Case Study: Somatic Sprouts and Halo-Like Amorphous Materials of the Purkinje Cells in Huntington’s Disease

Sakai, Kenji; Ishida, Chiho; Morinaga, Akiyoshi; Takahashi, Kazuya; Yamada, Masahito

doi:10.1007/s12311-015-0678-4

Cited by 5 publications

(7 citation statements)

References 13 publications

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“…Instead, localized changes and altered connectivity are more commonly reported than volume loss. For instance, Purkinje cell loss/degeneration is accompanied by somatic sprouts, halo-like amorphous materials, torpedoes, and granular cytoplasmic inclusions in Purkinje cells [73]. These changes localize to the oculomotor vermis (which would explain symptoms of impaired smooth eye pursuits, impaired Hoffman reflex, and hypotonia) [18].…”

Section: Cerebellar Dysfunction In Neurodegenerationmentioning

confidence: 99%

Neurodegeneration and the Cerebellum

Samson

Claassen²

2017

Neurodegener Dis

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Background: The cerebellum modulates diverse neuronal networks, regulating motor, cognitive, behavioral, and limbic circuits. Structural and functional changes to the cerebellum that occur with neurodegenerative conditions have not been systematically reviewed. Objective: We synthesize the current understanding of cerebellar somatotopy and function with previously described cerebellar changes in neurodegenerative diseases not associated with primary cerebellar pathology. Methods: A thorough literature review defines the role of the cerebellum in normal function and neurodegeneration, and we additionally provide exemplar cases of cerebellar dysfunction in neurodegenerative disorders. Results: Comparisons between normal function and neurodegenerative disease states emphasize how normal organization of the cerebellum is altered in neurodegenerative disease states. We illustrate key anatomic structures using novel cerebellar segmentation tools and images. Conclusions: Altered cerebellum in neurodegeneration can provide important diagnostic and pathologic insights into both predicting disease progression and network dysfunction.

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Section: Cerebellar Dysfunction In Neurodegenerationmentioning

confidence: 99%

Neurodegeneration and the Cerebellum

Samson

Claassen²

2017

Neurodegener Dis

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“…8,9 In addition, we previously demonstrated that Huntington's disease is one of the diseases showing somatic sprouting of Purkinje cells. 7 Direct damages to Purkinje cells could be proposed in these diseases, such as abnormal protein accumulation; however, no apparent α-synuclein accumulation has been described in Purkinje cells of MSA. A synucleinopathy with specific glioneuronal degeneration, which is associated with early myelin dysfunction and neuronal degeneration related to retrograde axonal disease, is suggested as a central role in the disease process in MSA.…”

Section: Discussionmentioning

confidence: 63%

“…Details of the pathomechanism behind developing somatic sprouts are still uncertain. Several degenerative disorders have been reported to be associated with somatic sprouts of Purkinje cells, [2][3][4][5][6][7] including hereditary spinocerebellar ataxia. [3][4][5] In patients with SCA31, a large number of Purkinje cells with the somatic sprouting and halo-like structures were observed.…”

Section: Discussionmentioning

confidence: 86%

“…Several degenerative disorders have been reported to be associated with somatic sprouts of Purkinje cells, [2][3][4][5][6][7] including hereditary spinocerebellar ataxia. The halo-like structures were observed around several Purkinje cells.…”

Section: Discussionmentioning

confidence: 99%

“…This case demonstrated typical clinical and neuropathological findings as MSA-P apart from a small number of Purkinje cells demonstrating somatic sprouts and halo-like structures. 7 Direct damages to Purkinje cells could be proposed in these diseases, such as abnormal protein accumulation; however, no apparent α-synuclein accumulation has been described in Purkinje cells of MSA. Several degenerative disorders have been reported to be associated with somatic sprouts of Purkinje cells, [2][3][4][5][6][7] including hereditary spinocerebellar ataxia.…”

Section: Discussionmentioning

confidence: 99%

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Somatic sprouts of the Purkinje cells in a patient with multiple system atrophy

et al. 2018

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We describe the post mortem case of a 71-year-old Japanese woman diagnosed as having multiple system atrophy (MSA), showing somatic sprouting formation of Purkinje cells. The patient had suffered from frequent falling episodes and clumsiness of the left hand since the age of 67 years. Orthostatic hypotension and parkinsonism subsequently emerged. Typical neuropathological features of MSA, including degeneration of the striatum, pontine base and cerebellum with abundance of phosphorylated α-synuclein-positive neuronal and glial cytoplasmic and nuclear inclusions in the brain, were observed. In addition to gliosis of the cerebellar white matter and notable loss of Purkinje cells, several Purkinje cells showed somatic sprouting. Somatic sprouting of Purkinje cells has been demonstrated in several specific conditions, such as developing brains and several neurodegenerative disorders, including Menkes kinky hair disease, familial spinocerebellar ataxia, acute encephalopathy linked to familial hemiplegic migraine, and Huntington's disease; however, no MSA cases have been reported with sprouting from the soma of Purkinje cells. Axonal damage caused by oligodendroglial dysfunction could be crucial in the development of Purkinje cell loss in MSA. Moreover, no apparent α-synuclein accumulation has been described in the Purkinje cells of MSA. We propose that MSA is another degenerative disorder associated with somatic sprouts of Purkinje cells.

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Cerebellum in Alzheimer's disease and other neurodegenerative diseases: an emerging research frontier

Yang,

Liu,

Chen

et al. 2024

MedComm

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The cerebellum is crucial for both motor and nonmotor functions. Alzheimer's disease (AD), alongside other dementias such as vascular dementia (VaD), Lewy body dementia (DLB), and frontotemporal dementia (FTD), as well as other neurodegenerative diseases (NDs) like Parkinson's disease (PD), amyotrophic lateral sclerosis (ALS), Huntington's disease (HD), and spinocerebellar ataxias (SCA), are characterized by specific and non‐specific neurodegenerations in central nervous system. Previously, the cerebellum's significance in these conditions was underestimated. However, advancing research has elevated its profile as a critical node in disease pathology. We comprehensively review the existing evidence to elucidate the relationship between cerebellum and the aforementioned diseases. Our findings reveal a growing body of research unequivocally establishing a link between the cerebellum and AD, other forms of dementia, and other NDs, supported by clinical evidence, pathological and biochemical profiles, structural and functional neuroimaging data, and electrophysiological findings. By contrasting cerebellar observations with those from the cerebral cortex and hippocampus, we highlight the cerebellum's distinct role in the disease processes. Furthermore, we also explore the emerging therapeutic potential of targeting cerebellum for the treatment of these diseases. This review underscores the importance of the cerebellum in these diseases, offering new insights into the disease mechanisms and novel therapeutic strategies.

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Case Study: Somatic Sprouts and Halo-Like Amorphous Materials of the Purkinje Cells in Huntington’s Disease

Cited by 5 publications

References 13 publications

Neurodegeneration and the Cerebellum

Neurodegeneration and the Cerebellum

Somatic sprouts of the Purkinje cells in a patient with multiple system atrophy

Cerebellum in Alzheimer's disease and other neurodegenerative diseases: an emerging research frontier

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