AI Article Synopsis
- Hereditary motor and sensory neuropathy (proximal dominant form, or HMSN-P), previously only reported in Okinawa, Japan, has now been documented in three Brazilian brothers of Japanese descent.
- The brothers exhibited typical symptoms of HMSN-P, and it is estimated that around 19-20 individuals with this condition exist among Okinawan emigrants in South America.
- Neurologists in regions with Okinawan populations should recognize HMSN-P to prevent misdiagnosis with other similar conditions like ALS or spinal muscular atrophy.
Article Abstract
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.
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| http://dx.doi.org/10.1016/j.clineuro.2007.07.015 | DOI Listing |
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