Neurological Involvement In Behcet's Disease Research Articles

Indications for Magnetic Resonance Imaging in Patients With Behcet Uveitis.

Behcet disease is a systemic vasculitis, which may involve the eyes and central nervous system. The true prevalence of neurological involvement is not precisely known but may be associated with ocular involvement. This study investigates the association between Behcet uveitis and neuro-Behcet disease. A retrospective single-center analysis was conducted for consecutive patients with Behcet uveitis at the Massachusetts Eye Research and Surgery Institution. Uveitis characteristics, neurological symptoms, fluorescein fundus angiography, and MRI results were recorded. Our population included 108 patients with Behcet uveitis, and 26 (24.1%) were found to have neurological involvement associated with Behcet disease. Optic nerve leakage on fundus angiography and neurological symptoms were associated with an increased risk of neurological involvement. Three cases (11.5%) were nonparenchymal, while 23 (88.5%) were parenchymal with lesions in the cortex, subcortical white matter, thalamus, basal ganglia, and brainstem. There is a high comorbidity between ocular and neurological involvement in Behcet disease. Careful assessment of neurological symptoms and baseline fluorescein fundus angiography are recommended for patients with Behcet disease. MRI has a high diagnostic yield and should be pursued if there is concern for progressive or pre-existing neurological involvement.

Neuro-Behcet: The Value of Transcranial Doppler

Abstract Abstract Neurological involvement in Behcet disease (BD) is one of the most serious causes of long term morbidity and mortality and can be found in up to 49% of cases. There are 2 main types of CNS involvement, parenchymal and non-parenchymal. Transcranial Doppler (TCD) ultrasonography provides a relatively inexpensive, noninvasive, evaluation of the haemodynamics within intracranial arteries. Material and Methods Case control study of 15 patients with NBD all of them fulfilled the criteria of the International Study Group for Behcet’s disease and fifteen apparently healthy volunteers age and sex matched. All patients and healthy controls were examined by TCD. Cranial Magnitic Resonance Imaging (MRI) examination was performed to the patients group. Results Transcranial Doppler showed significant decline in the mean velocity (MV) of all the examined cerebral arteries in all NBD patients with the highest significant decrease in the vertebral artery (VA). On the other hand, a significant increase in the pulsatility index (PI) was detected only in the posterior cerebral artery (PCA). Regarding MRI examinations of our patients, 7 of 15 (46.7%) patients had parenchymal lesions, 13% had vascular lesions coexisting with parenchymal lesions (mixed). The parenchymal were located in the periventricular and superficial cerebral white matter, thalamus and brainstem followed by the cerebellum. Vascular lesions were dural and transverse sinus thrombosis. Conclusion TCD provides a sensitive and accurate bed-side non-invasive imaging tool for the detection of flow pattern abnormalities in patients with NBD. MRI remains the gold standard for diagnosis.

Intestinal and neurological involvement in Behcet disease: a clinical case

BackgroundBehcet’s disease (BD) is a chronic immune-mediated, inflammatory disorder which may affect a number of different systems (oral and genital mucosa, eyes, skin, vascular district, joints, gastrointestinal tract and nervous system). Neurological manifestations are present in 5–10%, and gastrointestinal tract involvement in 10–15% of cases. The simultaneous involvement of two systems, neurological and gastrointestinal tract, is very rare and represents the aim of our case report.Case presentationWe describe a case of a 12-year-old girl with neurological (endocranial hypertension, papilledema, retinal vasculitis) and gastrointestinal tract (terminal ileum and cecum inflammation) involvement and with a history of recurrent oral aphthosis; therefore, according to both International Criteria for Behcet’s Disease (ICBD) and Paediatric Behcet’s Disease criteria (PEDBD) the diagnosis of BD was confirmed.ConclusionsThis case report is one of the few described in literature with simultaneous involvement of the two systems, neurological and gastrointestinal tract, in paediatric BD. The diagnosis is really difficult because there is no specific diagnostic test. We think that our clinical case should help clinicians to suspect a BD with an unusual onset.

