HHHH stands for hereditary
hemihypotrophy, hemiparesis, and hemiathetosis. Characterized by congenital
unilateral (left or right) hemiparesis with subsequent development of
hemihypoplasia and athetoid posturing of the left hand. Other clinical
features include hemiatrophy, involuntary movements, and seizures. It has
been suggested that either an autosomal dominant or X-linked inheritance
Very rare disease. X-linked or
autosomal dominant (variable expression in heterozygous females); autosomal
recessive inheritance also has been evocated.
Congenital unilateral (left or right) progressive
hemiparesis followed by hemihypotrophy with particular athetoid posturing of
the hand. Seizures are frequent.
Evaluate neurologic function (clinical,
history, CT, EEG). Careful intraoperative positioning is needed. Consider
interaction between antiepileptic treatment and anesthetic drugs.
Haar F, Dyken P: Hereditary nonprogressive athetotic hemiplegia: A new