[A family of hereditary communicating hydrocephalus with trigonocephaly]

We studied a family of hereditary hydrocephalus with trigonocephaly, affecting eight members (four males and four females) across two generations. A proband in this family was 51 year old man, who complained of nonprogressive gait disturbance after the age of 20 years. He was admitted to our hospital because of non-neurological disease. Accidentally urinary incontinence appeared and then CT scan was performed, which revealed marked dilatation of the lateral ventricles. He was short stature and showed trigonocephalic forehead, but his head circumference was 56 cm. Mild dementia, spastic gait and hyperreflexia were observed on neurological examinations. A lumbar puncture disclosed that cerebrospinal fluid pressure was 115 mm H2O and sugar, protein and cells were normal. His skull X-ray film demonstrated temporal buldging of the calvarium, but its breadth length and height were within normal limits. Sagittal, coronal and lomboid sutures were not fused. However, skull bones were thinned and the top of dorsum sellae was erosive. A marked symmetrical dilatation of lateral, third and fourth ventricles were found in CT scan, to the contrary, bilateral Sylvian fissures and cortical sulci were slightly widened. On R.I. cisternography, ventricular reflux was noted during 3 to 72 hours after the injection. No accumulation of R. I. was observed in the parasagital region. In the arterial phase of the right carotid angiography, slight displacement was noted in Sylvian branches of bilateral middle cerebral arteries.(ABSTRACT TRUNCATED AT 250 WORDS)