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

フォーマット:

論文

責任表示:

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

言語:

英語

出版情報:

Springer, 2015-05-12

著者名:

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

掲載情報:

Cerebellum

ISSN:

1473-4222      

巻:

14

通号:

6

開始ページ:

707

終了ページ:

710

バージョン:

author

概要:

We described a 63-year-old Japanese female with genetically confirmed Huntington’s disease who showed unusual pathological findings in the cerebellum. This case exhibited typical neuropathological features as Huntington’s disease, including severe degeneration of the neostriatum and widespread occurrence of ubiquitin and expanded polyglutamine-positive neuronal intranuclear and intracytoplasmic inclusions. The cerebellum was macroscopically … unremarkable; however, somatic sprouts and halo-like amorphous materials of Purkinje cell with a large amount of torpedoes were noteworthy. Furthermore, the Purkinje cells were found to have granular cytoplasmic inclusions. Somatic sprouting is a form of degenerated Purkinje cell exhibited in several specific conditions. Although this finding usually appeared in developmental brains, several neurodegenerative disorders, including Menkes kinky hair disease, familial spinocerebellar ataxia, acute encephalopathy linked to familial hemiplegic migraine, and several other conditions, have been reported showing sprouting from the soma of Purkinje cell. We propose that Huntington’s disease is another degenerative condition associated with these distinct neuropathological findings of Purkinje cell. Abnormally accumulated huntingtin protein in the cytoplasm could be related to the development of these structures. © 2015 Springer Science+Business Media New York<br />In Press 続きを見る

URL:

http://hdl.handle.net/2297/43020