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Table 2 Classification of hereditary sensory neuropathies type I (HSN I)

From: Hereditary sensory neuropathy type I

Subtype Hallmark/additional features Locus Gene OMIM #
HSN IA Predominant loss of pain and temperature sensation, sometimes initial sign with long preservation of vibration sense, burning and lancinating pain, variable distal motor involvement, which may be severe. 9q22.1–q22.3 SPTLC1 162400
HSN IB Predominant sensory neuropathy with cough and gastro-oesophageal reflux, rarely foot ulcerations; normal distal muscle strength. 3p24–p22 unknown 608088
HSN IC (= CMT2B, HMSN IIB) Prominent distal motor involvement, often as initial sign of the disease, sensory loss of all qualities, acro-mutilating complications. 3q21 RAB7 600882
HSN ID Prominent sensory loss and mutilations in hands and feet, acropathy; variable motor involvement. unknown unknown -