Diagnosis and management of Neuro-Behçet's disease: international consensus recommendations.

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.

Neuro-Behçet's disease: epidemiology, clinical characteristics, and management.

Behçet's disease (BD) is a multisystem relapsing inflammatory disorder of unknown cause. In neuro-BD (NBD), the CNS can be involved in one or both of two ways: first, and most commonly, through the development of an immune-mediated meningoencephalitis, which predominantly involves the brainstem, but can also involve the basal ganglia, thalamus, cortex and white matter, spinal cord, or cranial nerves; and second, as a consequence of thrombosis within the dural venous sinuses. Headache is a common symptom in BD and does not necessarily indicate CNS involvement. Peripheral nervous system involvement is rare. New treatment options have recently become available, which have led to an improvement in morbidity after meningoencephalitis. Most of the reported studies on NBD are retrospective. Collaborative prospective studies of the natural history of the disease, particularly the nature and treatment of progressive neurological disease, and evidence-based studies of treatment are needed.

Reappraisal of Lhermitte's sign in multiple sclerosis.

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.

Multiple sclerosis in Iraq: does it have the same features encountered in Western countries?

OBJECTIVES: To characterize the clinical, demographic and epidemiological features of MS patients from the only specialised MS centre in Iraq. METHODS: Data for consecutive Iraqi MS patients attending the Baghdad Multidiscipline MS Clinic between 2000 and 2002 who fulfilled Poser et al. criteria for clinically definite (CD) and clinically probable (CP) MS were reviewed. RESULTS: We identified a total of 300 MS patients (164 females, 54.7%; 136 males, 45.3%) with a mean age of onset being 29.2+/-7.8 years and the duration being 8.6+/-5.9 years. According to the year of clinical onset of MS, a progressive increase in cases in the last two decades and a trend towards more females was noted. Initial symptom was reported as motor in 31.7%, sensory in 28.3%, optic nerve in 24% and brainstem or cerebellar in 22.3% of patients. The course was relapsing-remitting in 199 (66.3%) patients, secondary progressive in 56 (18.7%) and primary progressive (PP) in 45 (15%) patients. CONCLUSIONS: MS is not rare in Iraq; its demographic and clinical data were, in general, similar to those reported in Caucasian populations. There was some evidence for North-South gradient and a possible increasing incidence characterized by an increase in female preponderance during the last 2 decades.

Autonomic dysfunction in multiple sclerosis.

OBJECTIVE: Multiple sclerosis (MS) is a demyelinating disorder of the central nervous system. Autonomic dysfunction in MS patients may cause significant morbidity. The aim of this controlled cross sectional study was to investigate the prevalence, pattern and severity of autonomic dysfunction in Iraqi MS patients and to correlate them with patient`s age, disease course, duration and severity. METHODS: Fifty-five patients with clinically definite MS according to Poser`s criteria attending Baghdad MS clinic at Baghdad Teaching Hospital were studied between July 2000 and August 2001. Each patient was assessed according to a detailed protocol paper. Expanded disability status scale was used to assess the severity of the disease. The severity of autonomic symptoms was classified according to autonomic nervous system disability scale (ANSDS). Five standardized autonomic cardiovascular (Ewing) tests were performed for every patient which included: heart rate responses to deep breathing, Valsalva maneuver and standing, and blood pressure responses to standing and sustained hand grip. Forty matched healthy subjects were studied as a control group who were assessed with the same protocol paper, ANSDS and Ewing tests. RESULTS: Autonomic symptoms were significantly more prevalent in MS patients than in the controls. Cardiovascular, urinary and gastrointestinal symptoms were highly prevalent. The severity of the different autonomic symptoms as assessed by ANSDS, were higher in the patients than the controls. All 5 Ewing tests in the patients showed highly significant abnormal results as compared to those of the control. Definite parasympathetic derangement was found in 45.5% of the patients while combined sympathetic and parasympathetic derangements were found in 34.5% of the patients. There were significant correlations between the finding of definite autonomic dysfunctions and the age of the patients at the time of assessment and the duration of the disease. CONCLUSION: Autonomic dysfunctions as assessed by a formal interview, ANSDS and by Ewing tests were common in Iraqi MS patients. Careful attention to autonomic disturbances should be considered in the routine evaluation of MS patients which might help in improving their quality of life.