Degenerative disorder	Developmental hereditary disorder	Hereditary disorder of nervous system	Intellectual disability	X-linked recessive hereditary disease
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X-linked neurodegenerative syndrome Hamel type (disorder)
	X-linked neurodegenerative syndrome Hamel type
	This syndrome is an X-linked neurodegenerative disorder with characteristics of intellectual deficit, blindness, convulsions, spasticity, mild hypomyelination and early death. It has been described in about ten male members from two generations of one family. The genetic defect responsible for the disorder is located in the pericentromeric region of the X chromosome, Xp11.3-q12.
	X-gebonden neurodegeneratief syndroom Hamel-type

Id	718847005
Status	Primitive

Associated morphology	Degenerative abnormality
Finding site	Structure of nervous system

Pathological process

Pathological developmental process

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	G31.8
Rule	TRUE
Advice	ALWAYS G31.8 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified