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Six cases of hydroencephalodysplasia were evaluated from the points of clinical signs and symptoms and neuroradiological procedures and diagnostic problems were discussed. All of our cases were characterized by extensive dysplasia of the brain and increased cerebrospinal fluid pressure. Deformity of the head and developmental arrest of the psychomotor function were observed in five cases. The side of the skull deformity was consisted with that of the brain defect. Pneumoencephalography and cerebral angiography are characteristic in this disease, but these procedures have to be carried out with caution in case of poor general condition. Transillumination and brain scan are also useful procedures and RI-cisternography is important for indication of shunting operation. Early rehabilitation for the motor dys function is main therapeutic trial, though the cases with increased intracranial pressure require cerebrospinal fluid shunt.
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M Tsuru
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