Charcot-Marie-Tooth disease type 4C
Charcot-Marie-Tooth disease type 4C
Definition
Charcot-Marie-Tooth disease type 4C (CMT4C) is a subtype of Charcot-Marie-Tooth type 4 characterized by childhood or adolescent-onset of a relatively mild, demyelinating sensorimotor neuropathy that contrasts with a severe, rapidly progressing, early-onset scoliosis, and the typical CMT phenotype (i.e. distal muscle weakness and atrophy, sensory loss, and often foot deformity). A wide spectrum of nerve conduction velocities are observed and cranial nerve involvement and kyphoscoliosis have also been reported.
Also known as Charcot-Marie-Tooth disease type 4 caused by mutation in SH3TC2, Charcot-Marie-Tooth disease type 4C, CMT4C, SH3TC2 Charcot-Marie-Tooth disease type 4 — per MONDO
Also identified as
- DOID 0110183 per MONDO
- MESH C535423 per MONDO
- NCIT C129864 per MONDO
- OMIM 601596 per MONDO
- Orphanet 99949 per MONDO
- SCTID 715797002 per MONDO
- UMLS C1866636 per MONDO
Affected Organs
| Organ | Involvement | Source |
|---|---|---|
| Musculature | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Musculature | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |
| Musculoskeletal system | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Musculoskeletal system | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |
| Nervous system | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Nervous system | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |
| Peripheral nervous system | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Peripheral nervous system | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |