Megalencephaly
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| Megalencephaly Classification and external resources | |
| ICD-10 | Q04.5 |
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| ICD-9 | 742.4 |
| OMIM | 155350 |
| DiseasesDB | 22519 |
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Overview
Megalencephaly is a type of cephalic disorder. Also called macrencephaly, this is a condition in which there is an abnormally large, heavy, and usually malfunctioning brain. By definition, 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.
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 is poor.
The prognosis for 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.
External links
Congenital malformations and deformations of nervous system (Q00-Q07, 740-742) | |
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| Brain | Anencephaly (Acephaly, Acrania, Iniencephaly) - Encephalocele - Microcephaly - Congenital hydrocephalus (Dandy-Walker syndrome) - other reduction deformities (Holoprosencephaly, Lissencephaly, Pachygyria, Hydranencephaly) - Septo-optic dysplasia - Megalencephaly - Congenital cerebral cysts (Porencephaly, Schizencephaly) - Congenital brain tumors |
| Brain stem | Arnold-Chiari malformation |
| Spinal cord | Spina bifida - Currarino syndrome - Sacrococcygeal teratoma - Diastematomyelia - Syringomyelia |
| see also non-congenital CNS and PNS (G, 320-359) | |
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
