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.

Comparison of the Oral Health-Related Quality of Life, Sleep Quality, and Oral Health Literacy in Sleep and Awake Bruxism: Results from Family Medicine Practice.

Objectives: Bruxism is a common oral behaviour. This study aimed to compare oral health-related quality of life, sleep quality, and oral health literacy in patients with and without possible sleep bruxism (SB) and awake bruxism (AB). Materials and Methods: A cross-sectional study including 249 volunteers was conducted in a family medicine clinic of a tertiary hospital in Istanbul, Turkey. The American Sleep Medicine Association Bruxism Diagnostic Criteria, Pittsburgh Sleep Quality Index (PSQI), Oral Health-Related Quality of Life (OHRQoL) tool, Decay Missing Filled Total Teeth (DMFT) score, and Health Literacy Dental Scale-Short Form (HeLD-14) were assessed by face-to-face interviews. Data were examined using Kruskal-Wallis and Mann-Whitney U tests, Spearman correlation, and logistic regression analysis. Results: The presence of SB and AB was detected as 41.4% and 21.7%, respectively, among 91 males and 158 females, with a mean age of 36.64 ± 11.60 years. Sleep and awake bruxers had a lower oral health-related quality of life (odds ratio (OR): 0.816, 95% confidence interval (CI) = 0.770-0.864 and OR: 0.923, 95% CI = 0.956-0.982, respectively). Poor sleep quality was detected 1.28 times higher in sleep bruxism (OR: 1.277, 95% CI = 1.152-1.415) and 1.14 times higher in awake bruxism (OR: 1.141, 95% CI = 1.230-1.058). The DMFT score was found to be 1.13 times higher in SB (OR: 1.129, 95% CI = 1.043-1.223). A higher HeLD-14 score was associated with a lower DMFT score (p < 0.001; r = -0.240). The oral health literacy score was lower in AB and SB groups than in patients without bruxism, but it was not statistically significant (p = 0.267, p = 0.376). Conclusion: A lower oral health-related quality of life and poor sleep quality would be expected in the presence of SB or AB. However, patients may not be aware of it unless asked by a physician regardless of oral health literacy level.

Long-term electrophysiological assessment after hypoglossal-facial anastomosis.

PURPOSE: To investigate and provide objective documentation of the possible differences in the axonal reinnervation process of facial muscles after hypoglossal-facial nerve anastomosis. Then, to search for the presence of the trigemino-hypoglossal reflex and determine whether it indicates better peripheral recovery. METHODS: Electrophysiological examination performed on 20 patients who had undergone VII-XII anastomosis, with follow-up periods of more than 2 years. RESULTS: The mean follow-up time after surgery was 4.1 ± 1.3 years (range 2-8 years). The degrees of axonal reinnervation for the orbicularis oculi (OOc) and orbicularis oris (OOr) were 46.91 ± 19.77 and 32.65 ± 14.85, respectively. And the difference between these muscles was statistically significant (p = 0.018) in favor of the OOc. In addition, R1 blink reflexes that were not followed by R2 components were observed in 30% of the patients. However, these 6 patients with short-latency potential did not differ from the others in terms of latency, the amplitude of compound muscle action potential (CMAP), and degree of axonal reinnervation (p > 0.05) at both muscles (OOc and OOr). CONCLUSION: The recoveries of the lower face and upper face are different after VII-XII anastomosis, and in our patients the OOc healed better. In addition, R1 blink reflexes that were not followed by R2 components were observed in 30% of the patients. However, the patients with these blink reflexes did not have better peripheral healing in their neuromuscular units, which suggests that the blink reflex is not an indicator for peripheral recovery.

Spinal excitability changes do not influence the mechanisms of split-hand syndrome in amyotrophic lateral sclerosis.

INTRODUCTION: We studied spinal excitability and its relation to motor unit action potential (MUAP) changes in the thenar and hypothenar muscles in patients with amyotrophic lateral sclerosis (ALS). METHODS: Quantitative MUAP and peak ratio interference analyses were performed, and cutaneous silent period (CutSP) was measured in the abductor digiti minimi (ADM) and first dorsal interosseous (FDI) muscles of patients with ALS and controls. RESULTS: Patients with ALS revealed more prominently increased duration (P = 0.002), number of phases (P = 0.02), size index (P = 0.02), and thickness (P = 0.02) in ADM than in FDI. The number of small segments was significantly decreased in ADM (P = 0.036) compared with controls. CutSP latency was prolonged in both ADM and FDI in ALS (P < 0.05). DISCUSSION: Although the reinnervation capacity was notably higher in the ADM of patients with ALS, the lack of a significant difference in CutSP measurements between ADM and FDI argues against spinal excitability changes in the development of split-hand syndrome. Muscle Nerve 58: 503-508, 2018.

Peripheral neuropathy in Tangier disease: A literature review and assessment.

