Distribution of cerebello-olivary degeneration in idiopathic late cortical cerebellar atrophy: clinicopathological study of four autopsy cases

Neuropathology : Official Journal of the Japanese Society of Neuropathology
Satoru OtaHaruhiko Akiyama

Abstract

Late cortical cerebellar atrophy (LCCA) is a neurodegenerative disease which presents with slowly progressive cerebellar ataxia as a prominent symptom and is characterized neuropathologically by a limited main lesion to the cerebellar cortex and inferior olivary nucleus. To elucidate the features of lesions in the cerebellar cortex and inferior olivary nucleus, four autopsy cases suffering from idiopathic LCCA without other cortical cerebellar atrophies, such as alcoholic cerebellar degeneration, phenytoin intoxication, or hereditary cerebellar atrophy including spinocerebellar ataxia type 6, were examined. All affected patients had identical distinct features of cerebellar cortical lesions. In all four cases, the most obvious pathological finding throughout the cerebellum was loss of Purkinje cells, but the rarefaction of granular cell layers was observed only where loss of Purkinje cells was very severe, and thinning of the molecular layer was seen only where the rarefaction of granular cell layers was moderate to severe. Two patients presented with vermis dominant cerebellar cortical lesions, but the other two patients showed hemispheric dominant pathological changes. Neuronal loss of the inferior olivary nucleus was observe...Continue Reading

References

Mar 14, 1998·Acta Neuropathologica·H SasakiK Nagashima
Jun 17, 1999·Journal of Neurology, Neurosurgery, and Psychiatry·K IshikawaH Mizusawa
Aug 6, 1999·Ryōikibetsu shōkōgun shirīzu·H Mizusawa
Apr 19, 2002·Brain : a Journal of Neurology·M AbeleT Klockgether
May 7, 2005·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Kuniaki TsuchiyaYoshio Hashizume

❮ Previous
Next ❯

Citations

Jun 10, 2011·The Cerebellum·Elan D LouisJean-Paul G Vonsattel
Nov 1, 1958·Electroencephalography and Clinical Neurophysiology·G OWENS, W M CLARK
May 1, 1966·Journal of the Neurological Sciences·J Allsop, B Turner
Jul 8, 2011·Current Opinion in Neurology·Thomas Klockgether
Sep 9, 2009·Clinical Neurology and Neurosurgery·Noriyuki KimuraToshio Okazaki
Aug 14, 2009·Journal of Neuroimaging : Official Journal of the American Society of Neuroimaging·Noriyuki KimuraToshio Okazaki
Mar 13, 2014·Movement Disorders : Official Journal of the Movement Disorder Society·David J LinJeremy D Schmahmann
Jan 20, 2010·Lancet Neurology·Thomas Klockgether
Jan 27, 2018·Cerebellum & Ataxias·Elan D LouisPhyllis L Faust
Jan 30, 2020·Movement Disorders : Official Journal of the Movement Disorder Society·Jennifer FaberThomas Klockgether
Nov 2, 2016·Movement Disorders Clinical Practice·Michael NockerChristoph Scherfler
Jul 5, 2018·Journal of Clinical Neurology·Ji Sun KimJin Whan Cho

❮ Previous
Next ❯

Related Concepts

Related Feeds

Ataxias

Ataxia is a neurological condition characterized by lack of voluntary coordination of muscle movements including loss of coordination, balance, and speech. Discover the latest research on different types of ataxias here.

Ataxia telangiectasia (MDS)

Ataxia telangiectasia is a rare neurodegenerative diseases caused by defects in the ATM gene, which is involved in DNA damage recognition and repair pathways. Here is the latest research on this autosomal recessive disease.