Objective:

We present a case with multiple instances of postpartum disease activity to discuss intrapartum and postpartum disease management and considerations for this condition.

Background:

Sarcoidosis is a chronic multisystem inflammatory condition predominantly affecting females, and often more severe in African and African-American women. An even smaller subset of these patients has neurosarcoidosis, contributing to the scarcity of knowledge of neurosarcoidosis as it pertains to pregnancy. Here we present a case diagnosed postpartum with neurosarcoidosis without other systemic manifestations.

Design/Methods:

A 29-year-old Nigerian female developed fever, severe back pain, photophobia and severe meningismus five months postpartum. After multiple consultations, lumbar puncture revealed WBC 203 (0-5/mm³) with 85% lymphocytes, protein 85, elevated IgG synthesis rate. Oligoclonal bands and infectious workup were negative. Neuroimaging revealed diffuse leptomeningeal enhancement involving the bilateral internal auditory canals, left trigeminal and glossopharyngeal nerves, anterior cervicomedullary junction. Spinal cord imaging revealed enhancement within the lower thoracic cord and of the conus medullaris. Comprehensive evaluation with CT scans revealed no evidence of systemic sarcoidosis. High dose intravenous steroids were administered with suspected inflammatory disease with significant improvement. CSF Angiotensin conversion enzyme (ACE) levels were elevated (3.6). Repeat images showed resolution of enhancing lesions. She was then transitioned to hydroxychloroquine with steroid taper. Five months after neurosarcoidosis diagnosis, she had another pregnancy resulting in abortion at six weeks, stopped hydroxychloroquine with worsening of prior urinary symptoms, since subsided after reinitiation of hydroxychloroquine. She subsequently continued her hydroxychloroquine throughout her third pregnancy with considerations for postpartum empiric treatment and close radiologic monitoring.

 

 

Results:

n/a

Conclusions:

While neurosarcoidosis is considered rare, it appears it may pose significant risk in pregnant populations—particularly in the postpartum setting. Intrapartum management of neurosarcoidosis should be planned in conjunction with a multidisciplinary team determining type and time of delivery; risk of relapse postpartum needs to be accounted for¹.