Megalencephaly Classification and external resources ICD-10 Q04.5 ICD-9 742.4 OMIM 155350 DiseasesDB 22519
Megalencephaly (or macrencephaly) is a condition in which there is an abnormally large brain. It is usually functioning abnormally. Megalencephaly is, by definition, when the brain weight is greater than average for the age and sex of the infant or child. Head enlargement may be evident at birth or the head may become abnormally large in the early years of life.
Symptoms of megalencephaly may include delayed development, convulsive disorders, corticospinal (brain cortex and spinal cord) dysfunction, and seizures.
Megalencephaly affects males more often than females.
Prognosis and treatment
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 1–2 years.
- ^ "megalencephaly" at Dorland's Medical Dictionary
- megalencephaly at NINDS
- Cleveland Clinic
Congenital malformations and deformations of nervous system (Q00–Q07, 740–742) BrainAnencephaly (Acephaly, Acrania, Acalvaria, Iniencephaly) · Encephalocele · Arnold–Chiari malformationOtherother reduction deformities (Holoprosencephaly, Lissencephaly, Pachygyria, Hydranencephaly)Septo-optic dysplasia · MegalencephalyCNS cyst (Porencephaly, Schizencephaly) Spinal cordOtherCategories:
- Congenital disorders of nervous system
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