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

We reported 9 cases of primary Sjogren syndrome (SS) who were complicated with nervous system involvement. All were women. Age between 24 to 58 years old. Their clinical symptoms of the nervous system varied widely, 7 of them manifested with the involvement of central nervous system, the main features were in case 1 bouts of seizures, case 2 multi-level damage of the brain and spinal cord which was similar to multiple sclerosis, case 3 recurrent hemiplegia caused by cerebral thrombosis then fatal vascular hemorrhage, case 4 sudden hemiplegia, case 5 persistent psychological disturbance, case 6 cranial neuropathy of V and VII, case 9 multi-focal symptoms of ataxia, myelopathy and transient blindness. 5 of the 9, case 3, 6-9 all appeared with the symptoms of peripheral neuropathy, case 7 also complicated with carpal tunnel syndrome. 4 of the 9 cases also complicated with renal tubular acidosis and/or chronic active hepatitis and/or fibrosing alveolitis and/or thrombocytopenic purpura and/or myositis. No differences of the positivity of autoantibodies was observed between those with or without nervous system involvement.
 ...
PMID:[The manifestations of the nervous system in primary Sjogren syndrome]. 158 41

The three major components of the Balint's syndrome are described: 1) visual disorientation or simultanagnosia, i.e. the inability to perceive the visual field as a whole, 2) ocular apraxia, a deficit of visual scanning and 3) optic ataxia, an impairment of pointing and reaching under visual guidance. Our description of the syndrome is illustrated by three case histories. The complete syndrome is rare, and partial syndromes have been reported more frequently. Sudden and severe hypotension resulting in bilateral borderzone infarction in the occipito-parietal region is reported to be the most frequent cause of complete Balint's syndrome. Lack of awareness of the syndrome may lead to misdiagnosis such as blindness, psychosis, or dementia.
 ...
PMID:[Balint's syndrome--visual disorientation]. 159 20

A 75-year-old patient with metastatic renal cell carcinoma was treated with recombinant interferon alpha-C and thereafter developed a neurologic syndrome of dementia, ataxia, confusional state, loss of concentration ability and cortical blindness. CT scan findings were compatible with leukoencephalopathy, which is reported as being a toxic effect of interferon.
 ...
PMID:Interferon-related leukoencephalopathy in a patient with renal cell carcinoma. 174 62

A metastasizing glioma in a 4-year-old boxer bitch is described. Clinical symptoms included ataxia, blindness, and increased cervical pain sensation. The tumor metastasized to an extraordinary extent via the cerebrospinal fluid. Tumor masses surrounded the whole spinal cord including the cauda equina. Histological examination revealed a variable morphology of the glioma. Immunohistochemical investigations showed some tumor cells reacting with antibodies specific to GFAP and S-100 protein. In contrast, NSE, 200 kd NF, vimentin, and desmin could not be demonstrated within tumor cells. The results are discussed in detail.
 ...
PMID:[Metastasizing glioma in a Boxer]. 194 88

Out of 604 Gambian children admitted with falciparum malaria to one hospital between September and December, 1988, 308 had cerebral malaria and 203 were severely anaemic (haemoglobin less than 60 g/l). 14% of those with cerebral malaria died, as did 7.8% of those with severe anaemia. 32 (12%) of children surviving cerebral malaria had residual neurological deficit. 69 other children were admitted with clinical features strongly suggestive of cerebral malaria but with negative blood films; 16 of these died and 3 had residual neurological deficits. The commonest sequelae of cerebral malaria were hemiplegia (23 cases), cortical blindness (11), aphasia (9), and ataxia (6). Factors predisposing to sequelae included prolonged coma, protracted convulsions, severe anaemia, and a biphasic clinical course characterised by recovery of consciousness followed by recurrent convulsions and coma. At follow up 1-6 months later over half these children had made a full recovery, but a quarter were left with a major residual neurological deficit. Cerebral malaria in childhood may be an important cause of neurological handicap in the tropics.
 ...
PMID:Neurological sequelae of cerebral malaria in children. 197 27

Biotinidase deficiency is an inborn error of metabolism that is transmitted as an autosomal recessive disorder. Symptoms include hearing loss, ataxia, blindness, mental retardation, and seizures. The metabolic defect is an inability to recycle the vitamin biotin, which is an important cofactor in key enzymes. We therefore sought to develop an animal model for this disorder by inducing biotin deficiency. Rat pups were divided into control and experimental groups. Control rats were fed a normal diet whereas experimental animals were given a diet deficient in biotin. Animals from both groups underwent brain stem auditory evoked potential testing at various ages. Wave I thresholds at various ages were similar in both groups. Latencies for wave I, however, and interpeak latencies (I-IV) were prolonged in the biotin-deficient groups compared to controls. Scanning electron microscopy of the organ of Corti in biotin-deficient animals showed no significant hair cell loss. The biotin-deficient developing rat appears to acquire functional changes in the auditory brain stem. These effects may be caused by defective myelination, since biotin is important in fatty acid metabolism.
 ...
PMID:Rat as a potential model for hearing loss in biotinidase deficiency. 201 87

