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Congenital Sensory Neuropathy

MARC J. SCHNEIDERMAN, MD
Am J Dis Child. 1986;140(11):1100. doi:10.1001/archpedi.1986.02140250026023.
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Sir.—The article "Paucifascicular Congenital Sensory Neuropathy in Identical Twins" by Croall et al1 was very informative and well written. We are learning that there are other variations of the classic four groups of congenital sensory neuropathies.2-4 We recently described a steroid-responsive pure sensory neuropathy.5 Only mixed motor and sensory congenital neuropathies have been successfully treated with steroids in the past.6 In a family of three, stocking/glove sensory neuropathy developed beginning in the third decade of life. A sural nerve biopsy specimen demonstrated vasculitis, and steroid treatment resulted in complete resolution of the neuropathy and its complications. We think that any clinician treating a congenital sensory neuropathy should be alerted that some forms may be steroid responsive and, therefore, treatable and that careful analysis of pathologic materials is essential to uncover a potentially treatable disease.

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