Acute hepatic failure	Autosomal recessive hereditary disorder	Digestive system hereditary disorder	Hereditary peripheral neuropathy	Spinocerebellar ataxia
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Acute infantile liver failure, cerebellar ataxia, peripheral sensory motor neuropathy syndrome (disorder)
	Acute infantile liver failure, cerebellar ataxia, peripheral sensory motor neuropathy syndrome
	Autosomal recessive spinocerebellar ataxia type 21
	A rare autosomal recessive axonal hereditary motor and sensory neuropathy with characteristics of infantile onset of recurrent episodes of acute liver failure (resulting in chronic liver fibrosis and hepatosplenomegaly), delayed motor development, cerebellar dysfunction presenting as gait disturbances and intention tremor, neurogenic stuttering and motor and sensory neuropathy with muscle weakness especially in the lower legs, and numbness. Mild intellectual disability was reported in some patients. MRI of the brain shows non-progressive atrophy of the cerebellar vermis and thinning of the optic nerve.
	syndroom van acuut infantiel leverfalen, cerebellaire ataxie en perifere sensorische en motorische neuropathie
	SCAR21 autosomaal recessieve spinocerebellaire ataxie type 21

Id	1187643003
Status	Primitive

Finding site

Peripheral nervous system structure

Finding site	Structure of parenchyma of liver
Occurrence	Infancy

Associated morphology	Degenerative abnormality
Finding site	Cerebellar structure

Associated morphology	Degenerative abnormality
Finding site	Spinal cord structure

ICD-10 complex map reference set

Target	G11.9
Rule	TRUE
Advice	ALWAYS G11.9 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified