Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0007758 (cerebellar ataxia)
 3,609 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 38 year-old laborer experienced solvent intoxication during each of two spray paintings of a dump truck and other heavy equipment in an enclosed, unventilated garage. The paint base consisted primarily of toluene and methyl ethyl ketone. Nausea, headaches, dizziness, respiratory difficulty and other symptoms began after exposures. Over the next several days he developed impaired concentration, memory loss and cerebellar signs including an intention tremor, gait ataxia and dysarthria. MRI of the brain and EGG early in the work-up were normal, although later MRIs demonstrated fluid collection over the left parietal area. Examination by a toxicologist and neurologist revealed likely toxic encephalopathy with dementia and cerebellar ataxia. Three formal neuropsychological assessments over 2 1/2 years quantified cognitive, motor and behavioral changes. Despite similar findings in chronic exposure to these solvents, lasting sequelae following acute exposure have not been widely reported.
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PMID:Chronic neuropsychological and neurological impairment following acute exposure to a solvent mixture of toluene and methyl ethyl ketone (MEK). 174 49

Five children with subacute or acute onset of cerebellar ataxia and opsoclonus are described. Two had cerebrospinal fluid pleocytosis at the onset of ataxia and were initially thought to have acute parainfectious cerebellar ataxia of childhood. All were found to have tumors of neural crest origin (two neuroblastomas, three ganglioneuroblastomas). Tumors were small and only found by computed tomographic techniques. Urinary catecholamine metabolites were elevated in only two of the patients. Four of the five failed to improve neurologically with resection of the tumor. All four have had a steroid-sensitive chronic ataxic syndrome that worsens with acute nonspecific illnesses and has resulted in long-term deficits, particularly in speech and gross motor function. This is a metabolic encephalopathy associated with permanent residual neurologic deficits but without visible lesions on neuroimaging studies. We stress the frequency of cerebrospinal fluid pleocytosis in patients with tumor-associated opsoclonus and the clinical difficulty in separating tumor-associated cases from those due to other causes [corrected].
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PMID:Opsoclonus-ataxia due to childhood neural crest tumors: a chronic neurologic syndrome. 234 82

Since the initial report of Beyers & Moll (1948), numerous cases of seizures and encephalopathy after pertussis immunization or DPT immunization have been reported. However, acute cerebellar ataxia and/or facial palsy after DPT immunization is unusual, although there have been several reports from Japan. We report a 1-year-11-month-old girl with acute cerebellar ataxia and facial palsy after DPT immunization. On admission, she was alert. She was active and had a 6-day history of an ataxic gait and asymmetric facial movement which had begun 5 hours after DPT immunization. Neurological examination revealed an ataxic gait, horizontal nystagmus and right facial palsy. A CT scan showed low density on the right side of the pons with marked contrast enhancement. A MRI scan indicated the involvement of not only the right side of the pons, but also of the bilateral cerebellar peduncles. The child did well subsequently and was neurologically normal 20 days after the initial symptoms. To our knowledge, the present case is probably the first reported one of acute cerebellar ataxia after DPT immunization with CT and/or MRI correlation.
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PMID:[Acute cerebellar ataxia and facial palsy after DPT immunization]. 280 99

Cerebellar ataxia and opsoclonus were the initial manifestations of an associated neuroblastoma in a 20-month-old girl. Two months after the initial symptomatology, a physical examination revealed an abnormal mass palpable left to the midline. Urinary catecholamines were within normal limits. The child's neurological findings improved immediately after surgery, and steroid treatment and the follow-up on her after 2 years revealed normal general and neurological development. The syndrome of myoclonic encephalopathy including cerebellar ataxia, myoclonus and opsoclonus, and its relationship to neuroblastoma is reviewed. Failure to recognize this association can result in delays in both diagnosis and treatment and could be fatal.
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PMID:Cerebellar ataxia and opsoclonus as the initial manifestations of myoclonic encephalopathy associated with neuroblastoma. 369 May 66

Increasingly vigorous chemotherapy of cancer including primary and metastatic central nervous system disease has resulted in prolonged good-quality survival. However, there has been an associated increase in neurotoxicity from both radiation therapy and chemotherapy. All classes of chemotherapeutic agents contain drugs that are potentially neurotoxic, often only at high doses. Mechlorethamine, the first nitrogen mustard, is not neurotoxic at conventional dosage, but at high doses, it may produce both an acute and a delayed encephalopathy. Methotrexate administered intrathecally often induces reversible aseptic meningitis, but chronic administration, either intrathecally or high-dose intravenously, may produce fatal leukoencephalopathy. 5-Fluorouracil at high dosage may cause cerebellar ataxia, but may also do so at low dosage when combined with thymidine infusions. Cytosine arabinoside at high dosage may also produce cerebellar ataxia. Vincristine produces a peripheral neuropathy, and less commonly causes both autonomic and cranial neuropathy. The enzyme L-asparaginase can produce a dose-related reversible encephalopathy. BCNU, now the mainstay of glioma chemotherapy, may combine with radiation to produce long-term cerebral atrophy. Both intracarotid and high-dose intravenous BCNU administration may cause encephalopathy. Several other chemotherapeutic agents have also been reported to cause neurotoxicity under certain circumstances.
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PMID:Neurological complications of antineoplastic therapy. 638 4

