Neurologic complications of lymphomatoid granulomatosis

Abstract

Lymphomatoid granulomatosis is a malignant form of pulmonary angiitis that has characteristics in common with Wegener granulomatosis and lymphoma. Neurologic symptoms occur in up to 30% of the cases and are the presenting complaints in up to 21%. CNS, cranial nerve, and peripheral nerve involvement occur singly or in combination. In the CNS there is a pathologic triad of angiitis, lymphoreticular infiltration, and necrosis. In the involved peripheral nerves there is demyelination and lymphoid cell infiltration. Neurologic involvement tends to indicate a worse prognosis. Treatment has been based on steroids and cytotoxic agents and may improve symptoms but does not seem to alter the disease process.