Distinctive sleep complaints and polysomnographic findings in antibody subgroups of autoimmune limbic encephalitis.

INTRODUCTION: Sleep disturbances are being increasingly recognized in association with autoimmune encephalitis (AIE). We investigated the prevalence of sleep-related symptoms and polysomnographic features of patients with AIE and the long-term outcomes in these patients in a multi-center, prospective study from Turkey. METHODS: We prospectively evaluated patients with definite AIE in a common database including demographics, AIE-related and sleep-related symptomatology. Maximum and latest modified Rankin scores (mRS) and Liverpool Outcome Score (LOS) were noted. RESULTS: Of 142 patients, 87 patients (61.3%) fulfilled the criteria for definite AIE (mean age, 46.8+18.8 years; 51.7% women; mean disease duration, 21.0+38.4 months). 78.9% of patients had at least one or more new onset or worsened sleep-related symptomatology: insomnia (55.3%), excessive daytime sleepiness (EDS, 28.0%), sleep apnea (18.7%), REM sleep behavior disorder (RBD, 17.3%), restless legs syndrome (10.7%) and oneiric stupor (9.3%). Sleep efficiency, N3 and REM sleep were decreased and N1 sleep was increased in patients with Ab[+] AIE. LOS points were highest in those with insomnia and sleep apnea, and lowest in those with EDS, RBD and oneiric stupor. RBD and sleep apnea were more common in anti-LG1 Ab[+] group than anti-NMDAR Ab[+] group. Index of periodic leg movements was highest in anti-LG1 Ab[+] group. Patients with EDS and oneiric stupor had more common memory problems. Maximum and latest mRS scores were positively correlated with EDS and oneiric stupor. EDS, RBD and oneiric stupor were negatively correlated with LOS points. CONCLUSION: Our study emphasizes the presence and importance of early diagnosis of sleep disturbances in AIE in regard to their deteriorative influences on disease prognosis.

Lateralizing value and clinicoradiological features of asymmetric last clonic jerks in temporal and extratemporal epilepsy.

Seizure semiology and electroencephalograph (EEG) are very important for determining seizure type, hemisphere lateralization, or localization. Clinical symptoms of focal seizures, as well as findings at the onset or end of a focal to bilateral tonic-clonic seizure (FBTCS), are highly informative for lateralization. This study aimed to investigate the relationship of asymmetric last clonic jerk in patients with temporal or extratemporal lobe epilepsy with pathologies, localization, lateralization, or other semiological findings detected in neuroimaging or neuro psychometric tests and its positive predictive value for the detection of hemisphere lateralization based on seizure onset ictal EEG activation. 44 patients with asymmetric last clonic jerks (aLCJ) who were followed up in our VEM unit were randomized 1:1 with epilepsy patients without. In patients with ipsilateral automatism and contralateral posture or gustatory and olfactory hallucinations aLCJ was less or absent. In patients with unilateral tonic activity, aLCJ was more common. The positive predictive value of aLCJ for ictal EEG activation lateralization was 86.36%. In conclusion, asymmetric last clonic beat is valuable for lateralization of FBTCS and should be considered. Its presence strongly and reliably lateralizes to the side of seizure onset.

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.

MRI findings in children with migraine or tension-type headache.

PURPOSE: Migraine and tension-type headache are common primary headaches in children. There is a risk of developing secondary headache in children. The current study was aimed to evaluate magnetic resonance imaging findings (MRI) in children with migraine or tension-type headache. METHODS: The study was planned in children with migraine or tension-type headaches who have been followed up in the pediatric neurology outpatient clinic with regular office visits for at least two years and had neuroimaging in the last year. RESULTS: 280 patients (187 female patients) datas were studied. 91 (61 female patients) were followed up with the diagnosis of migraine and 189 (126 female patients) with the diagnosis of tension-type headaches. The age of patients was found to be 13.1 ± 3.4 years. Brain tumor was found in one child with tension-type headache who had papilledema. Incidental MRI findings found 7.7% and 12.7% in migraine and tension-type headache, respectively. MRI findings in the study were arachnoid cyst (14), pituitary adenoma (6), mega cisterna magna (6), pineal cyst (3), non-specific gliosis (2) and tumor (1). CONCLUSION: Arachnoid cysts were found incidental as the most common MRI finding in children with migraine or tension-type headache. The rare life-threatening secondary headache may develop in children. The fundus examination as a complement to the neurological examination can be useful for requesting MRI.

Birth outcomes in pregnant women with epilepsy: A Nationwide multicenter study from Türkiye.

