The results of controlled investigations suggest that botulinumtoxin type A (BTA) leads to decrease headache intensity and prevent migraine attacks. The antinociceptive mechanisms of BTA action remain unclear. Modern and previous hypothesis of antinociceptive action BTA in chronic migraine (CM) are discussed in details. Recent experimental and clinical evidence strongly suggest that BTA has a
specific antinociceptive effect realized through inhibition of proinflammatory neurotransmitters release not only from the sensory terminals but from muscle nociceptors. The mechanism of the action of BTA in CM has more than one target and is considered to involve different pathophysiological levels CM: neurogenic inflammation, peripheral and central sensitization. The administration of BTA on the PREEMPT principle (paradigm) ensures optimal neurotoxin distribution in the anatomic areas in accordance with their sensory innervation by cervical segments and sensory fibers in the trigeminal system, the terminal branches which are the major target of BTA in the treatment of CM.

In the article there is a review the latest achievements in the field of physiotherapy Charcot–Marie–Tooth disease (CMT). Describes some of the techniques non-pharmacological treatment, the goal of physiotherapy application depending on the pathogenesis, clinical manifestations of the disease, electromiographic tests. Here there are recommendations for sanato¬rium treatment. On the basis of personal observations in the article presents the author's patterns of treatment for CMT patients.

Skeletal muscle biopsies of 7 patients with Lambert–Eaton syndrome were studied. Revealed that specific changes in the neuromuscular junctions are the complication of the postsynaptic region organization. Along with this, there is a destructive process in axon terminals, which may be terminated by the denervation of muscle fibers. Violations of the structure of muscle fibers were observed. A new hypothesis of the Lambert–Eaton syndrome pathogenesis was preposed.

Carpal tunnel syndrome (CTS) is a frequent syndrome in adults, but is very rare in children. CTS was described in children with mucopolysaccharidoses (MPS) as condition due to the deformation of carpal bones, deposition of glycosaminoglycans in tendosynovial tissue and connective tissue of flexor retinaculum. Electromyography is essential method for diagnostic CTS in children because typical symptoms of CTS (paresthesia, numbness of hand and fingers, atrophy and paresis of certain muscles) seen in adults are absent or not realized by children with MPS because of cognitive deficit despite the presence of nerve involvement. EMG results from 40 children with different types of MPS (age 1 year 8 months to 18 years) are presented. Neurophysiologic abnormalities related to CTS were found in every child with MPS I, in 80,9 % of cases – with MPS II and in every case – with MPS VI; no EMG signs of median nerve lesions in
carpal channel were detected in patients with MPS III and MPS IV. CTS was bilateral in children with MPS I, II and VI, but usually
there was an asymmetry of changes. We revealed CTS in one patient with MPS II as early as at the age 2 years 11 months. All children with MPS II had already CTS at the age of 4 years except one patient. Children with MPS I and MPS VI were not investigate before the age 4 years old, but one child 4 years old with MPS I had severe CTS. In children with MPS atrophy of thenar eminence muscles developed rapidly as complication of CTS. Therefore we recommend repeating of EMG regularly to identify earliest signs of median nerve disturbance in carpal channel and opportune surgical decompression of the entrapped nerve. It allows preserving normal function of hand that it is very important for adequate child development and quality of life.

A case of Pompe disease in infant in Orenburg is presented in this article. Clinical picture of the infantile form of the disease is described. Enzyme replacement therapy (ERT) with alglucosidase alfa is presented (alglucosidase alfa is not registered in Russia, was administered due to
life-threatening indication). Nowadays ERT is the only possible pathogenetic treatment of Pompe disease.

Letus present a unique clinical case course of autoimmune non paraneoplastic myasthenic syndrome of Lambert–Eaton on the background of pregnancy, in the 24 years old patient, with a 3-year history of the disease. Throughout the pregnancy, birth and early postpartum period, patient was taking a stable unmodified drug therapy, which allowed observing "natural" course for neuromuscular disease. The first trimester of pregnancy proceeded without deterioration of the myasthenic syndrome. From the middle of the second trimester (19–20 weeks gestation), and the entire third trimester until parturition, the patient's condition for neuromuscular disease has improved, which was accompanied by an increase in strength of proximal limb muscles and the positive dynamics of the basic parameters of neuromuscular transmission. Deliveries came on ime, preceeded safely through vaginal delivery, without weakness of generic forces. There was born clinically healthy male child, weighing 2970 g, growth of 52 cm (7/8 Apgar score). However , on the second day in the newborn was marked negative dynamics of muscular hypotonia, extinction of neonatal reflexes, sucking weakness, episodes of apnea, which required tube feeding and non invasive oxygen support. Neuromuscular defect was transient in character and resolved within 5–7 days with administration in the morning hours of 20 mg of prednisone parenteral. The child was discharged with full recovery of function of the neuromuscular system on the 13th day breastfed. Shortly after birth the mother appeared in a state of negative dynamics: the increased weakness of the proximal muscles of the legs and arms, deteriorated parameters of the neuromuscular conduct. By analogy with myasthenia there appeared the similar influence of pregnancy on the Lambert Eaton myasthenic syndrome: 1) improvmem of the neuromuscular disease in II–III trimester of pregnancy, 2) intact ability to birth vaginally, 3) no evidence of neuromuscular disease in newborn at birth, 4) one day delay in the development of transient neonatal myasthenic syndrome, 5) postpartum mothers deterioration that increases while breastfeeding.