Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pseudotumor was diagnosed in six patients aged three to 38 years during an eight year period. The diagnosis was based on headache, choked disks and normal cerebrospinal fluid and CT scans. Other symptoms were visual impairment, VIth nerve palsy and ataxia. Sagital sinus trombosis was excluded by angiography or magnetic resonance imaging. Pharmacological treatment with digitoxin, acetazolamide, furosemide or corticisteroids reversed clinical symptoms and signs in two patients. In the other four, shunt surgery with cerebrospinal fluid diversion to the right atrium or the peritoneal cavity rapidly reversed clinical symptoms and signs.
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PMID:[Pseudotumor cerebri]. 200 May 87

Pseudotumour was diagnosed in six patients aged 3-38 years during an 8 years period. The diagnosis was based on headache, papilloedema, normal CT scan and cerebrospinal fluid (CSF) composition. Additional clinical symptoms were nausea, VIth nerve palsy, ataxia, blurred vision and frank visual reduction over time. Sagittal sinus thrombosis was ruled out by angiography or magnetic resonance imaging. In five of the six patients lumbar steady state infusion tests were performed to evaluate intracranial hydrodynamics and CSF resorbtion. All patients demonstrated a markedly increased opening pressure (range 13 to 48 mm Hg). CSF outflow resistance ranged from upper normal to pathologically increased levels (8-19 mm Hg/ml/min). Combined epidural intracranial pressure/middle cerebral artery blood velocity monitoring in 3 patients revealed a great number of B waves and a labile cerebral vasomotor state. Pharmacological treatment was tried with digitoxin, acetazolamide, furosemide and/or corticosteroids. Two patients did well on long-term treatment with digitoxin and furosemide, respectively. In the other four patients the clinical development was unsatisfactory on medical treatment alone. They were subsequently operated with implantation of a lumboperitoneal, cisternoatrial or cisternoperitoneal shunt. Shunting rapidly reversed clinical signs and symptoms, except for a partial persistent visual loss in an 18 years old boy who had experienced symptoms for 3 years resistant to pharmacological treatment.
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PMID:Pseudotumour cerebri-neurosurgical considerations. 208 40

Primary amebic meningoencephalitis and granulomatous amebic encephalitis are well recognized clinicopathological entities caused by free-living amebas. Associated arteritis and "mycotic aneurysms" with infiltration of intracranial arteries by lymphocytes, amebic trophozoites and cysts have not been previously reported. A 26-month-old girl had a 3-week history of encephalitis, characterized, initially, by vomiting and low-grade fever. Subsequently, she developed ataxia, generalized weakness, lethargy, and esotropia. The first CSF showed 490 RBC/microliters, 705 WBC/microliters with 90% mononuclears. Her pupils reacted briskly to light. Moderate nuchal rigidity, nystagmus, fixed downward gaze, anisocoria, bilateral 6th nerve palsy, left arm monoparesis and left Babinski were present. CAT scan revealed slight symmetrical dilatation of anterior horns of lateral ventricles and an area of abnormal enhancement above the 3rd ventricle. She died 14 days after admission, 5 weeks after onset of symptoms. The brain showed focal necrotizing encephalopathy, involving thalami, cerebellum, brain stem, and cervical and upper thoracic spinal cord. Numerous free-living amebic trophozoites and cysts were present within a chronic granulomatous encephalitis. There were trombosis of basilar, posterior cerebral, and vertebral arteries with profuse chronic panarteritis, fibrinoid necrosis, and mycotic aneurysms.
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PMID:Granulomatous encephalitis, intracranial arteritis, and mycotic aneurysm due to a free-living ameba. 689 86

We report the case of a patient with a pontomedullary epidermoid cyst extending into the prepontine cistern. The patient presented with a progressive VIth nerve palsy, ataxia, and headache. Magnetic resonance imaging demonstrated a well-defined pontomedullary tumor with a high fat content. We excised the lesion using a microsurgical approach through the floor of the fourth ventricle. Postoperative magnetic resonance imaging confirmed the removal of both intra- and extra-axial components. We discuss the anatomic configuration, radiological appearances, and management of this unusual pathological finding.
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PMID:Microsurgical excision of a pontomedullary epidermoid cyst with prepontine extension: case report. 883 13

A 14-year-old boy had a 1-month history of diplopia (due to a VI nerve palsy), motor ataxia and dizziness. Brain MRI showed a 1.5-cm mass posterior to the pons. Histopathological examination of a biopsy specimen showed the lesion to be of viral origin. After 3 months, the ataxia and dizziness had resolved and the MRI findings returned to normal. By 5 months the abducens paralysis had also resolved. Viral encephalitis should be considered in the differential diagnosis of posterior fossa tumours.
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PMID:Posterior fossa pseudotumour due to viral encephalitis in a child. 932 41