Ahmet Gökçay

Headache in primary Sjögren’s syndrome: a prevalence study

Objectives –  To determine the prevalence of headache in patients with primary Sjögren’s syndrome (pSS) and to examine the relationship between headache types and clinical, serologic features of the disease.

Visual evoked potentials in children with occipital epilepsies

The objectives of this study are to see if any visual evoked potential (VEP) differences are present in two forms of occipital epilepsy, childhood epilepsy with occipital paroxysms (CEOP) and symptomatic occipital epilepsy (SOE) with respect to etiology, as CEOP is a benign age- and localization-related idiopathic epilepsy while SOE is a symptomatic form. Nineteen patients with CEOP and 13 patients with SOE were included in the study and P100 potential latency and amplitude values obtained from these patients were compared with the values recorded from normal controls. The amplitude values recorded from the patients with CEOP were significantly high (P=0.033). P100 potential latency values recorded in patients with SOE were significantly long (P=0.028). High amplitude VEP responses were mostly attributed to hyperexcitability of the occipital cortical structures whereas prolonged latency P100 responses were attributed to occipital structural changes.

Proton MR spectroscopy in Rett syndrome

Seven patients (mean age 7.7yr) with Rett syndrome, a condition with progressive regression of psychomotor development are included in this study. Proton MR spectroscopy images were obtained with the multivoxel chemical-shift imaging mode (TR=1500ms, TE=40ms). Spectra from 224 voxels in the brain parenchyma were studied. N-acetyl aspartate (NAA), creatine (Cr), choline (Cho), and myoinositol (mI) peaks were quantitatively evaluated, and NAA/Cr, NAA/Cho, and Cho/Cr, mI/Cr ratios were calculated. Five age-matched normal cases were available as controls. In three patients with Rett syndrome spectroscopy findings were normal, and the metabolite ratios were similar to control cases. In the remaining four patients with the syndrome prominent decrease of the NAA peak was the main finding resulting in decreases in NAA/Cr (1.14+/-17), and NAA/Cho (1.08+/-27) ratios (p<0.0001). Cho/Cr ratios (0.93+/-26), and mI/Cr ratios (0.88+/-36) were normal compared to controls. There was no correlation between spectroscopic changes and clinical status of the patients. The findings suggested that not only reduced neuronal-dendritic arborizations but also decreased neuronal function could contribute to spectroscopy changes in Rett syndrome.

Cognitive functions evaluated by P300 and visual and auditory number assays in children with childhood epilepsy with occipital paroxysms (CEOP)

PURPOSE This study was planned to evaluate cognitive functions, especially attention and immediate recall, in children with childhood epilepsy with occipital paroxysms (CEOP), by using P300 and neuropsychological tests, which included visual and auditory number assays. Thirty patients with CEOP, ages ranging from 5 to 17 years were enrolled in the study. Twenty-five healthy children were taken as the control group. METHODS Oddball paradigm was used in P300 recordings. The latency and the amplitude of the P300 wave recorded from Cz were taken into consideration. The neuropsychological test battery included visual and auditory number assays. RESULTS P300 latency was significantly longer in the CEOP group (p=0.014). The results of the visual and auditory number assay test showed significant decline in the patient group when compared with the normal controls. CONCLUSIONS Attention and immediate recall deficits as well as prolonged P300 latencies in children with CEOP can be due to an ongoing epileptic activity either influencing the whole brain or only the occipital lobe which can also be involved in the neuropsychological organization of the human cortex. Therefore, children with CEOP should be evaluated with more detailed neuropsychological tests for possible cognitive deficits.

Atypical EEG findings in subacute sclerosing panencephalitis

OBJECTIVE To evaluate atypical electroencephalographic features in subacute sclerosing panencephalitis (SSPE) and to detect its relation to clinical features. METHODS Twenty-two patients aged 2-17 years (mean 9.4 years) with definite diagnosis of SSPE were studied. Their clinical data and EEG records were reviewed retrospectively. All EEG records were analysed for features of periodic complexes (PCs) in relation to age, age at onset, clinical stage and the rate of progression as well as duration of the disease. RESULTS Classical periodic complexes of SSPE were found in EEGs of 13 patients (group I). Atypical patterns were observed in EEGs of nine patients (group II). Two new atypical findings were identified: prolonged discharges which include sharp waves and slow waves for 4-7 s followed by suppression for 1-4s; and periodic complexes which consist of four or five sharp waves in every 2 s. We observed atypical EEG patterns were more frequently in Stage III, acute form, and the disease duration was longer than in the typical group. CONCLUSIONS Atypical EEG patterns in SSPE might be related to the progression of the disease, but this theory needs further longitudinal studies. SIGNIFICANCE We suggest atypical EEG patterns might be observed more frequently in patients with severe neurologic disability, more rapidly progressive disease and longer duration of disease.

