Validation of the Brazilian Portuguese version of the Stanford cataplexy questionnaire.

INTRODUCTION: Cataplexy is a sudden and involuntary episode of loss of muscle tone during wakefulness. Cataplexy cannot be easily recognized when clinical features are atypical or when the physician is unfamiliar with its characteristics. The unstructured clinical interview is the only standard diagnostic method, but the use of a targeted questionnaire can help in the diagnosis of cataplexy. METHODS: The Stanford cataplexy questionnaire is a self-administered 51-question questionnaire. This validation consisted of an initial translation and back-translation of the questionnaire from English into Brazilian Portuguese, followed by a pilot study with 10 participants for the cultural adaptation of the scale. RESULTS: 155 consecutive patients aged 18-85 completed the questionnaire. The Brazilian version of the Stanford cataplexy questionnaire showed similar results to the original version with good metric properties (area under the curve), high internal consistency (Cronbach's alpha equal to 0.87), good reliability and reproducibility. CONCLUSIONS: The Brazilian Portuguese version of the Stanford Cataplexy questionnaire presented good accuracy satisfactory psychometric properties in identifying cataplexy.

The effects of the COVID-19 pandemic on patients with narcolepsy.

STUDY OBJECTIVES: To the best of our knowledge, there has not as yet been any study on the effects of the COVID-19 pandemic on patients with narcolepsy, in particular, in relation to its impact on sleep schedules, symptoms, the need for medication, work, income, and quality of life. This study therefore aimed to explore these factors and their possible influence on sleep, circadian timing, and narcolepsy symptoms during the pandemic. METHODS: Patients with narcolepsy who had been in quarantine for at least 3 months completed a 36-question online survey. Questions targeted the conditions of the quarantine, sleep-related behaviors, and factors known to affect sleep and circadian rhythms (work status, income, appetite, narcolepsy symptoms, and medication), as well as the quality of life during the quarantine period. RESULTS: The routines of the participants had been altered by quarantine, with changes in their place of work, and an increase in narcolepsy symptoms, such as cataplexy, sleep paralysis, hallucinations, nocturnal awakenings, and sleepiness. Sleep and wake times changed, resulting in altered sleep patterns in most of the sample. No association between changes in the place of work and narcolepsy symptoms was found. Regarding medication, the participants used fewer antidepressant pills but took more stimulants. Appetite was increased and self-reported quality of life decreased during the period. CONCLUSIONS: During the quarantine, the patients with narcolepsy reported changes in their bedtime and waking-up schedules, suggesting a tendency to circadian misalignment. In Brazil, the effects of the COVID-19 outbreak have gone beyond the direct action of the virus because of the collateral damage it has caused in respect to unemployment, financial hardship, and a reduction in quality of life. These impacts have been amplified in Brazil because of the level of social inequality found in the country, and they have particularly affected vulnerable patients with rare diseases, such as the narcolepsy population.

Is transcranial sonography useful for diagnosing Parkinson's disease in clinical practice?

OBJECTIVE: Transcranial sonography (TCS) is an emerging ancillary examination for diagnosing Parkinson's disease (PD). OBJECTIVE: To evaluate TCS features in patients with PD and its mimics, and establish their accuracy in predicting the final clinical diagnosis after follow-up. METHODS: We retrospectively studied 85 patients with an initial clinical suspicion of PD, atypical parkinsonism or essential tremor, all of whom underwent TCS. Two specialists reviewed the follow-up clinical visit records and determined the final clinical diagnosis. The accuracy analysis of the TCS was determined using Bayesian statistical methods. RESULTS: The finding of substantia nigra hyperechogenicity (> 20 mm2) showed high sensitivity (93.4%) and specificity (86.6%). The positive likelihood ratio showed 6.93-fold greater odds for diagnosing PD than an alternative condition when this finding was present. CONCLUSIONS: This study revealed the practical usefulness of TCS in differentiating PD from its prevalent mimics when the clinical diagnosis was initially unclear.

Is transcranial sonography useful for diagnosing Parkinson's disease in clinical practice? / O ultrassom transcraniano é útil para o diagnóstico da doença de Parkinson na prática clínica?

ABSTRACT Transcranial sonography (TCS) is an emerging ancillary examination for diagnosing Parkinson's disease (PD). Objective To evaluate TCS features in patients with PD and its mimics, and establish their accuracy in predicting the final clinical diagnosis after follow-up. Methods We retrospectively studied 85 patients with an initial clinical suspicion of PD, atypical parkinsonism or essential tremor, all of whom underwent TCS. Two specialists reviewed the follow-up clinical visit records and determined the final clinical diagnosis. The accuracy analysis of the TCS was determined using Bayesian statistical methods. Results The finding of substantia nigra hyperechogenicity (> 20 mm2) showed high sensitivity (93.4%) and specificity (86.6%). The positive likelihood ratio showed 6.93-fold greater odds for diagnosing PD than an alternative condition when this finding was present. Conclusions This study revealed the practical usefulness of TCS in differentiating PD from its prevalent mimics when the clinical diagnosis was initially unclear.

