Pelizaeus-Merzbacher spectrum disorder
Pelizaeus-Merzbacher spectrum disorder
Definition
An X-linked leukodystrophy characterized by developmental delay, nystagmus, hypotonia, spasticity, and variable intellectual deficit. It is classified into three sub-forms based on the age of onset and severity: connatal, transitional, and classic PMD.
Also known as diffuse familial brain sclerosis, HLD1, Pelizaeus-Merzbacher brain sclerosis, Pelizaeus-Merzbacher disease, Pelizaeus-Merzbacher disease, X-linked recessive, Pelizaeus-Merzbacher spectrum disorder, PMD, Sudanophilic leukodystrophy, Paelizeus-Merzbacher type — per MONDO
Also identified as
- DOID 3210 per MONDO
- MESH D020371 per MONDO
- NCIT C75487 per MONDO
- OMIM 312080 per MONDO
- Orphanet 702 per MONDO
- SCTID 64855000 per MONDO
- UMLS C0205711 per MONDO
Affected Organs
| Organ | Involvement | Source |
|---|---|---|
| Central nervous system | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Central nervous 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 |