[MIM 306 960]

Acronym for Hereditary Hemihypotrophy Hemiparesis Hemiathetosis

Extremely rare. X-inked recessive transmission of a mutation located on Xp21.1-q21.31. The expression is variable in heterozygous females. Association of unilateral hemiplegia resulting in a stunting of the affected side and athetoid movements of the hand. There are sometimes convulsions.

Some consider that this condition is very close to infantile hemiplegia with porencephaly.

Anesthetic implications: 

hemiplegia, convulsions. Monitoring of muscle relaxation should best be done on the healthy side..

References : 

Updated: March 2019