Neuromuscular Diseases (E-Journal) / Нервно-мышечные болезни
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Влияние клинических проявлений мигрени, депрессии и нарушения сна на качество жизни пациентов с хронической мигренью
Get PDFОЦЕНКА ДИНАМИКИ ПЛОЩАДИ ПОПЕРЕЧНОГО СЕЧЕНИЯ ПЕРИФЕРИЧЕСКИХ НЕРВОВ ПРИ МУЛЬТИФОКАЛЬНОЙ МОТОРНОЙ НЕЙРОПАТИИ ПО ДАННЫМ УЛЬТРАЗВУКОВОГО ИССЛЕДОВАНИЯ НА ФОНЕ ВНУТРИВЕННОЙ ТЕРАПИИ ИММУНОГЛОБУЛИНАМИ: ОПИСАНИЕ КЛИНИЧЕСКОГО СЛУЧАЯ
Get PDFBackground. Multifocal motor neuropathy (MMN) is а chronical dysimmune disease, which can be treated with intravenous human immunoglobulin (IVIG).Objectives. To follow up the sonographic changes of peripheral nerves in a patient with MMN treated with IVIG.Materials and methods. A 42 y.o. woman with MMN receiving IVIG is described. The cross-sectional area (CSA) of median, ulnar nerves and spinal roots was measured with high-resolution ultrasound (HRUS) after five IVIG courses.Results. Before treatment the asymmetrical CSA increase of median, ulnar nerves and C5, C6, C7 spinal roots was found. 7 weeks after the first IVIG, next 3 IVIG courses with 4 weeks’ intervals HRUS showed normalization of CSA of the peripheral nerves and significant decrease of CSA of the spinal roots; as soon as the interval between the fourth and fifth IVIG was prolonged up to 9 weeks the CSA of the peripheral nerves increased again.Conclusion. The follow up of a MMN patient treated with IVIG confirms the positive sonographic changes of the peripheral nerves and spinal roots. The possible role of HRUS as a method for monitoring changes in the peripheral nerves due to IVIG should be discussed after a larger cohort of patients with MMN and similar diseases is investigated
Особенности состояния мягких тканей на вершине деформации у больных кифосколиозом на фоне нейрофиброматоза 1-го типа
Get PDFObjective. Evaluation of skin sensitivity and analysis of morphological changes in paravertebral muscles and back skin in kyphoscoliotic deformity projection in patients with type 1 neurofibromatosis (NF-1).Materials and methods. Ten NF-1 patients who underwent surgery to treat kyphoscoliosis were examined. Using an electrical esthesiometer thermal pain sensitivity before the surgery was studied in dermatomes corresponding to the apex of the deformity. Skin and muscle biopsy samples were collected intraoperatively in the projection of the apex of the deformity curve and were subsequently analyzed by light and scanning electron microscopy.Results. Patients with kyphoscoliosis with underlying NF-1 were characterized by abnormal thermal pain sensitivity, pathological structural changes in skin and muscles accompanied by disrupted innervation and blood supply.Discussion. The observed changes may be responsible for lowered postoperative reparative potential of tissues and they must be considered in prevention and prognosis of treatment and rehabilitation efficacy
Стволовой энцефалит Бикерстаффа, острый поперечный миелит и острая моторная аксональная нейропатия: сложности диагностики и лечения пациентов с перекрестными синдромами. Клиническое наблюдение
Get PDFWe report a 40-year-old woman presented with consciousness disturbance, ataxia, asymmetrical limb weakness, hyperreflexia. Due to magnetic resonance imaging findings, the patient was diagnosed with Bickerstaff’s brainstem encephalitis overlapped with acute transverse myelitis. Later she developed distal muscles atrophy and the electroneuromyographic study revealed axonal motor neuropathy, therefore acute motor axonal neuropathy was diagnosed. The patient underwent one course of intravenous immunoglobulin therapy with the regression of symptoms and magnetic resonance imaging changes. Nine months after symptoms onset, the patient has completely recovered. This overlapping case of Bickerstaff’s brainstem encephalitis, acute transverse myelitis and acute motor axonal neuropathy provides further support that these conditions are part of the same spectrum.
