Case Presentation: 39 year-old African-American male presented with dizziness, worse with movement and short-term memory loss, remaining oriented, without visual changes, and no focal deficits. He complained of chronic sinusitis for the past year. CT head showed a hyperdense mass with surrounding white matter edema in the left basal ganglia. Confirmed by leptomeningeal enhancement by MRI. He was scanned to rule out malignancy, which was negative other than mediastinal lymphadenopathy Work up for infectious or autoimmune etiology was largely negative except a mildly positive ANA and elevated ACE levels. Bronchoscopy and lymph node biopsy was positive for granulomatosis changes indicating sarcoidosis. He was given high-dose corticosteroids for 6 weeks and repeat MRI showed resolution.
Discussion: First reported in 1869, sarcoidosis is an inflammatory disorder affecting multiple organ systems with histologically non-caseating granulomas (3), though etiology is unknown. Affecting all races, incidence is three times higher in African-Americans. It can present cutaneously (25%), in lymph nodes (40%), hepatic (20%), ocular (5%) and sinus complaints (1%) (3). NS presents in 5-10% of patients with sarcoidosis, isolated presentation in 0.2 per 100,000 (4,5). Cranial neuropathy is the most common presentation of NS, but may present as aseptic meningitis, hydrocephalus, peripheral neuropathy, and myopathy (6). CT of the brain is not sensitive and MRI with enhancement of the leptomeninges is seen in 40% of cases (4). It is necessary to rule out other infectious, autoimmune, and malignant etiologies. First-line treatment is high-dose corticosteroids for 4-6 weeks and currently the use of TNF-alpha, hydroxychloroquine, immunomodulators, and surgery in cases of severe organ damage are being investigated (7).
Conclusions: Vague symptomatology makes differentiating neurosarcoidosis from other neurological pathologies difficult and complex. Although rare as an initial presentation as it is seen in only a few cases, it is still imperative to be aware of this etiology given the limited treatment options and potential morbidity and mortality of the disease course. Further research into steroid-sparing therapies is needed.
To cite this abstract:Reddy, S; Mehdi, N; O'Kane, E; Kondaveeti, B. NEUROSARCOIDOSIS: THE LESS THAN 1%. Abstract published at Hospital Medicine 2019, March 24-27, National Harbor, Md. Abstract 890. https://www.shmabstracts.com/abstract/neurosarcoidosis-the-less-than-1/. Accessed January 21, 2020.