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

Jellinger, K. A.; Tarnowska-Dziduszko, E

doi:10.1007/bf00316407

Cited by 13 publications

(10 citation statements)

References 24 publications

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“…Several additional cases of such multisystematic degeneration of the CNS involving the thalamus have been reported [10,11,14,23], and it is well documented that the central nucleus and, less frequently, the anterior and medial nucleus of the thalamus are involved in OPCA [4,6]. The cases of Moriuchi et al [13] could be similar to those cases.…”

Section: Nosological Questionssupporting

confidence: 65%

Thalamus Degeneration in Japan

Oda¹

1976

Stereotact Funct Neurosurg

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12 Japanese cases with marked thalamus degeneration are reviewed. They are divided into 3 groups. The 4 cases of the first group showed a relatively short clinical course. Neuropathologically, the lesions of this group are selectively severe in the thalamus, and olivocerebellar atrophy is also observed. Because of the significance of the thalamic changes, this group is called ‘primary thalamus degeneration’. The cases of combined system degeneration and presenile dementia, such as Pick''s disease, are described in the 2nd and 3rd groups. In the thalamus, the developmentally younger, and medially and dorsolaterally located nuclei are most severely affected in all the cases. The substantia nigra, globus pallidus and cerebellar afferent systems, and occasionally the dentate nucleus, subthalamic nucleus and reticular formation, are also involved. In the cases of presenile dementia, in which the frontal and posterior parietal lobes are damaged, degenerated areas in the cerebrum and thalamus have a close relationship with each other genetically as well as in fiber connections. The clinicopathological relationship is discussed on the basis of those neurophathological findings.

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Section: Nosological Questionssupporting

confidence: 65%

Thalamus Degeneration in Japan

Oda¹

1976

Stereotact Funct Neurosurg

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“…Forty‐two postmortem‐confirmed cases of MSA from the files of the Institute of Clinical Neurobiology, Vienna, and the Department of Neurology, University Hospital, Innsbruck, Austria, were studied. The data for most of the SND cases and some of the OPCA cases were published previously 12, 13. Neuropathological examination was carried out by naked eye inspection and on formalin‐fixed sections from multiple blocks of cerebral hemispheres, brainstem, cerebellum, and, only in some cases, of the spinal cord, using routine stains, modified Bielschowsky silver technique, Perls' or Berlin blue stain for non‐heme iron, immunohistochemistry for glial fibrillary acidic protein (GFAP), HLA‐DR for microglia, tau protein (antibody AT‐8), ubiquitin, and α‐synuclein (for methods see Wenning and colleagues12).…”

Section: Methodsmentioning

confidence: 99%

“…The lateral reticular nucleus and accessory nuclei of the inferior olive sustain the most damage, followed by the pontobulbar body and arcuate nucleus 10. In the cerebellum, the Purkinje cells are affected more in the vermis than in the hemispheres,3, 11, 12 associated with atrophy of the inferior olivary nucleus, cerebellopontine fibers, and atrophy of the basis pontis 11, 13. Neuropathological diagnostic criteria for MSA have been proposed2, 3 and the degree of neuronal loss seems to be related to the duration of illness and to the type of MSA,3, 14 whereas the distribution and density of GCIs has been correlated with the degree of involvement of both major neuronal systems in SND and OPCA 15.…”

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confidence: 99%

Grading of neuropathology in multiple system atrophy: Proposal for a novel scale

Jellinger¹,

2005

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Multiple system atrophy (MSA), a sporadic progressive synucleinopathy of advanced age, is separated into two clinic opathological subtypes: MSA-P (striatonigral degeneration [SND]) with predominant parkinsonian features and MSA-C (olivopontocerebellar atrophy [OPCA]) with predominant cerebellar ataxia. We propose a novel morphological grading system for both subtypes to compare lesion intensities and their possible clinical validity. Forty-two autopsy cases of MSA were separated into four grades (SND 0-III and OPCA 0-III) based on semiquantitative assessment of neuronal loss, astrogliosis, and presence of alpha-synuclein-positive glial cytoplasmic inclusions (GCI) in striatum, globus pallidus, substantia nigra, pontine basis, cerebellum, and inferior olives. Whereas a recent grading system restricted to SND reflected disease progression and dopa-responsiveness, there was considerable variation in the morphological combination between SND and OPCA, with only around half the cases with OPCA II (moderate) and III (severe) showing comparable grades of both types, whereas OPCA 0 and I (no or little degeneration) was combined with all grades of SND. Twenty-two cases showing OPCA 0 + SND II (n = 3), OPCA I + SND I-II (n = 11), and OPCA I + SND III (n = 8) were classified as pure or predominant SND, consistent with MSA-P. Twenty cases showing OPCA II + SND II/III (n = 7) and OPCA III + SND III (n = 13) were classified as predominant OPCA, consistent with MSA-C. In MSA-P, the mean age of onset was higher than it was in MSA-C (55.1 vs. 50.5 years), but the mean duration of illness was shorter in MSA-P (5.3 vs. 6.7 years). Presenting symptoms in MSA-P were mainly parkinsonism, whereas in MSA-C they were mainly gait disorders (14 vs. 1; P < 0.001). Among clinical key symptoms, parkinsonism was more frequent than were cerebellar signs in MSA-P; in MSA-C it was the reverse (P < 0.01), whereas other symptoms (autonomic/urinary failure) showed no differences. Parkinsonism was infrequent in MSA-C even when OPCA was associated with SND, suggesting a masking effect by cerebellar system involvement. High terminal Hoehn and Yahr stages were more frequent in MSA-P (P < 0.01), some with good-to-moderate initial levodopa (L-dopa) response. Although the proposed morphological grading of both MSA-P and -C correlates well with initial symptoms and clinical key features of both types, further prospective studies are required to validate the clinical utility of the proposed MSA grading scales for future intervention studies.

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“…After dopa administration in combination with a decarboxylase inhibitor, a distinct preference in fluorescence was found in the lateral caudal part of the substantia nigra and the dorsolateral part of the striatum [Fuxe et al, 1974], IOH of the multisystem atrophy subtype appears to represent a more extensive disease process than usually seen in SND. This is probably also true for IOH when associated with olivo-ponto-cerebellar degenera tion [Jellinger and T arnowska-D ziduszko, 1971], T akei and Mirra [1973] found normal spinal cord gray matter in 4 of 5 cases of SND, but noted intermediolateral column cell loss in their one case with a his tory of orthostatic hypotension. One of the cases in Adams et al's [1964] original description of SND had hypotension, but the spinal cord was not examined [personal commun.].…”

Section: Discussionmentioning

confidence: 99%

Idiopathic Orthostatic Hypotension

Schober

Langston

Forno³

1975

Eur Neurol

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Two cases of idiopathic orthostatic hypotension are reported, one associated with striato-nigral degeneration (case 1) and the other with Lewy body parkinsonism (case 2). In case 1, cerebrospinal fluid monoamine studies were carried out before and during treatment with 5-HTP and L-dopa. Low values of 5-HIAA appeared to be associated with degeneration of the spinal cord intermedi-olateral column. L-dopa had a beneficial effect on both parkinsonism and hypotension over a period of 6 months. New pathologic observations included changes in the sacral cord and a topographically related involvement of the substantia nigra and striatum, consistent with recent experimental neuroanatomical data.

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

Cited by 13 publications

References 24 publications

Thalamus Degeneration in Japan

Thalamus Degeneration in Japan

Grading of neuropathology in multiple system atrophy: Proposal for a novel scale

Idiopathic Orthostatic Hypotension

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