Научные статьи \ Прикладные науки. Медицина. Технология \ Mедицинские науки \ Патология. Клиническая медицина \ Нервная система. Невропатология. Неврология

Редкая ангиопатия с поражением кожных покровов и центральной нервной системы: синдром и болезнь Снеддона

Редкая ангиопатия с поражением кожных покровов и центральной нервной системы: синдром и болезнь Снеддона

Автор: Грузманов А. К., Мазуренко С. О.

Журнал: Juvenis scientia @jscientia

Рубрика: Обзорные статьи

Статья в выпуске: 6 т.8, 2022 года.

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Синдром Снеддона - редкое, прогрессирующее заболевание, поражающее относительно молодых людей, преимущественно женского пола и проявляющееся двумя основными симптомами: сетчатое ливедо и повторные инфаркты головного мозга. Впервые описанный в 1965 году английским дерматологом Ian Bruce Sneddon, синдром, названный в его честь, объединяет гетерогенную группу заболеваний с различным патогенезом и сходными клиническими проявлениями. Кожные проявления заболевания в виде сетчатого ливедо носят доброкачественный характер, цереброваскулярные проявления сводятся к повторным инфарктам головного мозга, прогрессирующей деменции и могут приводить к гибели пациентов. В обзоре литературы описаны основные представления об этиологии, патогенезе первичной и вторичных форм синдрома Снеддона, предложена концепция болезни Снеддона для описания первичных форм синдрома. В статье приведены фотографии пациенток из клинической практики авторов статьи. Также в обзоре описаны методы диагностики, дифференциальной диагностики заболевания и рекомендуемые методы лечения, которое основывается на устранении дополнительных факторов риска повреждения сосудов и формирования тромбозов, контроле артериального давления, липидного и углеводного обмена, а также назначении антикоагулянтной и дезагрегантной терапии. Применение противовоспалительной или иммуносупрессивной терапии остается спорным. Основная цель лечения - профилактика органического повреждения головного мозга и нервно-психических осложнений. К сожалению, нейропсихиатрический прогноз синдрома Снеддона относительно плохой с развитием нарушения памяти, концентрации внимания и зрительно-пространственных навыков. В исключительных случаях прогрессирование заболевания может приводить к смерти.

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Синдром снеддона, ишемический инсульт, сетчатое ливедо, деменция

Короткий адрес: https://sciup.org/14126342

IDR: 14126342   |   DOI: 10.32415/jscientia_2022_8_6_15-29

A rare angiopathy with lesions of the skin and central nervous system: Sneddon syndrome and disease

Sneddon syndrome is a rare, progressive disease that affects relatively young people, mainly female, and manifests itself in two main symptoms: livedo reticularis and recurrent cerebral infarctions. First described in 1965 by the English dermatologist Ian Bruce Sneddon, the syndrome named in his honor unites a heterogeneous group of diseases with different pathogenesis and similar clinical manifestations. Skin manifestations of the disease in the form of reticular livedo are benign, cerebrovascular manifestations are reduced to repeated cerebral infarctions, progressive dementia and can lead to the death of patients. The literature review describes the basic concepts of the etiology, pathogenesis of primary and secondary forms of Sneddon syndrome, and proposes the concept of Sneddon’s disease to describe the primary forms of the syndrome. The article contains photographs of patients from the clinical practice of the authors of the article. The review also describes methods of diagnosis and differential diagnosis of the disease and recommended methods of treatment, which is based on the elimination of additional risk factors for vascular damage and thrombosis, blood pressure control, lipid and carbohydrate metabolism, as well as the appointment of anticoagulant and disaggregant therapy. The use of anti-inflammatory or immunosuppressive therapy remains controversial. The main goal of treatment is the prevention of organic brain damage and neuropsychiatric complications. Unfortunately, the neuropsychiatric prognosis of Sneddon syndrome is relatively poor with the development of impaired memory, concentration, and visual-spatial skills. In exceptional cases, the progression of the disease can lead to death.

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