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A necropsied case of Machado-Joseph disease with a hyperintense signal of transverse pontine fibres on long TR sequences of magnetic resonance images
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Machado-Joseph disease refers to autosomal dominant
spinocerebellar degeneration, and the gene responsible for the disease exhibits an expanded trinucleotide CAG repeat in chromosome
14q32.1.1 Machado-Joseph disease has a wide range of
clinical manifestations in addition to the cerebellar ataxia. The
diverse disorders are characterised neuropathologically by the
involvement of the pallidoluysian, dentatorubral, pontocerebellar,
cochleocerebellar, and spinocerebellar systems, lower motor neurons,
and dorsal root ganglia. Previous MRI studies disclosed only mild
cerebellar and brain stem atrophy in Machado-Joseph
disease.2 Our MRI examinations in 31 cases disclosed
atrophy of the pons, middle, and superior cerebellar peduncles, and
frontal and temporal lobes, together with fourth ventricular
dilatation.3 A third of the cases displayed a hyperintense signal of the transverse pontine fibres, which had been found previously in patients with olivopontocerebellar atrophy.4 Here, we report on a patient with Machado-Joseph disease with an
abnormal pontine signal on MRI, nine months before death, and pathological findings
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This article has been cited by other articles:
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Okamoto, K., Tokiguchi, S., Furusawa, T., Ishikawa, K., Quardery, A. F., Shinbo, S., Sasai, K.
(2003). MR Features of Diseases Involving Bilateral Middle Cerebellar Peduncles. Am. J. Neuroradiol.
24: 1946-1954
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