OBJECTIVE: The present study was aimed at investigating the effects of anti-seizure medications (ASMs), patient demographic characteristics, and the seizure type and frequency on the development of congenital malformations (CMs) in the infants of pregnant women with epilepsy (PWWE). METHODS: PWWE followed up at the neurology outpatient clinic of 21 centers between 2014 and 2019 were included in this prospective study. The follow-up of PWWE was conducted using structured, general pregnant follow-up forms prepared by the Pregnancy and Epilepsy Study Committee. The newborns were examined by a neonatologist after delivery and at 1 and 3 months postpartum. RESULTS: Of the infants of 759 PWWE, 7.2% had CMs, with 5.6% having major CMs. Polytherapy, monotherapy, and no medications were received by 168 (22.1%), 548 (72.2 %), and 43 (5.7 %) patients, respectively. CMs were detected at an incidence of 2.3% in infants of PWWE who did not receive medication, 5.7% in infants of PWWE who received monotherapy, and 13.7% in infants of PWWE who received polytherapy. The risk of malformation was 2.31-fold (95% confidence interval (CI): 1.48-4.61, p < .001) higher in infants of PWWE who received polytherapy. Levetiracetam was the most frequently used seizure medication as monotherapy, with the highest incidence of CMs occurring with valproic acid (VPA) use (8.5%) and the lowest with lamotrigine use (2.1%). The incidence of CMs was 5% at a carbamazepine dose <700 mg, 10% at a carbamazepine dose ≥700 mg, 5.5% at a VPA dose <750 mg, and 14.8% at a VPA dose ≥750 mg. Thus the risk of malformation increased 2.33 times (p = .041) in infants of PWWE receiving high-dose ASMs. SIGNIFICANCE: Birth outcomes of PWWE receiving and not receiving ASMs were evaluated. The risk of CMs occurrence was higher, particularly in infants of PWWE using VPA and receiving polytherapy. The incidence of CMs was found to be lower in infants of PWWE receiving lamotrigine.

Specificity and sensitivity of the SeLECT score in predicting late seizures in patients undergoing intravenous thrombolytic treatment and the effect of diabetes mellitus and leukoaraiosis.

BACKGROUND: Seizures after stroke can negatively affect the prognosis of ischemic stroke and cause a decrease in quality of life. The efficacy of intravenous (IV) recombinant tissue plasminogen activator (rt-PA) treatment in acute ischemic stroke has been demonstrated in many studies, and IV rt-PA treatment has been increasingly used around the world. The SeLECT score is a useful score for the prediction of late seizures after stroke and includes the severity of stroke (Se), large artery atherosclerosis (L), early seizure (E), cortical involvement (C), and the territory of the middle cerebral artery (T). However, the specificity and sensitivity of the SeLECT score have not been studied in acute ischemic stroke patients that received IV rt-PA treatment. OBJECTIVE: In the present study, we aimed to validate and develop the SeLECT score in acute ischemic stroke patients receiving IV rt-PA treatment. METHODS: The present study included 157 patients who received IV thrombolytic treatment in our third-stage hospital. The 1-year seizure rates of the patients were detected. SeLECT scores were calculated. RESULTS: In our study, we found that the SeLECT score had low sensitivity but high specificity for predicting the likelihood of late seizure after stroke in patients administered IV rt-PA therapy. In addition to the SeLECT score, we found that the specificity and sensitivity were higher when we evaluated diabetes mellitus (DM) and leukoaraiosis. CONCLUSION: We found that DM was an independent risk factor for late seizures after stroke in a patient group receiving thrombolytic therapy, and late seizures after stroke were less frequent in patients with leukoaraiosis.

ANTECEDENTES: As convulsões após o AVC podem afetar negativamente o prognóstico do AVC isquêmico e causar uma diminuição na qualidade de vida. A eficácia do tratamento com ativador do plasminogênio tecidual recombinante (rt-PA) intravenoso (IV) no AVC isquêmico agudo foi demonstrada em muitos estudos, e o tratamento com rt-PA IV tem sido cada vez mais usado em todo o mundo. A pontuação SeLECT é uma pontuação útil para a previsão de convulsões tardias após AVC e inclui a gravidade do AVC (Se), aterosclerose de grandes artérias (L), convulsão precoce (E), envolvimento cortical (C) e o território do meio artéria cerebral (T). No entanto, a especificidade e a sensibilidade do escore SeLECT não foram estudadas em pacientes com AVC isquêmico agudo que receberam tratamento IV com rt-PA. OBJETIVO: No presente estudo, objetivamos validar e desenvolver o escore SeLECT em pacientes com AVC isquêmico agudo recebendo tratamento IV com rt-PA. MéTODOS: O presente estudo incluiu 157 pacientes que receberam tratamento trombolítico IV em nosso hospital de terceiro estágio. As taxas de convulsão de 1 ano dos pacientes foram detectadas. Os escores SeLECT foram calculados. RESULTADOS: Em nosso estudo, descobrimos que o escore SeLECT apresentou baixa sensibilidade, mas alta especificidade para prever a probabilidade de convulsão tardia após AVC em pacientes que receberam terapia IV com rt-PA. Além do escore SeLECT, descobrimos que a especificidade e a sensibilidade foram maiores quando avaliamos diabetes mellitus (DM) e leucoaraiose. CONCLUSãO: Descobrimos que DM foi um fator de risco independente para convulsões tardias após AVC em um grupo de pacientes recebendo terapia trombolítica, e convulsões tardias após AVC foram menos frequentes em pacientes com leucoaraiose.

