Background: Migraine pain location and trigeminocervical convergence have limited diagnostic value and have usually been assessed using non-standard verbal descriptors in a small number of centers. Objective: To use non-verbal descriptors of migraine pain location to determine the prevalence of trigeminocervical convergence mechanisms in patients with episodic and chronic migraine. In addition, we explored the factors associated with the presence of convergence. Methods: A multicenter study was carried out. The explicit pain location was explored by asking subjects to indicate, on an electronic form, three points on the anterolateral side and three points on the posterolateral side of the head and neck that represented the common locations of their migraine pain. We evaluated associations of the pain pattern with demographic and psychological features, comorbidities, lifestyle and other headache characteristics. Results: 97 episodic and 113 chronic migraine patients were included. Convergence was present in 116 migraineurs (55%) who indicated dominance of pain in the posterior cervical region. This site was more often involved in the chronic migraine group (21 vs. 33%; p=0.034). The number of migrainous/altered sensitivity symptoms (OR=1.39; 95%CI 1.14–1.71) was associated with convergence independently of the chronification status. In this symptom group, there were statistical associations between convergence and vomiting (p=0.045), tactile allodynia (p<0.001), nuchal rigidity (p<0.001) and movement allodynia (p=0.031). Conclusions: Trigeminocervical convergence is common in migraineurs and, in practice, it might be found frequently in chronic migraineurs. Some features commonly found in this group, such as altered sensitivity symptoms, are associated with this phenomenon.

Introduction: When migraine evolves from episodic to chronic form, it becomes more disabling, due to refractory treatment and the arising of comorbidities. Bruxism has already been associated with migraine in adults, with a bidirectional relationship between sleep bruxism and chronic migraine. This study aimed to assess whether sleep and wake bruxism are more prevalent in chronic migraine when compared to episodic migraine and also to establish possible clinical correlations with chronification. Methods: 210 patients were allocated to the study, 97 with episodic migraine (EM) and 113 with chronic migraine (CM). The patients were submitted to face-to-face interviews with a neurologist to confirm the diagnosis and fill in the scales: specific questionnaire for the diagnosis of sleep and wake bruxism, PHQ-9 (depression), GAD-7 (anxiety), Epworth Scale (sleepness), MIDAS and HIT-6 scales to assess the migraine disability and the headache impact on patients. Results: The prevalence of sleep and wake bruxism was similar in patients with EM versus CM (p=0.300 and p=0.238). The correlation of patients with both bruxism forms at the same time with the high scores on the migraine disability and the headache impact, was higher among patients with chronic migraine than in patients with chronic migraine. episodic migraine (p <0.001). Conclusion: Sleep and wake bruxism alone aren’t more prevalent in chronic migraine when compared to episodic migraine. In patients affected with both bruxism forms, bruxism only causes a greater impact and disability on individuals with chronic migraine.

IntroduçãoOs ataques de migrânea são caracterizados por sintomas álgicos e não álgicos. Além disso, a migrânea ocorre associada a diversas comorbidades. Tanto os sintomas não álgicos como as comorbidades podem se caracterizar por sintomas autonômicos. Este trabalho tem por objetivo avaliar a disautonomia em migranosos e diferenças relacionadas entre os grupos episódico (ME) e crônico (MC).Material e MétodosEstudo transversal de pacientes atendidos em ambulatório especializado, diagnosticados pela International Classification of Headache Disorders 3 como ME ou MC, convidados de forma consecutiva e utilizando entrevistas semi-estruturadas. O questionário Composite Autonomic Symptom Score (COMPASS) foi utilizado para avaliação de sintomas autonômicos, o Patient Health Questionnaire (PHQ) 9 para avaliação de sintomas depressivos e o Short Form (SF) 36 para avaliação de qualidade de vida. O estudo foi aprovado pelo comitê de ética do HC- UFPR.ResultadosForam incluídos 210 pacientes dos quais 97 (46%) apresentavam ME e 113 (54%) MC. Destes, 78 (69%) consumiam analgésicos excessivamente. A média de idade era de 39,5±12,6 anos e 189 (90%) pacientes eram do sexo feminino. O grupo CM apresentou um escore COMPASS mais alto (34,7±18,3) que o grupo EM (26,4±14,8). A regressão múltipla foi empregada para analisar a associação das variáveis clínicas com o COMPASS. O modelo final ajustado mostra que para cada sintoma não álgico tipicamente vistos na aura de tronco encefálico, há um incremento médio de 2,17-6,3 no COMPASS. Para cada ponto a mais no PHQ9, o COMPASS eleva-se entre 0,78-1,84 em média. Todos os processos de inferência consideraram um valor alpha de 0.05.ConclusãoO processo de cronificação pode cursar com mais sintomas disautonômicos. Estes, por sua vez, estão associados a mais sintomas atribuídos a alterações de tronco e à depressão, indicando esta área como potencialmente envolvida nesta gama de sintomas.

