Seven-year follow-up of neurologic involvement in Behçet syndrome.

OBJECTIVE: To determine the long-term prognosis of neurologic involvement in Behçet syndrome. DESIGN: Forty-six patients with Behçet syndrome, who had been the subjects of a previous report with short-term follow-up, were reexamined 7 years later; 42 of them could be reexamined neurologically. Neuropsychological testing, magnetic resonance imaging, electromyography, and evoked potential studies were performed when available. RESULTS: Of the 27 patients who had had headaches without any neurologic symptoms or signs previously, 2 had developed an acute neurologic attack. In addition, 7 patients in this group showed minor abnormalities on neurologic examination and/or other laboratory investigations, without history of any attacks. Among the previous neuro-Behçet group (n = 15), as defined by the presence of neurologic signs or symptoms, other than headache, 7 had a stationary course, while 8 had been progressive. Three of the latter group had died. Patients with progressive course had had abnormal cerebrospinal fluid findings at the time of the previous report, whereas patients with a stationary course had not. CONCLUSIONS: Silent neurologic involvement may occur in Behçet syndrome. Patients should undergo periodic neurologic evaluation. The long-term prognosis in neuro-Behçet syndrome does not seem to be as favorable as we observed in short-term follow-up. Cerebrospinal fluid findings may predict prognosis.

HLA-DR and -DQ associations with multiple sclerosis in Turkey.

The DRB, DQA, and DQB subregions of the major histocompatibility complex (MHC) were investigated by polymerase chain reaction and sequence-specific oligonucleotide probe hybridization (PCR/SSO) in 103 multiple sclerosis (MS) patients and 101 healthy controls from Turkey. Significant differences were detected between MS and control populations in the frequencies of DRB1*1501 [29 vs. 14, p = 0.02, odds ratio (OR) = 2.4], DRB1*04 (35 vs. 18, p = 0.01, OR = 2.3), DQB1*0302 (30 vs. 15, p = 0.02, OR = 2.3), DQB1*0602 (27 vs. 10, p = 0.005, OR = 3.2), DQB1*0501 (10 vs. 24, p = 0.01, OR = 0.3), DQA1*0101 (16 vs. 31, p = 0.02, OR = 0.4), and DQA1*0103 (7 vs. 19, p = 0.02, OR = 0.3). These results confirm the proposed positive association of the Dw2 (DRB1*1501 DQA1*0102 DQB1*0602) haplotype with MS in Caucasians in our Turkish population (25 vs. 8, p = 0.003, OR = 3.7). Furthermore, the "putative" haplotype supposed to be more frequent in the MS population of Mediterranean countries, namely DRB1*04 DQA1*03 DQB1*0302, is also associated with MS in Turkey (29 vs. 12, p = 0.006, OR = 2.9). The presence of two different haplotypic associations in MS emphasizes the complexity of the genetic susceptibility to MS in different populations.

Neuropsychological follow-up of 12 patients with neuro-Behçet disease.

We analyzed the data obtained from neuropsychological evaluations of 12 neuro-Behcet Disease (NBD) patients who had been followed up for 35.6+/-23.7 months with successive neuropsychological testing by a comprehensive battery. Memory impairment, which seems to stem basically from a retrieval deficit, was the major finding in this series. The most severely affected memory process was delayed recall, being impaired in all of the patients in the verbal and/or visual modalities. This was followed closely by an impairment in the process of acquisition and storage. In addition to the memory impairment, a "clinical impression of personality change" toward either disinhibition or apathy was seen in 8 of the 12 patients. Attention deficit was of the third highest frequency and was present in 7 patients, followed by deficits of executive functions of frontal system which were present in 5. Other cognitive domains were rarely involved. Neuropsychological status deteriorated insidiously, regardless of the neurological attacks during the follow-up period in most of the patients. Furthermore, our observations also showed the presence of cognitive decline prior to detectable lesions on CT or MRI, emphasizing the need for neuropsychological testing in NBD patients. The late stages of the disease seem to be reflected in MRI as an enlargement of the third ventricle and atrophy of the upper brainstem, which could be compatible with memory loss. Our series, a rather selected group, suggests that NBD can be associated with a special pattern of cognitive deficit, especially memory loss and personality change. The designation of any specific neurobehavioral syndrome for NBD, however, awaits further study.

