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

• Published in: Journal of medical genetics Vol. 17; no. 6; pp. 424 - 429
• Main Authors: Tamari, I, Goodman, R M, Sarova, I, Hertz, M, Adar, R, Zvibach, T
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group Ltd 01.12.1980; BMJ Publishing Group LTD
• Subjects: Adolescent; Adult; Child; Consanguinity; Female; Genes, Recessive; Hereditary Sensory and Autonomic Neuropathies - classification; Hereditary Sensory and Autonomic Neuropathies - diagnostic imaging; Hereditary Sensory and Autonomic Neuropathies - genetics; Humans; Iraq - ethnology; Israel; Jews; Male; Pedigree; Radiography; Syria - ethnology
• ISSN: 0022-2593; 1468-6244; 1468-6244
• DOI: 10.1136/jmg.17.6.424

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 pain stimulation with normal motor and sensory nerve conduction velocity. This response may be an expression of the carrier state for this hereditary disease. Only five other families (non-Jewish) have been reported as having this form of peripheral hereditary sensory neuropathy. These observations suggest that one type, the progressive form, of peripheral hereditary sensory neuropathy may be more common in Oriental Jews.