Neurosarcoidois (NS) is an uncommon neuroinflammatory diseaseObjective
Describe the spectrum and frequency of NS in a tertiary neurology centreMethod
Medical record search for ‘sarcoidosis’/‘neurosarcoidosis’ from 1995–2016 followed by case note review.Results
73 patients had sarcoidosis, 34 (46%) had NS. 24 were male (M:F 2.4:1).The most frequently occurring presentations were weakness 41% (n=14), headaches 35% (n=12) and the most commonly affected structures were cranial nerves 32% (n=11) and meninges 17% (n=6). Serum ACE was abnormal in 38% (6/16), CSF ACE was abnormal in 60% (9/15). CSF protein was abnormal in 82% (14/17). Histology was available in 62% (n=21/34) of NS patients, only 6 involved neural tissue. Where treatment details were available (n=30) all patients received corticosteroids and 72% (n=21) required an additional immunosuppressant. The most commonly used second line immunosuppressant was methotrexate (n=10, 33%). Others included infliximab (n=7, 23%), azathioprine (n=5,16%), mycophenolate (n=2,10%), cyclophosphamide (n=2,6%) and rituximab (n=1,3%). At last follow up, 11 patients (32%) had stopped treatment as disease seemed to remit and 2 patients had died.Conclusion
Neurosarcoidosis is a rare disease being diagnosed at 1–2/year in a tertiary centre. Majority of patients need steroid sparing treatments but many do remit over time.