Porencephaly
232052
192323144
2008-02-18T16:09:08Z
VELVET
2397965
ICD9 ADDITION
{{DiseaseDisorder infobox |
Name = {{PAGENAME}} |
ICD10 = {{ICD10|Q|04|6|q|00}} |
ICD9 = {{ICD9|742.4 348.0}} |
ICDO = |
Image = |
Caption = |
OMIM = 175780 |
OMIM_mult = |
MedlinePlus = |
eMedicineSubj = |
eMedicineTopic = |
DiseasesDB = 33031 |
}}
'''Porencephaly''' is a type of [[cephalic disorder]]. This is an extremely rare disorder of the [[central nervous system]] involving a [[cyst]] or cavity in a [[cerebral hemisphere]]. The cysts or cavities are usually the remnants of destructive lesions, but are sometimes the result of abnormal development. The disorder can occur before or after birth.
==Causes==
Porencephaly most likely has a number of different, often unknown causes, including absence of brain development and destruction of brain tissue.
==Diagnosis==
The presence of porencephalic cysts can sometimes be detected by [[transillumination]] of the skull in infancy. The diagnosis may be confirmed by [[computed tomography|CT]], [[MRI]], or [[ultrasonography]].
==Presentation==
More severely affected infants show symptoms of the disorder shortly after birth, and the diagnosis is usually made before age 1. Signs may include delayed growth and development, [[Spasticity|spastic]] [[paresis]] (slight or incomplete paralysis), [[hypotonia]] (decreased muscle tone), seizures (often infantile spasms), and [[macrocephaly]] or [[microcephaly]].
Individuals with porencephaly may have poor or absent speech development, [[epilepsy]], [[hydrocephalus]], spastic contractures (shrinkage or shortening of muscles), and [[mental retardation]].
==Treatment and prognosis==
Treatment may include physical therapy, medication for seizure disorders, and a shunt for hydrocephalus. The prognosis for individuals with porencephaly varies according to the location and extent of the lesion. Some patients with this disorder may develop only minor neurological problems and have normal intelligence, while others may be severely disabled. Others may die before the second decade of life.
==External links==
* {{NINDS|Porencephaly}}
{{Congenital malformations and deformations of nervous system}}
[[Category:Congenital disorders]]
[[es:Porencefalia]]