Neurological involvement by Behçet’s syndrome: clinical features, diagnosis, treatment and outcome

Abstract

Neurological involvement in Behçet’s syndrome arises predominately through an inflammatory meningoencephalitis characterised by perivenular inflammation due to activation of Th-17 immunological pathways. The brainstem is involved in 50% of cases, the diencephalon and other areas of the brain in 30%, and the spinal cord in 10%. Movement disorders and epilepsy may occur. Psychiatric syndromes may arise with brain and brainstem involvement, and cognitive disorders relate to the brain disease, to circulating inflammatory factors, and to fatigue and despondency. Eighty per cent of cases begin with a relapsing disease course, of whom 70% have only one attack, and 30% have a progressive disease course either from onset or following an initially relapsing course. Venous thrombosis leading to intracranial hypertension and cerebral venous infarction is less common and caused by inflammation in affected veins and a circulating prothrombotic state. Arterial involvement is rare and relates to an arteritis affecting large-sized and medium-sized vessels within the brain leading to infarction, subarachnoid and parenchymal haemorrhage, aneurysm formation and arterial dissection. There is a newly recognised disorder of cerebral cortical hypoperfusion. Cranial neuropathy, peripheral neuropathy and myositis are rare. There has been significant progress in understanding the pathophysiology and treatment of the systemic disease, leading to improved outcomes, but there has been no randomised trial of treatment in the neurological disorder.

Behçet’s Disease, Pathogenesis, Clinical Features, and Treatment Approaches: A Comprehensive Review

“…The ulcers are often a result of vasculitis in the small or large intestine, leading to tissue necrosis and ulceration [31]. Neurological manifestations can include headaches, strokes, meningitis, or encephalitis [32][33][34]. These can be attributed to vasculitis affecting the central nervous system, with inflammation and possible thrombosis disrupting normal neurological function [35].…”

“…The interplay between innate immunity and adaptive immunity could sustain the long-term inflammatory condition seen in Behçet's. Behçet's disease has also been linked to regulatory T cell (Treg) dysfunction, which is implicated in immunological homeostasis maintenance [33]. The uncontrolled inflammation and autoimmunity seen in the condition may be attributed in part to impaired Treg activity.…”