Контакты: Евгений Дмитриевич Благовещенский zhenja@gmail.comВведение. Артрогрипоз -один из наиболее тяжелых врожденных пороков развития опорно-двигательного аппарата, характеризующийся наличием 2 и более контрактур крупных суставов, поражением мышц, а также передних рогов спинного мозга. Одной из основных проблем, обусловливающих ограничение или невозможность самообслуживания пациентов, является отсутствие активных движений в суставах верхних конечностей, которое восстанавливается путем аутотрансплантации мышц различных донорских областей. Процессы реабилитации после таких операций связаны в том числе и с нейрональными перестройками в центральной нервной системе как в спинном, так и в головном мозге, в частности в корковых его отделах. Цель исследования -оценить возможное отражение заболевания артрогрипозом у детей в амплитудных и нейродинамических показателях электроэнцефалограммы (ЭЭГ). Материалы и методы. Изучали электрофизиологические показатели активности коры головного мозга у детей с диагнозом артрогрипоза и здоровых детей сходного возраста. Оценивали такие показатели ЭЭГ, как мощность и длинновременные корреляции (метод оценки динамики нейрональной активности) в диапазонах 4-8, 8-12 и 12-16 Гц. Поражения оценивали на основе клинических шкал. Результаты. Анализ данных показал, что у детей с артрогрипозом, по сравнению с детьми без патологий, имеется достоверное снижение мощности ЭЭГ по всем исследованным частотным диапазонам. Кроме того, продемонстрирована достоверная корреляция мощности ЭЭГ со степенью восстановления двигательных функций верхних конечностей после операций по аутотрансплантации различных групп мышц в позицию двуглавой мышцы плеча. Полученные результаты отражают корреляцию электрофизиологических параметров коры головного мозга с процессами, связанными с патологией артрогрипоза. При этом нейродинамические параметры у детей с артрогрипозом не отличаются от таковых у здоровых детей. По результатам можно констатировать факт отражения заболевания артрогрипозом в снижении электрической активности коры больших полушарий головного мозга в частотном диапазоне 4-16 Гц при сохранении нейродинамических показателей, сходных с группой детей без заболевания. Заключение. В данной работе показано достоверное отличие мощности ЭЭГ в диапазонах 4-8, 8-12 и 12-16 Гц у детей с артрогрипозом и здоровых детей. Однако разницы в таком важном нейродинамическом показателе, как длинновременные корреляции, не обнаружено. Возможно, факт снижения амплитуды ритмов в ЭЭГ у больных детей объясняется их более низкой общей моторной активностью. Ключевые слова: артрогрипоз, электроэнцефалограмма, длинновременные корреляции, реабилитацияДля цитирования: Благовещенский Е.Д., Агранович О.Е., Кононова Е.Л. и др. Особенности электрофизиологической активности коры больших полушарий мозга у детей с артрогрипозом. Нервно-мышечные болезни 2018;8(2):25-32.
Цель. Оценить результаты восстановления активного сгибания в локтевом суставе путем монополярной транспозиции широчайшей мышцы спины (ШМС) у больных с артрогрипозом и выявить корреляцию с уровнем сегментарного поражения спинного мозга. Материалы и методы. С 2011 по 2018 год в ФБГУ "НИДОИ им. Г.И. Турнера" было выполнено восстановление активного сгибания в локтевом суставе у 30 больных с артрогрипозом (44 верхних конечности) путем монополярной пересадки ШМС. Возраст пациентов на момент операции составил от 1 года до 10 лет (средний возраст 3,98 ± 2,35). Проводились клиническое, неврологическое обследование пациентов. Полученные данные были подвергнуты статистической обработке. Результаты. По уровню сегментарного поражения спинного мозга все пациенты были разделены нами на следующие группы: уровень С6-С7 (8 больных, 13 конечностей)-29,6 %, С5-С7 (17 детей, 24 конечности)-54,5 %, С6 (5 пациентов, 7 конечностей)-15,9 %. Результаты лечения были изучены в сроки от 1 года до 7 лет после операции (3,2 ± 1,9). После операции пассивное сгибание в локтевом суставе составило 100 ± 7,0° (min 80°, max 110°), активное сгибание 90,5 ± 14,7° (min 40°, max 110). Дефицит разгибания в локтевом суставе увеличился в 18 случаях (51 %) на 12,8 ± 4,8° (min 10°, max 20°), что, однако, не вызвало ограничения возможности выполнения пациентом основных бытовых навыков. Амплитуда активных движений в локтевом суставе составила 75,4 ± 18,0° (min 40°, max 110°). Хорошие результаты были отмечены в 20 случаях (55,6 %), удовлетворительные-в 12 (33,3 %), неудовлетворительные-в 4 (11,1 %). При оценке разницы изменений активного сгибания после операции, а также дефицита разгибания в зависимости от уровня сегментарного поражения спинного мозга у пациентов с уровнями поражения С6-C7, С5-С7 корреляции не было выявлено. Заключение. ШМС является