Case Presentation: A 36 year old African-American gentleman with a history of asthma presented to the Emergency Department with dizziness, numbness of both lower extremities, gait imbalance, bowel and bladder incontinence which had been worsening since the last 2 months. MRI thoracic spine revealed Focus of abnormal T2 signal within the upper thoracic spinal cord extending from the mid T3 level inferiorly through the T5-6 level, with associated cord expansion and MRI brain revealed leptomeningeal enhancement in the posterior fossa. These findings were consistent with transverse myelitis and prompted a diagnostic work up to identify a specific cause. Cerebrospinal fluid(CSF) revealed elevated protein, low glucose, lymphocytic pleocytosis and elevated IgG level. Angiotensin converting enzyme level in the CSF was also elevated and autoimmune work up revealed negative ANA but elevated anti SSA titer. Given the laboratory studies, a provisional diagnosis of neurosarcoidosis versus extra-glandular Sjogren’s syndrome was made. Patient however denied any sicca symptoms and CT chest did not reveal hilar adenopathy to support the diagnosis of neurosarcoid. He was referred to a neuro-immunologist who recommended meningeal biopsy. The meningeal biopsy demonstrated non caseating granulomas and the diagnosis of neurosarcoidosis was confirmed. Patient was managed with intravenous methylprednisolone in the hospital, which lead to improvement in his symptoms. He was discharged on a long course of high dose oral prednisone to acute rehabilitation. Unfortunately, his disease continued to progress and he was started on rituximab and later, a trial of infliximab.

Discussion: HistopathologicalSarcoidosis is a multisystem disease caused by granulomatous inflammation of involved organs, most commonly presenting as bilateral hilar adenopathy. Neurological sarcoidosis can occur in approximately 5% of patients with sarcoidosis[1], although it is rarely the presenting complaint. Sarcoidosis can involve any part of the central nervous system and can present as cranial neuropathy, myelopathy/radiculopathy, encephalopathy, acute or chronic aseptic meningitis, seizures and peripheral neuropathy. The diagnosis is often missed unless it is strongly suspected. Histopathological demonstration of non caseating granulomas as in our case is the gold standard of diagnosis, although MRI findings, increased ACE levels may be supporting features. Extraglandular Sjogren’s syndrome, the second differential in this case given the positive SSA antibody can present very similarly to neurosarcoid. Our patient has not yet undergone a lip biopsy to demonstrate Sjogren’s syndrome but it is less likely since neurosarcoidosis has been confirmed. There have been a handful of case reports on an overlap syndrome between sarcoidosis and Sjogren’s syndrome and our case could be added to that number [2}

Conclusions: Our case highlights the difficulty of correctly diagnosing sarcoidosis especially when the disease is restricted to the central nervous system and the importance of always considering sarcoidosis in the differential diagnosis.