L1 syndrome

Orpha code: 275543OMIM code: 307000

Definition

A rare, congenital X-linked developmental disorder characterized by hydrocephalus of varying degrees of severity, intellectual deficit, spasticity of the legs, and adducted thumbs. The syndrome represents a spectrum of disorders including: X-linked hydrocephalus with stenosis of the aqueduct of Sylvius (HSAS), MASA syndrome, X-linked complicated hereditary spastic paraplegia type 1, and X-linked complicated corpus callosum agenesis.

Disease data
Classification

Malformation syndrome

Synonyms
CRASH syndrome
Zespół CRASH
Zespół hipoplazja ciała modzelowatego-opóźnienie-przywiedzione kciuki-spastyczność wodogłowie
Zespół L1CAM
Corpus callosum hypoplasia-retardation-adducted thumbs-spasticity-hydrocephalus syndrome
L1CAM syndrome
ORPHA code
275543
OMIM code
307000
ICD10 code
Q04.8
ICD11 code
LD90.Y

No additional description.

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