Historical Research Review


Hereditary Sensory Neuropathy Type I (HSN I) is a rare genetic disorder characterized by the loss of sensation, especially in the feet and legs, and progressing to the hands and forearms. The loss of sensation is caused by abnormal function of the autonomic nervous system, which controls responses to pain and temperature as well as other involuntary or automatic body processes.

The most common type of inherited sensory neuropathy is Charcot-Marie-Tooth (CMT) disease. Some texts do not differentiate between this more common neuropathy and any other sensory neuropathy. Doctors who are not specialists in this area may diagnose someone with HSN I as having CMT.

In 1922 E. P. Hicks published an article in an English medical journal describing an English family in which 10 members suffered from perforating ulcers of the feet, shooting pains and deafness. In 1951 D. Denny-Brown published a follow up report on one of the members of this family, identifying the disease as “hereditary sensory radicular neuropathy.” In 1939 Drs. Tocantines and Reimann published their first report on Harvey and Russell Deater and other members of the family. Drs. Reimann, McKechnie, and Stanisavljevic published a follow up report in 1968.

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