Neurosarcoidosis is a rare, chronic neuroinflammatory condition. It develops in approximately 5-15 percent of patients with systemic sarcoidosis. The origin of sarcoidosis is not fully understood but it probably stems from an exaggerated immune response (granuloma formation) against an unknown antigen. The disease may affect any part of the nervous system: meninges, brain, spinal cord, peripheral nerves; this explains why symptoms vary greatly from person to person and even from flare to flare. Left untreated, the disease has the potential to cause cumulative nervous damage and severe disability.
Common Symptoms
- Dor de cabeça
- Cognitive impairment
- Vision changes
- Facial palsy
- Vertigo, gait imbalance
- Hearing loss, tinnitus
- Weakness or numbness in face or extremities
- Difficulty ambulating
- Abnormal bladder and bowel function
Diagnóstico
- MRI of brain, orbits, and spine with IV contrast
- MR angiography / vessel wall imaging
- Punção lombar para análise do líquido espinhal
- Nerve conduction studies / electromyogram
- Skin biopsy for diagnosis of small fiber neuropathy
- Autonomic testing (done externally)
- Systemic imaging: body PET, chest tomography, cardiac echo/MRI, etc.
- Occasionally direct tissue exam (via surgical biopsy) may be necessary to confirm diagnosis
Tratamento
Neurosarcoidosis is a treatable condition. Given that the disease is caused by excessive immune activation, therapy is directed towards weakening the immune system. During acute flares, treatment is fundamentally high-dose intravenous steroids which is sometimes followed by an oral steroid taper. For maintenance and prevention of new flares, the usual medications used in systemic sarcoidosis (methotrexate, infliximab, adalimumab) are also effective in neurosarcoidosis. Some patients need little intervention to stabilize while others need aggressive immunosuppression; our goal is to achieve complete remission in every single one. Potential side effects of medications are closely monitored by our physicians.