Specificity and sensitivity of the SeLECT score in predicting late seizures in patients undergoing intravenous thrombolytic treatment and the effect of diabetes mellitus and leukoaraiosis / Especificidade e sensibilidade do escore SeLECT na predição de convulsões tardias em pacientes submetidos a tratamento trombolítico intravenoso e o efeito do diabetes mellitus e leucoaraiose

Abstract Background Seizures after stroke can negatively affect the prognosis of ischemic stroke and cause a decrease in quality of life. The efficacy of intravenous (IV) recombinant tissue plasminogen activator (rt-PA) treatment in acute ischemic stroke has been demonstrated in many studies, and IV rt-PA treatment has been increasingly used around the world. The SeLECT score is a useful score for the prediction of late seizures after stroke and includes the severity of stroke (Se), large artery atherosclerosis (L), early seizure (E), cortical involvement (C), and the territory of the middle cerebral artery (T). However, the specificity and sensitivity of the SeLECTscore have not been studied in acute ischemic stroke patients that received IV rt-PA treatment. Objective In the present study, we aimed to validate and develop the SeLECT score in acute ischemic stroke patients receiving IV rt-PA treatment. Methods The present study included 157 patients who received IV thrombolytic treatment in our third-stage hospital. The 1-year seizure rates of the patients were detected. SeLECT scores were calculated. Results In our study, we found that the SeLECT score had low sensitivity but high specificity for predicting the likelihood of late seizure after stroke in patients administered IV rt-PA therapy. In addition to the SeLECT score, we found that the specificity and sensitivity were higher when we evaluated diabetes mellitus (DM) and leukoaraiosis. Conclusion We found that DM was an independent risk factor for late seizures after stroke in a patient group receiving thrombolytic therapy, and late seizures after stroke were less frequent in patients with leukoaraiosis.

Resumo Antecedentes As convulsões após o AVC podem afetar negativamente o prognóstico do AVC isquêmico e causar uma diminuição na qualidade de vida. A eficácia do tratamento com ativador do plasminogênio tecidual recombinante (rt-PA) intravenoso (IV) no AVC isquêmico agudo foi demonstrada em muitos estudos, e o tratamento com rt-PA IV tem sido cada vez mais usado em todo o mundo. A pontuação SeLECT é uma pontuação útil para a previsão de convulsões tardias após AVC e inclui a gravidade do AVC (Se), aterosclerose de grandes artérias (L), convulsão precoce (E), envolvimento cortical (C) e o território do meio artéria cerebral (T). No entanto, a especificidade e a sensibilidade do escore SeLECT não foram estudadas em pacientes com AVC isquêmico agudo que receberam tratamento IV com rt-PA. Objetivo No presente estudo, objetivamos validar e desenvolver o escore SeLECT em pacientes com AVC isquêmico agudo recebendo tratamento IV com rt-PA. Métodos O presente estudo incluiu 157 pacientes que receberam tratamento trombolítico IV em nosso hospital de terceiro estágio. As taxas de convulsão de 1 ano dos pacientes foram detectadas. Os escores SeLECT foram calculados. Resultados Em nosso estudo, descobrimos que o escore SeLECT apresentou baixa sensibilidade, mas alta especificidade para prever a probabilidade de convulsão tardia após AVC em pacientes que receberam terapia IV com rt-PA. Além do escore SeLECT, descobrimos que a especificidade e a sensibilidade foram maiores quando avaliamos diabetes mellitus (DM) e leucoaraiose. Conclusão Descobrimos que DM foi um fator de risco independente para convulsões tardias após AVC em um grupo de pacientes recebendo terapia trombolítica, e convulsões tardias após AVC foram menos frequentes em pacientes com leucoaraiose.

Does Liver Transplant Improve Neurological Symptoms in Wilson Disease? Report of 24 Cases.

OBJECTIVES: Wilson disease is an inherited disorder that results in copper accumulation in the tissues with liver injury and failure. Orthotopic liver transplant is one of the treatments of choice for this disease. The aim of this study was to compare the neurological symptoms, before and after orthotopic livertransplant, of patients with liver cirrhosis due to Wilson disease, who represent a special group of patients with liver failure. MATERIALS AND METHODS: Between 2007 and 2020, there were 24 patients with Wilson disease resistant to medical treatment who underwent deceased donor orthotopic livertransplant and were followed up for 1 year, 5 years, and 10 years for evaluation with neurological scoring systems. Patients were also evaluated for postoperative complications and survival. RESULTS: Of the 24 patients evaluated, there were 13 (54.2%) female patients and 11 (45.8%) male patients, and the mean age was 34 years (range, 14-57 years). One of the patients died from early postoperative sepsis. After orthotopic livertransplant, disease scores returned to normal in 16 patients and improved in the remaining patients. Before transplant, all patients required help in their daily activities. After transplant, there were significant improvements in some symptoms, and the patients became more independent in their daily lives. CONCLUSIONS: Our study shows that orthotopic liver transplant provides significant improvement in neurological symptoms and quality of life in patients with Wilson disease.

A clinical evaluation of gelastic and dacrystic seizures: a multicenter study.

