K O'Connell1,2, L Williams3,4, J Jones3,4, D J H McCabe5,6,7,8,9, D Murphy4,10, R Killeen4,10, N Tubridy3,4, S O'Riordan3,4, C McGuigan3,4. 1. Department of Neurology, St Vincent's University Hospital, Elm Park, Dublin 4, Ireland. k.oconnell@svuh.ie. 2. School of Medicine, University College Dublin, Dublin, Ireland. k.oconnell@svuh.ie. 3. Department of Neurology, St Vincent's University Hospital, Elm Park, Dublin 4, Ireland. 4. School of Medicine, University College Dublin, Dublin, Ireland. 5. Department of Neurology, The Adelaide and Meath Hospital, Dublin, Incorporating the National Children's Hospital, Dublin, Ireland. 6. Stroke Service, The Adelaide and Meath Hospital, Dublin, Incorporating the National Children's Hospital, Dublin, Ireland. 7. Vascular Neurology Research Foundation, The Adelaide and Meath Hospital, Dublin, Incorporating the National Children's Hospital, Dublin, Ireland. 8. Department of Clinical Neurosciences, Royal Free Campus, UCL Institute of Neurology, London, UK. 9. Academic Unit of Neurology, School of Medicine, Trinity College Dublin, Dublin, Ireland. 10. Department of Radiology, St Vincent's University Hospital, Dublin, Ireland.
Abstract
BACKGROUND: The clinical manifestations of neurosarcoidosis are highly variable and it should be considered as a potential differential diagnosis in any neurological presentation. AIM: This study was designed to describe the clinical, diagnostic, and treatment patterns and functional outcome in a Caucasian neurosarcoidosis population. DESIGN: A retrospective analysis was performed on prospectively recorded data in patients attending our neurology clinic between 2008 and 2014 with a diagnosis of definite or probable neurosarcoidosis according to Zajiek criteria. METHODS: Detailed clinical features, baseline demographic data, results of investigations, treatment type and duration, and clinical outcomes were collated. RESULTS: Eleven patients were identified (55% men) with mean age 39 years (range 21-63). Four had a prior history of systemic sarcoidosis leading to earlier diagnosis (6.7 vs 13.1 months). Six were found to have evidence of systemic sarcoidosis on further investigation and one was biopsy proven isolated neurosarcoidosis. The commonest site of CNS involvement was the cranial nerves (64%), and headache (45%) was the most frequent presenting symptom. MRI abnormalities included leptomeningeal enhancement, white matter lesions, acute arteritis, spinal cord lesion, and cauda equina enhancement. The commonest CSF finding was raised protein (n = 6) and a lymphocytic pleocytosis (n = 7). Serum ACE was only elevated in three cases. Ten patients were treated with both corticosteroids and steroid-sparing agents 8 of whom went into remission. CONCLUSIONS: This series highlights the diverse nature of neurosarcoidosis. Early introduction of aggressive therapy with corticosteroids and steroid-sparing agents appears to improve clinical outcome.
BACKGROUND: The clinical manifestations of neurosarcoidosis are highly variable and it should be considered as a potential differential diagnosis in any neurological presentation. AIM: This study was designed to describe the clinical, diagnostic, and treatment patterns and functional outcome in a Caucasian neurosarcoidosis population. DESIGN: A retrospective analysis was performed on prospectively recorded data in patients attending our neurology clinic between 2008 and 2014 with a diagnosis of definite or probable neurosarcoidosis according to Zajiek criteria. METHODS: Detailed clinical features, baseline demographic data, results of investigations, treatment type and duration, and clinical outcomes were collated. RESULTS: Eleven patients were identified (55% men) with mean age 39 years (range 21-63). Four had a prior history of systemic sarcoidosis leading to earlier diagnosis (6.7 vs 13.1 months). Six were found to have evidence of systemic sarcoidosis on further investigation and one was biopsy proven isolated neurosarcoidosis. The commonest site of CNS involvement was the cranial nerves (64%), and headache (45%) was the most frequent presenting symptom. MRI abnormalities included leptomeningeal enhancement, white matter lesions, acute arteritis, spinal cord lesion, and cauda equina enhancement. The commonest CSF finding was raised protein (n = 6) and a lymphocytic pleocytosis (n = 7). Serum ACE was only elevated in three cases. Ten patients were treated with both corticosteroids and steroid-sparing agents 8 of whom went into remission. CONCLUSIONS: This series highlights the diverse nature of neurosarcoidosis. Early introduction of aggressive therapy with corticosteroids and steroid-sparing agents appears to improve clinical outcome.