Megalencephaly

Megalencephaly
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.[1] 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.

Contents

Symptoms

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

Epidemiology

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.

References

  1. ^ "megalencephaly" at Dorland's Medical Dictionary

External links

v · d · eCongenital malformations and deformations of nervous system (Q00–Q07, 740–742)
Brain
Other
Spinal cord
Other

M: CNS

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noco(m/d/e/h/v/s)/cong/tumr, sysi/epon, injr

proc, drug(N1A/2AB/C/3/4/7A/B/C/D)

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