Distribution of cerebellar cortical lesions in multiple system atrophy: a topographic neuropathological study of three autopsy cases in Japan.

Tsuchiya, Kuniaki; Watabiki, Sadakiyo; Sano, Mizuka; Iobe, Hiroaki; Shiotsu, Hidetoshi; Taki, Kazuhiro; Hashimoto, Keisuke

doi:10.1016/s0022-510x(97)00279-7

Cited by 15 publications

(6 citation statements)

References 13 publications

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“…There may be a specific and progressive cerebral atrophy affecting the frontal lobes and the motor/premotor areas . In the cerebellum, pathology is more evident in the vermis than the hemispheres . In the midbrain, the substantia nigra and locus coeruleus are often pale due to loss of pigment.…”

Section: General Featuresmentioning

confidence: 99%

Visual signs and symptoms of multiple system atrophy

Armstrong

2014

Clinical and Experimental Optometry

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Multiple system atrophy (MSA) is a rare movement disorder and a member of the ‘parkinsonian syndromes’, which also include Parkinson's disease (PD), progressive supranuclear palsy (PSP), dementia with Lewy bodies (DLB) and corticobasal degeneration (CBD). Multiple system atrophy is a complex syndrome, in which patients exhibit a variety of signs and symptoms, including parkinsonism, ataxia and autonomic dysfunction. It can be difficult to separate MSA from the other parkinsonian syndromes but if ocular signs and symptoms are present, they may aid differential diagnosis. Typical ocular features of MSA include blepharospasm, excessive square‐wave jerks, mild to moderate hypometria of saccades, impaired vestibular‐ocular reflex (VOR), nystagmus and impaired event‐related evoked potentials. Less typical features include slowing of saccadic eye movements, the presence of vertical gaze palsy, visual hallucinations and an impaired electroretinogram (ERG). Aspects of primary vision such as visual acuity, colour vision or visual fields are usually unaffected. Management of the disease to deal with problems of walking, movement, daily tasks and speech problems is important in MSA. Optometrists can work in collaboration with the patient and health‐care providers to identify and manage the patient's visual deficits. A more specific role for the optometrist is to correct vision to prevent falls and to monitor the anterior eye to prevent dry eye and control blepharospasm.

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Section: General Featuresmentioning

confidence: 99%

Visual signs and symptoms of multiple system atrophy

Armstrong

2014

Clinical and Experimental Optometry

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“…Spinocerebellar ataxia (SCA) or spinocerebellar degeneration (SCD) is a progressive neurodegenerative disorder, clinically characterized by ataxic gait, ataxia of the extremities, dysarthria, and nystagmus, with degeneration of the cerebellum, brainstem, and spinal cord 1–11 . SCA or SCD is usually divided into two groups: non‐hereditary (sporadic) SCD, including MSA, the most prevalent disorder in Japanese patients with SCA, in which the cardinal pathological hallmark is the presence of glial cytoplasmic inclusions (Papp‐Lantos inclusions) of the central nervous system, and late cerebellar cortical atrophy (LCCA) and hereditary SCD, consisting of autosomal dominant SCD, including SCA1, SCA2, SCA3 (MJD), SCA6, SCA7, DRPLA, and autosomal recessive SCD, including Friedreich's ataxia, which is very rare in Japan 12–31 …”

Section: Introductionmentioning

confidence: 99%

Degeneration of the inferior olive in spinocerebellar ataxia 6 may depend on disease duration: Report of two autopsy cases and statistical analysis of autopsy cases reported to date

