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Case Report

Isolated neurosarcoidosis presenting with

recurrent hydrocephalus

1
1
3
Frederick L. Hitti , Benjamin C. Kennedy , Yazmin Odia , Claire S. Riley , Sameer A. Sheth 1
2
1 Department of Neurological Surgery, Neurological Institute, Columbia University Medical Center, New York, NY 10032, USA.
2 Department of Neuro‑Oncology, Neurologic Institute of New York, Columbia University College of Physicians and Surgeons,
New York, NY 10032, USA.
3 Department of Neurology, Neurologic Institute of New York, Columbia University College of Physicians and Surgeons, New York,
NY 10032, USA.
ABSTRA CT

Sarcoidosis is an inflammatory process that is characterized by the formation of noncaseating granulomas. This protean disease
may afflict nearly any organ system, including the central nervous system. Here, we present a case of isolated neurosarcoidosis that
initially presented with hydrocephalus requiring ventriculoperitoneal shunt placement. The patient’s hydrocephalus recurred multiple
times and required two additional shunt placements over the 3‑year course of her illness. Due to the lack of systemic involvement,
sarcoidosis was only diagnosed after a tissue biopsy of a Cauda equine lesion. This case highlights the importance of tissue diagnosis
and the diagnostic workup for sarcoidosis in cases of cryptogenic hydrocephalus.

Key words: Biopsy, hydrocephalus, inflammatory, neurosarcoidosis

INTRODUCTION of isolated neurosarcoidosis among neurosarcoid
patients varies from series to series with older studies
Sarcoidosis is an inflammatory disease that may reporting 1‑3% and more recent studies reporting
present in a multitude of forms due to its ability to 11‑17%. [4,11] Here, we report a case of a patient
involve virtually any organ in the body. Involvement with isolated neurosarcoidosis that presented with
of the central nervous system (CNS) occurs in 5‑25% of hydrocephalus ultimately requiring placement of
sarcoidosis patients. [1‑3] The most common symptoms three ventriculoperitoneal shunts and eventually
associated with neurosarcoidosis are cranial nerve diagnosed by biopsy of Cauda equina lesions.
palsies, paresthesia, headache, weakness, ataxia,
aseptic meningitis, peripheral neuropathy, and CASE REPORT
cognitive impairment [Table 1]. Hydrocephalus is
a much rarer complication of neurosarcoidosis that A 38‑year‑old African‑American woman presented
was not found in one series of 91 cases and only to an outside hospital with a 2‑year history of
reported in a few case reports. [4,7‑10] Neurosarcoidosis headaches, a 5‑month history of difficulty walking,
patients commonly have evidence of the involvement dizziness refractory to meclizine, and a 3‑day history
of the thoracopulmonary system. Isolated of blurry vision. A neurological exam demonstrated
neurosarcoidosis (sarcoidosis limited solely to the nystagmus on upgaze, left gaze, and right gaze.
nervous system) is relatively rare. The prevalence She also had an ataxic gait and papilledema. Head
computed tomography (CT), and magnetic resonance
Corresponding Author: Dr. Frederick L. Hitti, imaging (MRI) revealed communicating hydrocephalus.
Department of Neurological Surgery, Neurological Institute, Analysis of cerebrospinal fluid (CSF) obtained by
Columbia University Medical Center, 4th Floor, 710, lumbar puncture (LP) demonstrated cell count, protein,
West 168th Street, New York, NY 10032, USA.
E‑mail: fhitti@gmail.com
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Cite this article as: Hitti FL, Kennedy BC, Odia Y, Riley CS, Sheth SA. Isolated
DOI: neurosarcoidosis presenting with recurrent hydrocephalus. Neuroimmunol
10.4103/2347-8659.167307 Neuroinflammation 2015;2:287-90.
Received: 15-04-2015; Accepted: 06-08-2015

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