Klinischer und anatomischer Beitrag zur Entstehung der extrapyramidalen Symptome bei cerebell�ren Erkrankungen

Töbel, Fr.

doi:10.1007/bf00367980

Archiv f�r Psychiatrie und Zeitschrift Neurologie

1952

DOI: 10.1007/bf00367980

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Klinischer und anatomischer Beitrag zur Entstehung der extrapyramidalen Symptome bei cerebell�ren Erkrankungen

Fr. Töbel

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1971

1997

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Cited by 6 publications

(1 citation statement)

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“…However, the analysis in the literature series was restricted to basic clinical features only, without consideration of underlying pathology. The correlation between pathology and clinical features has been evaluated only in the relatively small sample of 35 UKPDSBB cases. The present study therefore provides a more comprehensive survey of clinicopathological features reported in 203 pathologically proven MSA cases (including the 35…”

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confidence: 99%

Multiple system atrophy: A review of 203 pathologically proven cases

Tison²,

et al. 1997

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We report the clinicopathological features of 203 cases of pathologically proven multiple system atrophy (MSA) from 108 publications up to February 1995. The majority of patients showed symptoms in their early fifties, and men were more commonly affected than women (ratio of 1.3:1). Most patients suffered from some degree of autonomic failure (74%). Parkinsonism was the most common motor disorder (87%), followed by cerebellar ataxia (54%) and pyramidal signs (49%). The response to levodopa was poor in most patients, but there was a subgroup with a good response, who also often developed axial levodopa-induced dyskinesias. Other characteristic features included severe dysarthria, stridor, and, in a few patients, contractures and dystonia (antecollis). Mild or moderate intellectual impairment occurred in some cases, but severe dementing illness was most unusual. The main pathological change comprised cell loss and gliosis in the putamen, caudate nucleus, external pallidum, substantia nigra, locus ceruleus, inferior olives, pontine nuclei, cerebellar Purkinje cells, and intermediolateral cell columns of the spinal cord. However, other neuronal populations were also involved to varying degrees, such as the thalamus, vestibular nucleus, dorsal vagal nucleus, corticospinal tracts, and anterior horn cells. Characteristic glial and/or neuronal cytoplasmic inclusions were identified in all cases in which they were sought, irrespective of clinical presentation. Akinesia correlated with the degree of nigral and putaminal cell loss, whereas rigidity was related only to the later. Tremor was unrelated to cell loss at any site. Ataxia correlated with the degree of olivopontocerebellar atrophy. Pyramidal signs were associated with pyramidal tract pallor. Our analysis also confirmed an association of postural hypotension with intermediolateral cell column degeneration.

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confidence: 99%

Multiple system atrophy: A review of 203 pathologically proven cases

Tison²,

et al. 1997

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Late Onset Ataxia, Rigidity, and Peripheral Neuropathy

Ziegler¹

1972

Arch Neurol

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A family had a polymorphous neurological syndrome transmitted in autosomal dominant fashion.The clinical syndrome is one of late onset cerebellar ataxia, muscular rigidity, bradykinesia, dysarthria, fasciculations, muscle atrophy, and spasticity appearing in various combinations in affected individuals. Pathological studies on one case have revealed degeneration in spinocerebellar tracts, Purkinje cells and dentate nuclei of cerebellum, dorsal root ganglion cells, and cauda equina nerve roots. Peripheral neuropathy was present in several cases, as evidenced by demyelinating changes on sural nerve biopsy, and by characteristic changes in nerve conduction times and electromyography. Such changes on occasion preceded clinical signs of peripheral nerve disease. Several cases showed a therapeutic response to levodopa; only the symptom of rigidity was alleviated.

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Die ZNS-Ver�nderungen bei den olivo-ponto-cerebellaren Atrophien

Jellinger

Tarnowska-Dziduszko

1971

Z. Neurol.

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Klinischer und anatomischer Beitrag zur Entstehung der extrapyramidalen Symptome bei cerebell�ren Erkrankungen

Cited by 6 publications

References 13 publications

Multiple system atrophy: A review of 203 pathologically proven cases

Multiple system atrophy: A review of 203 pathologically proven cases

Late Onset Ataxia, Rigidity, and Peripheral Neuropathy

Die ZNS-Ver�nderungen bei den olivo-ponto-cerebellaren Atrophien

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