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Hydranencephaly

Hydranencephaly is a type of Cephalic disorder. This is a rare condition in which the cerebral hemispheres are absent and replaced by sacs filled with cerebrospinal fluid. Usually the cerebellum and brainstem are formed normally. An infant with hydranencephaly may appear normal at birth. The infant's head size and spontaneous reflexes such as sucking, swallowing, crying, and moving the arms and legs may all seem normal. However, after a few weeks the infant usually becomes irritable and has increased muscle tone (hypertonia). After several months of life, seizures and hydrocephalus may develop. more...

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Other symptoms may include visual impairment, lack of growth, deafness, blindness, spastic quadriparesis (paralysis), and intellectual deficits.

Hydranencephaly is an extreme form of porencephaly, which is characterized by a cyst or cavity in the cerebral hemispheres, and may be caused by vascular insult or injuries, infections, or traumatic disorders after the 12th week of pregnancy.

Diagnosis may be delayed for several months because the infant's early behavior appears to be relatively normal. Transillumination, an examination in which light is passed through body tissues, usually confirms the diagnosis. Some infants may have additional abnormalities at birth including seizures, myoclonus (involuntary sudden, rapid jerks), and respiratory problems.

There is no standard treatment for hydranencephaly. Treatment is symptomatic and supportive. Hydrocephalus may be treated with a shunt.

The outlook for children with hydranencephaly is poor. Death generally occurs before age 1.

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The Official Parent's Sourcebook on Hydranencephaly: A Revised and Updated Directory for the Internet Age $28.95 Living a normal life with the nondominant hemisphere: magnetic resonance imaging findings and clinical outcome for a patient with left-hemispheric hydranencephaly. : An article from: Pediatrics $5.95
Characterization of the transfer ribonucleic acids isolated from individuals with Down's syndrome, Hunter's syndrome and hydranencephaly (Kent State University. ... theses : Department of Biological Sciences) Akabane disease: congenital arthrogryposis and hydranencephaly syndrome in cattle =: Showa 47 Nen Irai Honkyo ni Taihatsu Shita Ushi no Ijosan - Ijosan no biyogen
Outbreaks of abortion-arthrogryposis-h ydranencephaly syndrome in cattle in Japan, 1972/73: VII. Clinical findings of defective calves collected for two years after the end of the prevalence

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