Accumulation of tubular structures in oligodendroglial and neuronal cells as the basic alteration in multiple system atrophy

Papp, M

doi:10.1016/0022-510x(92)90286-t

Cited by 184 publications

(102 citation statements)

References 25 publications

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“…However, variable immunolabeling of GCI was obtained with antitau antibodies [40-42, 44, 46, 47]. Furthermore, GCI are not stained with antibodies directed to actin, desmin, cytokeratin and, as in our own case, to phosphorylated and not phosphorylated NF proteins [48][49][50].…”

Section: Discussionmentioning

confidence: 96%

Primary Progressive Aphasia with Glial Cytoplasmic Inclusions

Molina

Probst²,

Villanueva

et al. 1998

Eur Neurol

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We report a 80-year-old woman who suffered from primary progressive aphasia for 3 years. Cranial CT and MRI studies showed moderate cerebral atrophy, more marked in the left frontal and temporal lobes, and SPECT brain scans revealed marked hypometabolism in the left frontal and temporal lobes. Neuropathologic examination of a temporal lobe biopsy demonstrated Gallyas-positive intracytoplasmic inclusions looking like fibrillary tangles and of Gallyas-positive cell processes, probably from glial cells. Glial intracytoplasmic inclusions were immunolabelled with antibodies to ubiquitin and with phosphorylion-dependent antitau antibodies, indicating the presence of hyperphosphorylated tau in the inclusions. There was only mild pathology of cortical neurons consisting in rare perikarya diffusely stained with antitau antibodies. There were no senile plaques, neurofibrillary tangles, ‘achromatic’ neurons, ballooned cells, Pick or Lewy bodies, nor microvacuoles or spongiform changes of the neuropil. The glial intracytoplasmic inclusions found in this case were similar to those found in multiple system atrophy, and differ from the cortical changes hitherto reported in primary progressive aphasia.

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Section: Discussionmentioning

confidence: 96%

Primary Progressive Aphasia with Glial Cytoplasmic Inclusions

Molina

Probst²,

Villanueva

et al. 1998

Eur Neurol

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“…Pathologic and positron emission studies have recently shown that nonfamilial OPCA and MSA are the same disease with variable manifestations. [10][11][12] In a series of 47 nonfamilial OPCA, only seven had the cerebellar syndrome whereas others had a combination of a rigid-akinetic syndrome. 9 In this series 9 and another recent series of 100 cases of MSA, 13 no patients had either PT or extensive multiple cranial nerve palsies.…”

Section: Discussionmentioning

confidence: 99%

Palatal tremor, progressive multiple cranial nerve palsies, and cerebellar ataxia: A case report and review of literature of palatal tremors in neurodegenerative disease

et al. 1999

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“…The GCIs were seen with a frequency of no more than one per 20X field and thus were less common than those found in the MSA patients of Papp and Lantos [2O]. Moreover, the patient evaluated neuropathologically had GCIs, which are currently thought to be an important marker for the diagnosis of MSA [ 13-17, [19][20][21][22].…”

Section: Gross Examinationmentioning

confidence: 98%

Spinocerebellar ataxia type 1 with multiple system degeneration and glial cytoplasmic inclusions

Gilman

Sima

Junck

et al. 1996

Annals of Neurology

137

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Spinocerebellar ataxia type 1 (SCA1) is a dominantly inherited progressive neurological disorder characterized by neuronal degeneration and reactive gliosis in the cerebellum, brainstem, spinocerebellar tracts, and dorsal columns. Multiple system atrophy is a sporadic progressive neurological disorder with degeneration and gliosis in the basal ganglia, cerebellum, brainstem, and spinal autonomic nuclei, and with argyrophilic glial cytoplasmic inclusions. We describe 4 members of a family with the SCA1 mutation and a dominantly inherited progressive ataxia in which autopsy examination of 1 member showed neuropathological changes typical of multiple system atrophy, including glial cytoplasmic inclusions. In this patient, magnetic resonance imaging revealed marked brainstem and cerebellar volume loss and mild supratentorial generalized volume loss. Positron emission tomography with [18F]fluorodeoxyglucose revealed widespread hypometabolism in a pattern found in sporadic multiple system atrophy and not in dominantly inherited olivopontocerebellar atrophy. Positron emission tomography with [11C]flumazenil revealed normal benzodiazepine receptor distribution volumes, similar to those seen in sporadic multiple system atrophy. Two other family members still living had similar changes in the imaging studies. The findings in this family suggest that the SCA1 gene mutation can result in a disorder similar to multiple system atrophy, both clinically and neuropathologically.

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Accumulation of tubular structures in oligodendroglial and neuronal cells as the basic alteration in multiple system atrophy

Cited by 184 publications

References 25 publications

Primary Progressive Aphasia with Glial Cytoplasmic Inclusions

Primary Progressive Aphasia with Glial Cytoplasmic Inclusions

Palatal tremor, progressive multiple cranial nerve palsies, and cerebellar ataxia: A case report and review of literature of palatal tremors in neurodegenerative disease

Spinocerebellar ataxia type 1 with multiple system degeneration and glial cytoplasmic inclusions

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