Long-latency reflexes in patients with Behçet's disease

Objective: Recent studies demonstrate that the subclinical involvement of motor pathways is frequently observed in patients with Behçet's disease (BD). Long-latency reflexes (LLR) provide information about the continuity of both ascending and descending neural pathways. Our aim was to evaluate the utility of LLR and somatosensory-evoked potentials (SEP) in demonstrating subclinical neural involvement in patients with BD. Methods: Twenty-nine patients with BD were studied by means of SEP and LLR. Bilateral median nerve SEPs and LLRs evoked by electrical stimulation of both median nerves were recorded. The latency of second component of LLR (LLR2), the duration of LLR2–HR (Hoffmann reflex, spinal reflex component of LLR) interval, peak to peak amplitude of LLR2 and the amplitude ratio of LLR2/HR were analyzed. The data obtained from patients were compared with those of 20 control subjects. Results: LLR2 latencies and the durations of LLR2–HR interval were significantly prolonged in patients with BD (p=0.001 for both parameters). Increased duration of LLR2–HR interval was the most frequent abnormality observed in the study (37.9%). Conclusion: Our findings suggest that LLR is a useful technique to demonstrate subclinical neural involvement in patients with BD.     

Optic neuropathy in Behçet's disease. Report of two cases

Orbital magnetic resonance imaging demonstrated increased signal of the optic nerve in short time inversion recovery (STIR) images of two young women with unilateral visual blurring. In both, recurrent oral and genital ulcerations and papulopustular lesions appeared within the next 14–15 months, respectively, allowing a diagnosis of Behçet's disease. Optic neuropathy may be an early manifestation of Behçt's disease and clinical follow-up is crucial for its diagnosis.     

Epilepsia partialis continua in a patient with Behçet's disease

Behçet's disease (BD) is a multisystemic, recurrent, inflammatory disorder of unknown aetiology. Neurological involvement is characterised either by primary parenchymal lesions or secondary to major vascular involvement. Seizures are rarely seen in BD and their occurrence can be related to seizure provoking factors or exacerbation of the disease. We experienced a case of neuro-BD presenting with subacutely developing mental and behavioral changes, followed by left dominant tetraparesis with bilateral pyramidal signs, fever and left hand focal motor seizures with elementery clonic motor signs which later evolved into right hand epilepsia partialis continua (EPC) of Kojevnikov. The seizures were very resistant to antiepileptic drugs and 8 months after neurological involvement the patient died. The EPC evolving after neurological involvement is associated with high mortality rate.     

Subclinical neurological involvement in Behçet's disease

CONTEXT: Beh?et's disease (BD) is a multisystem inflammatory disorder with unknown etiology characterized by recurrent oral and genital aphthous ulcers and uveitis. Beh?et's disease can affect the central nervous system. AIMS: We aimed to investigate subclinical neurological involvement in patients who were suffering from BD and who had no neurological symptoms. SETTINGS AND DESIGN: A total of 49 patients were included in the study. For the investigation of subclinical neurological involvement, the patients received imaging and/or neurophysiologic evaluations. MATERIALS AND METHODS: The evaluation techniques were as follows: single photon emission computed tomography, 33 patients; cranial magnetic resonance imaging (MRI), 25 patients; brainstem auditory evoked potential examination, 36 patients; and electroencephalography (EEG), 30 patients. STATISTICAL ANALYSIS USED: The Mann-Whitney U test and Wilcoxon Rank-Sum W test were used. RESULTS: Patients in the MRI and EEG groups showed significantly more abnormalities than did age- and gender-matched controls. CONCLUSIONS: Early diagnosis of neurological involvement in BD is important in reducing or preventing complications. Cranial MRI and EEG were found to be useful for detecting subclinical neurological abnormalities in patients with Beh?et's disease.     

Thrombin activatable fibrinolysis inhibitor in Behçet's disease

INTRODUCTION: Thrombin activatable fibrinolysis inhibitor (TAFI) is a procarboxypeptidase downregulating plasmin formation, thereby causing a tendency for thrombosis development. Since, Beh?et's disease (BD) is a systemic vasculitis, which is commonly complicated by arterial and venous thrombosis, we aimed to find out plasma TAFI levels in BD, compared with healthy controls. We also searched whether plasma TAFI levels were significantly different between Beh?et's subgroups with and without thrombosis. MATERIALS AND METHODS: In this study, 105 BD patients (M/F: 64/41; mean age 36+/-1 years), followed up by Ege University Rheumatology Department were enrolled. The exclusion criteria were hemophilia, hyperlipidemia, diabetes mellitus, hepatic diseases renal failure, antiphospholipid positivity, oral contraceptive use and pregnancy. Age-and sex-matched healthy controls (n=53) were also included. Plasma TAFI levels were measured by ELISA. Since TAFI is also an acute-phase reactant, we also measured other inflammatory markers such as C-reactive protein (CRP). RESULTS: Plasma TAFI levels were significantly higher in Beh?et's patients (91.1+/-7.4 ng/ml) compared with healthy controls (14.3+/-4.5 ng/ml) (P<0.001), but there were no significant difference between the subgroups with and without thrombosis. In BD, there was no correlation between plasma TAFI levels and CRP. CONCLUSIONS: Regardless of manifest thrombosis, plasma TAFI levels in BD were significantly higher than in healthy controls. High TAFI levels might possibly contribute to the thrombotic tendency in BD. Future studies investigating TAFI gene polymorphism and functional activity are clearly needed, to clarify the exact role of TAFI in Beh?et's thrombosis.     

