Asymmetrical temporal lobe atrophy with massive neuronal inclusions in multiple system atrophy

Shibuya, Kazuhiko; Nagatomo, H; Iwabuchi, K; Inoue, M.; Yagishita, Saburo; Itoh, Yoshimitsu

doi:10.1016/s0022-510x(00)00364-6

Cited by 32 publications

(35 citation statements)

References 21 publications

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“…1b). This type of NCI occurred infrequently in the griseum pontis and none or only few of typical NCI were found in the other brain regions except for one case (case 16), in which numerous NCI were found in the neurons in the dentate layer of the hippocampal formation 9 …”

Section: Resultsmentioning

confidence: 88%

Distribution and dynamic process of neuronal cytoplasmic inclusion (NCI) in MSA: Correlation of the density of NCI and the degree of involvement of the pontine nuclei

Yokoyama¹,

Kusunoki²,

Hasegawa³

et al. 2001

Neuropathology

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MSA is a sporadic degenerative disease that occurs in striatonigral degeneration (SND), SDS and most cases of sporadic OPCA. Oligodendroglial inclusion is a hallmark of MSA. Recently there have been a small number of reports of neuronal argyrophilic inclusions. To clarify the distribution and dynamic process of neuronal cytoplasmic inclusions (NCI), 31 cases of MSA were studied using histology, immunohistochemistry, and electron microscopy. The inclusions were exclusively found in the pontine nucleus and there was a correlation between the incidence of NCI and the severity of OPCA, but not of SND. NCI were increased to some extent in the cases with moderate OPCA and decreased in number in proportion to devastation of the pontine nuclei. Immunohistochemical and ultrastructural features of NCI were virtually identical to those of glial cytoplasmic inclusions (GCI), which gives some clues to the pathogenesis of MSA. It is tempting to interpret this as NCI playing a significant role in the degenerative changes of the neurons at least in the pons. Further systematic studies on NCI in the other brain regions are necessary to elucidate the pathogenesis of neuronal degeneration in MSA.

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Section: Resultsmentioning

confidence: 88%

Distribution and dynamic process of neuronal cytoplasmic inclusion (NCI) in MSA: Correlation of the density of NCI and the degree of involvement of the pontine nuclei

Yokoyama¹,

Kusunoki²,

Hasegawa³

et al. 2001

Neuropathology

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“…Another intriguing neuropathological finding was the presence of many intraneuronal inclusions in the dentate fascia. Two reports have previously described such inclusions in dentate fascia in MSA [10,11]. In one of those cases, cognitive impairment and asymmetrical temporal atrophy was observed [11].…”

Section: Antibodies Used In This Investigation and Results Of Immunohmentioning

confidence: 99%

Synucleinopathy with features of both multiple system atrophy and dementia with Lewy bodies

Sikorska

Papierz

Preusser

et al. 2007

Neuropathology Appl Neurobio

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“…Unusual for PD, however, was the severe neuronal loss in the CA2/3 subregions of the hippocampus and the extensive accumulation of neuronal α-synuclein in the hippocampus, including the DF, and striatum [7, 8, 38, 52]. Involvement of the DF by neuronal α-synuclein inclusions has been described previously in the case of DLB with additional MSA-type pathology and in some cases of MSA [73, 74, 78]. The severe neuronal loss observed in CA2/3 of this case bears similarity to that described in association with A53T SNCA mutation [48, 77] and in cases of multiplication of SNCA , though this pattern of loss was suggested to be a unique feature of the latter [21] (Table 2).…”

Section: Discussionmentioning

confidence: 96%

α-Synucleinopathy associated with G51D SNCA mutation: a link between Parkinson’s disease and multiple system atrophy?

Kiely

Asi

Kara³

et al. 2013

Acta Neuropathol

401

318

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We report a British family with young-onset Parkinson’s disease (PD) and a G51D SNCA mutation that segregates with the disease. Family history was consistent with autosomal dominant inheritance as both the father and sister of the proband developed levodopa-responsive parkinsonism with onset in their late thirties. Clinical features show similarity to those seen in families with SNCA triplication and to cases of A53T SNCA mutation. Post-mortem brain examination of the proband revealed atrophy affecting frontal and temporal lobes in addition to the caudate, putamen, globus pallidus and amygdala. There was severe loss of pigmentation in the substantia nigra and pallor of the locus coeruleus. Neuronal loss was most marked in frontal and temporal cortices, hippocampal CA2/3 subregions, substantia nigra, locus coeruleus and dorsal motor nucleus of the vagus. The cellular pathology included widespread and frequent neuronal α-synuclein immunoreactive inclusions of variable morphology and oligodendroglial inclusions similar to the glial cytoplasmic inclusions of multiple system atrophy (MSA). Both inclusion types were ubiquitin and p62 positive and were labelled with phosphorylation-dependent anti-α-synuclein antibodies In addition, TDP-43 immunoreactive inclusions were observed in limbic regions and in the striatum. Together the data show clinical and neuropathological similarities to both the A53T SNCA mutation and multiplication cases. The cellular neuropathological features of this case share some characteristics of both PD and MSA with additional unique striatal and neocortical pathology. Greater understanding of the disease mechanism underlying the G51D mutation could aid in understanding of α-synuclein biology and its impact on disease phenotype.Electronic supplementary materialThe online version of this article (doi:10.1007/s00401-013-1096-7) contains supplementary material, which is available to authorized users.

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Asymmetrical temporal lobe atrophy with massive neuronal inclusions in multiple system atrophy

Cited by 32 publications

References 21 publications

Distribution and dynamic process of neuronal cytoplasmic inclusion (NCI) in MSA: Correlation of the density of NCI and the degree of involvement of the pontine nuclei

Distribution and dynamic process of neuronal cytoplasmic inclusion (NCI) in MSA: Correlation of the density of NCI and the degree of involvement of the pontine nuclei

Synucleinopathy with features of both multiple system atrophy and dementia with Lewy bodies

α-Synucleinopathy associated with G51D SNCA mutation: a link between Parkinson’s disease and multiple system atrophy?

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