Severe X-linked mitochondrial encephalomyopathy

Orpha code: 238329OMIM code: 300816

Definition

Severe X-linked mitochondrial encephalomyopathy is an extremely rare mitochondrial respiratory chain disease resulting in a neurodegenerative disorder characterized by psychomotor delay, hypotonia, areflexia, muscle weakness and wasting in the two patients reported to date.

Disease data
Classification

Disease

Synonyms
Mitochondrial encephalomyopathy due to COXPD6
Encefalopatia mitochondrialna spowodowana COXPD6
Encefalomiopatia mitochondrialna spowodowana złożonym defektem fosforylacji oksydacyjne 6
Mitochondrial encephalomyopathy due to combined oxidative phosphorylation defect 6
ORPHA code
238329
OMIM code
300816
ICD10 code
E88.8
ICD11 code
-

No additional description.

Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl