Which factors affect phenoconversion in isolated rapid eye movement sleep behavior disorder?

AIM: Isolated REM sleep behavior disorder (IRBD) is characterized by loss of the normal atonia of REM sleep. Patients with IRBD are at substantial risk of developing the synuclein-related neurodegenerative diseases (NDD). Few predictors of phenoconversion (from IRBD to NDD) have been identified such as age >65 years, hyposmia, constipation, elevated Epworth sleepiness scale (ESS). We aimed to detect rate and risk factors of phenoconversion. METHOD: The study designed as retrospectively. NDD was developed in 18 (27.27%) patients while NDD wasn't developed in 48 (72.73%) patients after ten years. The data of the first visit (age, gender, hyposmia, constipation, ESS, comorbidities, physical/neurological examinations, laboratory, and polysomnography) were compared between NDD (n:18) and IRBD (46) groups. The statistical program IBM SPSS Statistics Version 20.0 was used for all analyzes. The threshold for statistical significance for each test was set at 0.05. RESULTS: Although, most first-visit data (age, gender, hyposmia, constipation, ESS, laboratory, polysomnography) were not different between NDD (n:18) and IRBD (n:48) groups, diabetes mellitus (DM) frequency (p:0.021), mean duration of DM (0.027), chest circumference (p:0.017), and hip circumference (p:0.045) were found higher in NDD than IRBD. If the risk of phenoconversion calculated by logistic regression analysis was different only in terms of DM frequency (p:0.030) [odds ratio: 4.909 (1.17-20.19)]. CONCLUSION: The present study showed that the phenoconversion rate for ten years is 27.27%, and IRBD patients with diabetes mellitus increase the phenoconversion risk nearly five times.

Postural stability in blepharospasm: the effects of dual-tasking and botulinum toxin therapy.

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.

Oromandibular Dystonia: Clinical and Demographic Data from Eight-Two Patients.

Objective: This study aimed to determine the demographic and clinical characteristics of patients with oromandibular dystonia (OMD). Background: Dystonia is a movement disorder characterized by sustained involuntary muscle contractions that often cause abnormal postures. OMD is a rare focal dystonia that affects the tongue, jaw, and mouth. OMD, which is a rare public health problem, is often recognized as psychogenic and there are delays in its diagnosis and treatment. Methods: Patients with OMD, both isolated and combined, followed at our Movement Disorders Outpatient Clinic between 2004 and 2021 were enrolled in this study. Age, sex, age at onset, and disease duration were recorded. The type of OMD, affected muscles, etiologies of accompanying neurological disorders, and treatment were noted. Results: A total of 82 patients (44 women, 38 men) were included in this study. Among these, 39 patients had isolated OMD, and 43 patients had either segmental or generalized dystonia. Seven patients reported a family history of dystonia. Only nine patients reported a sensory trick. The average disease duration was 6.01 ± 3.73 (range, 1-29) years, and the average age at onset was 43.34 ± 18.24 (range, 1-78) years. The disease etiology was unknown (idiopathic) in most patients. Fifteen patients reported task-specific dystonia. The most common type of dystonia was jaw-opening dystonia. Conclusion: OMD is focal dystonia that significantly affects the quality of life. This study adds more data to the literature by defining the clinical features of this rare disorder and draws attention to this neglected type of dystonia.

Frequency and economic burden of psychogenic non-epileptic seizures in patients applying for disability benefits due to epilepsy.

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: 103,52­1601,3). 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.

Frequency and economic burden of psychogenic non-epileptic seizures in patients applying for disability benefits due to epilepsy / Frequência e impacto econômico das crises psicogênicas não epilépticas em pacientes requerentes de aposentadoria por invalidez devido a epilepsia

Abstract 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.

Resumo 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.

Central Aortic Pressure and Arterial Stiffness in Parkinson's Disease: A Comparative Study.

Background: Cardiovascular autonomic dysfunction, which leads to hemodynamic disorders, is commonly observed in patients with Parkinson's disease (PD). Central aortic pressure (CAP) is the systolic blood pressure (SBP) at the root of the aorta. In young people, CAP is lower than peripheral arterial blood pressure. In older people, the difference between CAP and peripheral arterial blood pressure decreases depending on the extent of arterial stiffness (AS). In patients with AS, CAP increases. CAP is thus regarded as an indicator of AS. Objective: To compare CAP and other hemodynamic parameters for AS between patients with Parkinson's disease and control group. We also aimed to evaluate changes in these hemodynamic parameters after the levodopa (LD) intake. Methods: We included 82 patients with PD and 76 healthy controls. Age, sex, disease duration, disease subtype, Hoehn-Yahr stage (H&Y), and nonmotor symptoms (NMS) were documented. TensioMed Software v.3.0.0.1 was used to measure CAP, peripheral arterial blood pressure, pulse pressure (PP), heart rate (HR), mean arterial pressure (MAP), augmentation index (AI), pulse wave velocity, and ejection time. All patients were being treated with LD, and measurements were performed 1 h before and 1 h after LD intake. Results: Baseline peripheral arterial blood pressure and CAP values were significantly higher in the PD group than in the control group (p < 0.001 and p=0.02, respectively). Most cardiac hemodynamic parameters, including peripheral arterial blood pressure and CAP, decreased significantly (p < 0.02 and p < 0.001, respectively) after LD intake in the PD group. Disease subtype, duration, and severity did not affect any of the hemodynamic parameters. When NMS were evaluated, patients with psychosis and dementia showed higher baseline parameters. Conclusion: Loss of postganglionic noradrenergic innervation is well-known with PD. Several cardiac hemodynamic parameters were affected, suggesting cardiac autonomic dysfunction in these patients. The data obtained were independent of disease severity, duration, and subtype. After LD intake, most of these parameters decreased, which might have a positive effect on the vascular burden.

Coexistence of restless legs syndrome and multiple sclerosis aggravates anxiety and depression.

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.

Coexistence of restless legs syndrome and multiple sclerosis aggravates anxiety and depression / A coexistência da síndrome das pernas inquietas e esclerose múltipla agrava ansiedade e depressão

ABSTRACT 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.

RESUMO Antecedentes: Considerando-se as comorbidades que acompanham a esclerose múltipla (EM), a síndrome das pernas inquietas (SPI) é uma das mais comuns, e ansiedade e depressão são comorbidades psicológicas comuns que afetam a qualidade de vida de pacientes com EM, bem como de pacientes com SPI. Objetivo: Investigar a carga psiquiátrica da coexistência de EM e SPI por meio de uma pesquisa nacional, multicêntrica e transversal. Métodos: Os participantes foram avaliados por parâmetros demográficos e clínicos, além da versão turca das escalas de ansiedade e depressão de Hamilton (HAM-A e HAM-D). Resultados: Dos 1.068 participantes, 173 (16,2%) apresentaram SPI [SPI (+)] e 895 (83,8%) não [SPI (-)]. As pontuações médias no HAM-A e no HAM-D foram significativamente maiores em indivíduos com SPI (+) do que naqueles com SPI (-) (p <0,001 para todas as variáveis). Conclusões: De acordo com nossos dados, a presença de SPI na EM pode aumentar a ocorrência de sintomas de ansiedade e depressão. A conscientização e o tratamento da SPI na EM podem reduzir os sintomas de comorbidades psiquiátricas originadas da SPI.

The effect of cognitive task on postural stability in cervical dystonia / O efeito da tarefa cognitiva sobre a estabilidade postural na distonia cervical

ABSTRACT 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.

RESUMO Introdução: A distonia cervical (DC) é a forma mais comum de distonia focal. Não se sabe exatamente se posturas anormais da cabeça na DC causam problemas de equilíbrio. A execução de duas tarefas simultaneamente é situação comum da vida cotidiana. Objetivo: Avaliar a estabilidade postural (EP) em pacientes com DC e o efeito da tarefa cognitiva na EP. Como objetivo secundário, avaliamos o efeito da toxina onabotulínica A (BoNT) na EP. Métodos: Foram incluídos 24 pacientes com DC em tratamento com BoNT por pelo menos um ano e 23 controles saudáveis. As análises posturográficas foram realizadas em todos os sujeitos na plataforma de posturografia estática sob quatro condições diferentes: olhos abertos, olhos fechados, postura tandem e tarefa cognitiva. Nos pacientes, a análise posturográfica foi realizada imediatamente antes das injeções de BoNT e após quatro semanas. Resultados: Antes do tratamento, a oscilação ântero-posterior era significativamente maior nos pacientes com DC com os olhos abertos quando comparados aos controles (p=0,03). A tarefa cognitiva interferiu significativamente nas velocidades de oscilação. A postura tandem afetou significativamente muitos parâmetros de oscilação, enquanto a condição de olhos fechados não. Após o tratamento, apenas dois parâmetros na posição tandem e um na tarefa cognitiva melhoraram no grupo de pacientes. Conclusões: O controle postural é prejudicado em pacientes com DC, provavelmente devido à comprometida integração proprioceptiva e sensório-motora. Em referência às teorias de dupla-tarefa, possivelmente devido à atenção dividida e à priorização de tarefas, a dupla-tarefa cognitiva e a tarefa postural mais difíceis perturbam o EP nesses pacientes.

The effect of cognitive task on postural stability in cervical dystonia.

