Abstract
A 10-month-old boy with Alexander disease is described. He was clinically characterized
by early onset of megalencephaly, psychomotor retardation, spasticity and recurrent
seizure attacks. Pathological examination of the biopsied cerebrum revealed a large
number of Rosenthal fibers in the white matter and beneath the pia in association
with advanced demyelination.
The motor nerve conduction velocity was remarkably delayed, suggesting a possible
change of the peripheral nerve. The computerized tomography demonstrated megalencephaly
and bilateral symmetrical low density areas in the cerebral white matter, particularly
in the frontal region. The preferential involvement of frontal lobes was also confirmed
by electroencephalogram and brain scan. The preferential damage of the frontal region
in the early stage of the disease may be an important clue to a diagnosis of Alexander
disease by computerized tomography, brain scan and electroencephalogram.
Key words
Alexander disease - motor nerve conduction velocity (MCV) - computerized tomography
(CT)
