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)

Haemorrhages into the subarachnoid space lead to scarlike changes in the leptomeninges in some patients. If these changes reach a certain severity, circulation of the C.S.F.. is compromised and absorption reduced. This eventually results in ventricular dilatation which, in severe cases, is clinically manifested by dementia, motor ataxia and incontinence. The early stages of the disturbance in C.S.F. circulation were examined scintigraphically in 165 patients with spontaneous traumatic or operative subarachnoid bleeding. In the presence of abnormal findings which suggested the need for C.S.F. shunting, air encephalography was carried out in addition. Special attention was paid to the demonstration of the extent and localisation of occlusions of the basal cisterns and of the cortical subarachnoid spaces. Typical scintigraphic and pneumoencephalographic findings are demonstrated and their diagnostic value compared.
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PMID:[Disturbances in C.S.F. circulation after subarachnoid bleeds--a comparison of pneumoencephalographic and scintigraphic findings (author's transl)]. 15 65

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

An epileptic patient on chronic anticonvulsant drug therapy is described, in whom anaemia and neurological abnormalities including progressive dementia, bilateral pyramidal tract signs, incontinence and ataxia developed. Vitamin B12 serum levels and absorption were normal, but serum folic acid levels were low. Both the neurological disturbances and anaemia resolved following oral folic acid administration. This sequence of events in our patient suggests a cause and effect relationship between the folate deficiency and the coexistent, transient neurological syndrome.
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PMID:Reversible central nervous system dysfunction in folate deficiency. 114 59

A 40-year-old man was admitted after 8 months of speech disturbance and locomotive ataxia. He had no seizures, lightning pains, paresthesia, visual loss, bladder disturbance or rectal incontinence. He had never been neurologically or psychiatrically ill and had no history of syphilis. When the patient was admitted, his general physical examination including blood pressure and dermatologic examination was normal. His consciousness was alert. He was found to have a deterioration of mental status such as inability to concentrate, failing memory, amnesia and circumstantiality. His pupils were anisocoric and Achilles jerks were absent. No rigidity of the neck muscles, paralysis and sensory disturbance were recognized. Romberg's sign was absent. The right pupil was 7.0 mm and the left was 6.0 mm in room illumination. The pupils were nonreactive to bright light and both did not constricted to near stimuli. 0.125% pilocarpine eyedrops produced bilateral pupillary constriction. The results indicated bilateral tonic pupils. Laboratory data revealed white cell count of 12,600/mm3 and normal erythrocyte sedimentation rate of 8 mm/hr. Cerebrospinal fluid (CSF) examination revealed the following: opening pressure, 140 mm of water; cell counts, 76/mm2 (mononuclear cells); total protein, 116 mg/dl; glucose, 57 mg/dl. A serum venereal disease research laboratories (VDRL) test was positive in a 1:32 titer confirmed by positive treponema pallidum hemagglutination (TPHA) test in a 1:40,960 titer and positive fluorescent treponemal antibody-absorption (FTA-ABS) test. Serum TPHA-IgM was positive in a 1:320 titer but TPHA-IgG was negative. CSF examination revealed positive TPHA test (titer of 1:2,560) and positive FTA-ABS test.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of early syphilis presenting general paresis-like symptoms and bilateral tonic pupils]. 130 Feb 74

We studied the clinical records of 18 patients (11 female and 7 male), from 42-83 years old (average 66.1) who were operated for Normal Pressure Hydrocephalus. The etiology was idiopathic in 9 (50%), post-subarachnoid hemorrhage in 6 patients (33.3%), post-cerebral trauma in 2 (11.1%) and post-meningitis in 1. The patients were divided in 4 categories according to their symptoms, as follows: Group A: 9 patients (50%) with dementia, ataxia and incontinence. Group B: 1 patient (5.6%) with dementia-ataxia. Group C: 4 patients (22.2%) with ataxia and incontinence. Group D: 4 patients (22.2%) with ataxia only. 6 patients had a ventriculo peritoneal shunt, and 12 had a ventriculo-atrial shunt placed. All received a Hakim valve of low, medium or high pressure, according to the pre-op ventricular pressure. According to Stein and Langfitt scale for recovery 12 patients (66%) improved and 6 (33.3%) did not change. None was worse. There were no complications.
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PMID:[Normal pressure hydrocephalus]. 176 37

