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Abstract
Cases of Behcet’s disease (BD) cluster along the ancient Silk Road, which extends from eastern Asia to the Mediterranean basin (Kenneth, 2008). The prevalence of BD per 100,000 populations has been reported to be as high as 370 in Turkey and as low as 0.64 in Western Europe (Davatchi, 2007). The age of onset in Egyptian patients with BD is between (17 and 37) years with higher male-to-female ratio. The initial presenting and commonest manifestation of BD in Egypt is oral ulcer with higher incidence of vascular and neurological lesions (El-Menyawi et al., 2009). The pathogenesis of BD remains uncertain and most recent studies focus on new genetic markers, the function of T and B cells, infectious agents and the mechanisms involved in thrombophilia (Mendoza-Pinto et al., 2010).
Neuro-Behçet’s disease (NBD) is any neurological complications due to definite Behcet’s disease (BD) other than isolated headache (Al-Araji and Kidd, 2009). It is apparent that the main pathological feature of CNS lesions caused by BD is the perivascular infiltration of mononuclear and polymorphonuclear cells (Haghighi et al., 2007). Both the CNS and PNS can be involved in BD. The CNS manifestations can be divided into two main groups: Parenchymal mani-festations that include brainstem presentations, hemispheric manifestations, spinal cord lesions and meningoencephalitic presentations; Nonparenchymal manifestations, such as dural sinus thrombosis, pseudotumor cerebri, arterial occlusion and/or aneurysms (Al-Kawi et al., 1991; Suda et al., 2008). There are some patients with mixed features of parenchymal involvement and intracranial hypertension (Al-Araji et al., 2003).
The characteristic MRI lesion in NBD is an upper brainstem lesion that extends into the thalamus and basal ganglia (Tali et al., 1997; Borhani et al., 2005). Cerebral atrophy (especially in the brainstem) can also be seen in patients with chronic disease (Akman-Demir et al., 2003). Spinal cord MRI reveals similar signal characteristics and enhancement properties. Segmental enlargement of the spinal cord has also been reported (Yesilot et al., 2007).
ESR has been found to be associated with disease activity (Yazici, et al., 2007). CSF protein is modestly raised in most cases of parenchymal NBD, sometimes to over 1 g/dL (Siva et al., 2001), however CSF is often normal in non-parenchymal (Houman et al., 2007). NBD should be kept in mind for any patient who develops stroke at a young age, intracranial hypertension and intracerebral venous occlusive disease, multiple sclerosis and spinal cord syndromes, especially in countries where BD is prevalent (Haghighi et al., 2009).
According to publish EULAR recommendations for BD treatment, there are no controlled data to guide the management of CNS involvement in BD. For parenchymal involvement agents to be tried may include corticosteroid, INF-α, azathioprine, cyclophosphamide, methotrexate and TNF-α antagonist. For CVT, corticosteroids are recommended. Ciclosporine A should not be used in NBD patients unless necessary for intraocular inflammation (Hatemi et al., 2008; Siva and Hirohata, 2010).
Poor prognostic factors include multifocal involvement, spinal presentations, more than two attacks per year, progressive course and increased cerebrospinal fluid cell count and protein content at the time of neurologic manifestations (Haghighi et al., 2005).