Basilar artery aneurysm associated with Behçet's Disease: a case report

Vascular involvement is common in Beh?et's Disease (BD). The venous system is often affected, while arterial system involvement is a rare occurrence. Intracranial aneurysm formation associated with BD is extremely rare. A 38- year-old male patient with BD under the follow-up of the Dermatology department presented with acute headache. The cranial computed tomography revealed subarachnoid bleeding. A basilar artery aneurysm, which was ruptured during the procedure, was found on the cerebral angiography and the patient died the next day. This report adds our case to the literature of posterior cerebral circulation aneurysms associated with BD among the limited number of articles on this topic.     

Discrepancies of NKT cells expression in peripheral blood and in cerebrospinal fluid from Behçet's disease

The precise role of natural killer T (NKT) cells in the pathogenesis of Beh?et's disease (BD) remains unknown. The frequency, cytokine profile and heterogeneity of NKT cells were studied in peripheral blood mononuclear cells (PBMC) from 42BD patients and in cerebrospinal fluid (CSF) samples from 9 neuro-BD patients. Flow cytometry revealed a decreased frequency of NKT cells in PBMC from BD patients (median: 0.06%; range: 0%-0.3%) when compared to healthy controls (median: 0.23%; range: 0.1%-0.7%; P<0.01). NKT cells were biased toward a Th(1)-like phenotype, with a significant decrease of IL-4/IFN-gamma ratio in BD (median: 0.049; range: 0.01-0.13) vs. healthy controls (median: 0.82; range: 0.4-1.33; P<0.01). NKT cells were increased in CSF-BD samples (median: 0.18%; range: 0.1%-0.4%), when compared to CSF-NIND patients (median: 0.05%; range: 0.01%-0.09%; P<0.01). Based on the reactivity of PBMC-derived NKT cells toward alpha-galactosylceramide (alpha-GalCer), 80% of BD patients were non-responsive. At the opposite, the reactivity of NKT cells in CSF from BD patients was not impaired. BD-CSF NKT cells exhibited an increased expression of IFN-gamma-producing cells, demonstrating that CSF-NKT cells were functional, and biased toward a Th(1)-like phenotype. These data suggest that functional NKT cells are recruited into BD inflammatory sites contributing to BD pathogenesis.     

Optic neuropathy in Beh?et's disease. Report of two cases

Orbital magnetic resonance imaging demonstrated increased signal of the optic nerve in short time inversion recovery (STIR) images of two young women with unilateral visual blurring. In both, recurrent oral and genital ulcerations and papulopustular lesions appeared within the next 14-15 months, respectively, allowing a diagnosis of Beh?et's disease. Optic neuropathy may be an early manifestation of Beh?t's disease and clinical follow-up is crucial for its diagnosis.     

Histopathology of central nervous system lesions in Behçet's disease

Central nervous system (CNS) involvement in Beh?et's disease, usually called neuro-Beh?et's syndrome (NB), is one of the most serious complications of the disease. In the present study, we carried out immunohistological examination of biopsied or autopsied brain tissues from 3 patients with different types of NB, acute NB, chronic progressive NB, and NB in a long-term remission. Histopathology of mass lesion in acute NB revealed infiltration of mononuclear cells around small vessels, consisting of CD45RO+ T lymphocytes and CD68+ monocytes with few CD20+ B lymphocytes. Of interest, TUNNEL staining disclosed that most neurons were undergoing apoptosis in the inflammatory lesion. In chronic progressive NB, similar histopathological changes were noted in pons, cerebellum, medulla, internal capsule, and midbrain, although the degree of mononuclear cell infiltration was modest. There were also scattered foci of neurons undergoing apoptosis with formation of a few binucleated neurons. The most prominent feature of NB in a long-term remission was atrophy of basal pons with formation of cystic or moth-eaten lesions, consisting of isomorphic gliosis with viable neurons. There were still scattered foci of perivascular cuffing of T lymphocytes and monocytes. These results emphasize the common features throughout the courses of NB, perivascular cuffing of T lymphocytes and monocytes, irrespective of the clinical phenotypes. More importantly, it is suggested that soluble factors produced by infiltrating cells, including IL-6, might play a role in the induction of apoptosis of neurons in NB.     

Behçet’s disease presenting as a peripheral vestibulopathy

Prolonged acute spontaneous vertigo can be secondary to acute vestibular neuritis or posterior circulation ischaemia. We present a 66-year-old man who first developed an acute vestibular syndrome with profound unilateral hearing loss 34 years ago. First treated as vestibular neuritis, he subsequently developed manifestations of Behçet’s disease, including mouth ulcers, genital ulcers and erythema nodosum over a period of 10 years. Subsequently, sudden sensorineural hearing loss affecting his only hearing ear responded to immunomodulation, confirming an autoimmune cause for the audiovestibular symptoms. This report serves as a reminder that vestibular neuritis seldom causes hearing loss; ischaemic, infective and autoimmune causes should be sought when an acute vestibular syndrome is accompanied by hearing impairment.