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Objective.—To study the frequencies and characteristics of different headache types seen in patients with Behcet's syndrome (BS) in a large cohort of patients. Background.—Patients with BS may present with different neurological problems, related either directly or indirectly to the disease, with headache being the most common neurological symptom seen in this syndrome, as well as independent from neurologic involvement. Method.—This study was carried out at the multidisciplinary Behcet outpatient clinic of the Behcet's Syndrome Research Center. Every fifth admitted patient who had fulfilled the International Study Group for Behcet's Disease classification criteria was recruited for this study. Each patient was interviewed by one of the examining neurologists through a semistructured questionnaire. In case of the presence of more than one headache type, the best defined one was diagnosed and evaluated. All patients received a complete neurological and physical examination. A χ2 test was used to evaluate the differences between frequencies. One sample t-test was used to compare means. The prevalence rates of primary headaches were compared with results of the Turkish Headache Epidemiology study by using goodness-of-fit test. Results.—Two hundred and twenty-eight patients were studied. Headache was reported in 66.2% (151/228) of the study cohort. Primary headaches were seen in 38.6% of the patient population, which corresponds to 58% of BS patients with any type of headache. Tension-type headache (TTHA) and migraine were seen in 23.6% and 14.9% of the whole study cohort, respectively. In 5.2% of the study cohort, the headache was associated with neurological involvement and in 3.9% to uveal inflammation. A predominantly frontal, bilateral paroxysmal throbbing pain of moderate severity was reported in 18.4% of the study cohort who did not fulfill the criteria of the International Headache Society (IHS) for any of the primary headaches. It was commonly associated with exacerbations of the mucocutaneous symptoms of the syndrome. The neurological examination was normal in all of these patients. This type of headache was categorized as the nonstructural headache of Behcet. Conclusion.—Headache is the most common neurological symptom seen in BS, both in patients with and without neurological involvement. The results of our study reveal that headache is seen in a majority of patients with neurological involvement due to BS and in a minority with uveal inflammation. The prevalence of migraine and TTHA are close to the population in general, but a nonstructural migrainous headache, which is commonly associated with exacerbations with some of the systemic symptoms of the syndrome, is noteworthy in patients with BS. This form of headache is not specific for this disorder, but may be explained by a vascular headache triggered by the immunomediated disease activity in susceptible individuals.

Patients with Behcet’s Syndrome (BS) may present with different neurological problems, which are either primary or secondary consequences of the systemic disease. The clinical and imaging evidence suggests that primary neurological involvement in BS may be divided into two major forms: The first form, seen in the majority of patients, is the parenchymal central nervous system (CNS) involvement. This is likely to be due to inflammatory small vessel disease, and may present as an acute disorder or may have a chronic progressive form. It’s named as intra-axial “neuro-Behcet’s syndrome” (NBS). The second form, which has few symptoms and a better neurological prognosis, is caused by isolated cerebral venous sinus thrombosis and presents with symptoms and signs of intracranial hypertension, and it’s named as extra-axial “neuro-Behcet’s syndrome.” These two types rarely occur in the same individual, and their pathogenesis is likely to be different. Isolated behavioral syndromes and peripheral nervous system involvement are rare, whereas a nonstructural vascular type headache is relatively common and independent from neurological involvement. Neurologic complications secondary to systemic involvement of BS such as cerebral emboli from cardiovascular complications of BS and increased intracranial pressure due to superior vena cava syndrome, as well as neurologic complications related to BS treatments such as CNS neurotoxicity with cyclosporine and peripheral neuropathy with the use of thalidomide or colchicine are considered as secondary neurological complications. Acute episodes of NBS are treated with high-dose i.v. methylprednisolone followed by an oral tapering. As the neurological involvement in this syndrome is so heterogeneous, it is difficult to predict its course and prognosis, and response to treatment. A number of immunosuppressive agents as well as the immunomodulatory agent interferon-alpha have been used for the long-term treatment of intra-axial NBS, but none of them have been shown to be specifically beneficial. The monoclonal anti-TNF alpha antibody treatment emerges as a new option for the same purpose, but further data is needed. Currently, treatment options are limited to attack and symptomatic therapies, with no evidence for the efficacy of any long-term preventive treatment.

Interferon beta (IFN-β) is the most widely prescribed disease-modifying drug for multiple sclerosis (MS). Therapy with IFN-βmay be associated with a number of adverse reactions. The development or exacerbation of other autoimmune diseases is a rare but reported side effect of IFN-βtherapy. In this case report, we present clinical and laboratory findings of two MS patients who developed arthritis during IFN-β1b treatment, probably of autoimmune origin.

We present four incidental cases that developed partial myelitis following the administration of hepatitis B vaccine in 1998. The first two cases, a 33-year-old man and a 42-year-old woman developed progressive sensory symptoms without motor involvement within 4 weeks following the vaccination. Their magnetic resonance imaging (MRI) disclosed similar lesions consistent with myelitis at their cervical spinal cord. A comparable inflammatory lesion was seen at the T9-T10 levels of the spinal cord in the third case, who was a 40-year-old woman presenting with numbness in her legs and urinary retention following the vaccination. The fourth case who was a 42-year-old woman, presented with sensory symptoms in her left extremities, which developed 3 months after the vaccination. Her MRI showed a hyperintense lesion at C6. She also had two tiny lesions in her cranial MRI. In all cases, there was no history of preceding infections and no clinical evidence suggestive of any other disorders that may cause myelopathy. All patients recovered completely within 3 months with the exception of the third patient who developed new neurological symptoms after 12 months. Similar clinical and imaging presentation of myelitis following hepatitis B vaccination within a 1 year period with no other demonstrable clinical and laboratory evidence for any other disorder raise the probability of a causal link between these two events.

Summary: Baló's concentric sclerosis (BCS) is a rare demyelinating disease considered to be a variant of multiple sclerosis. Five BCS cases were diagnosed antemortem based on their typical concentric mass patterns on MR images and based on clinical and CSF findings. Histopathologic investigation was also performed in one case. Our case report supports the concept that BCS may be a self-limited disease that is not always fatal. Characteristic MR imaging findings may allow antemortem diagnosis of BCS when performed at the onset of the disease.

This study was conducted to describe clinical and prognostic aspects of neurological involvement in Behçet's disease (BD). Patients referred for neurological evaluation fulfilled the criteria of the International Study Group for Behçet's Disease. We analyzed disability and survival by the Kaplan-Meier method, using Kurtzke's Extended Disability Status Scale (modified for BD) and the prognostic effect of demographic and clinical factors by Cox regression analysis. We studied 164 patients; of the 107 diagnostic neuroimaging studies: 72.1% showed parenchymal involvement, 11.7% venous sinus thrombosis (VST) and the others were normal. CSF studies were performed in 47 patients; all with inflammatory CSF findings (n = 18) had parenchymal involvement. An isolated increase in pressure was compatible with either VST or normal imaging. The final diagnoses were VST (12.2%), neuro-Behçet syndrome (NBS) (75.6%), isolated optic neuritis (0.6%), psycho-Behçet syndrome (0.6%), and indefinite (11%). VST and NBS were never diagnosed together. Ten years from onset of BD 45.1% (all NBS) reached a disability level of EDSS 6 or higher, and 95.7 +/- 2.1% of the patients were still alive. Having accompanying cerebellar symptoms at onset or a progressive course is unfavorable. Onset with headache or a diagnosis of VST is favorable. Two major neurological diagnoses in BD are NBS and VST. These are distinct in clinical, radiological, and prognostic aspects, hence suggesting a difference in pathogenesis.