SHM Converge Scientific Abstract ePoster Gallery-Unveiling Neurosarcoidosis: A Rare Case of Leptomeningitis and Central Diabetes Insipidus

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A 49-year-old woman with alcohol use disorder, hypertension, asthma, obesity, and prior lacunar stroke presented with seizure-like activity, encephalopathy, right gaze deviation, and a year of progressive cognitive, bladder, and gait decline leading to wheelchair dependence. She was initially found to have ESBL Klebsiella urosepsis, which was treated successfully, but her mental status remained impaired.<br /><br />Brain and spinal MRI showed diffuse nodular leptomeningeal enhancement involving the brain and spinal cord, with subependymal nodules. CSF studies were negative for infection and malignancy but showed elevated ACE. Chest imaging showed no hilar adenopathy or pulmonary disease, and CT chest/abdomen/pelvis did not reveal malignancy. Broad autoimmune and infectious testing was largely unrevealing, though inflammatory markers and hypergammaglobulinemia were present.<br /><br />She also had central diabetes insipidus, with very high urine output and an abnormal water deprivation test. Given the imaging pattern, CSF findings, and clinical presentation, she was treated empirically for neurosarcoidosis with high-dose glucocorticoids and IV immunoglobulin, resulting in rapid improvement in encephalopathy. A leptomeningeal biopsy did not show granulomas, likely because steroids had already been started. Repeat MRI showed resolution of most leptomeningeal enhancement, though cauda equina enhancement persisted. She was discharged on a prednisone taper, desmopressin, and infliximab.<br /><br />This case highlights a rare presentation of presumed neurosarcoidosis with diffuse leptomeningeal involvement and central diabetes insipidus. It emphasizes the need to consider inflammatory causes of leptomeningeal enhancement after infection and malignancy are excluded, and to pursue early biopsy when possible.

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Jose Medina

Meta Tag

Author List Bharath Ganesh, Crystal Benjamin, Jose Medina, Linda Mwamuka, Newton Mei

Category Clinical Vignettes

Concept Neurosarcoidosis

Concept Central Diabetes Insipidus

Concept Leptomeningeal Enhancement

Concept Cerebrospinal Fluid Studies

Concept Magnetic Resonance Imaging

Distinguished Non-Finalist

Presenter Organization Thomas Jefferson University

Presenting Author Jose Medina

Track Adult

Keywords

neurosarcoidosis

leptomeningeal enhancement

central diabetes insipidus

encephalopathy

seizure-like activity

CSF ACE elevation

MRI brain and spine

cauda equina enhancement

glucocorticoid therapy

infliximab

Neurosarcoidosis

Central Diabetes Insipidus

Leptomeningeal Enhancement

Cerebrospinal Fluid Studies

Magnetic Resonance Imaging