Abstract
Arnold-Chiari malformation (ACM) is a group of congenital hindbrain malformations affecting the structural relationships between the cerebellum, brain stem, top cervical spinal cord, and bones of the skull base. In clinical practice, ACM types 0 and 1 are more common and types 2, 3, and 4 belong to rare severe (often fatal) congenital malformations. Objective: to study gender differences in the incidence of ACM types 0 and 1 in outpatient neurological practice. Patients and methods. A total 2039 case records of outpatients who had visited a neurologist of the Krasnoyarsk University clinic in 2008–2014 were analyzed. Neurological and neuroradiological (1.5 Tesla brain magnetic resonance imaging, phase-contrast spinal cerebral fluid flow imaging) diagnostic techniques were used. 3.4% (70/2039) of cases were randomized according to the criteria of inclusion and exception. An entire sample included 70 ACM patients (median age, 25 [17; 34] years) (30 (42.8–7.1%) men and 40 (57.2–7.1%) women). Results. There was an increase in the incidence of ACM type 1 in the women than in the men. Conclusion. It is necessary to develop a new strategy for the prophylactic medical examination of patients with ACM to improve primary and specialized outpatient health care.
Highlights
Аномалия Арнольда–Киари (ААК) – это группа врожденных аномалий развития заднего мозга, влияющих на структурные взаимоотношения мозжечка, ствола мозга, верхних шейных отделов спинного мозга и костей основания черепа
Arnold-Chiari malformation (ACM) is a group of congenital hindbrain malformations affecting the structural relationships between the cerebellum, brain stem, top cervical spinal cord, and bones of the skull base
There was an increase in the incidence of ACM type 1 in the women than in the men
Summary
Кантимирова Е.А., Шнайдер Н.А., Петрова М.М., Строцкая И.Г., Дутова Н.Е., Алексеева О.В., Шаповалова Е.А. В клинической практике чаще встречается ААК 0-го и 1-го типов, а 2–4-й типы заболевания относятся к редким тяжелым (часто несовместимым с жизнью) врожденным порокам развития. Objective: to study gender differences in the incidence of ACM types 0 and 1 in outpatient neurological practice. There was an increase in the incidence of ACM type 1 in the women than in the men. It is necessary to develop a new strategy for the prophylactic medical examination of patients with ACM to improve primary and specialized outpatient health care. Традиционное разделение ААК на четыре типа основано на количественном определении дислокации (смещения) миндалин мозжечка в большое затылочное отверстие ниже линии Чемберлена В клинической практике чаще встречается ААК 0-го и 1 типов, а ААК 2-го–4-го типов относится к редким тяжелым (часто несовместимым с жизнью) врожденным порокам развития. Что величина дислокации миндалин мозжечка не всегда соответствует тяжести симптомов или ответу на лечение
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