Amyotrophic lateral sclerosis (ALS) is a fatal disease characterized by progressive degeneration of the motor neurons. It is difficult to define the severity of the clinical findings of this destructive disease owing to its rapid progression, which presents serious alterations in a short time even in the same patient. The present study was designed to evaulate the validity of the Turkish version of the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R), which has been used in various countries for measuring the functional status of ALS patients. The ALSFRS-R scores of 41 ALS patients (24 male), in any stages of illness, were simultaneously assessed by two physicians. The functional status of the patients (motor system, bulbar, and pulmonary functions) was evaluated under 12 titles. In every subtitle, the functional status was evaluated as 0 for the worst functional status and 4 for the best functional status. The mean differentials for both subtitles and global scores and the 95% confidence bounds of these means were detected. The coherence was defined as the states in which the coefficient is above 0.80 and is statistically significant. From the data obtained, the correlation between the two physicians was found to be statistically significant (p=0.000) in terms of the means of both subtitles and total scores. It was shown in the present study that in the clinical follow-up of the disease, the Turkish version of ALSFRS-R is a simple, reliable, and easily applicable.

Bu çalışmada, Adana il merkezinde epilepsi prevalansı, hastaların demografik verileri ve epilepsi gelişimindeki risk faktörlerinin belirlenmesi amaçlanmıştır. Gereç ve yöntem: Çalışma, toplum temelli kesitsel olarak planlandı ve Adana il merkezi nüfusu üzerinden ‘'orantılı tabakalı örneklem yöntemi'' kullanılarak hedef nüfus 7052 kişi olarak belirlendi. Toplumun, sosyoekonomik düzeyini yansıtacak şekilde rastgele örneklem yöntemi ile üç mahalle belirlendi. Hazırlanan anket formları bir doktor ve iki anketör tarafından yüz yüze görüşme yöntemi ile dolduruldu. Hedef nüfus olan 7052 kişiye ulaşıldığında çalışma sonlandırıldı. Daha önceden epilepsi tanısı almış olan veya öykü özellikleri ile epilepsi olabilecek olan grup, hazırlanan diğer anket formlarının doldurularak ileri tetkiklerin yapılması için kliniğimize davet edildi. Bulgular: Anket formu doldurulan 7052 kişinin, 3577'si erkek (%50,7), 3475'i (%49,3) kadındı. Taranan nüfus içerisinde 52 kişi epilepsi olarak tanındı ve aktif epilepsi prevalansı %0,7 olarak bulundu. Epilepsi tespit edilen grubun 31'i (%59,6) erkek 21'i (%40,4) ise kadındı. Sosyoekonomik durumu yüksek olan mahallede prevalans %0,3 sosyoekonomik durumu orta düzeyde olan mahallede %0,7 ve sosyoekonomik durumu düşük olan mahallede ise prevalans %1,4 olarak bulundu. Sosyoekonomik düzey düştükçe, epilepsi prevalansında artış saptandı ve bulgular istatiksel olarak anlamlı bulundu (p<0,001). Sonuç: Elde ettiğimiz veriler, daha önce ülkemizde ve dünyada yapılan prevalans çalışmalarını destekler nitelikte bulunmuştur. Sosyoekonomik düzey yükseldikçe prevalansta düşme saptanmıştır. Bu durum, düşük sosyoekonomik düzeye sahip kişilerin yaşam koşulları nedeniyle prenatal dönemde ve çocukluk çağlarında daha az tıbbi bakım almaları, çalışma şartları nedeniyle daha çok travmaya maruz kalmaları ile açıklanabilir. Ülkemizde epilepsi prevalansı ile ilgili yapılan çalışmalar arttıkça, hastalığın gelişimine katkıda bulunan faktörler daha iyi tanınarak toplumda epilepsi farkındalığının artacağı kuşkusuzdur.

Background Blepharospasm is a focal dystonia that presents as involuntary, intermittent, continuous contractions of the eyelids. Abnormal eyelid contractions in blepharospasm are expected to cause balance problems, but there is no clear information. Objective This study was designed to evaluate the effect of blepharospasm on postural stability (PS) in patients with blepharospasm. As a secondary endpoint, the efficacy of botulinum toxin type-A (BoNT-A) treatment on static balance in patients with blepharospasm was investigated. Methods Twenty-four patients with blepharospasm receiving regular BoNT-A injections and 20 age-matched and sex-matched healthy controls were included in the study. All subjects were evaluated on a static posturography force platform performing four tasks (eyes open (EO), eyes closed (EC), tandem Romberg (TR) and verbal cognitive task (COGT)). Evaluations of the patients were repeated 4 weeks after the injection. Results Pretreatment lateral and anterior–posterior sways, sway area and velocities of the sways were significantly higher in patients than controls during the COGT and TR (p<0.05). In the patient group, with EO and EC, a few parameters improved after BoNT-A injection. On the other hand, in the TR and COGT, most of the sway parameters and velocities improved significantly after treatment (p<0.05). Conclusions Blepharospasm may cause functional blindness in patients. This study demonstrated that PS worsens in patients with blepharospasm under dual-task conditions. BoNT-A injection treats the disease itself and, thus, markedly improves PS under dual-task conditions in blepharospasm.