Adrenoleukodystrophy, an X-linked recessive disorder characterized by progressive demyelination of the central nervous system and adrenal insufficiency, usually manifests at 4-8 years of age. We report a 20-month-old male who presented with the sudden onset of status epilepticus and cortical blindness; initially, he had complete resolution of these findings, but experienced a relapse 3 months later. The initial computed tomographic scans depicted cerebral edema and possible "watershed infarcts:" however, over the next 2 weeks before discharge from the hospital, the cortical blindness and ataxia both resolved. During the next 2 months, he exhibited no symptoms: he had no seizures and his neurologic examinations were normal. Three months after the initial hospitalization, he developed what the mother believed was "a weakness on his right side." Magnetic resonance imaging confirmed severe white matter disease. Adrenoleukodystrophy was clinically suspected and an assay of plasma levels confirmed an elevation of C26 long-chain saturated fatty acid levels. After the patient's diagnosis of adrenoleukodystrophy was confirmed, long-chain fatty acid levels were obtained on his 5-year-old brother and his mother. This child had the earliest known onset of X-linked adrenoleukodystrophy.
 ...
PMID:Variable phenotypes in a family kindred with adrenoleukodystrophy. 202 94

Between 1986 and 1988 a door-to-door survey was conducted on a stable rural population of 60,820 in central Ethiopia. Trained lay health workers made a complete census and identified cases with symptoms and signs of neurological disorders, using specially designed questionnaires which, in a previous pilot study, were found to have a sensitivity of 91% and specificity of 85%. Neurological disorders in the rural population were epilepsy, postpoliomyelitis paralysis, mental retardation, peripheral neuropathy (mainly due to leprosy), and deaf-mutism with prevalence rates (cases/100,000 population) of 520, 240, 170, 150 and 130, respectively. The prevalence rates of the other less common neurological disorders were 62 for hemiparesis (15 of which were for cerebrovascular accidents), 20 for cerebral palsy, 16 for optic atrophy, 12 for perceptive deafness, 10 for tropical spastic paraparesis, 7 for Parkinson's disease and 5 for motor neuron disease, ataxia and chorea/athetosis. Among related non-neurological conditions, blindness, locomotor disability and deafness were predominant. The significance and role of such a neuroepidemiological study in laying the strategies for the prevention of neurological disorders and rehabilitation of patients are discussed in the context of a developing country.
 ...
PMID:Community-based study of neurological disorders in rural central Ethiopia. 208 51

Review of all reports involving anthelmintics in dogs and cats to the IAPIC between January 1, 1986 and August 10, 1988, revealed that ivermectin (extra-label use) and piperazine accounted for over 50% of the calls assessed as toxicoses and suspected toxicoses. Both ivermectin and piperazine are gamma-aminobutyric acid (GABA) agonists and their major effects appear to be on the central nervous system. Ivermectin toxicoses at estimated doses of greater than or equal to 100-less than 500 micrograms/kg were reported more than once only in the collies (n = 25) and Australian shepherds (n = 10); these two breeds accounted for 46% (69 of 150) of the toxicoses and suspected toxicoses calls in dogs. Ataxia, behavioral disturbances, tremors, mydriasis, weakness/recumbency, apparent blindness, hypersalivation/drooling (dogs only), and coma were the most commonly reported clinical signs in dogs and cats with suspected ivermectin toxicoses. Shock, dyspnea, vomiting, and ataxia were the most common clinical signs attributed to the microfilaricidal activity of ivermectin. Piperazine was the anthelmintic with the greatest number of reports of toxicoses and suspected toxicoses in cats. Piperazine neurotoxicity in cats and dogs usually was manifested by muscle tremors, ataxia, and/or behavioral disturbances within 24 hours after estimated daily dose(s) between 20 and 110 mg/kg.
 ...
PMID:Ivermectin and piperazine toxicoses in dogs and cats. 218 Jan 89

Chronic cysticercal meningitis was diagnosed in 6 of 260 cases of neurocysticercosis. Clinical features usually associated with meningitis, such as fever, cranial nerve palsies and nuchal rigidity, were absent and the diagnosis was reached because of persistently abnormal cerebrospinal fluid (CSF) associated with active neurocysticercosis on computed tomography. Other causes of chronic meningitis were excluded. Hydrocephalus, either obstructive or communicating, was present in all 6 patients. The results of medical treatment with praziquantel were poor and there was no improvement either clinically or in the CSF. Severe clinical sequelae, such as dementia, blindness and gait ataxia, were common despite protracted medical treatment and ventriculoperitoneal shunting.
 ...
PMID:Cysticercal meningitis--a pernicious form of neurocysticercosis which responds poorly to praziquantel. 234 53


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>