Context: Pelizaeus-Merzbacher Disease (PMD) is a rare X-linked recessive hypomyelinating leukodystrophy caused by mutations in the proteolipid protein 1 (PLP1) gene, associated with myelin sheath development and stability. The result is a broad spectrum of clinical phenotypes. Diagnosis is confirmed by genetic testing. Clinical features include hypotonia followed by progressive spasticity, nystagmus, ataxia and cognitive impairment. Males are more affected. Females are asymptomatic or present milder symptoms. Most cases arise from duplications, point and null mutations. Null mutations are associated with milder phenotypes. Brain Magnetic Resonance Imaging (MRI) may reveal hypomyelination. There is no disease modifying treatment for PMD. We aim to present the case of a woman with a novel variant of the PLP1 gene. Case report: A 38-year-old female presented with 23 years of progression of upper limb tremor, speech impairment, lower limb rigidity and urinary incontinence. She reported abnormal development of reading and writing skills. She had a brother with cognitive impairment, delayed motor development, gait disorder and generalized tonic-clonic seizures; and a sister with upper limb tremor, dysarthria and behavioral disorder. Hypomyelination was detected on brain MRI. Complete exome sequencing detected a novel likely pathogenic variant of PLP1 gene: ChrX(GRCh37):NC_000023.10:g.103041651del:NM _000533.3:c449del, p.Asp150AlafsTer10, heterozygous. Conclusions: The patient’s case resembles a milder form of PMD. This is supported by literature linking deletions and female sex to milder phenotypes. In 20 to 40% of cases with suggestive clinical findings, no PLP1 mutation is found. New studies are needed to identify other variants associated with PMD.

Introduction: Functional dependence is a common condition poststroke. The specialized care offered at the stroke unit can provide patients with better rehabilitation. We designed a study to evaluate risk factors for functional dependence at hospital discharge in a stroke unit. Design and setting: Prospective cohort study at Hospital Geral Roberto Santos. Methods: Patients were admitted within 72h of ictus. Functional dependence was defined as scores 3-5 on the modified Rankin Scale (mRS). Results: 389 patients were enrolled, with a mean age of 59.3 (±17.9) years, of whom 55.8% were men. The frequency of functional dependence at discharge was 57.1%, 50.3% at 30 days, and 38.8% at 90 days of the ictus. Higher risk of dependence at hospital discharge were associated with stroke recurrence [RR 1.2 (1.0 - 1.5)], polypharmacy [RR 1.3 (1.1 - 1.6)], female sex [RR 1.3 (1.1 - 1.6)], hemorrhagic stroke [RR 1.4 (1.2 - 1.7)], large artery atherosclerosis by TOAST [RR 1.6 (1.2 - 2.0)], hemorrhagic transformation [RR 1.57 (1.3 - 1.9)], infection [RR 1.7 (1.5 - 2.0)] and delirium [RR 1.8 (1.5 - 2.0)] during hospitalization. Conclusions: We have demonstrated a high frequency of functional dependence at hospital discharge from the stroke unit. Understanding which patients may have a worse functional outcome can be a good strategy to articulate access to the rehabilitation.