Neurological symptoms and signs in Behçet disease: a Western Turkey experience.

AimTo assess the clinical patterns of neurologic involvement and the frequencies and characteristics of different types of headaches in patients with Behçet disease. MethodsTotal 530 patients with Behçet disease were evaluated for the presence of neurologic involvement and/or headache. ResultsOf the 54 patients (10.2%) with neurological involvement, 36 (66.7%) had parenchymal and 16 (29%) had vascular involvement. There was a patient with optic neuropathy and another patient with peripheral nerve involvement. There were 46 patients (8.7%) with headache without any evidence of neurological involvement and 25 of them (4.7%) were diagnosed with migraine. ConclusionsThe prevalence of neurologic involvement in our group was higher than the studies published previously from the same country. Migraine prevalence on the other hand was low.

Acquired Chiari I Malformation Changes Postendoscopic Third Ventriculostomy

Accessible online at: www.karger.com/journals/pne A 12-year-old boy presented with unsteadiness and neck pain for the past month. He was alert and cooperative. On neurological examination, ataxic gait and dysarthria were noted. Magnetic resonance imaging (MRI) scans revealed Chiari I malformation and ventricular dilatation (fig. 1). The foramen Magendie seemed to be obstructed. We decided to perform endoscopic third ventriculostomy (ETV). ETV was done, using a zero-degree rigid channel neuroendoscope (Medtronic PS Medical, Goleta, Calif., USA). His symptoms disappeared shortly after ETV. A slight ascent of the cerebellar tonsils and cerebrospinal fluid flow void through the third ventricular floor were noted on the postoperative MRI scans (fig. 2). The patient has been symptom free and without neurological deficit for 6 months.

ADEM: diffusion MRI findings

Abstract Acute disseminated encephalomyelitis (ADEM) usually reveals patchy demyelinated lesions with high signal on T2-weighted sequences. Diffusion MRI in a 10-year-old patient with ADEM demonstrated two distinct lesion patterns occurring simultaneously in the cerebral white matter. These were a restricted diffusion pattern (high-signal on b =1000 mm 2 /s images with low apparent diffusion coefficient (ADC) values, and a demyelinating pattern (negative b =1000 mm 2 /s images with high ADC values). The patient responded well to intravenous methylprednisolone therapy. Further studies are required for detailed evaluation of such patterns in ADEM, and their clinical relevance, and for discrimination from other inflammatory encephalopathies.

Four novel and two recurrent NHLRC1 (EPM2B) and EPM2A gene mutations leading to Lafora disease in six Turkish families

Lafora disease (LD) is a type of autosomal recessive, progressive myoclonus epilepsy resulting mostly from mutations in the EPM2A and NHLRC1 genes. Mutational analysis in both genes was initiated with the aim of establishing LD DNA diagnosis in Turkey. Four novel NHLRC1 (p.G131X, p.P69S and p.D82H) and EPM2A (p.V7A) and two recurrent NHLRC1 (p.D146N) and EPM2A (p.R241X) mutations were identified in six families. The delineation of causative mutations in patients provided early disease diagnosis for other family members and contributed to the knowledge of LD pathogenesis.

Sydenham chorea and Hashimoto thyroiditis: an unusual association.

Sydenham chorea is an immune-mediated neuropsychiatric disorder associated with group A β-hemolytic streptococci infection. The authors present a 12-year-old female patient with Sydenham chorea and Hashimoto thyroiditis. Although Hashimoto thyroiditis has been associated with other autoimmune disorders, the association of Sydenham chorea and Hashimoto thyroiditis has not been previously reported. Immunological processes are suggested to be implicated in the pathogenesis of this association; however, the exact mechanism remains unclear.