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Megalencephaly

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Megalencephaly
Classification and external resources
ICD-10 Q045
ICD-9 742.4
OMIM 155350
DiseasesDB 22519

Megalencephaly (or macrencephaly) is a condition in which the brain is abnormally large.[1] It usually functions abnormally, as well. Head enlargement may be evident at birth or the head may become abnormally large in the early years of life.

SymptomsEdit

Symptoms of megalencephaly may include delayed development, convulsive disorders, corticospinal (brain cortex and spinal cord) dysfunction, and seizures.

EpidemiologyEdit

Megalencephaly affects males more often than females.

Prognosis and treatmentEdit

The prognosis for individuals with megalencephaly largely depends on the underlying cause and the associated neurological disorders. Treatment is symptomatic. Megalencephaly may lead to a condition called macrocephaly. Unilateral megalencephaly or hemimegalencephaly is a rare condition characterized by the enlargement of one-half of the brain. Children with this disorder may have a large, sometimes asymmetrical head. Often they suffer from intractable seizures and mental retardation. The prognosis for those with hemimegalencephaly ranges from poor to highly functioning. In some cases, children with hemimegalencephaly are severely mentally retarded and must undergo a hemispherectomy to stop seizure activity. In others, seizure activity can be controlled by medication; and the affected child develops mentally and physically trailing normal development by one to two years.

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