Background Psychogenic non-epileptic seizures (PNES) resemble epileptic seizures and are often misdiagnosed as epilepsy. Objective To investigate the frequency of PNES and to calculate the economic burden of the patients who admitted to video-electroencephalographicmonitoring (VEM) to obtain a diagnosis of epilepsy in order to apply for disability retirement. Methods The present retrospective study included 134 patients who required disability reports between 2013 and 2019 and had their definite diagnoses after VEM. Following VEM, the patients were divided into three groups: epilepsy, PNES, and epilepsy + PNES. Results In total, 22.4% (n = 30) of the patients were diagnosed with PNES, 21.6% (n = 29) with PNES and epilepsy, and 56% (n = 75), with epilepsy. The frequency of PNES among all patients was of 44% (n = 59). In patients with PNES alone, the annual cost of using anti-seizure medication was of 160.67 ± 94.04 dollars; for psychostimulant drugs, it was of 148.3 ± 72.48 dollars a year; and the mean direct cost for diagnostic procedures was of 582.9 ± 330.0 (range: 103.52–1601.3) dollars. Conclusions Although it is challenging to determine the qualitative and quantitative total cost in these patient groups, early diagnosis and sociopsychological support will reduce the additional financial burden on the health system and increase the quality of life of the patients. Antecedentes As crises psicogênicas não epilépticas (CPNE) se assemelham a crises epilépticas, e muitas vezes são diagnosticadas erroneamente como epilepsia. Objetivo Investigar a frequência de CPNE e calcular o impacto econômico dos pacientes internados para serem submetidos a monitoramento videoencefalográfico (MVE) para obter um diagnóstico de epilepsia e requerer aposentadoria por invalidez. Métodos Este estudo retrospectivo incluiu 134 pacientes que solicitaram laudo médico de incapacidade entre 2013 e 2019, e obtiveram seus diagnósticos definitivos após serem submetidos a MEV. Os pacientes foram divididos em três grupos: epilepsia, CPNE, e epilepsia + CPNE. Resultados Após o MEV, 22,4% (n = 30) dos pacientes foram diagnosticados com CPNE, 21,6% (n = 29), com CPNE + epilepsia, e 56%, com epilepsia. A frequência de CPNE entre todos os pacientes foi de 44% (n = 59). Em pacientes somente com CPNE, o custo anual do uso de anticonvulsivantes foi de US$ 160,67 ± 94,04; para os psicoestimulantes, o custo anual foi de US$ 148,3 ± 72,48; e a média do custo direto de procedimentos diagnósticos foi de US$ 582,9 ± 330,0 (variação: 103,52–1601,3). Conclusões Embora seja um desafio determinar o custo total qualitativo e quantitativo nesses grupos de pacientes, o diagnóstico precoce e o apoio sociopsicológico reduzirão o impacto financeiro adicional ao sistema de saúde e aumentarão a qualidade de vida dos pacientes. Received: 01 December 2021 Accepted: 29 May 2022 Article published online: 28 December 2022 © 2022. Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/) Thieme Revinter Publicações Ltda. Rua do Matoso 170, Rio de Janeiro, RJ, CEP 20270-135, Brazil

Background: Among the comorbidities that accompany multiple sclerosis (MS), restless legs syndrome (RLS) is one of the most common. Anxiety and depression are common psychological comorbidities that impact the quality of life of patients with MS (PwMS), as well as patients with RLS. Objective: To investigate the psychiatric burden of MS and RLS coexistence, we conducted a nationwide, multicenter and cross-sectional survey. Methods: Participants were assessed by using demographic and clinical parameters along with the Hamilton Anxiety and Hamilton Depression Scales (HAM-A and HAM-D). Results: Out of the 1,068 participants, 173 (16.2%) were found to have RLS [RLS(+)] and 895 (83.8%) did not [RLS(-)]. The mean scores for HAM-A and HAM-D were significantly higher among RLS(+) subjects than among RLS(-) subjects (p<0.001 for all variables). Conclusions: According to our data, the presence of RLS in PwMS may increase the occurrence of both anxiety and depression symptoms. Awareness and treatment of RLS in PwMS could possibly reduce the symptoms of psychiatric comorbidities originating from RLS.

Background: Cervical dystonia (CD) is the most common form of focal dystonia. It is not known exactly whether abnormal head postures in cervical dystonia cause balance problems. Dual-tasking is a common every-day life situation. Objective: We aimed to evaluate postural stability (PS) in patients with CD and the effect of cognitive task on PS. As a secondary aim, we evaluated the effect of onabotulinum toxin A (BoNT) injection on PS. Methods: A total of 24 patients with CD who were on BoNT treatment for at least one year and 23 healthy controls were included. Posturographic analyses were carried out in all the subjects on static posturography platform under four different conditions: eyes open, eyes closed, tandem stance and cognitive task. In patients, posturographic analysis was carried out just before the BoNT injections and was repeated four weeks later. Results: Before treatment, the anterior-posterior sway was significantly higher in CD patients with the eyes open condition compared to the controls (p=0.03). Cognitive task significantly affected several sway velocities. Tandem stance significantly affected many sway parameters, whereas the eyes closed condition did not. After treatment, only two parameters in tandem stance and one in cognitive task improved within the patient group, in a pairwise comparison. Conclusions: Postural control is impaired in CD patients probably due to the impaired proprioceptive and sensorimotor integration. In reference to dual task theories possibly due to divided attention and task prioritization, cognitive dual-task and harder postural task disturbes the PS in these patients.