Porencephaly

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Porencephaly
ICD-10 Q04.6
ICD-9 742.4
OMIM 175780
DiseasesDB 33031

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Overview

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 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, 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

Cost Effectiveness of Porencephaly

| group5 = Clinical Trials Involving Porencephaly | list5 = Ongoing Trials on Porencephaly at Clinical Trials.govTrial results on PorencephalyClinical Trials on Porencephaly at Google


| group6 = Guidelines / Policies / Government Resources (FDA/CDC) Regarding Porencephaly | list6 = US National Guidelines Clearinghouse on PorencephalyNICE Guidance on PorencephalyNHS PRODIGY GuidanceFDA on PorencephalyCDC on Porencephaly


| group7 = Textbook Information on Porencephaly | list7 = Books and Textbook Information on Porencephaly


| group8 = Pharmacology Resources on Porencephaly | list8 = AND (Dose)}} Dosing of PorencephalyAND (drug interactions)}} Drug interactions with PorencephalyAND (side effects)}} Side effects of PorencephalyAND (Allergy)}} Allergic reactions to PorencephalyAND (overdose)}} Overdose information on PorencephalyAND (carcinogenicity)}} Carcinogenicity information on PorencephalyAND (pregnancy)}} Porencephaly in pregnancyAND (pharmacokinetics)}} Pharmacokinetics of Porencephaly


| group9 = Genetics, Pharmacogenomics, and Proteinomics of Porencephaly | list9 = AND (pharmacogenomics)}} Genetics of PorencephalyAND (pharmacogenomics)}} Pharmacogenomics of PorencephalyAND (proteomics)}} Proteomics of Porencephaly


| group10 = Newstories on Porencephaly | list10 = Porencephaly in the newsBe alerted to news on PorencephalyNews trends on Porencephaly


| group11 = Commentary on Porencephaly | list11 = Blogs on Porencephaly

| group12 = Patient Resources on Porencephaly | list12 = Patient resources on PorencephalyDiscussion groups on PorencephalyPatient Handouts on PorencephalyDirections to Hospitals Treating PorencephalyRisk calculators and risk factors for Porencephaly


| group13 = Healthcare Provider Resources on Porencephaly | list13 = Symptoms of PorencephalyCauses & Risk Factors for PorencephalyDiagnostic studies for PorencephalyTreatment of Porencephaly

| group14 = Continuing Medical Education (CME) Programs on Porencephaly | list14 = CME Programs on Porencephaly

| group15 = International Resources on Porencephaly | list15 = Porencephaly en EspanolPorencephaly en Francais

| group16 = Business Resources on Porencephaly | list16 = Porencephaly in the MarketplacePatents on Porencephaly

| group17 = Informatics Resources on Porencephaly | list17 = List of terms related to Porencephaly


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