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Treatment of Neurosarcoidosis
Abstract and Commentary
By Joseph E. Safdieh, MD, Assistant Professor of Neurology, Weill Medical College, Cornell University. Dr. Safdieh reports no financial relationships relevant to this field of study.
Synopsis: Patients with high-risk neurosarcoidosis who receive combination therapy with corticosteroids and additional immunosuppressive agents seem to have favorable outcomes.
Sources: Scott TF, Yandora K, Valeri A, et al. Aggressive Treatment for Neurosarcoidosis : Long-term Follow-up of 48 Treated Patients. Arch Neurol 2007;64:691-696
Neurological involvement is encountered in approximately 5% of cases of systemic sarcoidosis. Both the central and peripheral nervous systems can be involved. The more common central nervous system manifestations include cranial neuropathies, chronic meningitis, myelopathy, and intraparenchymal lesions. Due to the relative rarity of neurosarcoidosis, there have been no randomized, controlled, treatment trials published. Corticosteroids are generally used as first-line agents in symptomatic neurosarcoidosis. The role of alternative immunosuppressive agents has traditionally been limited to corticosteroid refractory cases, or in those patients who cannot tolerate corticosteroid treatment. In this retrospective study, the treatment response of 48 patients with neurosarcoidosis is reviewed. The practice of the authors is to use combination therapy with corticosteroids plus additional immunosuppressive agents at diagnosis, in patients who are considered to be high-risk for progression. High-risk patients were defined as those with intracranial lesions, hydrocephalus, myelopathy, seizures, or encephalopathy. Patients who were not considered to be high-risk were treated with corticosteroids alone. Alternative immunosuppressive agents used in this study included methotrexate, azathioprine and cyclophosphamide. Adverse events in the combination group were generally mild and included moderate leukopenia in 2 patients, mild leukopenia in 6 patients and elevated liver function tests in 11 patients.
In the study, 26 of the 48 patients were deemed high-risk and treated with combination therapy. Of the high-risk patients, 69% improved, 15% remained stable, and 15% worsened. Of the 19 standard risk patients treated with corticosteroids alone, 35% improved, 55% remained stable, and 10% worsened. Additionally, retrospective assignment of disability scores demonstrated a significant decline in disability over the course of treatment with combination immunosuppressive therapy, but not in the group treated with corticosteroids alone. However, the group treated with corticosteroids alone had lower disability scores at baseline.
Of note, in this study, CSF angiotensin-converting enzyme levels were obtained in 12 patients and elevated in only 2. The most common symptoms of neurosarcoidosis in this cohort included cranial neuropathy, headache, ataxia, cognitive complaints, and paresthesias. Other less common symptoms included weakness, peripheral neuropathy, seizures and endocrine dysfunction. The majority of biopsy proven cases were demonstrated by lymph node or lung biopsy. Ten patients were diagnosed based on brain or meningeal biopsy. The mean time from onset of symptoms to diagnosis of neurosarcoidosis was 37 months.
The authors suggest that high risk neurosarcoidosis patients should be treated more aggressively than standard risk patients. Although the follow-up data that they present suggests that patients on combination therapy do quite well, the lack of a control group consisting of corticosteroid monotherapy in high-risk patients significantly limits the conclusions of their findings. However, this is an important study that describes the clinical presentation, diagnostic testing, and follow-up of neurosarcoidosis, contributing a large number of cases to the literature about a relatively rare disorder. Overall, the study confirms that neurosarcoidosis is a treatable disorder, and most treated patients stabilize or improve. In addition, the study notes that CSF angiotensin-converting enzyme has a low sensitivity and its absence does not rule out the diagnosis of neurosarcoidosis.