Electrodiagnostic methods to verify Guillain-Barré syndrome subtypes in Istanbul: A prospective multicenter study.

BACKGROUND AND AIMS: This study aimed to identify the clinical characteristics and electrodiagnostic subtypes of Guillain-Barré syndrome (GBS) in Istanbul. METHODS: Patients with GBS were prospectively recruited between April 2019 and March 2022 and two electrodiagnostic examinations were performed on each patient. The criteria of Ho et al., Hadden et al., Rajabally et al., and Uncini et al. were compared for the differentiation of demyelinating and axonal subtypes, and their relations with anti-ganglioside antibodies were analyzed. RESULTS: One hundred seventy-seven patients were included, 69 before the coronavirus disease 2019 pandemic (April 2019-February 2020) and 108 during the pandemic (March 2020-March 2022), without substantial changes in monthly frequencies. As compared with the criteria of Uncini et al., demyelinating GBS subtype diagnosis was more frequent according to the Ho et al. and Hadden et al. criteria (95/162, 58.6% vs. 110/174, 63.2% and 121/174, 69.5%, respectively), and less frequent according to Rajabally et al.'s criteria (76/174, 43.7%). Fourteen patients' diagnoses made using Rajabally et al.'s criteria were shifted to the other subtype with the second electrodiagnostic examination. Of the 106 analyzed patients, 22 had immunoglobulin G anti-ganglioside antibodies (14 with the axonal subtype). They had less frequent sensory symptoms (54.5% vs. 83.1%, p = 0.009), a more frequent history of previous gastroenteritis (54.5% vs. 22.9%, p = 0.007), and a more severe disease as compared with those without antibodies. INTERPRETATION: Serial electrodiagnostic examinations are more helpful for accurate subtype diagnosis of GBS because of the dynamic pathophysiology of the disease. We observed no significant increase in GBS frequency during the pandemic in this metropolis.

The effect of sleep disorders on quality of life in patients with epilepsy: A multicenter study from Turkey.

OBJECTIVE: We aimed to investigate sleep disorders in patients with epilepsy (PWE) and to investigate the effects of sleep disorders on quality of life. METHODS: In our multicenter study conducted in Turkey, 1358 PWE were evaluated. The demographic and clinical data of the patients were recorded. The Insomnia Severity Index (ISI), Epworth Sleepiness Scale (ESS), Pittsburgh Sleep Quality Index (PSQI), Beck Depression Inventory (BDI), and Quality of Life in Epilepsy Inventory-10 (QOLIE-10) were administered. RESULTS: The mean age of 1358 patients was 35.92 ±â€¯14.11 (range, 18-89) years. Seven hundred fifty-one (55.30 %) were women. Some 12.7 % of the patients had insomnia (ISI > 14), 9.6 % had excessive daytime sleepiness (ESS > 10), 46.5 % had poor sleep quality (PSQI > 5), and 354 patients (26.1 %) had depressive symptoms (BDI > 16). The mean QOLIE-10 score was 22.82 ±â€¯8.14 (10-48). Resistant epilepsy was evaluated as the parameter with the highest risk affecting quality of life Adjusted odds ratio (AOR = 3.714; 95 % confidence interval (CI): [2.440-5.652] < 0.001)). ISI (AOR = 1.184; 95 % CI: [1.128-1.243]; p < 0.001), ESS (AOR = 1.081; 95 % CI: [1.034-1.130]; p < 0.001), PSQI (AOR = 0.928; 95 % CI: [0.867 - 0.994]; p = 0.034), BDI (AOR = 1.106; 95 % CI: [1.084-1.129]; p < 0.001), epilepsy duration (AOR = 1.023; 95 % CI: [1.004-1.041]; p = 0.014), were determined as factors affecting quality of life. SIGNIFICANCE: Sleep disorders are common in PWE and impair their quality of life. Quality of life can be improved by controlling the factors that may cause sleep disorders such as good seizure control, avoiding polypharmacy, and correcting the underlying mood disorders in patients with epilepsy.

Evaluating vertebrobasilar insufficiency and Meniere's disease: Insights from cervical vestibular evoked myogenic potential and video head impulse test.

Background and purpose:

This prospective study aimed to investigate diffe-rences in video head impulse test (vHIT) and cervical vestibular evoked myogenic poten-tial (cVEMP) findings between patients with vertebrobasilar insufficiency (VBI) and Meniere’s disease (MD) who experience episodic vertigo attacks.

A total of 27 patients with VBI and 37 patients with MD were enrolled into the study in a tertiary referral center. Inclusion criteria consisted of patients with a minimum of two previous vertigo attacks, unaccompanied by any neurological symptoms during an attack. All patients underwent horizontal canal h-vHIT and c-VEMP assessments following pure sound audiometric examinations. First, vHIT and cVEMP results for low and high flow sides in VBI patients were analyzed. Subsequently, data from the low-flow side in VBI patients and the affected side in MD patients were compared.

The mean vHIT values for low and high-flow volume sides in VBI patients were 0.68 and 0.88, respectively. In MD patients, mean vHIT values for affected and healthy sides were measured as 0.77 and 0.87, respectively. Abnormal results were observed in 66.7% of VBI patients and 51.4% of MD patients, with no statistically significant difference between the findings (p> 0.05). Upon examining the affected side, c-VEMP responses were absent in 41% of MD patients and 48% of VBI patients, with no statistically significant difference between the groups (p> 0.05).

vHIT and cVEMP assessments can be utilized as supplementary tools to radiologic investigations for the clinical diagnosis and follow-up of VBI. However, no significant differences were observed between vHIT and cVEMP findings in patients with MD and VBI.

