HMSN-P proximal dominant among Brazilians of Japanese ancestry

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Clin Neurol Neurosurg. 2007 Aug 29

Hereditary motor and sensory neuropathy (proximal dominant form,

HMSN-P) among Brazilians of Japanese ancestry.

Maeda K, Sugiura M, Kato H, Sanada M, Kawai H, Yasuda H.

Division of Neurology, Department of Medicine, Shiga University of

Medical Science, Seta-Tsukinowa, Otsu, Shiga 520-2192, Japan.

Hereditary motor and sensory neuropathy (proximal dominant form,

HMSN-P) has been reported exclusively from Okinawa Prefecture in

Japan. We herein report three brothers with HMSN-P who are among

Brazilians of Japanese ancestry. They showed the typical clinical

manifestations and were compatible with HMSN-P.

Okinawa Prefecture has been a site of emigration to other countries,

mainly in South America, since 1908. Although this is the first

reported familial case of HMSN-P occurring outside Japan, it is

estimated that there are 19 or 20 individuals with HMSN-P among

these emigrants.

Since HMSN-P might be misdiagnosed as familial amyotrophic lateral

sclerosis or spinal muscular atrophy, neurologists in countries

where individuals of Okinawan extraction reside should be aware of

this hereditary neuropathy. HMSN-P should no longer be regarded as

an endemic condition limited to Okinawa.