BACKGROUND: Gelastic seizures are extremely rare, short-lasting, unprovoked, and uncontrollable laughing attacks. We conducted this retrospective evaluation to determine whether these symptoms, manifesting in different forms, such as cheerful laughter, laughing, smiling, and sobbing had any value in terms of etiology or localization. METHODS: A total of 31 patients who exhibited bouts of laughing or crying and who were under follow-up between 2000 and 2019 at tertiary epilepsy centers were included in the study. Laughing seizures were divided into three groups in terms of semiology (i.e., laughter with mirth, laughter without mirth, and smile). Dacrystic seizures were accompanied by some gelastic seizures and were divided into two groups in terms of semiology (i.e., weeping loudly [motor and voice-sobbing] and crying). RESULTS: Of the 27 patients with laughing seizures, 12 had seizures that manifested with smiling, 7 had seizures that manifested with laughing and mirth, and 8 had seizures that manifested with laughter without mirth. Dacrystic-gelastic seizures were observed in four patients, among whom 2 patients had crying and laughter without mirth and 2 patients had weeping loudly and laughter without mirth episodes. CONCLUSION: Gelastic and dacrystic seizures often suggest hypothalamic hamartomas, in the literature. This rare ictal behavior can originate from different cortical locations and lesions of a different nature. However, we found that gelastic seizures with smiling were a more homogenous group with regard to location in the temporal lobe, which we aimed to show by evaluating the patients included in this study.

ANTECEDENTES: Crises gelásticas são ataques de riso extremamente raros, de curta duração, não provocados e incontroláveis. Realizamos esta avaliação retrospectiva para determinar se esses sintomas, manifestando-se de diferentes formas, como riso alegre, riso, sorriso e soluço, tinham algum valor em termos de etiologia ou localização. MéTODOS: Foram incluídos no estudo 31 pacientes que apresentavam crises de riso ou choro e que estavam em acompanhamento entre 2000 e 2019 em centros terciários de epilepsia. As crises de riso foram divididas em três grupos em termos de semiologia (ou seja, riso com alegria, riso sem alegria e sorriso). As crises dacrísticas foram acompanhadas por algumas crises gelásticas e foram divididas em dois grupos em termos de semiologia (ou seja, choro alto [motor e soluçar a voz] e choro). RESULTADOS: Dos 27 pacientes com crises de riso, 12 tiveram crises que se manifestaram com sorriso, 7 tiveram crises que se manifestaram com riso e alegria e 8 tiveram crises que se manifestaram com riso sem alegria. Crises dácristico-gelásticas foram observadas em quatro pacientes, sendo 2 pacientes com choro e riso sem alegria e 2 pacientes com choro alto e riso sem alegria. CONCLUSãO: Crises gelásticas e dacrísticas frequentemente sugerem hamartomas hipotalâmicos, na literatura. Este comportamento ictal raro pode ter origem em diferentes localizações corticais e lesões de natureza diversa. No entanto, verificamos que as crises gelásticas com sorriso foram um grupo mais homogêneo quanto à localização no lobo temporal, o que buscamos evidenciar avaliando os pacientes incluídos neste estudo.

A clinical evaluation of gelastic and dacrystic seizures: a multicenter study / Uma avaliação clínica das crises gelásticas e dacrísticas: um estudo multicêntrico

Abstract Background Gelastic seizures are extremely rare, short-lasting, unprovoked, and uncontrollable laughing attacks. We conducted this retrospective evaluation to determine whether these symptoms, manifesting in different forms, such as cheerful laughter, laughing, smiling, and sobbing had any value in terms of etiology or localization. Methods A total of 31 patients who exhibited bouts of laughing or crying and who were under follow-up between 2000 and 2019 at tertiary epilepsy centers were included in the study. Laughing seizures were divided into three groups in terms of semiology (i.e., laughter with mirth, laughter without mirth, and smile). Dacrystic seizures were accompanied by some gelastic seizures and were divided into two groups in terms of semiology (i.e., weeping loudly [motor and voice-sobbing] and crying). Results Of the 27 patients with laughing seizures, 12 had seizures that manifested with smiling, 7 had seizures that manifested with laughing and mirth, and 8 had seizures that manifested with laughter without mirth. Dacrystic-gelastic seizures were observed in four patients, among whom 2 patients had crying and laughter without mirth and 2 patients had weeping loudly and laughter without mirth episodes. Conclusion Gelastic and dacrystic seizures often suggest hypothalamic hamartomas, in the literature. This rare ictal behavior can originate from different cortical locations and lesions of a different nature. However, we found that gelastic seizures with smiling were a more homogenous group with regard to location in the temporal lobe, which we aimed to show by evaluating the patients included in this study.

