Infantile-onset axonal motor and sensory neuropathy-optic atrophy-neurodegenerative syndrome

Orpha code: 457205OMIM code:

Definition

A rare neurologic disease characterized by axonal sensorimotor neuropathy, progressive optic atrophy, cognitive deficit, bulbar dysfunction, seizures, and early hypotonia and feeding difficulties. Additional possible features include dystonia, scoliosis, joint contractures, ocular anomalies, and urogenital anomalies. Brain MRI reveals variable degrees of cerebral atrophy. The disease is fatal in childhood due to respiratory failure.

Disease data
Classification

Disease

Synonyms
ANOAC
ANOAC
Zespół neuropatii aksonalnej, zaniku nerwu wzrokowego i deficytu poznawczego
Axonal neuropathy-optic atrophy-cognitive deficit syndrome
ORPHA code
457205
OMIM code
-
ICD10 code
G60.8
ICD11 code
-

No additional description.

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