Definition:
Schizencephaly is a rare cephalic disease characterized by anomalous clefts (or slits) in the cerebral hemispheres.
Diagnosis:
An ultrasound can reveal the occurrence of clefts in a fetus' brain. If cerebrospinal clefts are identified, the infant will be born with Schizencephaly.
Treatment:
Treatment for patients with Schizencephaly is generally supportive. Physical therapy may be integrated along with appropriate medications to treat seizures and other symptoms.
Symptoms and Signs:
A characteristic sign of Schizencephaly is the appearance of a cerebrospinal fluid–filled cleft, which is lined by gray matter and extends throughout the cerebral hemisphere, starting from the ependyma (ventricular surface) to the brain's periphery (pial surface).
Clinical symptoms vary, but typical presentations include hemiparesis, seizures, and developmental deficits. The severity of symptoms also varies according to the extent of the brain affected with the abnormality.
The clefts may occur unilaterally or bilaterally. If occurring in both hemispheres (bilateral), Schizencephaly leads to delayed language comprehension, developmental impairment, and corticospinal defects. If affecting only one hemisphere (unilateral), the disease causes a weakness on only one side of the body, with affected patients having average or near-average intelligence.
In general, individuals with Schizencephaly suffer from varying severities of mental retardation, microcephaly, hemiparesis/quadriparesis, and reduced muscle tone.
Causes:
Schizencephaly has no known cause to date, although several theories about its etiology have been proposed. A popular hypothesis points to a link between Schizencephaly and an early, focal damage to the germinal matrix and surrounding brain at a time when the hemispheres are not yet fully developed. In other words, the brain may have suffered from severe impact or trauma at a critical time and in a critical location during normal brain development.
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