Acquired hepatocerebral degeneration.

Acquired hepatocerebral degeneration is a rare, mostly irreversible neurological syndrome that occurs in patients with chronic liver disease, particularly in those with surgically or spontaneously induced portosystemic shunts. Typical magnetic resonance findings are T1 hyperintensity in the pallidum, substantia nigra, periaquaductal gray matter. In this paper, we report a case of a 51-year-old woman presented with hepatic encephalopathy episodes and typical magnetic resonance findings, who does not develop any neurological signs or symptoms, nor cognitive decline in the follow up period, lasting for 3 years.

An association analysis at 2q36 reveals a new candidate susceptibility gene for juvenile absence epilepsy and/or absence seizures associated with generalized tonic-clonic seizures.

PURPOSE: To further evaluate the previously shown linkage of absence epilepsy (AE) to 2q36, both in human and WAG/Rij absence rat models, a 160-kb region at 2q36 containing eight genes with expressions in the brain was targeted in a case-control association study involving 205 Turkish patients with AE and 219 controls. METHODS: Haplotype block and case-control association analysis was carried out using HAPLOVIEW 4.0 and inhibin alpha subunit (INHA) gene analysis by DNA sequencing. KEY FINDINGS: An association was found between the G allele of rs7588807 located in the INHA gene and juvenile absence epilepsy (JAE) syndrome and patients having generalized tonic-clonic seizures (GTCS) with p-values of 0.003 and 0.0002, respectively (uncorrected for multiple comparisons). DNA sequence analysis of the INHA gene in 110 JAE/GTCS patients revealed three point mutations with possible damaging effects on inhibin function in three patients and the presence of a common ACTC haplotype (H1) with a possible dominant protective role conferred by the T allele of rs7588807 with respective p-values of 0.0005 and 0.0014. SIGNIFICANCE: The preceding findings suggest that INHA could be a novel candidate susceptibility gene involved in the pathogenesis of JAE or AE associated with GTCS.

The Prevalence of Headache in Crohn's Disease: Single-Center Experience.

Objectives. This study is aimed at studying the prevalence and characteristics of different types of headaches in patients with Crohn's disease. Materials and Methods. 51 patients in Crohn's disease group (F/M: 26/25) and 51 patients in control group (F/M: 27/24) were involved. Patients in Crohn's disease group were diagnosed and monitored according to European Crohn's and Colitis Organization diagnostic criteria. The control group composed of healthy subjects with similar age and sex to Crohn's disease group. Headache was classified using the International Headache Society II criteria. Results. Headache was reported by 35/51 (68.6%) patients in Crohn's disease group and 21/51 (41.2%) patients in the control group. The prevalence of headache was statistically high in the group with Crohn's disease (OR: 3.125 (95% CI: 1.38-7.04); p = 0.01). Comparing two groups with respect to their subtypes of headaches resulted in that the tension-type headache was statistically (p = 0.008) higher in Crohn's disease group (26/51) than in the control group (12/51). However, no significant difference was found in the migraine-type headache (p = 1). Conclusions. This study indicates that the prevalence of headache is high in patients with Crohn's disease and most commonly associated with the tension-type headache.

Tonic seizures in subacute sclerosing panencephalitis: a video-illustration of two cases.

Subacute sclerosing panencephalitis is a progressive disorder which also presents with various types of seizures, mainly myoclonic jerks, atonic attacks and tonic-clonic seizures. We report two cases, documented by video-EEG that during the course of the disease also presented with tonic seizures. The differential diagnosis of non-epileptic paroxysmal events might prove to be a problem.

Myoclonus-dystonia syndrome: case report.

Myoclonus-dystonia syndrome (MDS) is a rare disease manifesting myoclonus as the only neurological symptom which may be accompanied by dystonia. It usually starts in the first or second decade of life. It has a benign course with spontaneous remissions but can cause functional disability in some patients. In this paper, we report a patient diagnosed as probable MDS on the basis of clinical and electrophysiological features who showed marked improvement under levetiracetam treatment.

Triphasic EEG pattern in bilateral paramedian thalamic infarction.

Two cases of bilateral paramedian thalamic infarction (BPTI) showing triphasic waves (TWs) on the electroencephalogram (EEG) at acute stage are presented in this study. BPTI is a rare syndrome with decreased level of consciousness, gaze abnormalities and cognitive deterioration. TWs are nonspecific EEG findings occurring in both metabolic and nonmetabolic conditions. The TWs in BPTI might be related to level of consciousness and does not always predict a poor prognosis in BPTI.

Hot water epilepsy with cerebral lesion: a report of five cases with cranial MRI findings.

Hot water epilepsy (HWE) is included in the reflex epilepsies. Although, in general, not common, HWE is concentrated in certain regions of the world. Different bathing habits and genetic factors may be responsible for the high incidence of HWE in these regions. However, the exact pathogenesis of HWE is not known. The facts that complex partial seizures are the most common clinical presentation and EEG recordings show an epileptic focus in the temporal lobe suggested the presence of a structural lesion in the temporal lobe. To our knowledge, however, there were no demonstrable structural changes on MRI and CT scans except in a few case reports. Here, we describe an additional five cases of HWE having an intracranial pathology, for example, hippocampal sclerosis, dysplasia, and a huge cystic lesion. We believe that investigations with new detailed neuroimaging techniques, in addition to experimental and clinical studies, might help us to understand the mechanism of this reflex epilepsy.