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Selective enlargement of the fourth ventricle in Machado‐Joseph disease: With special reference to the neuroradiological and neuropathological correlation
Author(s) -
Tsuchiya Kuniaki,
Ikeda Kenji,
Sugihara Hiroshi,
Watabiki Sadakiyo,
Ohbu Sadayoshi,
Abe Mitsubumi,
Takahashi Ryosuke,
Tadokoro Mamoru,
Shimada Hiroyuki
Publication year - 1997
Publication title -
neuropathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.701
H-Index - 61
eISSN - 1440-1789
pISSN - 0919-6544
DOI - 10.1111/j.1440-1789.1997.tb00032.x
Subject(s) - machado–joseph disease , correlation , ventricle , fourth ventricle , medicine , disease , pathology , anatomy , mathematics , spinocerebellar ataxia , geometry
This report concerns a study of serial brain computed tomography (CT) images including neuroradiological and neuropathological correlations in three patients with Machado‐Joseph disease (MJD). The diagnosis of MJD was confirmed by neuropathological examination in two patients (Cases 1 and 2), and by molecular analysis in one (Case 3). Serial CT images of Case 1 were obtained for nine years, and of Cases 2 and 3 for three years. The results indicate that in the initial stages of MJD only enlargement of the fourth ventricle is seen and that in the advanced stages of the disease, atrophy of the cerebellar hemispheres also becomes evident. These findings are essentially in agreement with the previously described sequential neuropathological changes of the spinocerebellar pathways in MJD patients. Enlargement of the fourth ventricle in the initial stages of the disease is probably due to the involvement of the dentate nucleus with degeneration of the efferent fibers. The atrophy of the cerebellar hemispheres seen in the advanced stages of MJD is probably caused by alterations of the pontine nucleus with degeneration of the cerebellar white matter.