A necropsied case of Machado-Joseph disease with a hyperintense signal of transverse pontine fibres on long TR sequences of magnetic resonance images

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...

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Veröffentlicht in:Journal of neurology, neurosurgery and psychiatry neurosurgery and psychiatry, 1998-01, Vol.64 (1), p.140-141
Hauptverfasser: IMON, YUKARI, KATAYAMA, SADAO, KAWAKAMI, HIDESHI, MURATA, YOSHIO, OKA, MASAKI, NAKAMURA, SHIGENOBU
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Sprache:eng
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Zusammenfassung: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 of the necropsied brain. [...]the frontal atrophy commonly seen in Machado-Joseph disease may be caused by demyelination in the deep white matter and the hyperintense signal of transverse pontine fibres, visible on long TR sequences of MRI, is presumably due to the characteristic atrophy of the pons in Machado-Joseph disease.
ISSN:0022-3050
1468-330X
DOI:10.1136/jnnp.64.1.140