et al. 2005

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This report concerns a clinicopathological study of two autopsied patients with spinocerebellar ataxia 6 (SCA6), and a statistical analysis between neuronal loss of the inferior olive and disease duration of 15 SCA6 autopsy cases reported to date, including the two cases reported in this study. Cases 1 and 2 came from independent Japanese families. Case 1 developed gait disturbance at age 35 years and died at age 78 years; she had a CAG-repeat expansion of the SCA6 gene (25/13). Case 2 presented with gait disturbance at age 68 years and died at age 78 years; he had an expanded CAG-repeat of the SCA6 gene (22/13). Neuropathological examination of both cases disclosed not only neuronal loss of the Purkinje cells and inferior olive, but also some unnoticed features, including cactus-like expansion of the dendrite of Purkinje cells and relative preservation of Golgi cells in the granular layer of the cerebellum. Exploratory statistical analysis between 11 SCA6 autopsy cases with neuronal loss in the inferior olive (average disease duration: 27 years) and four SCA6 autopsy cases without neuronal loss in the olive (average disease duration: 14.5 years) was investigated by Kaplan-Meier estimates of survival and log-rank test, retrospectively. Kaplan-Meier estimates of survival revealed an obvious difference between the two groups. Survival of 10 years after the disease onset was 90.9% in the former 11 SCA6 autopsy cases, but was 50% in the latter four SCA6 autopsy cases. Furthermore, a log-rank test on the two groups disclosed a significant difference (P=0.0450). We postulate that the neuronal loss of the inferior olive in SCA6 may depend on disease duration.

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“…In addition, MSA is characterized by neuronal loss associated with astrogliosis. The degree of neuronal loss appears to be related to the duration of illness 25 and to the type of MSA 26 . Degenerative changes in our case were very severe, but mainly restricted to the systematically favorable site of MSA in spite of the long clinical course, excepting the hypoxic‐damaged sites.…”

Section: Discussionmentioning

confidence: 53%

Extensive distribution of glial cytoplasmic inclusions in an autopsied case of multiple system atrophy with a prolonged 18‐year clinical course

Masui¹,

Nakata²,

Fujii³

et al. 2011

Neuropathology

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We describe herein an autopsied case of multiple system atrophy (MSA) with prolonged clinical course of 18 years, and evaluate the extent of neurodegeneration and glial cytoplasmic inclusions (GCIs) in the entire brain of this rare case. A 64-year-old woman presented with typical neurological symptoms and imaging features of MSA. Thereafter, she became bedridden, and breathing was assisted through a tracheostomy for 12 years. She died at the age of 82 after 18 years from the initial symptom. Post mortem examination revealed severe neurodegeneration in the inferior olive, pontine nuclei, substantia nigra, locus ceruleus, putamen and cerebellum. Notably, phosphorylated α-synuclein (p-α-syn)-positive GCIs were found in these areas, but their number was very low. In contrast, the density of GCIs was much higher in such regions as the tectum/tegmentum of the brainstem, pyramidal tracts, neocortices and limbic system, which usually contain a small number of GCIs. Another constituent of GCIs, ubiquitin (Ub) and Ub-associated autophagy substrate p62, were also positive in some GCIs, and distribution of Ub/p62 immunoreactivity was proportionate to that of p-α-syn+ GCIs despite the very long duration of the disease. Furthermore, this case had complicated hypoxic encephalopathy, but p-α-syn+ GCIs were also found in the damaged white matter, indicating the contribution of α-syncleinopathy as well as hypoxic effect to the secondary myelin and axonal loss in the white matter. Together, this rare case suggests the contribution of the disease duration to the prevalence of GCIs, and the possible involvement of the limbic system in extensive-stage disease.

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Distribution of cerebellar cortical lesions in multiple system atrophy: a topographic neuropathological study of three autopsy cases in Japan.

Cited by 15 publications

References 13 publications

Visual signs and symptoms of multiple system atrophy

Visual signs and symptoms of multiple system atrophy

Degeneration of the inferior olive in spinocerebellar ataxia 6 may depend on disease duration: Report of two autopsy cases and statistical analysis of autopsy cases reported to date

Extensive distribution of glial cytoplasmic inclusions in an autopsied case of multiple system atrophy with a prolonged 18‐year clinical course

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