Neuro-Behcet's disease: An update on diagnosis, differential diagnoses, and treatment

Abstract

Neuro-Behcet's disease (NBD) is defined as a combination of neurologic symptoms and/or signs in a patient with Behcet's disease (BD). Relevant syndromes include brainstem syndrome, multiple-sclerosis like presentations, movement disorders, meningoencephalitic syndrome, myelopathic syndrome, cerebral venous sinus thrombosis (CVST), and intracranial hypertension. Central nervous involvement falls into parenchymal and non-parenchymal subtypes. The parenchymal type is more prevalent and presents as brainstem, hemispheric, spinal, and meningoencephalitic manifestations. Non-parenchymal type includes CVST and arterial involvement. Perivascular infiltration of polymorphonuclear and mononuclear cells is seen in most histo-pathologic reports. In parenchymal NBD, cerebrospinal fluid (CSF) generally exhibits pleocytosis, increased protein and normal glucose. In NBD and CVST, CSF pressure is increased but content is usually normal. The typical acute NBD lesions in brain magnetic resonance imaging (MRI) are mesodiencephalic lesions. The pattern of extension from thalamus to midbrain provides a cascade sign. Brain MRI in chronic NBD usually shows brain or brainstem atrophy and/or black holes. The spinal MRI in the acute or subacute myelopathies reveals noncontiguous multifocal lesions mostly in cervical and thoracic lesions. In chronic patients, cord atrophy can also be seen. Brain MRI (particularly susceptibility-weighted images), MR venography (MRV) and computerized tomographic venography (CTV) can be used to diagnose CVST. Parenchymal NBD attacks can be treated with glucocorticoids alone or in combination with azathioprine. For patients with relapsing-remitting or progressive courses, shifting to more potent immunosuppressive drugs such as mycophenolate, methotrexate, cyclophosphamide, or targeted therapy is warranted. For NBD and CVST, immunosuppressive drugs with or without anticoagulation are suggested.

Keywords: Behcet Syndrome; Cerebral Venous Sinus Thrombosis; Diagnosis; Differential Diagnosis; Intracranial Thrombosis; Multiple Sclerosis; Neuro-Behcet's disease; Therapeutics.