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Neuro-Behçet’s disease (NBD) is one of the more serious manifestations of Behçet’s disease (BD), which is a relapsing inflammatory multisystem disease with an interesting epidemiology. Though NBD is relatively uncommon, being potentially treatable, neurologists need to consider it in the differential diagnosis of inflammatory, infective, or demyelinating CNS disorders. Evidence-based information on key issues of NBD diagnosis and management is scarce, and planning for such studies is challenging. We therefore initiated this project to develop expert consensus recommendations that might be helpful to neurologists and other clinicians, created through an extensive literature review and wide consultations with an international advisory panel, followed by a Delphi exercise. We agreed on consensus criteria for the diagnosis of NBD with two levels of certainty in addition to recommendations on when to consider NBD in a neurological patient, and on the use of various paraclinical tests. The management recommendations included treatment of the parenchymal NBD and cerebral venous thrombosis, the use of disease modifying therapies, prognostic factors, outcome measures, and headache in BD. Future studies are needed to validate the proposed criteria and provide evidence-based treatments.

Objectives: To determine the prevalence of neurological involvement in Behcet's disease in a prospective study, and to describe the clinical patterns of neurological presentation in this disease in patients attending a multidisciplinary clinic in Baghdad. Methods: All patients attending the clinic who fulfilled the international study group criteria for the diagnosis of Behcet's disease were studied during a two year period starting in April 1999. Patients were assessed neurologically by a neuro-Behcetologist. All those with clinical neurological manifestations were sent for CSF examination, cranial magnetic resonance imaging, and magnetic resonance venography and were followed up to explore the patterns of neurological relapse. Results: 140 patients with Behcet's disease were studied. Their mean age was 34.2 years (range 16 to 66); 105 (75%) were men and 35 (25%) were women. The mean duration of the disease was 4.2 years (range 0.4 to 26). Twenty patients (14%) had neurological involvement (neuro-Behcet's disease); 14 of these (70%) were men and six (30%) women. The mean age at the first neurological presentation was 34.1 years. The mean duration of follow up of patients with neuro-Behcet's disease was 20.7 months. Ten patients with neuro-Behcet's disease (50%) presented with parenchymal CNS involvement, six (30%) with intracranial hypertension, and four (20%) with a mixed pattern of both parenchymal CNS involvement and intracranial hypertension. Conclusions: Careful neurological assessment of patients with Behcet's disease may show a relatively high prevalence of neuro-Behcet features, and though the clinical patterns of presentation are characteristic a mixed pattern may occur.

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Lhermitte's sign (LS) is strongly linked to multiple sclerosis (MS). Our aim is to reassess its frequency, natural history, various characteristics and neuroradiological correlation in a cohort of MS patients attending our specialized MS clinic and to propose a working definition. Consecutive patients with CDMS and normal controls were interviewed using a structured questionnaire. Cervical MRIs were reviewed when available. There were 300 MS patients and 100 normal controls. Forty-one per cent of the patients and none of the controls reported having LS during the course of their illness. In 53% of those who reported LS, it started in the first three years of the illness and began as an isolated symptom in 64% and was polysymptomatic in 36%. In all patients LS was a short-lasting sensation in all patient who experienced it and was mostly stereotyped in individual patients. Characteristics varied widely between patients. Forty-three patients had cervical MRIs; 17 out of 18 patients who reported LS had abnormalities, whereas only 13 out of the 25 with no LS had abnormalities. The results indicate that LS is highly prevalent in MS, is commonly stereotyped in individual patients, has a variable natural course and correlates significantly with cervical MRI abnormalities. A working definition is proposed.