Table 3

Summary and treatment recommendations

Neurological disorderImaging appearancesCSF findingsTreatment
MeningoencephalitisBrain
Brainstem
Cord
Lesions with enhancementActive, leucocytosis
OB rarely present
Intravenous corticosteroid
Immediate institution or escalation of immunosuppressive therapy
Chronic progressive diseaseAtrophy without enhancementLess active, IL-6 elevatedCorticosteroid and immunosuppression
Role of TNFα antagonists to be defined
Venous diseaseCVST
Intracranial hypertension
Venous sinus thrombosis
Normal
Normal
Normal
Intravenous corticosteroid
Immediate institution or escalation of immunosuppressive therapy
Role of TNFα antagonists to be defined
Corticosteroid and immunosuppression
Diuretic therapy
Arterial diseaseCerebral infarction
Cerebral haemorrhage
Aneurysm
Infarction
Haemorrhage within a lesion
Subarachnoid or parenchymal
Beading or occlusion on angiography
On MRA; may be multiple
Intravenous corticosteroid
Immediate institution or escalation of immunosuppressive therapy
TNFα antagonist immediately
Role of IL-6 antagonists to be defined
Perfusion disorderRegional perfusion defects on SPECT and PETPathogenesis and treatment to be defined
Cranial neuropathyOptic nerve
Others
Vestibulocochlear
Lesion in nerve or sheath
Lesion in brainstem, meningeal enhancement or normal
Normal
Corticosteroid and immunosuppression
Movement disorders and epilepsyAssociated brainstem and/or cerebral lesionTreatment of the meningoencephalitis
Cognitive and psychiatric disordersNormal or lesion in brainstemCareful multidisciplinary assessment to define relationship to Behçet’s syndrome
Treatment of meningoencephalitis
Treatment of systemic disease without brain disorder
Treatment of underlying psychiatric disorder
Peripheral neuropathy
Myositis
Corticosteroid and immunosuppression
Corticosteroid and immunosuppression