A HIV-2 strain named HIV-2ben was isolated from peripheral blood lymphocytes of a patient who, since 1984, had developed neurological symptoms such as Raynaud's syndrome, followed by paresthesia of extremities and ataxia, and finally paraparesis of the legs and incontinence. This new isolate could be distinguished from HIV-2rod by antibody-binding epitopes, peptide maps of core p24 and p18 polypeptides and restriction endonuclease cleavage pattern.
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PMID:Isolation and characterization of HIV-2ben obtained from a patient with predominantly neurological defects. 211 42

The authors report on the case of a woman, aged 29, who was suffering for 15 years from an unipolar manic psychosis and for 5 years from ataxia, speech troubles and urine incontinence. The diagnosis of MS was supported by the presence of a marked increase of liquoral IgG as well as Link's and Tourtelotte's index. CT scan, EEG and VEPs were normal. The relationships between the two clinical conditions are briefly discussed on the view of data of the literature.
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PMID:[Description of a case of multiple sclerosis with psychotic disorders at the onset]. 357 64

Of 70 autopsied patients with the acquired immune deficiency syndrome (AIDS), 46 suffered progressive dementia that was frequently accompanied by motor and behavioral dysfunction. Impaired memory and concentration with psychomotor slowing represented the most common early presentation of this disorder, but in nearly one half of the patients either motor or behavioral changes predominated. Early motor deficits commonly included ataxia, leg weakness, tremor, and loss of fine-motor coordination, while behavioral disturbances were manifested most commonly as apathy or withdrawal, but occasionally as a frank organic psychosis. The course of the disease was steadily progressive in most patients, and at times was punctuated by an abrupt acceleration. However, in 20% of patients a more protracted indolent course was observed. In the most advanced stage of this disease, patients exhibited a stereotyped picture of severe dementia, mutism, incontinence, paraplegia, and in some cases, myoclonus. The high incidence and unique clinical presentation of this AIDS dementia complex is consistent with the emerging concept that this complication is due to direct brain infection by the retrovirus that causes AIDS.
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PMID:The AIDS dementia complex: I. Clinical features. 372 8

Three patients with hydrocephalus aged 16, 26, and 33 years demonstrated memory deficits before or after the insertion of a ventricular shunt but at no time demonstrated ataxia, incontinence, or dementia. Following shunt surgery, memory functions improved rapidly in the patient who had had a recent acute onset of hydrocephalus, but in the two patients with a long period of hydrocephalus before shunting, memory impairments did not recover significantly in one patient, and in the other, memory impairments had become more severe by 12 months postshunt. Other neuropsychological and psychological sequelae of adult hydrocephalus are discussed, and it is suggested that for patients with chronic hydrocephalus, an extended period of neurological and neuropsychological monitoring may be advantageous before a decision is made to perform shunt surgery.
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PMID:Neuropsychological and psychological sequelae of shunt surgery in young adults with hydrocephalus. 378 46

From a review of our experience in the past 4 years and of the literature generally, the following comments can be made about selecting patients with idiopathic NPH for a shunt procedure. (a) In the clinical presentation, either significant gait difficulty or the full triad of dementia, ataxia, and incontinence should be present. If dementia occurred first or is the major symptom, shunting may not improve the patient. (b) A CT scan with periventricular low density and/or small sulci along with expansion of the entire ventricular system (especially the temporal horns) is strongly associated with good shunt outcome. However, presence of significant atrophy does not prevent shunt success if the clinical picture is appropriate. Some surgeons now feel that the clinical presentation and CT scan findings are enough in themselves to indicate a shunt. If further testing is desired, the following may be useful: Lumbar puncture: A pressure over 100 mm is associated with better chances of improvement. Improvement after lumbar puncture is associated with high likelihood of shunt success, but lack of improvement after lumbar puncture is not useful as a predictor. Isotope or metrizamide cisternography: A typical NPH pattern suggests a good response; a mixed or normal pattern is irrelevant to shunt outcome. Overnight recording of CSF pressure: If pressure is above 180 mm at night, or if there are frequent B-waves, shunting is likely to be helpful. Lumboventricular perfusion: This technique appears to give the most accurate prediction but requires special expertise and probably human studies approval to be done, as it is still an experimental procedure. These features make it difficult to use as a routine test. With regards to results of shunting once accomplished, it is important to follow patients carefully to exclude a chronic subdural collection. If a shunted patient fails to improve with persistent large ventricles and a medium or high pressure valve was used, consideration should be given to shunt revision with insertion of a lower pressure valve.
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PMID:CSF shunts for dementia, incontinence, and gait disturbance. 390 56


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