Autosomal recessive peripheral sensory neuropathy in 3 non-Ashkenazi Jewish families.

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Autosomal recessive peripheral sensory neuropathy in 3 non-Ashkenazi Jewish families.

Three unrelated Oriental Jewish families with a total of eight subjects with progressive hereditary sensory neuropathy are reported. The parents were all unaffected and because of parental consanguinity in each of the three families it is postulated that this rare neurological disorder is transmitted in an autosomal recessive manner. In one family both parents showed an abnormal response to pai...

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Six families are described with hereditary motor and sensory neuropathy (HMSN) of probable autosomal recessive inheritance. Four of these were classified as HMSN type I and two as type II. The consanguinity rate in this series was high, suggesting that these recessive genes are rare. In comparison with the dominantly inherited forms of these disorders, the mean age of onset was significantly ea...

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Autosomal recessive motor and sensory neuropathy with excessive myelin outfolding.

Two Japanese persons with consanguinous parents had a motor and sensory neuropathy of the hypertrophic type with excessive myelin outfolding in the myelinated fibers. A morphometric analysis of the biopsied sural nerve was made. Excessive myelin outfolding, segmental demyelination, and remyelination and decrease in the density of both large and small myelinated fibers were evident. Using linear...

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Investigating Seven Recently Identified Genes in 100 Iranian Families with Autosomal Recessive Non-syndromic Hearing Loss

Objectives: Hearing loss (HL) is the most common sensory disorder, and affects 1 in 1000 newborns. About 50% of HL is due to genetics and 70% of them are non-syndromic with a recessive pattern of inheritance. Up to now, more than 50 genes have been detected which are responsible for autosomal recessive non-syndromic hearing loss, (ARNSHL). In  Iran, HL is one of the most common disabilitie...

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Department of Pediatric Neurology, Hospital Universitário de Santa Maria, Santa Maria RS, Brazil. 1Pediatric Neurologist at Hospital Universitário de Santa Maria, Santa Maria RS, Brazil; 2Resident of Pediatrics Neurology at Hospital Universitário de Santa Maria, Santa Maria RS, Brazil; 3Resident of Pediatrics at Hospital Universitário de Santa Maria, Santa Maria RS, Brazil; 4Medicine Graduation...

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ژورنال

عنوان ژورنال: Journal of Medical Genetics

سال: 1980

ISSN: 1468-6244

DOI: 10.1136/jmg.17.6.424