Sylvian aqueduct syndrome with slit ventricles in shunted hydrocephalus due to adult aqueduct stenosis.

[hydrocephalus with stenosis of the aqueduct of sylvius]

The authors report on 3 patients who developed sylvian aqueduct syndrome (SAS ) in the context of shunt dysfunction and slit ventricles . All 3 patients had received shunts for adult onset hydrocephalus due to aqueduct stenosis and were stable for years before presenting with loss of upward gaze , convergence-retraction nystagmus , and slit ventricles , all due to shunt overdrainage . All 3 improved after either shunt revision or a third ventriculostomy procedure . Although it is well known that SAS can be caused by shunt blockage producing a transtentorial pressure gradient , these cases emphasize that an identical clinical pattern can occur with a reverse transtentorial pressure gradient and slit ventricles due to shunt overdrainage . The authors propose a simple management plan for patients with shunted hydrocephalus who develop SAS .

Diseases
Validation

Diseases presenting "nystagmus" symptom

aniridia

gm1 gangliosidosis

homocystinuria without methylmalonic aciduria

hydrocephalus with stenosis of the aqueduct of sylvius

kabuki syndrome

oculocutaneous albinism

primary hyperoxaluria type 1

well-differentiated liposarcoma

wolf-hirschhorn syndrome

zellweger syndrome

This symptom has already been validated