Neurosarcoidosis

Abstract

Neurosarcoidosis occurs in 3% to 10% of patients with sarcoidosis. Cranial neuropathy and meningeal involvement are the most common manifestations, but any part of the nervous system can be affected. Definite diagnosis requires the presence of noncaseating granuloma in the nervous system, although histopathologic confirmation is often not obtainable. Moderate to high dose of glucocorticoids is the main therapy for neurosarcoidosis. Relapse often occurs after the dose of glucocorticoids is tapered down, often necessitating the use of steroid-sparing immunosuppressive agents.

Application of the modified Zajicek criteria to diagnose probable spinal cord neurosarcoidosis

“…Immediate treatment for patients with confirmed systemic sarcoidosis with evidence of CNS involvement has been advocated, as unlike pulmonary sarcoidosis spontaneous resolution of neurosarcoidosis is rare, and there is significant risk of morbidity and mortality . Treatment with corticosteroids, typically 1 mg/kg/d is first line for neurosarcoidosis.…”

“…1,17 Indications for immediate treatment of sarcoidosis are generally recognized as functional respiratory impairment or extrapulmonary manifestations including neurological, cardiac, or renal involvement, ocular disease refractory to topical therapy, or symptomatic refractory hypercalcaemia 1,15,16,18 Immediate treatment for patients with confirmed systemic sarcoidosis with evidence of CNS involvement has been advocated, 4,15,16,18 as unlike pulmonary sarcoidosis spontaneous resolution of neurosarcoidosis is rare, and there is significant risk of morbidity and mortality. 19 Treatment with corticosteroids, typically 1 mg/kg/d is first line for neurosarcoidosis. For patients presenting with severe disabling disease such as brain or spinal cord lesions, leptomeningeal involvement or hydrocephalus, aggressive management with pulsed IV methylprednisolone followed by prolonged corticosteroid wean over six to twelve months is recommended.…”

Association Between Treatment-Resistant Sarcoid Myopathy and Inclusion Body Myositis

“…A systemic review and meta-analysis of 1,088 neurosarcoidosis patients showed a mean age of presentation to be 43 with variable outcomes to include remission, the stability of the disease, and in a minority of cases, deterioration or death [11]. Most patients with sarcoidosis respond well to glucocorticoid therapy [12].…”

Laboratory Evaluation of Peripheral Neuropathy

“…Sarcoidosis can be evaluated with serum angiotensin-converting enzyme (ACE) or cerebrospinal fluid (CSF), although specificity is limited and further supportive clinical evidence is often necessary. 25 The yield of testing without a corresponding history is likely to be low, although, as previously discussed, no formal study has proven this. Based on a prospective study, if initial diagnostics do not yield an etiology, repeat laboratory testing is unlikely to elucidate a cause at a later time.…”