Abstract Details

Title Acute Hydrocephalus as a Complication of Neurosarcoidosis: A Case Report

Topic Autoimmune Neurology

Presentation(s) P1 - Poster Session 1 (12:00 PM-1:00 PM)

Poster/Presentation
Number 057

Objective N/A

Background Sarcoidosis is a multisystem immune-mediated disorder, pathologically characterized by non- or minimally-caseating granulomas in involved tissues. It can affect several organs including the skin, eyes, heart, kidneys, and rarely the central nervous system. Neurosarcoidosis (NS) often presents with symptoms such as headaches, seizures, and cranial nerve deficits. Hydrocephalus is a severe complication of neurosarcoidosis and is reported in approximately one-tenth of neurosarcoidosis cases.

Design/Methods A 27-year-old female with a history of neurosarcoidosis presented to the emergency department with lightheadedness, intractable headaches, and vomiting for 2 weeks. She had been off immunotherapy for neurosarcoidosis for 3 years and was currently on prednisone monotherapy. Neurological examination revealed diffuse hyperreflexia as the only notable finding. Initial serum studies were unremarkable, including angiotensin-converting enzyme of 20 U/L. Brain MRI showed new mild to moderate ventriculomegaly in the lateral, third, and fourth ventricles consistent with acute hydrocephalus, and irregular/nodular basilar leptomeningeal enhancement and thickening consistent with neurosarcoidosis. She was treated with 3 days of 1000 mg parenteral methylprednisolone followed by 60 mg prednisone daily with clinical improvement. Per Rheumatology, she began 1000 mg parenteral cyclophosphamide, every 4 weeks. The Neurosurgery team recommended ventriculoperitoneal shunt placement outpatient.

Results N/A

Conclusions This case demonstrates that acute hydrocephalus is a feared complication of neurosarcoidosis. We emphasize the early necessity of obtaining brain imaging in patients with neurosarcoidosis (NS) who exhibit symptoms indicative of elevated intracranial pressure. Timely identification of hydrocephalus is paramount, given its associated mortality rate of 22%.

Authors/Disclosures
Emily Guagliardo, MD PRESENTER	Emily Guagliardo has nothing to disclose.
Justin Anderson, Student	No disclosure on file
Divya Singh, MD (Saint Louis University)	Dr. Singh has nothing to disclose.
Wilson E. Rodriguez, MD	Dr. Rodriguez has nothing to disclose.
Pratap R. Chand, MD, FRCP, FAAN (St Louis University School of Medicine)	Dr. Chand has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Boston Scientific. An immediate family member of Dr. Chand has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Acorda. Dr. Chand has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Teva Neuroscience.
Lissette Orozco (Trinity Health Ann Arbor Hospital)	No disclosure on file

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