Resumo Antecedentes Crises gelásticas são ataques de riso extremamente raros, de curta duração, não provocados e incontroláveis. Realizamos esta avaliação retrospectiva para determinar se esses sintomas, manifestando-se de diferentes formas, como riso alegre, riso, sorriso e soluço, tinham algum valor em termos de etiologia ou localização. Métodos Foram incluídos no estudo 31 pacientes que apresentavam crises de riso ou choro e que estavam em acompanhamento entre 2000 e 2019 em centros terciários de epilepsia. As crises de riso foram divididas em três grupos em termos de semiologia (ou seja, riso com alegria, riso sem alegria e sorriso). As crises dacrísticas foram acompanhadas por algumas crises gelásticas e foram divididas em dois grupos em termos de semiologia (ou seja, choro alto [motor e soluçar a voz] e choro). Resultados Dos 27 pacientes com crises de riso, 12 tiveram crises que se manifestaram com sorriso, 7 tiveram crises que se manifestaram com riso e alegria e 8 tiveram crises que se manifestaram com riso sem alegria. Crises dácristico-gelásticas foram observadas em quatro pacientes, sendo 2 pacientes com choro e riso sem alegria e 2 pacientes com choro alto e riso sem alegria. Conclusão Crises gelásticas e dacrísticas frequentemente sugerem hamartomas hipotalâmicos, na literatura. Este comportamento ictal raro pode ter origem em diferentes localizações corticais e lesões de natureza diversa. No entanto, verificamos que as crises gelásticas com sorriso foram um grupo mais homogêneo quanto à localização no lobo temporal, o que buscamos evidenciar avaliando os pacientes incluídos neste estudo.

The Efficacy and Safety of Lumbar Accessed Catheter-Assisted Epidural Blood Patch in Cervical and Thoracic Cerebrospinal Fluid Leakage.

OBJECTIVE: To evaluate the clinical and radiological results of lumbar accessed catheter-assisted epidural blood patch (LACA-EBP) technique in treatment of spontaneous intracranial hypotension secondary to cervicothoracic cerebrospinal fluid leakage. METHODS: Patients presenting with spontaneous intracranial hypotension where magnetic resonance imaging cisternography confirmed and localized the cerebrospinal fluid leakage at cervicothoracic levels were treated with LACA-EBP. Visual analog scale (VAS) score for headache at baseline, third day after treatment, and during long-term follow-up was used to assess the treatment response. Posttreatment VAS score ≤3 on the third day defined treatment success, and VAS score ≥4 indicated treatment failure. RESULTS: LACA-EBP was performed in 10 patients (7 females, 3 males) with a mean age of 38.4 ± 10.5 years. Orthostatic headache was the most common presenting symptom (10/10; 100%) followed by nausea (5/10; 50%). Pituitary gland enlargement was the most commonly observed finding on pretreatment cranial magnetic resonance imaging (9/10; 90%). On magnetic resonance imaging cisternography, 8 patients had cervical and/or thoracic fistulas, whereas 2 patients had multiple-level fistulas. LACA-EBP was successful in all patients with no complications. All patients showed a treatment response with a 72-hour VAS score ≤3. Follow-up VAS scores were available for 8 patients with a clinical follow-up duration of 1-74 months (median 7.5 months). During clinical follow-up, headache recurred in 2 patients. CONCLUSIONS: In our study cohort, LACA-EBP was a safe and efficacious technique in treatment of cerebrospinal fluid leakage at cervical and thoracic levels.

Neuro-Behçet is a rare disease but should be considered in all kinds of neurological findings, even in childhood.

OBJECTIVES: Behçet's disease (BD) is a vasculitis characterised by eye, musculoskeletal, neurological and gastrointestinal involvement, in addition to recurrent oral ulcers. Neuro-Behçet is the term used to define the nervous system involvement in BD and is very rarely seen in childhood. This study aims to show that neuro-Behçet can manifest a clinical course involving all kinds of neurologic findings in the paediatric population. METHODS: The Clinic of Paediatric Neurology at Uludag University provides tertiary treatment for children up to eighteen years of age in Bursa, Turkey. Five patients who were clinically diagnosed with Neuro-Behçet in the last 5 years were included in the study. RESULTS: Seizure, myopathy, transverse myelitis, polyneuropathy, venous thrombosis and facial nerve paralysis were respectively seen in the patients. CONCLUSIONS: Neuro-Behçet is rare in children, but it is important to know that it can cause various neurological findings, and also systemic findings should be taken into consideration in the diagnosis of neurological diseases. Studies on the neurological involvement of BD in children are inadequate. We believe that paediatric neurologists should be more aware of the neuro-Behçet condition.

The REM-sleep-related characteristics of narcolepsy: a nation-wide multicenter study in Turkey, the REMCON study.