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.

'Is RLS a harbinger and consequence of MS?: Striking results of the 'RELOMS-T' study'.

BACKGROUND: Although studies report a high prevalence rate of restless legs syndrome (RLS) among patients with multiple sclerosis (PwMS) ranging from 13.3 to 65.1%, little is known about the causes of this relationship. METHODS: To ascertain the prevalence, features and impact of RLS among PwMS a nation-wide, multicenter, prospective and a cross-sectional survey, designed to reflect all of the PwMS throughout Turkey, was conducted in 13 centers. Exploring the relationship of the two conditions could possibly contribute to the understanding of the causes of the high and wide-ranging prevalence rates and the pathophysiology of both diseases. RESULTS: Of the 1068 participants 173 (16,2%) found to have RLS [RLS(+)] and 895 (83,8%) did not [RLS(-)]. Among the RLS(+) 173, all but 8 patients (4,6%) were underdiagnosed in terms of RLS. More than half of the patients with RLS had 'severe' or 'very severe' RLS. The onset of RLS was before or synchronous with the onset of MS in about a half of our patients. CONCLUSION: We conclude that RLS should be meticulously investigated in PwMS and MS can be a direct cause of RLS at least in part of PwMS. Our data about the timing of the onset of MS and RLS, along with the high prevalence of RLS in PwMS suggest that the pathologic changes in the initial phases of MS can possibly trigger RLS symptoms.

Clinical features and risk factors of angiography headache and evaluation of its relationship to primary headaches.

INTRODUCTION: Angiography headache is defined as a new headache caused directly by cerebral angiography. We aimed to investigate the prevalence and clinical characteristics of angiography headache, and its relationship with primary headaches. MATERIAL AND METHODS: This single-centre prospective cohort study was carried out in our tertiary healthcare centre. Patients who had undergone a diagnostic cerebral angiography procedure between March 2016 and June 2017 were included in the study. During and after the procedure, we evaluated the patients in terms of occurrence of headache, and, if present, details about time to onset of headache after cerebral angiography, duration of headache, accompanying symptoms (nausea, vomiting, photophobia and phonophobia), and degree and severity of pain. RESULTS: A total of 226 patients were included in the study. The prevalence of angiography headache was found to be 22.1% (n = 50). While angiography headache started in the first six hours in 92% (n = 46) of the patients, it started after 24 hours of the angiography procedure in only 4% (n = 2). Compressive headache was the most common type, described by 64% of the patients, whereas 4% of the patients described pricking or stabbing sensations. Angiography headache was associated with female gender, higher educational level, and a history of primary headache. CONCLUSION: Angiography headache is an important clinical entity that must be considered during and after angiography. Experiencing angiography headache is more common among patients with a history of primary headache.

Evaluation of the Innermost Retinal Layers and Visual Evoked Potentials in Patients with Multiple Sclerosis.

PURPOSE: The present study was conducted to investigate alterations in the innermost layers of the retina using optical coherence tomography (OCT) and to assess potential associations of structural measures with functional markers in patients with Multiple Sclerosis (MS). MATERIALS AND METHODS: Ninety-four eyes of 47 MS patients and 60 eyes of 30 healthy individuals were included in the study. All patients underwent complete ophthalmological examination and OCT imaging to analyze peripapillary retinal nerve fiber layer (RNFL) and ganglion cell-inner plexiform layer (GCIPL) thickness. Visual evoked potentials (VEPs) and expanded disability status scale (EDSS) score were assessed for MS patients. RESULTS: The average RNFL and GCIPL thicknesses were thinner in MS patients (86.2 ± 11.9 µm and 73.6 ± 9.7 µm, respectively) when compared with those of healthy controls (96.7 ± 8.2 µm and 85.9 ± 4.6 µm, respectively, p < 0.001 for both). Within MS patients, the average RNFL and GCIPL thicknesses were lower in eyes with a prior history of optic neuritis (MS ON) than in eyes with no optic neuritis history (MS non-ON) (p = 0.012 and p < 0.001, respectively). RNFL and GCIPL thicknesses were inversely correlated with VEP latency (r = -0.40, p < 0.001 and r = -0.36, p < 0.001, respectively) in MS patient eyes. There was a correlation between GCIPL thickness and VEP amplitude in eyes with previous ON history (r = 0.34, p = 0.035). No significant correlations were found between OCT measurements and EDSS score. CONCLUSIONS: Innermost layers of the retina are highly affected by the pathophysiologic process in MS disease, manifesting as a reduction in RNFL and GCIPL thickness. The structural retinal changes show correlation with alterations in potentials showing the optic pathway function.

Adaptation to Turkish and Reliability Study of the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R).

INTRODUCTION: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.