Two patients are reported in whom the presence of triventricular hydrocepha
lus and aqueductal obstruction or stenosis due to multiple expanding lacuna
e in the mesencephalothalamic region possibly corresponds to abnormally dil
ated perivascular spaces. Placement of a ventriculoperitoneal cerebrospinal
fluid (CSF) shunt in one patient and the per formance of a third ventricle
cisternotomy in the other reversed the hydrocephalic syndrome, but did not
modify the complex neuroophthalmological disturbance and rubral tremor pre
sumably related to the compressive effects of the lacunae on adjacent paren
chyma. In one patient the number and size of the lacunae were increased 4 y
ears after CSF shunt placement. A review of the literature revealed two cas
es in which magnetic resonance imaging demonstrated a similar, poorly under
stood pathological condition.