INTRODUCTION: Narcolepsy type 1 (NT1) is caused by hypocretin deficiency, the pathophysiology of narcolepsy type 2 (NT2) has not been delineated. Except for the hypocretin deficiency and cataplexy, all clinical and laboratory features used in the diagnosis of NT2 are identical to those used for NT1. The aim of this study was to assess the rapid eye movement (REM) sleep-related characteristics in the patients with narcolepsy; the characteristics of REM sleep in polysomnography (PSG) and multiple sleep latency test (MSLT) recordings, the quantification of REM sleep without atonia (RSWA) and atonia index, and the analysis of rapid eye movements (REMs) during REM sleep. MATERIALS AND METHODS: This study was planned by the Sleep Medicine Study Group of the Turkish Neurology Society, and conducted in 11 centers in eight cities in Turkey. The analysis of RSWA was analyzed by reviewing all REM sleep periods on nocturnal PSG and MSLT recordings per standard criteria. The total duration of the increased muscle tone during REM sleep in the chin and bilateral leg electromyography (EMG) recordings was calculated as RSWA index. The REMs index was also investigated the relation to the RSWA. RESULTS: A total of 274 patients were involved; 147 patients (53.6%) were males and 127 patients (46.4%) were females; the mean age was 29.1 ± 12.0 years. The diagnosis of NT1 was made in 166 patients (60.6%), and 108 patients (39.4%) were diagnosed as having NT2. The mean Epworth sleepiness scale score was significantly higher in patients with NT1 than the patients with NT2 (P = 0.001). The diagnosis of REM sleep behavior disorder (RBD) was made in 19.3% of the patients with NT1 versus in 2.8% of the patients with NT2 (P < 0.001). The percentage of SOREMP in PSG recordings was significantly higher in patients with NT1 (37.1%) than those with NT2 (18.9%, P = 0.001). MSLT showed that the mean sleep latency was shorter in patients with NT1 compared to those with NT2 (P < 0.001). The total duration of REMs on electrooculography recordings was also significantly higher in patients with RSWA in comparison with the patients without RSWA (P = 0.002). Total duration of REMs was significantly and positively correlated with the duration of RSWA on chin-EMG and leg-EMG recordings (P = 0.001). ROC analyses showed an RSWA index of ≥2% for the RSWA on chin-EMG with a sensitivity of 86.7% and a specificity of 71.3% (P < 0.001). The REMs index ≥20% was associated with the presence of RSWA with a sensitivity of 70.0% and a specificity of 57.1% (P = 0.008). CONCLUSIONS: In this nation-wide study, we identified for the first time that the increase in REMs density during REM sleep may be a major correlate of the RSWA. Significant positive correlations were demonstrated between the total duration of REMs on electrooculography recordings and the mean durations of RSWA in both chin and leg EMG recordings. A REMs index of >20% was demonstrated to have a moderate sensitivity and specificity in the diagnosis of RSWA. As observed in chin RSWA index, REMs index also showed a significantly high association with RBD, in comparison to RSWA per standard criteria.

Effects of quarantine applied during the COVID-19 pandemic on mental health and quality of life in patients with multiple sclerosis and healthy controls.

BACKGROUND: The coronavirus outbreak, which emerged in Wuhan, China, in late 2019 and spread to the world, has changed each of our lives. OBJECTIVE: To investigate the effects of quarantine on depression, anxiety, sleep quality, fatigue, and SF-36 of multiple sclerosis (MS) patients during the COVID-19 outbreak and differences between healthy controls (HC). METHODS: Eighty-six MS patients and 65 HC patients were included in the study. Participants filled out the various scales through face-to-face interviews for mental health assessment from January 15 to February 15, 2021. RESULTS: When both groups were compared in terms of BECK-D inventory (p < 0.001), BECK-A inventory (p = 0.010), and FS (p < 0.001), the patient group had significantly higher results. Physical functioning (p < 0.001), physical role limitation (p = 0.001), energy vitality rates (p = 0.010), and general health perception (p < 0.001) were higher in the HC group. When MS patients were divided according to EDSS scores, BECK-A (p < 0.001), BECK-D (p = 0.001), and PSQI (p = 0.006) scores of the patients with EDSS > 3 were higher, while emotional role restriction rates (p = 0.006), energy and vitality (p = 0.018), and pain (p = 0.005) were significantly lower than those with EDSS ≤ 3. When MS patients were divided into two groups as who had COVID-19 and who did not and compared SF-36 subscale scores, pain, (p = 0.049) and mental status (p = 0.030) were obtained significant differences in the two groups. CONCLUSIONS: Our study revealed that MS patients, who are more susceptible to the new 'normal' that emerged during the pandemic period, are among the priority groups that should be supported in terms of mental health as well as physical health.

Traveling Volunteers: A Multi-Vendor, Multi-Center Study on Reproducibility and Comparability of 4D Flow Derived Aortic Hemodynamics in Cardiovascular Magnetic Resonance.

