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CMT 1

Autosomal DominantNeuropathy (Hereditary)OMIM 618682
W
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Overview

From WUSTL Neuromuscular

● Epidemiology: 2 patients, 1 family Genetics Mutation: K28I (c.83A>T) Allelic disorder: CMT-DI c1orf194 protein Calcium regulator in neurons Clinical Onset age: 6 & 40 years Weakness Arms & Legs Distal Gait: Steppage Course: Progressive Sensory loss Distal Arms Modalities: Large & Small fiber Tendon reflexes: Absent in Legs Pes cavus Laboratory Nerve conduction Velocities: Demyelinating; Velocity 11 M/s SNAPs: Absent c1orf194 variant: CMT-DI Epidemiology: 6 patients. 1 family Genetics Mutation: I122N (c.365T>A) Inheritance: Dominant Clinical Onset age: 20 to 33 years Weakness Distal Legs Gait

OMIM Entries

OMIM #618682

Related Conditions

Shared genes
Distal Myopathy: MPD3
HNRNPA1EPIDEMIOLOGY
HEREDITARY MOTOR SENSORY NEUROPATHIES (HMSN; CMT)
DIFCMT1ATP6
Myokymia & Benign Neonatal Epilepsy (BFNS1)
KCNEPIDEMIOLOGY

External Resources

WUSTL Neuromuscular
Washington University Disease Center
OMIM
Online Mendelian Inheritance in Man
PubMed
Biomedical Literature
GeneReviews
NCBI Expert-Authored Reviews
Orphanet
Portal for Rare Diseases
NORD
National Organization for Rare Disorders

Data sourced from the Washington University Neuromuscular Disease Center. For clinical use, always refer to primary sources.