Behçet's Disease

NeuroLearn NeuroHelp Meningoencephalitis without identified infectious agent @

Background    Neuroimaging    Gross Pathology    Histopathology & Immunohistochemistry    Differential Diagnosis    Reference

BACKGROUND AND CLINICAL INFORMATION: Head

Geographic distribution: Most common in Turkey, other Near East countries and Japan. Commonly presents as recurrent ulceration of the mouth and genitalia associated with uveitis or iridocyclitis. About one fifth of cases affect the brain.

Clinical features:

• Diagnosis is largely relied on features outside the CNS.

• Neurologically can present as recurrent meningoencephalitis, cranial nerve palsies, cerebellar ataxia, and cortical-spinal tract signs.

• Symptoms usually have an abrupt onset and brisk pleocytosis in the CSF.

• As a rule, neurologic symptoms clear up in a few weeks.

• Clinically may mimic multiple sclerosis and minor strokes of the brain stem.

Anatomic locations:

• Multifocal:  can involve both the gray and whit matter. No part of the brain is spared but the brain stem, hypothalamus, and internal capsule are most frequently involved.

• Systemic: A rare vasculitic disorder involving multiple organs and characterized by recurrent ulcers of oral and genital mucous membranes, uveitis and arthritis.This is a systemic disease that involves kidney, intestines, mucosa, brain and other organs.

Rhomboencephalitis: The CNS is affected inabout 30-40% of patients and most often present with meningoencephalitis with accentuation in the brain stem region.

NEUROIMAGING: Head

GROSS PATHOLOGY: Head

HISTOPATHOLOGY AND IMMUNOHISTOCHEMISTRY: Head  

• Multifocal necrotizing lesions with marked inflammatory cell infiltration.

• Microglial nodules and diffuse glial proliferation.

• There is also loss of myelin, axons, and neurons.

• Relatively mild involvement of neurons that may be spared in necrotic foci.

DIFFERENTIAL DIAGNOSIS: Head