title: The clinical spectrum and morphology of type II hereditary sensory neuropathy.
abstract: Consistent morphological features were found in sural nerve biopsies of four sporadic cases of sensory neuropathy. The morphological finding of marked fascicular atrophy, a profound loss of myelinated fibres and a relative preservation of unmyelinated fibres are typical of those previously found in the recessive form  of hereditary sensory neuropathy (HSN-II). Variable clinical manifestations contrasted with this morphological specificity. Where clinical doubt exists, the value of nerve biopsy in distinguishing HSN types I and II is emphasized. Long-term clinical follow-up and a comparison of repeat nerve biopsy data indicated that HSN-II is a progressive disorder.
mesh_terms: D000328:Adult; D001706:Biopsy; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D009477:Hereditary Sensory and Autonomic Neuropathies / Q000175:diagnosis / Q000473:pathology* / Q000503:physiopathology; D006801:Humans; D008297:Male; D009413:Nerve Fibers, Myelinated / Q000473:pathology; D009431:Neural Conduction; D013127:Spinal Nerves / Q000473:pathology*; D013497:Sural Nerve / Q000473:pathology*