Karakis I, Nuccio A, Amadio J, Fountain A

BACKGROUND Posterior reversible leukoencephalopathy syndrome (PRES) is linked to various etiologies, foremost systemic hypertension. Its association with intracranial hypotension (IH), a potential sequela of various neurosurgical procedures, is under-recognized. We report a case of lumbo-peritoneal shunt induced IH resulting in PRES with the goal to increase awareness, and elaborate on the potential biological mechanism, based on the Monro-Kellie hypothesis.

CASE DESCRIPTION A 26-year-old woman with acquired immunodeficiency syndrome and epilepsy was admitted for recurrent cryptococcal meningitis and breakthrough seizures. There was radiologic evidence of ventricular enlargement and opening pressure on serial lumbar punctures was constantly elevated. Due to persistently elevated, symptomatic intracranial pressure, and transient relief with serial spinal taps, a lumbo-peritoneal shunt was placed. Subsequently, the patient had a breakthrough seizure and became encephalopathic. Repeat head imaging showed reduced ventricular size, engorged venous sinuses and tonsillar herniation in keeping with IH, coupled with extensive white matter abnormalities in bilateral parieto-occipital lobes indicative of PRES. The patient had an emergent programmable valve placed in the lumbo-peritoneal shunt to prevent excessive cerebrospinal fluid (CSF) drainage, leading in clinical and radiological improvement. Subsequent CSF leak resulted in recurrent presentation.

CONCLUSIONS IH appears to be a distinct cause of PRES, previously not reported in the neurosurgical literature. It occurs in susceptible patients, on average 1-5 days after the IH trigger, and seems clinically and radiologically similar to the more common hypertensive cases, both in terms of initial presentation and prognosis. Increased vigilance is required to allow for prompt recognition and management.

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