BACKGROUND: Implementation of four-dimensional flow magnetic resonance (4D Flow MR) in clinical routine requires awareness of confounders. PURPOSE: To investigate inter-vendor comparability of 4D Flow MR derived aortic hemodynamic parameters, assess scan-rescan repeatability, and intra- and interobserver reproducibility. STUDY TYPE: Prospective multicenter study. POPULATION: Fifteen healthy volunteers (age 24.5 ± 5.3 years, 8 females). FIELD STRENGTH/SEQUENCE: 3 T, vendor-provided and clinically used 4D Flow MR sequences of each site. ASSESSMENT: Forward flow volume, peak velocity, average, and maximum wall shear stress (WSS) were assessed via nine planes (P1-P9) throughout the thoracic aorta by a single observer (AD, 2 years of experience). Inter-vendor comparability as well as scan-rescan, intra- and interobserver reproducibility were examined. STATISTICAL TESTS: Equivalence was tested setting the 95% confidence interval of intraobserver and scan-rescan difference as the limit of clinical acceptable disagreement. Intraclass correlation coefficient (ICC) and Bland-Altman plots were used for scan-rescan reproducibility and intra- and interobserver agreement. A P-value <0.05 was considered statistically significant. ICCs ≥ 0.75 indicated strong correlation (>0.9: excellent, 0.75-0.9: good). RESULTS: Ten volunteers finished the complete study successfully. 4D flow derived hemodynamic parameters between scanners of three different vendors are not equivalent exceeding the equivalence range. P3-P9 differed significantly between all three scanners for forward flow (59.1 ± 13.1 mL vs. 68.1 ± 12.0 mL vs. 55.4 ± 13.1 mL), maximum WSS (1842.0 ± 190.5 mPa vs. 1969.5 ± 398.7 mPa vs. 1500.6 ± 247.2 mPa), average WSS (1400.0 ± 149.3 mPa vs. 1322.6 ± 211.8 mPa vs. 1142.0 ± 198.5 mPa), and peak velocity between scanners I vs. III (114.7 ± 12.6 cm/s vs. 101.3 ± 15.6 cm/s). Overall, the plane location at the sinotubular junction (P1) presented most inter-vendor stability (forward: 78.5 ± 15.1 mL vs. 80.3 ± 15.4 mL vs. 79.5 ± 19.9 mL [P = 0.368]; peak: 126.4 ± 16.7 cm/s vs. 119.7 ± 13.6 cm/s vs. 111.2 ± 22.6 cm/s [P = 0.097]). Scan-rescan reproducibility and intra- and interobserver variability were good to excellent (ICC ≥ 0.8) with best agreement for forward flow (ICC ≥ 0.98). DATA CONCLUSION: The clinical protocol used at three different sites led to differences in hemodynamic parameters assessed by 4D flow. LEVEL OF EVIDENCE: 2 TECHNICAL EFFICACY STAGE: 2.

A Rare Cause of Epilepsy: Ulegyria Revisited in a Series of 10 Patients.

Introduction. Ulegyria results from perinatal hypoxic-ischemic brain injury in term infants. The specific mushroom-shaped configuration of ulegyria results from small atrophic circumvolutions at the bottom of a sulcus underlying an intact gyral apex. Clinically, ulegyria is generally associated with epilepsy. Here, we aimed to delineate the characteristics of patients with ulegyria and the epileptic seizures they experience. Material and methods. Medical records including radiology and pathology reports, video-electroencephalographic (EEG) analysis, operative notes, hospital progress and outpatient clinic notes were reviewed retrospectively in a total of 10 ulegyria patients. Results. Patients ages ranged between 24 and 58 years (mean, 32 ± 9.8 years). Past medical history was confirmed for neonatal asphyxia in 2 (20%). Neurological examination was remarkable for spastic hemiparesis in 1 (10%) patient with perisylvian ulegyria and for visual field deficits in 2 patients (20%) with occipital ulegyria. Ulegyria most commonly involved the temporoparietal region (n = 5, 50%) followed by the perisylvian area (n = 2, 20%). Except the one with bilateral perisylvian ulegyria, all patients had unilateral lesions (n = 9, 90%). Hippocampal sclerosis accompanied ulegyria in 2 patients (20%). All patients experienced epileptic seizures. Mean age at seizure onset was 8.8 ± 5.4 years (range, 2-20 years). Interictal scalp EEG and EEG-video monitoring records demonstrated temporoparietal and frontotemporal activities in 5 (50%) and 2 (20%) patients, respectively. The seizures were successfully controlled by antiepileptic medication in 8 patients (n = 8, 80%). The remaining 2 patients (%20) with concomitant hippocampal sclerosis required microsurgical resection of the seizure foci due to medically resistant seizures. Discussion. Ulegyria is easily recognized with its unique magnetic resonance imaging characteristics and clinical presentation in the majority of cases. It is highly associated with either medically resistant or medically controllable epileptic seizures. The treatment strategy depends on the age at onset and extends of the lesion that has a significant impact on the severity of the clinical picture.

The evaluation of psychogenic non-epileptic seizures (PNES) cases with saline injection method in video-EEG monitorization unit.

BACKGROUND: It has been reported that 10 to 30% of patients sent to epilepsy centers with a diagnosis of refractory epilepsy are diagnosed with psychogenic non-epileptic seizure (PNES). A wide variety of provocative methods are used to assist PNES diagnosis. OBJECTIVE: To investigate the effect of seizure induction on the diagnosis and prognosis of PNES. METHODS: We retrospectively examined 91 patients with PNES complaints in our video-EEG laboratory. Intravenous saline was administered to all patients for induction of seizures. RESULTS: Saline injection was performed in 91 patients referred to our EEG lab with PNES initial diagnosis, 57 of whom were female and 34 male. Saline injection triggered an attack in 82 patients (90%). CONCLUSIONS: In this study we have concluded that provocative methods are practical, cheap and, most of all, effective for patient diagnosis. In clinical practice, explaining the diagnosis is the first and most important step of the treatment, and careful patient-doctor communication has a positive impact on patient prognosis.