A fatal case of probable neurobehcet

Abstract Behcet's disease (BD) is a systemic vasculitis characterized by recurrent oral and genital ulcers, with a frequent ocular and skin involvement. Neurological involvement in BD has been reported in 4–49% of cases and determines functional and vital prognosis of the disease. Based on clinical and imaging evidence, two major forms can be identified: a parenchymal or an extra parenchymal involvement essentially represented by cerebral vein thrombosis. Awareness disorders are rarely reported as tell-tale signs of neurological involvement in BD. We hereby report the peculiar case of a young patient, 39 years old, admitted to the emergency department for an acute onset of altered mental status in a non-traumatic setting where investigations led to the diagnosis of neurobehcet disease. Patient was placed on high doses corticosteroids with unfavorable outcome; patient passed away after 01 week.

Association of amyotrophic lateral sclerosis and Behcet’s disease: is there a relationship? A multi-national case series

Neurological involvement may be seen in 5-30% of the patients with Behcet's disease (BD). Occasionally, parenchymal neurological involvement in BD can present as a spinal cord syndrome. However, motor neuron disease-like presentation is extremely uncommon. Here we are reporting five patients (all male; median age, 38) fulfilling both International Study Group criteria for BD and El Escorial criteria for amyotrophic lateral sclerosis (ALS). These patients were identified by a questionnaire sent to the members of the Neuro-Behcet Study Group of the International Study Group for BD. Three out of five patients had only motor presentations. In two patients, sensory and urinary manifestations were present as well. Spinal cord MRIs were normal in all, and brain MRIs were normal in four patients; one patient had nonspecific white matter changes. Two patients passed away 1-3 years after diagnosis of ALS, and two patients were lost to follow-up 3 and 11 years after admission; one patient is still alive 3 years after onset. The patients that are presented here might represent a rare form of neurological involvement in BD as well as sole coincidence. Larger prospective series are needed to further answer this issue.

Neurologic Soft Signs in Behcet Disease

Patients with Behcet disease have an excess of minor neurologic abnormalities (neurologic soft signs). (a) To investigate the neurologic soft signs (NSS) in Behcet disease (BD) patients who had no neurologic symptoms, by using the neurologic evaluation scale (NES). (b) To evaluate the effect of silent infarction on NES scores in BD patients. Thirty six BD patients without neurologic symptoms and 36 healthy controls were included in the study. NSS were assessed with the NES. Cranial magnetic resonance imaging was conducted to determine the presence of silent cerebral infarcts. Patients with BD had significantly higher scores overall and on each subscale (except for subscale "others") of the NES than the control group's. Tandem walk, adventitious overflow, tremor, graphesthesia, fist edge palm test, Ozeretski test, finger thumb opposition, mirror movements, extinction, synkinesis, convergence, finger nose test, glabellar reflex, grasp reflex, and suck reflex were also significantly higher in patients with BD than in the healthy control group. There were no significant differences in the total NES scores, total subscale scores and each of the NES items between BD patients with silent infarction and those without infarction. Early diagnosis of neurologic involvement in BD is important and the NES is a useful instrument for detecting subclinical neurologic involvement in BD patients.

Subclinical cerebral involvement in Behcet's disease: a SPECT study.

Neurologic involvement has been reported in Behcet's disease (BD) with prevalence rates of 4-49%. Involvement of the central nervous system (CNS) usually follows systemic manifestations of BD by months to years, but as the initial future in only 5% of cases. The variance of the prevalence rates of neurologic involvement in BD raises the possibility of subclinical neurologic involvement. For the purpose of explaining the variance in the prevalence rates, 20 patients with BD, but without neurological symptoms and signs, were investigated by using cerebral single photon emission computed tomography (SPECT) which seems to be more convenient for BD than other scanners. A control group of patients with various diseases that were not expected to influence the cerebral blood flow was included. Brain magnetic resonance imaging (MRI) scans were performed in cases in which abnormal SPECT findings were obtained. Decreased and asymmetrical tracer uptakes were detected in 35% of patients with BD. MRI scans were normal in these patients. We concluded that functional imaging using SPECT may detect abnormalities at an initial stage prior to their progression to morphological damage detectable by MRI, and this imaging modality can be used even in cases which show no neurologic symptom to indicate the subclinical neurologic involvement.