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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Observation of neurosarcoidosis in a thirty-year-old black female characterised predominantly by bilateral facial nerve paralysis gave rise to a review of literature since 1978 and also to a comparison with an early study in 1963. As described 1963 the clinical picture is characterized by increased protein content of the CSF (33.8%), facial nerve paresis (25.5%), pleocytosis (23%), diabetes insipidus (21%), hemiparesis (17.2%), organic psychosis (16.9%), papilloedema (15.5%), ataxia (13%), convulsive seizures (12.5%), optic atrophy (12.5%), loss of hearing (12.2%), nystagmus (8.6%) and numerous other symptoms more rarely found. This corresponds to the symptoms of chronic basal meningitis with an infiltration in the neighbouring structures of brain and less frequently the spinal cord. In only 58.7% of the cases (presumably at the onset of sarcoidosis) was the bronchial tract (or the lungs) affected, in 11.5% the skin or the eyes. Although the clinical picture is clear enough the etiology has yet to be determined. Evidence of a pathogen or a pathogenic agent (analogous to berylliosis) has never been established to date. On the other hand there are some indications of a disturbance in the immune system, perhaps of a particular genetic foundation since sarcoidosis strikes black patients with conspicuous frequency. There exist more cases in one family. Exceeding expected random distribution, many patients have the HLA-Factor B 8 (on the chromosome 6) and DR 3. The Kveim-Test was in 71 cases positive, in 12 cases negative. The possibilities of carrying out studies of CSF - analogous to the studies of bronchial lavage - in the most cases of neurosarcoidosis have not been exhausted as to determine the activity of the T-lymphocytes, the interleucines, the angiotensin-converting enzyme while the Gallium 67 scintigraphy and other methods to determine the non-specific activity of the inflammation. The efficiency of the treatment with corticosteroids (Prednison or Triamcinolonacetonid) depends of the phase of the inflammatory process. 12% of the registered cases died.
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PMID:[Neurosarcoidosis. Comparative analysis of the clinical profile based on 537 cases from the world literature up to 1963 and from 1976-1988]. 240 26

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

A patient is described who 32 years after thyroidectomy developed a chronic progressive syndrome due to hypoparathyroidism, with multiple extrapyramidal signs (faciobuccolingual dyskinesias, choreiform and athetotic movements of upper extremities, tremor of hands, cogwheel phenomenon), cerebellar manifestations (dysarthria, dysgraphia, mild gait ataxia), and pyramidal signs as well as an organic psychosis and epilepsy. A CT scan showed calcification of the basal ganglia. Therapy for hypoparathyroidism produced partial regression of the disorder. To explain the physiopathologic mechanism of the syndrome, Selye et al. advanced an attractive hypothesis of calciphylaxis, which assumes that a sensitizer and a challenger together induce development of calcification. In the case reported calcification involved the periventricular region and was not limited to the basal ganglia. It is suggested that either a sensitizer or a challenger may enter the periventricular tissue via the ependyma.
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PMID:Calcification of basal ganglia, postoperative hypoparathyroidism and extrapyramidal, cerebellar, pyramidal motor manifestations. 618 Dec 25

We report on two young patients with an encephalitic syndrome and bilateral thalamic lesions following a presumably viral or mycoplasma respiratory tract infection with the main clinical symptoms of organic psychosis in the first and a prolonged amnestic syndrome and ataxia in the second case. Four months later the patients had recovered clinically and the thalamic lesions had resolved on magnetic resonance imaging in one case and almost completely in the other. We interpret the patients' illness as rare cases of a post-infectious acute thalamic encephalitis in adults. The cases and their relationship to possible post-infectious autoimmune inflammatory or toxic pathophysiological mechanisms are discussed and a review of the literature is provided.
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PMID:Adult post-infectious thalamic encephalitis: acute onset and benign course. 1474 75