The evaluation of psychogenic non-epileptic seizures (PNES) cases with saline injection method in video-EEG monitorization unit / Avaliação de casos de crises não epilépticas psicogênicas (CNEPs) pelo método de injeção de solução salina em unidade de monitoramento por vídeo-EEG

ABSTRACT Background: It has been reported that 10 to 30% of patients sent to epilepsy centers with a diagnosis of refractory epilepsy are diagnosed with psychogenic non-epileptic seizure (PNES). A wide variety of provocative methods are used to assist PNES diagnosis. Objective: To investigate the effect of seizure induction on the diagnosis and prognosis of PNES. Methods: We retrospectively examined 91 patients with PNES complaints in our video-EEG laboratory. Intravenous saline was administered to all patients for induction of seizures. Results: Saline injection was performed in 91 patients referred to our EEG lab with PNES initial diagnosis, 57 of whom were female and 34 male. Saline injection triggered an attack in 82 patients (90%). Conclusions: In this study we have concluded that provocative methods are practical, cheap and, most of all, effective for patient diagnosis. In clinical practice, explaining the diagnosis is the first and most important step of the treatment, and careful patient-doctor communication has a positive impact on patient prognosis.

RESUMO Antecedentes: Há relatos de que 10 a 30% dos pacientes com epilepsia refratária enviados a centros de epilepsia são diagnosticados com crise não epiléptica psicogênica (CNEP). Uma ampla variedade de métodos provocativos é usada para auxiliar no diagnóstico de CNEP. Objetivo: Investigar o efeito da indução de convulsões no diagnóstico e no prognóstico de CNEP. Métodos: Examinamos 91 pacientes com queixas de CNEP em nosso laboratório de vídeo-EEG. Foi administrada solução salina intravenosa a todos os pacientes para indução de convulsões. Resultados: A injeção de solução salina foi realizada em 91 pacientes com diagnóstico inicial de CNEP encaminhados ao nosso laboratório de EEG, 57 dos quais eram mulheres e 34 homens. A injeção de solução salina desencadeou um ataque em 82 pacientes (90%). Conclusões: Neste estudo, concluímos que os métodos provocativos são práticos, baratos e, acima de tudo, eficazes para o diagnóstico de pacientes. Na prática clínica, a explicação do diagnóstico é a primeira e mais importante etapa do tratamento, e a comunicação cuidadosa entre médicos e pacientes tem um impacto positivo em seu prognóstico.

Myelitis After Liver Transplant: A Case Report.

We report a case of neurotoxicity as a side effect of a calcineurin inhibitor (tacrolimus), which is used as an immunosuppressive drug after liver transplant. Our patient had chronic hepatic failure due to Budd-Chiari syndrome and underwent a liver transplant after an appropriate deceased donor organ was obtained. After organ transplant surgery, he was kept under the effect of an immunosuppressive drug (tacrolimus) with daily control of the level of drug in his blood to avoid drug toxicity. Despite the level of drug in his blood being within the ideal range, the patient developed neurotoxicity that presented as weakness of his extremities. Appropriate diagnostic tests were done, and all proved that these signs and symptoms were related to the use of tacrolimus. Therefore, the drug was changed to cyclosporine. After a few months, the patient regained normal neurological functions of his extremities. We should take precautions to monitor neurological symptoms and signs while we administer calcineurin inhibitors.

Impact of sequence type and field strength (1.5, 3, and 7T) on 4D flow MRI hemodynamic aortic parameters in healthy volunteers.

PURPOSE: 4D flow magnetic resonance imaging (4D-MRI) allows time-resolved visualization of blood flow patterns, quantification of volumes, velocities, and advanced parameters, such as wall shear stress (WSS). As 4D-MRI enters the clinical arena, standardization and awareness of confounders are important. Our aim was to evaluate the equivalence of 4D flow-derived aortic hemodynamics in healthy volunteers using different sequences and field strengths. METHODS: 4D-MRI was acquired in 10 healthy volunteers at 1.5T using three different prototype sequences, at 3T and at 7T (Siemens Healthineers). After evaluation of diagnostic quality in three segments (ascending-, descending aorta, aortic arch), peak velocity, flow volumes, and WSS were investigated. Equivalence limits for comparison of field strengths/sequences were based on the limits of Bland-Altman analyses of the intraobserver variability. RESULTS: Non-diagnostic quality was found in 10/144 segments, 9/10 were obtained at 7T. Apart for the comparison of forward flow between sequence 1 and 3, the differences in measurements between field strengths/sequences exceeded the range of agreement. Significant differences were found between field strengths/sequences for forward flow (1.5T vs. 3T, 3T vs. 7T, sequence 1 vs. 3, 2 vs. 3 [P < .001]), WSS (1.5T vs. 3T [P < .05], sequence 1 vs. 2, 1 vs. 3, 2 vs. 3 [P < .001]), and peak velocity (1.5T vs. 7T, sequence 1 vs. 3 [P > .001]). All parameters at all field strengths/with all sequences correlated moderately to strongly (r ≥ 0.5). CONCLUSION: Data from all sequences could be acquired and resulting images showed sufficient quality for further analysis. However, the variability of the measurements of peak velocity, flow volumes, and WSS was higher when comparing field strengths/sequences as the equivalence limits defined by the intraobserver assessments.