надежным аутотрансплантатом, позволяющим восстановить активное сгибание в локтевом суставе у больных артрогрипозом с уровнями сегментарного поражения С6, С6-С7, C5-C7 в тех случаях, когда сила донорской мышцы составляет 4 балла и более, а пассивное сгибание в локтевом суставе не менее 90°. Ключевые слова: артрогрипоз, локтевой сустав, широчайшая мышца спины, трансплантат Objective To evaluate active elbow flexion restored with latissimus dorsi (LD) transfer in patients with arthrogryposis and determine the correlation with the level of segmental injury to the spinal cord. Material and methods Active elbow flexion was restored in 30 patients with arthrogryposis (44 upper limbs) using unipolar LD transfer performed between 2011 and 2018 at the Turner Scientific and Research Institute for Children's Orthopaedics. The patients' age at the time of surgery ranged from 1 year to 10 years with the mean age of 3.98 ± 2.35 years. Clinical and neurological assessment was performed for the patients. Statistical data analysis was produced. Results The patients were subdivided into three groups with regard to the level of segmental injury to the spinal cord including С6-С7 (n = 8, 29.6 %, 13 limbs), С5-С7 (n = 17, 54.5 %, 24 limb...
Klippel-Feil syndrome is a congenital malformation, the leading component of which is a violation of segmentation of the cervical vertebral bodies. The syndrome can be combined with other skeletal anomalies: skull asymmetry, scoliosis, high shoulder blades, and cervical ribs. Treatment of the syndrome is usually symptomatic; indications for surgical treatment are progressive neurological disorders and persistent pain syndrome, which usually develop due to instability of unblocked segments, or neurogenic pain. A clinical case of treatment of a 17-year-old patient with Klippel-Feil syndrome who developed a picture of severe upper limb monoparesis during three years due to compression of the brachial plexus associated with cervical ribs is presented. Decompression of the brachial plexus was performed, which led to rapid relief of pain syndrome and gradual partial regression of motor disorders. Due to incomplete restoration of the gripping function, tendon-muscle plasty of the right hand was performed, which significantly improved the possibility of self-care. The results of radiation and staged neurophysiological studies are described, as well as a review of the literature on the Klippel-Feil syndrome.
This article analyzes the literature related to flaccid paresis and paralysis of the upper extremities in children during the first months of life. This pathology is a heterogeneous group of diseases with different etiopathogenesis. There are various courses of flaccid paresis and paralysis of the upper extremities in children: damage to the spinal cord, brachial plexus, peripheral nervous system to the level of the brachial plexus, and isolated damage to peripheral nerves. According to the time of occurrence, flaccid paresis and paralysis can be divided into three groups: antenatal, intranatal, and postnatal pathology. The main mechanism of occurrence of this pathology is intranatal trauma. More rare causes of flaccid paresis and paralysis of the upper extremities are antenatal conditions of dysplastic and traumatic origin, postnatal damage to the peripheral nervous system due to trauma or infection. Congenital contractures of the upper extremities combined with flaccid paralysis are connected with genetically determined diseases of the lower motor neurons and congenital myopathies, intrauterine injuries of the brachial plexus peripheral nerves. This article discusses the issues of topical and differential diagnosis of this pathology, the clinical picture suitable for each period of the childs life, and the prognosis of the disease. This research will be useful not only for neurologists, but also for specialists of related specialties: orthopedists, physiotherapists, and neonatologists for making correct the diagnosis, providing adequate treatment, and predicting its results.
Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease. Congenital contractures are a heterogeneous group of various diseases with different etiology and pathogenesis. The article describes a family case of hereditary sensory-motor polyneuropathy caused by a mutation with 943GA (p Arg315Trp) in the TRPV4 gene (transient receptor potential vanilloid cation channel 4, NM_021625. 4). The article presents the clinical and neurological characteristics of the patient, the results of genetic and neurophysiological examination of the patient and his parents, differential diagnosis of this disease.
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