Isolated cerebellar hypoplasia/agenesis
Isolated cerebellar hypoplasia/agenesis
Definition
Hypoplasia of the cerebellum that is associated with inherited metabolic disorders and neurodegenerative disorders. Signs and symptoms include mental and developmental delays, walking and balance difficulties, floppy muscle tone, and seizures.
Also known as cerebellar hypoplasia/atrophy, epilepsy, and global developmental delay, Chiari 4 malformation, Chiari IV malformation, congenital cerebellar Hypoplasia, near total absence of cerebellum, subtotal absence of cerebellum — per MONDO
Also identified as
- DOID 0070338 per MONDO
- MESH C562568 per MONDO
- NCIT C98890 per MONDO
- OMIM 213000 per MONDO
- Orphanet 1398 per MONDO
- SCTID 16026008 per MONDO
- UMLS C5231391 per MONDO
Affected Organs
| Organ | Involvement | Source |
|---|---|---|
| Brain | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Brain | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |
| 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 |