two, cerebral infarction occurred. Profound hypotension, bradyarrhythmias or hypoxia were not associated with any of these episodes. These events may have been related to infused DMSO, which in the non-transplant setting has been associated with neurological toxicity and local infusion of which has resulted in acute vasospasm in animal models. These cases suggest that infusion of cryopreserved stem cells may result in cerebrovascular ischaemia. bone marrow transplantation (2000) 25, 1285-1287.
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6/165. Neurological accidents caused by repetitive breath-hold dives: two case reports.

    We report two Japanese male professional breath-hold divers (33 and 39 years of age) who experienced neurological disorders during repetitive dives to over 20 m of seawater. One patient had right homonymous hemianopsia, and the other presented with right hemiparesis with facial involvement and sensory deficit. In addition, they each had a history of neurological problems following such dives. Magnetic resonance images of their brains disclosed multiple T2-weighted hyperintensities corresponding to their neurological symptoms. Their brain lesions suggest a multiple cerebral infarction caused by occlusion of the cerebral arteries. We conclude that the repetitive deep breath-hold dives induced the brain involvement.
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7/165. Bullous pemphigoid in a leg affected with hemiparesia: a possible relation of neurological diseases with bullous pemphigoid?

    We report a typical case of bullous pemphigoid (BP) associated with a neurological disorder and study a possible link between neurological disorders and BP. An 84-year-old hemiplegic woman presented with unilateral BP on the hemiparetic side. BP was confirmed by histological and immunofluorescence data. The medical records of the previous 46 consecutive patients with BP were retrospectively analyzed (average age: 79; median age: 85). Thirty of the 46 patients with BP had neurological disorders. These disorders included dementia, epilepsy, multiple sclerosis, cerebral stroke, Parkinson's disease, gonadotropic adenoma, trembling, dyskinesia, lumbar spinal stenosis. In a control group of the 46 consecutive oldest patients (older than 71; average age: 82,5; median age: 80) with another skin disease referred during the previous two-year-period to our one-day-unit only, 13 patients had a neurological disorder. This study demonstrates that there is a high prevalence of neurological disorders in patients with BP (p = 0.0004). A prospective case control study with neurological examination and psychometrical evaluation is warranted to confirm these data. We speculate that neuroautoimmunity associated with the aging process or neurological disorders may be involved in pemphigoid development via an autoimmune response against dystonin which shares homology with bullous pemphigoid antigen 1. Bullous pemphigoid could be considered to be a marker of neurological disorder.
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keywords = cerebral
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8/165. Familial dysautonomia: a diagnostic dilemma. chronic lung disease with signs of an autoimmune disease.

    We present an 11-year-old girl with sensory and autonomic neurological dysfunction, and respiratory insufficiency caused by recurrent aspiration. The diagnosis of familial dysautonomia (FD) was confirmed by a missing axonal flare to histamine, miosis in response to conjunctival methacholine and homozygous polymorphic linked markers DS58(18) and DS159(7) on chromosome 9. Ashkenazi Jewish descent could not be ascertained by history. A variety of positive tests for autoantibodies were initially interpreted as evidence for systemic lupus erythematosus vs. overlap syndrome with pulmonary, cerebral, skin, and ocular involvement. The diagnosis of FD was delayed because of the rarity of this disorder in germany (second case reported). We discuss possible explanations for the misleading immunological findings, including interference by antibodies binding to milk proteins used as blocking reagents in enzyme-linked immunoassays and circulating immune-complexes due to chronic aspiration pneumonitis.
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keywords = cerebral
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9/165. Delayed, transient neurological deterioration after mild head injury--case report.

    A 16-year-old boy presented with delayed, transient neurological deterioration 18 days after mild head injury. Left hemiparesis and left homonymous hemianopsia appeared after right frontal contusional and mild subdural hematomas subsided. neuroimaging examinations including cerebral angiography, magnetic resonance imaging, and single photon emission computed tomography showed vasodilation and hyperemia in the right cerebral hemisphere. The present case is not typical of acute "juvenile head trauma syndrome," but may represent a possible pathophysiology of the delayed type of transient neurological deterioration after mild head injury.
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keywords = cerebral
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10/165. Giant frontal mucocele extending into the anterior cranial fossa.

    The authors report a patient with a huge frontal mucocele presenting with transient left hemiparesis. magnetic resonance imaging and computed tomography showed the huge frontal mucocele arising from the frontal sinus extending to the anterior cranial fo ssa. Right middle cerebral artery stenosis was seen on the carotid angiography. The mucocele was treated with Killian's surgery. On the postoperative seventh day, the patient showed massive cerebrospinal fluid leakage that ceased with conservative management. The patient was discharged from the hospital without any neurological deficit. We discuss the clinical symptoms and postoperative course of the huge frontal mucocele with intracranial extension.
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