Cognitive impairment in Behçet's disease patients without overt neurological involvement

We investigated the prevalence of cognitive impairment in patients with Behçet's disease (BD) without overt neurological involvement. The influence of disease duration, disease activity, prednisone dosage, and anxiety and depression levels was evaluated. Twenty-six consecutive BD outpatients and 26 healthy controls matched for age, education and sex completed a comprehensive neuropsychological battery including tests of memory, visuospatial and constructional abilities, language, attention and psychomotor speed, non-verbal reasoning and executive functioning. The Hamilton scales for anxiety and depression were administered. Disease activity was assessed using the Behçet's Disease Current Activity Form (BDCAF). Compared to controls, BD patients were significantly impaired on tasks evaluating long-term verbal and non-verbal memory, and visuospatial skills. In addition, BD patients were significantly more anxious and depressed than controls. Cognitive impairment was evident in 46.1% of BD patients compared with none of control subjects (p<0.0001), with memory representing the cognitive domain most affected. Both high disease activity (OR 1.3, 95% CI 1.0–1.5, p<0.04) and high prednisone dosage (OR 1.3, 95% CI 1.0–1.7, p<0.03) were independently associated with cognitive impairment in BD after adjustment for demographic variables. Cognitive impairment, involving mainly memory functions, occurs frequently in BD patients. It may occur independently of clinically overt neurological involvement, and is more common in patients with an active disease and in those receiving prednisone.     

Executive dysfunctions and depression in Behçet's disease without explicit neurological involvement

Abstract This study aims to assess the executive functions and depression status in patients with Behçet’s disease without explicit neurological involvement and to evaluate cognitive functions in this group of patients independent of accompanying depression. In the present study, 30 patients with Behçet’s disease in the non‐active phase of their illness and 30 healthy volunteers were included. In the evaluation of depression levels, Beck Depression Inventory was employed. The executive functions of the patients were evaluated by Wisconsin Card Sorting Test (WCST) and Stroop Test (ST). Beck Depression Inventory scores measured in the Behçet’s disease group, in which no clinically serious depression was observed, were higher than the control group. Then, it was observed that the scores of neuropsychological tests of the Behçet’s disease group were found to be significantly lower, especially in WCST, while significant relations were revealed between Beck Depression Inventory scores and WCST, ST scores. The authors’ findings suggest that there may be factors other than depression causing executive impairment in patients with Behçet’s disease without explicit neurological symptoms. Executive dysfunctions may occur in association with mild depressive states due to chronic disease stress or silent and/or future neurological involvement of Behçet’s disease, especially in the frontal lobes.     

Behçet's disease: diagnostic and prognostic aspects of neurological involvement

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's syndrome (NBS) (75.6%), isolated optic neuritis (0.6%), psycho-Behçet's 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.     

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.     

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.     

Central nervous system involvement in Beh?et's disease]

Central nervous system involvement in Beh?et's disease, usually called neuro-Beh?et's syndrome (NB), includes acute type and chronic progressive type. Acute NB is characterized by acute meningoencephalitis with focal lesions, presenting high intensity areas in T2-weightened images or FLAIR images on MRI scans. Cyclosporin A frequently causes acute NB. Acute NB responds to steroid therapy, and is usually self-limiting. By contrast, chronic progressive NB is characterized by intractable slowly progressive dementia, ataxia and dysarthria with persistent elevation of cerebrospinal fluid IL-6 activity (more than 20 pg/ml). In our series, most patients (approximately 90%) with chronic progressive NB were HLA-B51 positive, and had history of attacks of acute NB prior to the development of progressive neurological symptoms. Chronic progressive NB is resistant to conventional treatment with steroid, cyclophosphamide, or azathioprine. However, recent studies suggest the efficacy of low dose methotrexate in chronic progressive NB.     

Behçet's disease associated with peripheral neuropathy

INTRODUCTION: The involvement of the peripheral nervous system in Beh?et's disease is very rare. CASE REPORT: We report a case of a 47-year-old man with a six-year history of Beh?et's disease and a two-year history of peripheral nervous system involvement. This patient presented with paraesthesia and weakness of the upper and lower limbs, diarrhea and erectile dysfunction. The electromyogram showed evidence of an axonal sensorimotor neuropathy and the nerve biopsy showed an axonal neuropathy. Routine blood tests were normal, there was no increase of serum creatine kinase, aspartate aminotransfease or lactate dehydrogenase and no signs of hyperthyroidism. Fibroscopy and colonoscopy showed no signs of entero-Beh?et. The patient was treated with prednisone, cyclophosphamide and carbamazepine with an improvement of paraesthesia. CONCLUSION: The mechanism of the peripheral neuropathy in Beh?et's disease is still unknown, it might be due to vasculitis of the vasa nervorum or to the side effects of colchicine. Our report is particular by the association of sensorimotor and autonomic involvement of peripheral neuropathy in a patient with Beh?et's disease.     

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.     

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.     

Endovascular treatment of carotid and pulmonary aneurysms in Behçet's disease

Arterial aneurysm formation is a rare manifestation in Beh?et's disease (BD). Aneurysm rupture is the main cause of death among BD patients. In recent years, endovascular treatment has been proposed as an effective and less invasive procedure compared with classical surgical treatment. We report the case of a 29-year-old man with BD who had two syncopes precipitated by pressure on a left cervical mass and hemoptysis. Cerebral magnetic resonance imaging (MRI) showed acute infarction of left basal ganglia. Other examinations demonstrated carotid and pulmonary aneurysms that were treated by endovascular methods. The patient received treatment with corticosteroids and immunosuppressive therapy. First of all, the pulmonary aneurysm was treated by means of coil embolization. After anticoagulant therapy was initiated, a covered stent was placed to exclude the carotid aneurysm at the level of extracranial internal carotid artery (ICA), with no immediate complications. After twenty-seven months of follow-up the patient remains asymptomatic. Endovascular treatment may be a definite therapeutic option for extracranial carotid aneurysms in BD, although longer term follow-up studies are needed.     

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.