Abstract Details

Title A travelling salesman’s long journey toward a diagnosis of neurosarcoidosis

Topic Multiple Sclerosis

Presentation(s) P1 - Poster Session 1 (5:30 PM-6:30 PM)

Poster/Presentation
Number 15-066

Objective To report an atypical case of neurosarcoidosis that was diagnosed and treated as blastomycosis.

Background Neurosarcoidosis is a rare condition that occurs in 5-10% of patients with sarcoidosis, affecting any part of the nervous system resulting in variable presentations. Clinical overlap with neoplastic, infectious and inflammatory conditions, makes diagnosis challenging. Blastomycosis is a pyogranulomatous infection primary involving lungs which rarely may affect the central nervous system.

Design/Methods Case Report

Results A 48-year-old African American salesman who travels for work, developed 6 months of fatigue, weight loss, urinary symptoms, hoarseness, intermittent vertigo, and recurrent left ptosis, followed by 3 days of neck pain with headache, confusion, gait imbalance, and bowel incontinence. MRI showed extensive, heavy leptomeningeal enhancement. CSF revealed elevated protein (845), hypoglycorrhacia (<20), 97 cells, with 93% lymphocytes. Cytology showed atypical inflammatory cells with lymphocyte predominance. Labs showed lymphopenia and anemia without leukopenia with normal ACE level. CT chest revealed bilateral hilar lymphadenopathy. Neurological exam showed positive Romberg and dysmetria. FNA of hilar lymph nodes was non-diagnostic. Craniotomy with meningeal biopsy showed no granulomas. Hilar lymph node biopsy revealed extensive necrotizing granulomas, with occasional broad budding yeast. Given travel history, positive lymph node biopsy and otherwise non-diagnostic work-up, diagnosis of disseminated blastomycosis was given leading to one year of anti-fungal therapy. MRI continued to show impressive leptomeningeal disease with no clinical improvement. NCS revealed distal symmetric axonal length-dependent polyneuropathy. The lymph node biopsy was reviewed by Mayo Clinic, and consistent with neurosarcoidosis. Patient was started on high dose steroids and methotrexate, subsequently switched to Humira. He improved significantly and resumed his full-time work duties.

Conclusions

Diagnosis of atypical neurosarcoidosis can be challenging due to overlap of pulmonary conditions with other fungal infections. Hamazaki-Wesenburg bodies can be found in sarcoidosis and may be mistaken for budding yeast. For rare diagnoses multiple reviewers improve diagnostic certainty.

Authors/Disclosures
Sahar H. Osman, MD (PPMG) PRESENTER	Dr. Osman has nothing to disclose.
Xiao-Tang Kong, MD, PhD (UC Irvine)	Dr. Kong has nothing to disclose.

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