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.     

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.     

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.     

Enhanced complement consumption in neuromyelitis optica and Behçet's disease patients

The complement system is essential in the pathogenesis of inflammatory central nervous system disorders. To investigate the involvement of complement pathways in neuromyelitis optica (NMO), levels of breakdown products for classical (C4d), alternative (FBb) and common (sC5b-9) pathways were measured in the sera of 28 NMO and control patients (30 Beh?et's disease (BD), 29 multiple sclerosis (MS)) and 31 healthy controls by ELISA. Classical and/or alternative pathway consumption was enhanced in NMO and BD patients as compared to MS patients and healthy controls. Our results suggest that NBD and NMO differ from MS by the predominance of complement system involvement.     

Pathological haemostasis and "prothrombotic state" in Behçet's disease

Beh?et's disease (BD) is a widespread occlusive-type vasculitis with life-threatening manifestations. The vasculopathy of BD is unique and any type of vessel can be involved. Moreover, vascular lesions in BD represent an occlusive nature suggesting a hypercoagulable/prothrombotic state. The data concerning the genetic defects of the coagulation cascade are expanding. There is evidence of universal activation of haemostatic system in BD. Procoagulant markers of thrombosis are elevated reflecting intravenous excessive thrombin formation. Defective fibrinolysis with impaired fibrinolytic kinetics may have a role in the hypercoagulable/prothrombotic state of BD. Endothelial cell injury and/or pathological activation is well documented in BD. The aim of this paper is to review current literature knowledge and our experience regarding the unresolved complicated issues of genetic thrombotic defects, in vivo haemostatic markers, coagulation inhibitors, impaired fibrinolysis, and endothelial injury/dysfunction of the hypercoagulable/prothrombotic state of BD. The clinical aspects of vascular thrombosis, the genetic basis of coagulation, coagulation inhibitors, fibrinolysis inhibitors, and endothelial dysfunction are reviewed. Challenges and future prospects regarding the prothrombotic state of BD are discussed together with new promising antithrombotic and antiplatelet treatment strategies. Better understanding of the exact pathogenesis of the hypercoagulable/prothrombotic state of this disease may help to develop novel therapeutic approaches offering a better outcome for Beh?et's patients with thrombosis.     

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.     

Brachial plexopathy caused by subclavian artery aneurysm in Behçet's disease

As the cause of brachial plexopathy, an aneurysm of the subclavian artery is rare and mostly related to trauma. Early diagnosis and treatment is very important because the arterial aneurysm itself is life-threatening and nerve injury can be reversible in cases of early treatment. We report a patient with Beh?et's disease having a right brachial plexopathy caused by a nontraumatic aneurysm of the right subclavian artery.     

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.     

Epileptic seizures in Behçet disease

Summary: Purpose: To outline the clinical characteristics of seizures in our large series of Behçet disease (BD) patients with neurologic involvement. Methods: All files of 223 patients with neuro‐BD were evaluated retrospectively, and the group with clearly documented seizures was included in the current study. Clinical characteristics, EEG, neuroimaging and cerebrospinal fluid findings were reevaluated, and the seizures were classified according to the new proposed criteria of the International League Against Epilepsy. On excluding the patients in whom the seizures were due to possible seizure‐provoking factors, the seizures that appeared during a neurologic exacerbation were noted. Results: Seizures were seen in 10 (4.48%) of 223 patients. There were one female and nine male patients. In five of the patients, seizures occurred during neurologic exacerbation. Therefore, the actual prevalence of seizures due to BD in our group is 2.2%. In the remaining five patients, the seizures were not related to neurologic BD attacks, but probably were due to some seizure‐provoking factors. The predominating seizure type was generalized tonic–clonic convulsions accompanied by focal motor seizures. It is notable that four patients died 1–5 years after the onset of the seizures. Conclusions: Our study showed that seizures are rare in BD. As the seizures due to some interventions and drugs are as frequent as neuro‐BD–related seizures, seizure‐provoking factors must be considered before attributing them to the pathogenetic mechanism of BD. The occurrence of seizures seems to be associated with a high mortality rate.