Cerebellar hemorrhage complicating methylmalonic and propionic acidemia
P. Dave, R. G. Curless and L. Steinman
Methylmalonic (MMA) and propionic (PPA) acidemias are inherited disorders
characterized by a wide spectrum of clinical and biochemical findings. We
report the neuropathologic findings in two young infants with MMA and PPA
in which death probably resulted from cerebellar hemorrhage. When combined
with a previous report of isovaleric acidemia complicated by diffuse
subarachnoid and parenchymal cerebellar hemorrhage, it is apparent that
cerebellar hemorrhage must be considered in any infant with a fulminating
organic acidemia. Rapid neurologic deterioration despite an improvement in
acidosis and hyperammonemia is an indication for computed tomography of
brain. Surgical evacuation of the blood may improve the chance for survival
in this catastrophic illness.