Tangier disease (TD) (OMIM#205400) is a rare cause of inherited metabolic neuropathies characterized by marked deficiency of high-density lipoproteins and accumulation of cholesterol esters in various tissue resulting from reverse cholesterol transport deficiency. We report a case of a patient with TD with multifocal demyelinating neuropathy with conduction block who presents with winging scapula, tongue, and asymmetric extremity weakness. We also present a review of all studies published from 1960 to 2017 regarding peripheral neuropathy in TD. Our search identified 54 patients with TD with peripheral neuropathy. Syringomyelia-like neuropathy subtype (52.4%) was more frequent than multifocal sensorial and motor neuropathy subtype (26.2%), focal neuropathy subtype (19.1%), and distal symmetric polyneuropathy subtype (2.4%). Splenomegaly was the most common (40.7%) clinical manifestation in these patients. The pattern of electrodiagnostic abnormalities are: (1) demyelinating abnormalities were more predominant in the upper extremities than in the lower extremities and (2) slowing of motor nerve conduction was more prominent in the intermediate segment than in distal nerve segments. The sural-sparing pattern was present in 34.6% and conduction block was present in 11.5% of the patients. Our literature review and our case showed the clinical spectrum of TD neuropathy is quite wide and that it should be considered in the differential diagnosis of non-uniform demyelinating neuropathies.

Ictal body turning in focal epilepsy.

Despite the explanations of many lateralization findings, body turning in focal epilepsy has been rarely investigated. One of the aims of this study was to evaluate the role of ictal body turning in the lateralization of focal epilepsies. The records of 263 patients with focal epilepsy (temporal lobe epilepsy (TLE), n=178; extratemporal lobe epilepsy (ETLE), n=85) who underwent prolonged video-EEG monitoring during presurgical epilepsy evaluation were reviewed. Preoperative findings (TLE, n=16; ETLE, n=6) and postoperative outcomes (TLE, n=7) of patients with focal epilepsy with ictal body turning were assessed. For the evaluation of ictal body turning, two definitions were proposed. Nonversive body turning (NVBT) was used to denote at least a 90° nonforced (without tonic or clonic component) rotation of the upper (shoulder) and lower (hip) parts of the body around the body axis for a minimum of 3s. Versive body turning (VBT) was used to denote at least a 90° forced (with tonic or clonic component) rotation of the upper (shoulder) and lower (hip) parts of the body around the body axis for a minimum of 3s. Nonversive body turning was observed in 6% (n=11) of patients with TLE and 2% (n=2) of patients with ETLE. For VBT, these ratios were 5% (n=8) and 7% (n=6) for patients with TLE and ETLE, respectively. Nonversive body turning was frequently oriented to the same side as the epileptogenic zone (EZ) in TLE and ETLE seizures (76% and 80%, respectively). If the amount of NVBT was greater than 180°, then it was 80% to the same side in TLE seizures. Versive body turning was observed in 86% of the TLE seizures, and 55% of the ETLE seizures were found to be contralateral to the EZ. When present with head turning, NVBT ipsilateral to the EZ and VBT contralateral to the EZ were more valuable for lateralization. In TLE seizures, a significant correlation was found between the head turning and body turning onsets and durations. Our study demonstrated that ictal body turning is a rarely observed but reliable lateralization finding in TLE and ETLE seizures, which also probably has the same pathophysiological mechanism as head turning in TLE seizures.

The Profile of Guillain-Barré Syndrome Before and During COVID-19 Pandemic: A 5-Year Experience.

Introduction: We aimed to evaluate and compare the clinical and electrophysiological features of post-COVID-19 Guillain-Barré syndrome (GBS) and non-COVID-19 GBS patients over the last five years. Methods: We retrospectively analyzed patients diagnosed with GBS between October 2016 and September 2021. They were divided into five groups according to the flu season and the COVID-19 pandemic. Groups 1-4 were identified as non-COVID-19 groups, whereas Group 5 constituted the post-COVID-19 group. At the sixth month the Hughes functional grading scale score (HFGSS) was noted. Clinical and electrophysiological findings were compared between the groups. Results: Fifty-nine GBS patients were enrolled in this study. Post-COVID-19 GBS patients had more facial diplegia than non-COVID-19 GBS patients. Except for facial diplegia, post-COVID-19 GBS patients did not differ from non-COVID-19 GBS patients regarding the need for mechanical ventilation, loss of ambulation, type of GBS, response to treatment, and patient outcomes. In 67% of post-COVID-19 GBS patients, HFGSS was ≤2. Acute inflammatory demyelinating polyneuropathy (AIDP) was the most common subtype in post-COVID-19 GBS patients. The 2018-2019 flu season saw more ambulation loss than other flu seasons. The 2017-2018 influenza season had the highest number of patients (39%). Conclusion: The clinical and electrophysiological features of GBS may differ according to year, infectious etiology, and severity of seasonal viral infections. Post-COVID-19 GBS patients mostly had the AIDP subtype with frequent facial diplegia. The prognosis of post-COVID-19 GBS patients was good. The patients responded well to treatment with intravenous immunoglobulin and plasma exchange.

CD4+ T Cells of Myasthenia Gravis Patients Are Characterized by Increased IL-21, IL-4, and IL-17A Productions and Higher Presence of PD-1 and ICOS.

