Clinical case of child with myoclonicastaticepilepsy (Doose syndrome)

A clinical survey of a child's behavior, who has had multiple episodes of flinches and falls from the age of 3, is presented. At the age of 3,5 began generalized tonic-clonic seizures and a status of myoclonic seizures. Patient was experiencing loss of speech, self-service skills and walking skills. EEG has revealed an epileptic status of slow-wave sleep. Based on the clinical electroencephalographic data, a child was diagnosed with Doose syndrome, or epilepsy with myoclonic-astatic seizures. Despite the difficulties in assigning the treatment, seizures have regressed and epileptic status of sleep was blocked, with only a slight lag in neuro-mental development.Представлено клиническое наблюдение ребенка, у которого в возрасте трех лет появились эпизоды вздрагиваний, падений. В 3,5 года появились генерализованные тонико-клонические судороги, статусное течение миоклонических приступов. У пациента наблюдалась утрата речевых навыков, навыков самообслуживания и ходьбы. При ЭЭГ-исследовании был выявлен эпилептический статус медпенноволнового сна. Основываясь на данных клинико-электроэнцефалографической картины, ребенку был выставлен диагноз синдром Дузе, или эпилепсия с миоклонически-астатическими приступами. Несмотря на трудности в подборе терапии, приступы регрессировали, блокировался эпилептический статус сна с исходом в легкое отставание в нейропсихическом развитии

Description of clinical observation families with CADASIL syndrome

Clinical family attendance, three members of which had migraine attacks with aura, lacunar strokes in youth, cognitive impairment was presented in the report. Multiple sclerosis was misdiagnosed in the one patient, he received interferon during a few years. Then cerebral autosomal dominant ateriopathy with subcortical infracts and leukoencephalopathy was diagnosed due to MRI and genetic test.Представлено клиническое наблюдение семьи, три члена которой имели приступы мигрени с аурой, лакунарные инсульты в молодом возрасте, когнитивные нарушения. У одного из больных ошибочно был диагностирован рассеянный склероз, данный пациент получал препараты интерферона в течение нескольких лет. Благодаря магнитно-резонанс­ ному и генетическому обследованию ему был выставлен диагноз церебральной аутосомно-доминантной артериопатии с субкортикальными инфарктами и лейкоэнцефалопатией (CADASIL)

Burden and attitude to resistant and refractory migraine: a survey from the European Headache Federation with the endorsement of the European Migraine & Headache Alliance

Background: New treatments are currently offering new opportunities and challenges in clinical management and research in the migraine field. There is the need of homogenous criteria to identify candidates for treatment escalation as well as of reliable criteria to identify refractoriness to treatment. To overcome those issues, the European Headache Federation (EHF) issued a Consensus document to propose criteria to approach difficult-to-treat migraine patients in a standardized way. The Consensus proposed well-defined criteria for resistant migraine (i.e., patients who do not respond to some treatment but who have residual therapeutic opportunities) and refractory migraine (i.e., patients who still have debilitating migraine despite maximal treatment efforts). The aim of this study was to better understand the perceived impact of resistant and refractory migraine and the attitude of physicians involved in migraine care toward those conditions. Methods: We conducted a web-questionnaire-based cross-sectional international study involving physicians with interest in headache care. Results: There were 277 questionnaires available for analysis. A relevant proportion of participants reported that patients with resistant and refractory migraine were frequently seen in their clinical practice (49.5% for resistant and 28.9% for refractory migraine); percentages were higher when considering only those working in specialized headache centers (75% and 46% respectively). However, many physicians reported low or moderate confidence in managing resistant (8.1% and 43.3%, respectively) and refractory (20.7% and 48.4%, respectively) migraine patients; confidence in treating resistant and refractory migraine patients was different according to the level of care and to the number of patients visited per week. Patients with resistant and refractory migraine were infrequently referred to more specialized centers (12% and 19%, respectively); also in this case, figures were different according to the level of care. Conclusions: This report highlights the clinical relevance of difficult-to-treat migraine and the presence of unmet needs in this field. There is the need of more evidence regarding the management of those patients and clear guidance referring to the organization of care and available opportunities