RESUMO A ultrassonografia transcraniana (UTC) é um exame complementar para diagnóstico de doença de Parkinson (DP). Objetivo Avaliar as características da UTC em pacientes com DP e seus diagnósticos diferenciais e estabelecer a precisão desse exame para o diagnóstico clínico de DP após seguimento. Métodos Avaliou-se retrospectivamente 85 pacientes com suspeita clínica inicial de DP, parkinsonismo atípico (PA) ou tremor essencial (TE), todos submetidos a UTC. Um consenso de dois especialistas determinou o diagnóstico clínico final após revisar os registros médicos das consultas de seguimento. A precisão do UTC foi calculada usando métodos estatísticos Bayesianos. Resultados O achado de hiperecogenicidade da substância negra (> 20 mm2) mostrou alta sensibilidade (93,4%) e especificidade (86,6%). A razão de verossimilhança positiva mostra 6.93 vezes mais chances de diagnosticar DP do que uma condição alternativa, se o achado estiver presente. Conclusões Este estudo demonstra a utilidade prática do UTC na diferenciação de DP de condições clínicas similares quando o diagnóstico clínico é inicialmente pouco claro.

Suicidal ideation, anxiety, and depression in patients with multiple sclerosis.

INTRODUCTION: Psychiatric disorders frequently occur in patients with multiple sclerosis (MS); however, limited reports are available on these comorbidities. We aimed to investigate the relationships among MS, anxiety, depression, and suicidal ideation. METHODS: One hundred and thirty two patients with relapsing-remitting MS were evaluated using the Expanded Disability Status Scale, Beck Depression Inventory-II (BDI-II), Beck Scale for Suicide Ideation (BSI), and Hospital Anxiety and Depression Scale. RESULTS: A hierarchical regression analysis was performed to evaluate the variables. The regression equation significantly predicted the BSI score (R2 = 0.306; adjusted R2 = 0.273; F (9, 125) = 9.18; p < 0.0005), and the BDI-II score was the only variable that contributed significantly to this model (p < 0.0005). CONCLUSIONS: A high prevalence of depression and anxiety, and a higher rate of suicidal ideation were identified in MS patients compared to the general population. The presence of depressive symptoms appeared to have a direct influence on the risk of suicide.

Suicidal ideation, anxiety, and depression in patients with multiple sclerosis / Ideação suicida, ansiedade e depressão em pacientes com esclerose múltipla

ABSTRACT Psychiatric disorders frequently occur in patients with multiple sclerosis (MS); however, limited reports are available on these comorbidities. We aimed to investigate the relationships among MS, anxiety, depression, and suicidal ideation. Methods: One hundred and thirty two patients with relapsing-remitting MS were evaluated using the Expanded Disability Status Scale, Beck Depression Inventory-II (BDI-II), Beck Scale for Suicide Ideation (BSI), and Hospital Anxiety and Depression Scale. Results: A hierarchical regression analysis was performed to evaluate the variables. The regression equation significantly predicted the BSI score (R2 = 0.306; adjusted R2 = 0.273; F (9, 125) = 9.18; p < 0.0005), and the BDI-II score was the only variable that contributed significantly to this model (p < 0.0005). Conclusions: A high prevalence of depression and anxiety, and a higher rate of suicidal ideation were identified in MS patients compared to the general population. The presence of depressive symptoms appeared to have a direct influence on the risk of suicide.

RESUMO Transtornos psiquiátricos frequentemente ocorrem em pacientes com esclerose múltipla (EM). No entanto, os artigos sobre estas comorbidades são limitados. Pretendemos investigar as relações entre EM, ansiedade, depressão e ideação suicida. Métodos: Cento e trinta e dois pacientes com EM remitente-recorrente foram avaliados usando a Escala de Estado de Incapacidade Expandida, Inventário de Depressão de Beck-II (IDB-II), Escala de Beck para Ideação de Suicídio (BSI) e Escala de Ansiedade e Depressão. Resultados: Uma análise de regressão hierárquica foi realizada para avaliar as variáveis. A equação de regressão previu significativamente o escore BSI (R2 = 0,306; R2 ajustado = 0,273; F (9,125) = 9,18; p < 0,0005) e o escore no IDB-II foi a única variável que contribuiu significativamente para este modelo (p < 0,0005). Conclusões: Uma alta prevalência de depressão e ansiedade e uma maior taxa de ideação suicida foram identificadas em pacientes com EM em comparação com a população em geral. A presença de sintomas depressivos pareceu ter uma influência direta no risco de suicídio.

Oral L-Carnitine used to treat narcoleptic type 1 patient during pregnancy - A case report.

Narcolepsy type 1 is a sleep disorder characterized by excessive daytime sleepiness (EDS), sleep fragmentation, hypnagogic hallucinations, sleep paralysis, and cataplexy. Stimulant medications such as modafinil and amphetamines are the first-line medications for treating sleepiness. However, the management of narcolepsy during special circumstances of life such as pregnancy is complex. MMDM is a 34-year-old female with Narcolepsy type 1 treated with modafinil (400mg/d) and citalopram (20mg/d). Before she become pregnant, modafinil and citalopram were replaced for L-Carnitine 510mg/d with good outcome. She underwent an usual pregnancy and was submitted to a term cesarean delivery without child-birth complications. This is the first description of oral L-Carnitine such an alternative to treatment narcolepsy type 1 during pregnancy. Treat these patients is not easy but care narcolepsy pregnant is a challenging even bigger. In a disease without many treatment options, L-Carnitine can be used to treat daytime sleepiness during pregnancy in narcolepsy.