Myasthenia gravis (MG) is an autoimmune disease mediated by autoantibodies predominantly against the acetylcholine receptor (AChR). Specific T cell subsets are required for long-term antibody responses, and cytokines secreted mainly from CD4+ T cells regulate B cell antibody production. The aim of this study was to assess the differences in the cytokine expressions of CD4+ T cells in MG patients with AChR antibodies (AChR-MG) and the effect of immunosuppressive (IS) therapy on cytokine activity and to test these findings also in MG patients without detectable antibodies (SN-MG). Clinically diagnosed AChR-MG and SN-MG patients were included. The AChR-MG patients were grouped as IS-positive and -negative and compared with age- and sex-matched healthy controls. Peripheral blood mononuclear cells were used for ex vivo intracellular cytokine production, and subsets of CD4+ T cells and circulating follicular helper T (cTfh) cells were detected phenotypically by the expression of the chemokine and the costimulatory receptors. Thymocytes obtained from patients who had thymectomy were also analyzed. IL-21, IL-4, IL-10, and IL-17A productions in CD4+ T cells were increased in AChR-MG compared to those in healthy controls. IS treatment enhanced IL-10 and reduced IFN-γ production in AChR-MG patients compared to those in IS-negative patients. Increased IL-21 and IL-4 productions were also demonstrated in SN-MG patients. Among CD4+ T cells, Th17 cells were increased in both disease subgroups. Treatment induced higher proportions of Th2 cells in AChR-MG patients. Both CXCR5+ and CXCR5- CD4+ T cells expressed higher programmed cell death protein 1 (PD-1) and inducible costimulatory (ICOS) in AChR-MG and SN-MG groups, mostly irrespective of the treatment. Based on chemokine receptors on CXCR5+PD-1+ in CD4+ T (cTfh) cells, in AChR-MG patients without treatment, the proportions of Tfh17 cells were higher than those in the treated group, whereas the Tfh1 cells were decreased compared with those in the controls. The relevance of CXCR5 and PD-1 in the pathogenesis of AChR-MG was also suggested by the increased presence of these molecules on mature CD4 single-positive thymocytes from the thymic samples. The study provides further evidence for the importance of IL-21, IL-17A, IL-4, and IL-10 in AChR-MG. Disease-related CD4+T cells are identified mainly as PD-1+ or ICOS+ with or without CXCR5, resembling cTfh cells in the circulation or probably in the thymus. AChR-MG and SN-MG seem to have some similar characteristics. IS treatment has distinctive effects on cytokine expression.

Chronic pain after Lichtenstein and preperitoneal (posterior) hernia repair.

BACKGROUND: Hernia repair is one of the most common surgical procedures, and some patients suffer from chronic pain after hernia surgery. The aim of the present study was to evaluate chronic postherniorrhaphy pain in men who underwent Lichtenstein mesh repair or preperitoneal (posterior) repair. METHODS: Our study included 94 male inpatients. Two surgeons experienced in both Lichtenstein and preperitoneal hernia repair performed the procedures. We controlled postoperative pain with systemic analgesic therapy. We evaluated the patients over the subsequent 12 months, using a questionnaire to focus on chronic pain and its limitations to their quality of life. RESULTS: The overall incidence of chronic pain at 2 months was 5%. About 6% of patients who underwent Lichtenstein repair (n = 70) and 4% of patients who underwent preperitoneal repair (n = 24) experienced chronic pain. All patients with chronic pain rated their pain as slight or moderate. Their pain was present occasionally and was related to physical stress. None of the patients were unable to work. After 12 months of follow-up, the overall incidence of chronic pain decreased to 3%, with 3 patients in Lichtenstein group reporting chronic pain with slight limitations in sports and social activities. CONCLUSION: The incidence rates of chronic pain after Lichtenstein and preperitoneal repair were 6% and 4%, respectively. Inpatient status might have resulted in low incidences with both approaches.

A case of sarcoidosis of the central nervous system and orbita.

Sarcoidosis is a multisystemic disease characterized by granulomatous inflammation. Lung or lymph node involvement is common. We present a rare case of sarcoidosis that began with orbital involvement, and a month later, due to insufficient treatment, it involved the central nervous system. A 49-year-old female patient began suffering from swelling in her right eye, redness, ptosis, and limited eye movements two months ago. Gadolinium-enhanced orbital magnetic resonance imaging showed thickening of the lacrimal gland and the right medial rectus muscle. After three weeks of local antibiotic and steroid treatments, her symptoms were resolved. One month ago, the patient reported sudden weakness in her right arm and leg. After laboratory tests and imaging studies, the patient was diagnosed with probable neurosarcoidosis using the Zajicek criteria and treated with prednisone (1 mg/kg/day). Although sarcoidosis frequently presents with lung and lymph node involvement, it is rarely accompanied by orbital involvement. Patients with orbital symptoms may receive a late diagnosis and insufficient central nervous system treatment. Involvement of the central nervous system in sarcoidosis leads to high morbidity and mortality rates. Therefore, early diagnosis and treatment are very important.