Abstract | March 24, 2024

Communicating Hydrocephalus in Neurosarcoidosis, a Case Report and Review of Current Literature

Preston M. Terle, BS, MS3, Tulane University School of Medicine, New Orleans, LA

Dylan R. Gonzales, MD, PGY3, Neurology, Tulane University School of Medicine, New Orleans, LA; Justin A. Salerian, MD, Assistant Professor, Director of Tulane Comprehensive Stroke Center, Neurology, Tulane University School of Medicine, New Orleans, LA

Learning Objectives

  1. Test for, diagnose, and treat cerebral salt wasting due to neurosarcoidosis

Introduction: Sarcoidosis is a rare granulomatous, systemic disease of unknown origin that commonly affects the lungs. Neurosarcoidosis typically affects 5% of sarcoid patients and is associated with a high mortality rate. Diagnostic criteria are based upon clinical syndrome, histopathological imaging, laboratory testing, and the exclusion of other diagnoses. Neurosarcoidosis can lead to non-communicating or communicating hydrocephalus in 5-7% of cases. Treatment varies but typically involves corticosteroids, immune-modulating/cytotoxic agents, chloroquine/hydroxychloroquine, or radiotherapy.

Case Report: A 39-year-old man with a past medical history of autism, bipolar disorder, and schizophrenia was transferred to Tulane Medical Center from an outside hospital. The patient presented with a three-month history of progressively worsening altered mental status, ataxia, fatigue, nausea, and urinary incontinence. On examination he was oriented to self; however unable to walk, hypokinetic, and significantly hyperreflexic with clonus at bilateral patella. Completed metabolic panel revealed a serum sodium level of 120mEq/L and computed tomography (CT) findings were concerning for moderate hydrocephalus and Fahr’s disease with bilateral basal ganglia, thalamic, and cerebellar calcifications. Initial lumbar puncture (LP): opening pressure of 29mmHg, protein 371mg/dL, white blood cell (WBC) 41/mm3, and glucose 23mg/dL. Magnetic resonance imaging with contrast supported moderate hydrocephalus, however, signs of sarcoid lesions, enhancements, or obstructions of the ventricular system were absent. For approximately five days the patient was treated with 3g NaCl tablets tid with continuous infusion of 250mL/hr of 0.9%, however his sodium remained near 120mEq/L.

Neurosurgery was consulted for VP shunt consideration. A large volume (26mL) LP was performed with opening pressure 16mmHg, WBC 130/mm3, and protein 300mg/dL. Additional CSF testing done at this time showed ACE>11.9, sIL-2r: 2,811 (normal<26.8), infectious work up was negative as was flow cytometry. CT chest guided lymph node biopsy was performed revealing granulomatous lymphadenitis.

Final Diagnosis: Neurosarcoidosis with Communicating Hydrocephalus

Management: With strong evidence for sarcoidosis and lack of infection or malignancy, the patient was started on solumedrol IV 1g daily for 5d. Thereafter the patient’s symptoms rapidly improved. At discharge the patient’s sodium normalized to 134mEq/L and he left the hospital on an extended steroid taper (prednisone 80mg) scheduled to decrease over 4 months.

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