Refractory Neurosarcoidosis Research Articles

Optic neuropathy in refractory neurosarcoidosis treated with TNF-α antagonist

Case report: To report a rare case of optic neuropathy in refractory neurosarcoidosis treated with tumour necrosis factor-α antagonist (infliximab) perfusion. A 41-year-old man with diabetes presented with an abrupt, painless, unilateral visual loss. Thoracic computed tomography disclosed mediastinal lympha-denopathy, and evidence from histopathology confirmed sarcoidosis. Comments: Corticosteroids alone and with immunosuppressive agents failed to suppress the active disease. Alternative therapy of infliximab perfusions resulted in disease remission and relative visual improvement, and this treatment may warrant further clinical studies.

Optic neuropathy in refractory neurosarcoidosis treated with TNF-α antagonist

To report a rare case of optic neuropathy in refractory neurosarcoidosis treated with tumour necrosis factor-alpha antagonist (infliximab) perfusion. A 41-year-old man with diabetes presented with an abrupt, painless, unilateral visual loss. Thoracic computed tomography disclosed mediastinal lymphadenopathy, and evidence from histopathology confirmed sarcoidosis. Corticosteroids alone and with immunosuppressive agents failed to suppress the active disease. Alternative therapy of infliximab perfusions resulted in disease remission and relative visual improvement, and this treatment may warrant further clinical studies.

Neurosarcoidosis.

Neurosarcoidosis is an uncommon disorder and requires a careful clinical evaluation to reach a diagnosis. Generally, patients with peripheral symptoms, which include paresthesias, painful patches over extremities, and stocking glove deficits, have a better outcome when compared with those with central nervous system (CNS) involvement. Patients with mass lesions or hydrocephalus tend to have more relapses and are often more resistant to routine therapy. Neurosarcoidosis often responds to glucocorticoids, usually within days or weeks of initiating therapy. Patients are usually maintained on 40 to 80 mg per day for 4 to 6 weeks, then tapered slowly. The use of alternative treatments for refractory neurosarcoidosis, or to reduce or eliminate steroids, includes methotrexate, cyclophosphamide, azathioprine, cyclosporine, chlorambucil, chloroquine, and hydroxychloroquine.

Therapeutic Considerations in Patients With Refractory Neurosarcoidosis

To assess the effectiveness of alternative treatments for patients with refractory neurosarcoidosis. Nonrandomized, retrospective patient survey. Multicenter, involving patients cared for by their primary physicians and neurologists, and referred for management advice to a neurology consultant. Patients were treated with corticosteroids and alternative treatments, including azathioprine, cyclosporine, cyclophosphamide, chlorambucil, methotrexate, and radiation therapy. Prednisone dosage was successfully tapered to 10 to 20 mg/d without worsening symptoms in 10 (38%) of the 26 patients studied. Six (23%) patients had improved conditions while receiving alternative medication and nine (35%) patients' conditions remained stable with no further progression of their symptoms. Radiotherapy was beneficial for one of three patients. Four (15%) patients did not respond to alternative treatment and died of worsening symptoms or infection. Adverse effects of the alternate medications resolved on discontinuing treatment with the offending agent. Alternative treatment is an effective adjunct to corticosteroid therapy for some patients with refractory neurosarcoidosis. Clinical deterioration may occur despite combined therapy. Choice of alternative therapy should be determined, in part, by its potential adverse effects.