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)

A total of 160 patients suspected of having acquired hydrocephalus were studied either by quantitative isotope ventriculography (QIV) or by lumbar isotope cisternography (LIC). Of these patients, 56 had hydrocephalus. Mental deterioration, gait disturbances, ataxia, spasticity, and incontinence were most frequently present in the hydrocephalic patients, but none of the signs or combinations thereof are pathognomonic of acquired hydrocephalus. These signs are independent of the intracranial pressure (ICP) and the type of hydrocephalus. Surgical shunt procedures were in most cases followed by the disappearance of mental deterioration, gait disturbances, ataxia, and spasticity.
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PMID:Acquired hydrocephalus. I. A clinical analysis of 160 patients studied for hydrocephalus. 31 40

Mannosidosis, an inherited lysosomal storage disease, was diagnosed in two purebred Angus calves in the United States. Calf 1 was 5 months old and calf 2 was 7 months old when they were necropsied. Both calves had a history of progressive incoordination and ataxia. Gross postmortem findings included moderate hydrocephalus. Histologic examination revealed intracytoplasmic vacuolation of neurons throughout spinal cord and brain as well as in exocrine pancreatic cells, reticuloendothelial cells of the liver, and medullary sinusoids of lymph nodes. Biochemical study of 61 blood samples from the first calf's herd revealed bimodal distribution of enzyme activity for mannosidase. Two populations could be distinguished in that herd, those with normal mannosidase activity and those heterozygous for the disease.
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PMID:Mannosidosis of Angus calves. 50 Apr 78

Seven patients with histologically confirmed cerebellar hemangioblastomas are reviewed in order to establish computed tomographic (CT), angiographic and clinical characteristics and correlations. Noncontrast CT scans demonstrated most cystic lesions but did not always depict nodules on the cyst wall and small solid tumors. Contrast-enhanced CT scans demonstrated the solid and cystic types of tumors but in some cases angiography better delineated them and the presence of nodules. The CT appearance reflected the pathologic characteristics, which included vascular channels, cyst formation and nodules. Solid tumors enhanced homogeneously and could not be definitively distinguished from other cerebellar neoplasms by CT alone. One atypical undifferentiated tumor appeared as a solid mass with a necrotic center. The CT appearance of associated retinal angiomatosis is also described. Hydrocephalus, gait or appendicular ataxia was the initial clinical presentation in these patients.
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PMID:Cerebellar hemangioblastoma: computed tomographic, angiographic and clinical correlation in seven cases. 57 Jan 2

Severe dementia and ataxia in an elderly woman were related to hydrocephalus resulting from Paget disease of the skull, with basilar impression. Ventriculoatrial shunting resulted in dramatic improvement. This procedure, rather than suboccipital decompression, is probably the operation of choice, and should be carried out at an early stage.
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PMID:Hydrocephalic dementia in Paget disease of the skull: treatment by ventriculoatrial shunt. 57 51

A 65-year-old female patient with Paget's disease displayed dementia, a bilateral Parkinsonian syndrome, gait ataxia, bilateral grasp reflex of the feet, urinary incontinence and a left hemicerebellar syndrome. She had a marked basilar impression; the radionuclide cisternogram was suggestive of a communicating hydrocephalus with ventricular dilatation. After a shunting procedure there was improvement in both neurological and mental function.
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PMID:Parkinsonism--dementia complex, hydrocephalus and Paget's disease. 85 13

A description of the clinical symptoms of the communicating hydrocephalus (normal-pressure hydrocephalus = NPH) is given: progressive psycho-organic alterations, ataxia, tetraspastic signs, urinary incontinence, without increased intracranial pressure. Diagnosis can be confirmed by use of pneumencephalography, isotope-cisternography and tomography of the brain by use of a water-solubile contrast fluid. Skull radiographs. EEG, testing of liquor and psychological examinations are of less diagnostic value. The illness is the result of a disturbance of liquor resorption and circulation. In this context there is to differentiate between cases of known (Hydrocephalus communicans obstructivus = HCO) and unknown etiology (Hydrocephalus communicans non obstructivus = HCNO). The group of cases with unknown etiology represents the central part of cases of normal-pressure hydrocephalus (NPH). The prognosis after a ventricular shunt-procedure is here worse than in the group of cases with known etiology. For differential diagnosis presenil dementia, cerebrovascular insufficiency, intracranial tumors and reactive and psychotic depressions are to be considered.
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PMID:[Communicating hydrocephalus. Clinical aspects, diagnosis, pathogenesis]. 86 58