Three children with myoclonic encephalopathy (Kinsbourne's disease) are described, in which one of them was shown to have ganglioneuroblastoma. Symptoms were opsoclonus, polymyoclonia of the striated muscles and cerebellar ataxia. Treatment consisted in corticosteroids and adrenocorticotropic hormone respectively in all patients, the patient with ganglioneuroblastoma also had a resection of the tumor. All patients responded to therapy, however recurrence of myoclonia and of the opsoclonus were seen after discontinuation or reduction of the corticosteroid dose, as well as following the course of intercurrent viral infections. Neurologic symptoms eventually disappeared after 3 1/2-5 1/2 years, however in two children behavioural abnormalities and disorders of speech and cognitive development remained.
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PMID:[Myoclonic encephalopathy (Kinsbourne syndrome)]. 647 68

Nineteen children aged 8-14 years were admitted over a six-year period with an acute encephalopathy due to toluene intoxication. Seven had a history of euphoria and hallucinations. The remainder presented with coma (4), ataxia (3), convulsions (3), and behaviour disturbance with diplopia (2), A history of glue sniffing was elicited in 14, but in the remainder toluene assay confirmed the diagnosis. Thirteen children recovered completely; five still had psychological impairment and personality change on discharge from hospital but were lost to follow-up, and one has a persistent cerebellar ataxia one year after the acute episode, despite absence of further exposure. Toluene inhalation is an important cause of encephalopathy in children and may lead to permanent neurological damage. Diagnosis is most important if further damage due to continued abuse is to be prevented, and toluene assay is a valuable aid to diagnosis.
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PMID:Solvent encephalopathy. 679 Jan 21

Disulfiram is known to produce toxic encephalopathy and peripheral neuropathy. The case of a 37-year-old alcoholic who attempted to commit suicide by taking 22.5 g disulfiram is described. During the first 6 days after the intoxication he was stuporous and had cerebellar ataxia and dysarthric speech. Then he became comatose, and as he recovered from coma, he showed peripheral neuropathy including diplegia faciei and severe tetraparesis. Denervation potentials were detected in both facial muscles and distal muscles of the upper and lower limbs, while conduction velocity was normal. Axonal degeneration was verified by sural nerve biopsy. In addition neurofilamentous axonopathy was documented. The recovery from his paresis lasted 2 years.
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PMID:[Polyneuropathy caused by disulfiram poisoning]. 686 Jan 22

1 Twenty patients, aged eight to 14 years, were admitted to hospital over a six year period with an acute encephalopathy following toluene abuse. 2 In all cases toxicity was confined to the central nervous system. Electroencephalography performed in ten patients was abnormal in three. 3 Fourteen patients made a complete recovery, five had evidence of personality impairment but were lost to follow-up, one had a persistent cerebellar ataxia one year after admission, despite absence of exposure to toluene. 4 Solvent abuse may be impossible to establish on history, as in six of these patients. Blood assay of toluene is vital in such cases, as is an awareness that toluene abuse may lead to acute encephalopathy.
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PMID:Neurological sequelae of toluene abuse. 717 9

The epidemiological and clinical presentation in 37 patients (males/females 1.5:1, median age 37 years, range 12-85 yrs) with a seasonal ataxic syndrome of unknown etiology peculiar to parts of Western Nigeria is reported. All patients belonged to the low socio-economic strata, and all subsisted on a monotonous diet of high-carbohydrate meals with minimal protein supplementation. All patients consumed a stew containing the roasted larvae of Anaphe venata, a seasonal protein supplement peculiar to the area of endemicity in their last meals prior to the onset of disease. The clinical features seen in the patients studied were mainly those of acute cerebellar ataxia in all, with ophthalmoplegias and encephalopathy in the more severe cases. These features are remarkably similar to those of acute thiamine deficiency and the results in concert provide clinical and epidemiological support for an etiological hypothesis of acute thiamine deficiency in this seasonal ataxic syndrome.
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PMID:Epidemiology and clinical presentation of a seasonal ataxia in western Nigeria. 793 28


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