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Palmoplantar keratoderma-hereditary motor and sensory neuropathy syndrome

MedGen UID:
322722
Concept ID:
C1835671
Disease or Syndrome
Synonyms: Axonal neuropathy with palmoplantar keratoderma; Charcot-Marie-Tooth disease with palmoplantar keratoderma and nail dystrophy; Keratoderma palmoplantar spastic paralysis; Keratoderma, palmoplantar, with nail dystrophy and hereditary motor-sensory neuropathy
SNOMED CT: Palmoplantar keratoderma, Charcot-Marie-Tooth syndrome (1222646006); Palmoplantar keratoderma, hereditary motor and sensory neuropathy syndrome (1222646006)
 
Monarch Initiative: MONDO:0007853
OMIM®: 148360
Orphanet: ORPHA538574

Definition

A rare genetic autosomal dominant hereditary axonal motor and sensory neuropathy disorder with characteristics of childhood-onset palmoplantar keratoderma associated with motor and sensory polyneuropathy manifesting with late-onset, predominantly distal, lower limb muscle weakness and atrophy (later associating mild proximal weakness and upper limb involvement), moderate sensory impairment and normal or near normal nerve conduction velocities. Additional variable manifestations include impaired vibratory sensation, reduced tendon reflexes, pain, talipes equinovarus, pes cavus and nail dystrophy. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPalmoplantar keratoderma-hereditary motor and sensory neuropathy syndrome
Follow this link to review classifications for Palmoplantar keratoderma-hereditary motor and sensory neuropathy syndrome in Orphanet.

Recent clinical studies

Clinical prediction guides

Tolmie JL, Wilcox DE, McWilliam R, Assindi A, Stephenson JB
J Med Genet 1988 Nov;25(11):754-7. doi: 10.1136/jmg.25.11.754. PMID: 2976839Free PMC Article

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    Palmoplantar keratoderma-hereditary motor and sensory neuropathy syndrome
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