Sixty-seven defects of the nervous system of lambs were examined during a 3-year investigation. Thirteen (19.4 percent) were single defects, and 54 (80.6 percent) were associated with defects of other body systems. Twice as many male lambs as females were affected. The various defects were: hydrocephalus 15, spina bifida 9, meningocoele 8, perosomus elumbis 8, cyclopia 6, cerebellar agenesis 3, aprosopia 3, cranium bifidum 3, anencephaly 2, cerebellar fusion 2, meningo-encephalocoele 2, congenital ataxia 2, micrencephaly 2, cerebellar hypoplasia 1, and acrania 1. A number of lambs had more than 1 defect of the nervous system. Congenital defects of the nervous system were found in 1.5 percent of the 4,417 lambs autopsied.
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PMID:Congenital defects of the nervous system of lambs. 110 13

A 48-year-old man, who took by mistake a sip of ointment containing dichloroethane, survived, and showed a course of two phases of toxic symptoms. After an initial narcosis and an interval with few pathological symptoms seizures, myoclonia and somnolence occurred. Irreversible final disturbances were lasting mental defects, cerebellar dysarthria, ataxia, and hydrocephalus. Concomitant diseases were acute liver dystrophy, nephropathy, and anemia. The clinical picture of dichoroethane posoning is outlines, the pathogenesis of this particular cerebral lesion described, and the therapy discussed.
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PMID:[Dichloroethane poisoning with myoclonic syndrome, seizures and irreversible cerebral defects (author's transl)]. 122 Jun 46

In a retrospective study of 624 elderly patients referred with falls and gait disorders, 45 patients were found to have ataxia. Cerebrovascular diseases were the most common underlying cause of ataxia (15 patients, 37%). Nine patients had hereditary/degenerative cerebellar ataxia. History suggesting alcohol as an underlying cause was established in two patients with cerebellar ataxia. Three patients had normal pressure hydrocephalus and their condition improved remarkably after surgery. No definite cause was found in five patients. Cranial computed tomography (CT) showed cerebral atrophy in 16 patients and in three patients there was evidence of atrophy of the cerebellar vermis. Four patients had femoral neck fractures and three patients had other fractures. In a 5-year follow-up five patients died with bronchopneumonia (11% mortality) and patients with dementia showed rapid deterioration. All patients were referred to the day hospital for rehabilitation. The best treatment outcome was achieved in patients who had a single cerebrovascular accident with no cognitive impairment and in those whose ataxia was secondary to medication. Fourteen patients (44%) moved to residential care while 27 (66%) continued to live in their homes with community support. We concluded that there is no evidence of increased mortality in the elderly patients with cerebellar ataxia. CT scan is mainly helpful in diagnosing specific diseases such as tumours or hydrocephalus. A significant proportion of elderly patients with ataxia may have reversible or treatable conditions and can pursue an independent life.
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PMID:Cerebellar ataxia in the elderly. 140 89

A pediatric neurologist analyzed the case histories of 30 4-16 year old children diagnosed with cerebellar ataxia in the pediatric neurology unit at the Royal Hospital for Sick Children in Edinburgh, Scotland to examine its clinical features, investigative findings, and etiology. Previous unfavorable events happened to 14 children (46.6%). Yet only 6 (42.8%) of these 14 children had unfavorable events of etiological significance. These previous unfavorable events occurred during the perinatal period (48%). These events in order of significance were asphyxia, prematurity, neonatal jaundice, and trauma. 66.6% of all children had an unsteady gait. The 2nd and 3rd most common signs of cerebellar ataxia were truncal ataxia (53.3%) and hypotonia (36.6%). The next most common symptom was considerable delay in reaching gross motor milestones (50%) such as not sitting until 2 years old. 23 (76.6%) of the children had dysfunctions in 1 of the cerebellar divisions. Clinical examination found dysfunctions most often in the paleocerebellum (86.6) followed by the neocerebellum (70%) and archicerebellum (56.6%). The paleocerebellum and the archicerebellum were the only divisions involved in 6 and 1 of the remaining children, respectively. The most common cause of ataxia was hydrocephalus (23.3%) followed by perinatal problems (20%). 70% of the patients also experienced other central nervous system conditions such as macrocephaly and mental retardation. 5 children had normal investigative findings, 3 of whom had cerebellar ataxia syndrome, 1 had congenital ataxic cerebral palsy, and 1 had familiar ataxia.
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PMID:Cerebellar ataxia in childhood: a review of clinical features, investigative findings and aetiology in 30 cases. 150 89


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