Eye closure related spike and wave discharges: clinical and syndromic associations.

Precipitation of spike and wave (SW) discharges in some epileptic patients by eye closure (EC) has rarely been reported. To disclose the clinical characteristics and classification of syndromes of epileptic patients with SW discharges induced by EC, we investigated 10 patients (1 M, 9 F) showing this peculiar EEG feature. The patients aged between 9-39 years (mean 20.6 +/- 9.058), underwent short-term (1-3.5 hr) video-EEG investigations in order to document the appearance of the SW discharges within 3 seconds of the act of EC, in at least two occasions. Clinical analysis showed that 5 female patients who had the syndrome of juvenile myoclonic epilepsy (JME) had a later onset of epilepsy (13-15 years) than the 3 patients (3 girls) with eyelid myoclonia with absences (EMA) (3-8 years of age at onset). The remaining 2 patients who were diagnosed as childhood absence epilepsy (CAE) and juvenile absence epilepsy (JAE) according to the international classification, did not show photosensitivity on the video-EEG. All but one of the 5 JME patients had experienced myoclonic seizures in intermittent photic stimulation (IPS) at the time of EC, associated with multiple spike and wave discharges. Two of the 3 EMA patients exhibited typical absences with eyelid myoclonia during the act of EC. The high rate of family history of epilepsy in first degree relatives of our patients was an outstanding feature, which could have future implications in research of the genetic basis of epilepsy patients with ECS.

A clinically recognizable neuronal migration disorder: congenital bilateral perisylvian syndrome. Case report with long-term clinical and EEG follow-up.

Congenital bilateral perisylvian syndrome (CBPS) is a recently described, neuronal migration disorder, characterized by pseudobulbar palsy, epilepsy and mental retardation and bilateral perisylvian dysplasia. A 15-year-old boy was diagnosed with CBPS according to the typical clinical, and magnetic resonance imaging (MRI) features. The patient was suffering from atypical absence seizures, repeating daily in spite of antiepileptic drug therapy, since age 7 years. He had also experienced rare generalized tonic-clonic seizures and complex partial seizures. Neurological examination showed severe restriction of tongue movements, severe dysarthria, dysphagia, facial diplegia, mild pyramidal signs and moderate mental retardation. A computed tomographic (CT) scan demonstrated bilateral perisylvian enlargement. The diagnosis was corrected with MRI after six years. Frequent irregular generalized spike and wave abnormalities and focal sharp and slow waves over the posterior regions of both hemispheres were shown by electroencephalograms (EEG). The patient was treated with Na-Valproate, carbamazepine and lamotrigine but did now show any significant change in seizure frequency in the eight-year follow-up period. Intractable seizures, mental retardation and particularly congenital pseudobulbar palsy suggest this congenital entity. Those patients who exhibit these typically clinical features, must have MRI.

A controlled study of reliability and validity of MRI findings in neuro-Behçet's disease.

Our aim was to test the reliability of interpreting MRI studies in neuro-Behçet's disease (NBD) and to determine the sensitivity and specificity of different MRI findings. We prospectively studied 50 patients: 24 had chronic NBD, 12 multiple sclerosis, 5 vasculitis other than Behçet's disease (BD) and 9 patients had BD without neurological involvement. MRI studies were performed according to a standard protocol with a 0.2 T imager. Two neuroradiologists, blinded to the diagnosis, age and sex of the subjects, reviewed the films independently, twice. Separate assessments were made for a set of items: dural sinus pathology, widening of ventricles and sulci, brain stem atrophy, lesions of the cerebral cortex, discrete lesions of deep white matter, basal ganglia, brain stem and cerebellum and the presence of smooth periventricular high-signal foci. Intraobserver agreement was substantial or better, and interobserver agreement moderate to substantial for most items. In these patients with chronic NBD we found low sensitivity on all assessed items. Dural sinus pathology or brain stem atrophy were highly specific, but parenchymal lesions in different sites had uniformly low specificity.