Семейная амилоидная полинейропатия TTR Cys 114 у монозиготных братьев-близнецов (клинический случай)
Get PDFTransthyretin amyloidosis (ATTR) is a hereditary autosomal dominant disease. Its symptoms depend on polymorphisms of the transthyretin gene and include disorders of the peripheral nervous system and internal organs. One of the rarest mutations of the transthyretin gene is tyrosine substitution for cysteine in position 114 (Tyr114Cys). One of the described characteristics of ATTR is the discordant phenotype in monozygotic twins. We present a case of ATTR Cys 114 in a Russian family with a pair of monozygotic twins discordant for ATTR.Транстиретиновый амилоидоз (transthyretin amyloidosis, ATTR) – наследственное аутосомно-доминантное заболевание, проявления которого зависят от полиформизма гена транстиретина и включают поражение периферической нервной системы и внутренних органов. К наиболее редким мутациям гена транстиретина относится замена цистеина на тирозин в позиции 114 (Tyr114Cys). Одной из описанных особенностей течения ATTR является дискордантность симптоматики у монозиготных близнецов. Представлен случай ATTR Cys 114 в российской семье у монозиготных братьев-близнецов, дискордантных по течению данного заболевания
Ботулинотерапия в лечении спастичности нижней конечности
Get PDFThe article reviews the current concept of lower extremity spasticity, which is a frequent disabling consequence of stroke. Gait biomechanics, step cycle and main pathologic patterns of lower extremity are described (hip adduction, knee flexion, knee extension, foot plantar flexion, equinovarus foot position, toes flexion, hallux extension), including muscles involved in the pathological process. Additionally the article contains detailed information on pathologic principles of lower extremity spasticity development. Special focus is given to sarcomeregenesis as an essential element of the development of potential conditions for muscle tissue adaptation to a new state and restoration of muscle length and strength. At present Botulinum toxin A (BTA) is used in a complex spasticity management programs. The results of clinical studies performed in the last decade supporting the efficacy of Botox® (Onabotulinumtoxin A) in the treatment of spasticity are reviewed. Effective BTA doses are proposed. Authors came to the conclusion that BTA as a part of complex rehabilitation in patients with poststroke spasticity of lower extremity promotes treatment efficacy due to a decrease of muscle tone and increase of range of movements in the joints. BTA should be regarded as an essential part of standard rehabilitation programs. Further studies to define optimal muscles for intervention, BTA doses and rehabilitation schemes are still needed.
Клинический опыт ведения кризов у больных миастенией в Амурской области
Get PDFBackground. Management of the myasthenic crisis remains one of the issues in clinical neurology.Objective. Analysis of the timeframes of the myasthenic exacerbation since the disease onset, sex distribution, age predominance, specific clinical features, precipitating factors in order to investigate the efficacy of the management algorithm of the myasthenic crisis.Materials and methods. Medical histories of 33 female and 19 male patients with myasthenia for the period of 2000 to 2003 were analyzed.Results. In comparison with the literature data the number of mysthenic crisis in myasthenic patients was lower and did not exceed 25 %. Sex distribution 1,2 (male): 1 (female). In 70 % worsening of myasthenia appeared in the first two years of the disease: in 6 patients (46.2 %) at the age of 22–35 y. o.; in 5 patients (38.5 %) – at the age of 36–60 y. o. All patients suffered from generalized myasthenia, in three of them ocular and bulbar muscle weakness predominated. In 46.2 % of patients with crisis, thymus abnormalities were present (thymoma / hyperplasia). The most common precipitating factors were: infection (36.9 %), stress (26.3 %), misuse of the anticholinesterase drugs (15.8 %). In 5.2 % the worsening of myasthenia followed the delivery. The crisis triggering factor was not identified in 15.8 % of cases.Conclusion. In order to prevent the exacerbation of myasthenia, generalized forms of myasthenia with poor response to anticholinesterase drugs require special attention towards patients in their first two years of the disease, including patient’s educational program on appropriate mode of drug administration and avoiding initiating factors, as well as thymectomy