[Modern aspects of diagnosis and treatment of chronic inflammatory demyelinating polyneuropathy in children]. / Sovremennye aspekty diagnostiki i lecheniya khronicheskoi vospalitel'noi demieliniziruyushchei polineiropatii u detei.

OBJECTIVE: Analysis of demographic, clinical, laboratory, electrophysiological and neuroimaging data and pathogenetic therapy of pediatric patients with chronic inflammatory demyelinating polyneuropathy (CIDP). MATERIAL AND METHODS: Patients (n=30) were observed in a separate structural unit of the Russian Children's Clinical Hospital of the Russian National Research Medical University named after. N.I. Pirogova Ministry of Health of the Russian Federation in the period from 2006 to 2023. The examination was carried out in accordance with the recommendations of the Joint Task Force of the European Federation of Neurological Societies and the Peripheral Nerve Society on the Management of CIDP (2021). All patients received immunotherapy, including intravenous immunoglobulin (IVIG) (n=1), IVIG and glucocorticosteroids (GCS) (n=17, 56.7%), IVIG+GCS+plasmapheresis (n=12, 40.0%). Alternative therapy included cyclophosphamide (n=1), cyclophosphamide followed by mycophenolate mofetil (n=1), rituximab (n=2, 6.6%), azathioprine (n=3), mycophenolate mofetil (n=2, 6.6%). RESULTS: In all patients, there was a significant difference between scores on the MRCss and INCAT functional scales before and after treatment. At the moment, 11/30 (36.6%) patients are in clinical remission and are not receiving pathogenetic therapy. The median duration of remission is 48 months (30-84). The longest remission (84 months) was observed in a patient with the onset of CIDP at the age of 1 year 7 months. CONCLUSION: Early diagnosis of CIDP is important, since the disease is potentially curable; early administration of pathogenetic therapy provides a long-term favorable prognosis.

[Neuropathy in n-hexane poisoning]. / Neiropatiya pri otravlenii n-geksanom.

N-Hexane is a solvent widely used in manufacturing as a cleaner, degreaser and component of rubber cement. Chronic exposure to n-hexane either through contact with unprotected skin or inhalation can lead to the development of clinical symptoms and electrophysiological changes similar to those of inflammatory demyelinating polyneuropathy which requires careful differential diagnosis. This article presents three cases of severe predominantly motor polyneuropathy with demyelinating features in 15- and 16-year-old adolescents. The results of laboratory tests were within normal limits; electroneuromyography revealed symmetrical involvement of sensory and motor fibers of the nerves of the legs and arms with a decrease in the speed of propagation of excitation and conduction blocks. Sural nerve biopsy revealed intraneural and perineural swelling without any signs of inflammation or fibrosis confirming the genesis of the neuropathy. Despite a relatively favorable prognosis there is no specific therapy for hexane poisoning and the recovery period can last up to several years.

[Guillain-Barre syndrome in children]. / Sindrom Giiena­Barre u detei.

Guillain-Barré syndrome (GBS) is an immune-mediated disease of the peripheral nervous system that can occur in both children and adults. The classic presentation of GBS is characterized by progressive symmetrical, ascending muscle weakness. Patients with GBS require meticulous monitoring due to the risk of bulbar syndrome, respiratory failure and autonomic dysfunction, which can be life-threatening. Early diagnosis and timely prescription of pathogenetic therapy for GBS are particularly important, especially in young children. Meanwhile, the spectrum of disorders covered by GBS has expanded significantly; its eponym is now designate any variant of acute dysimmune polyneuropathy, and its atypical forms pose a serious diagnostic problem for clinicians. This review article provides an analysis of the data available in the medical literature on GBS in children and discusses the tactics for diagnosing and managing patients with GBS, taking into account the Russian and European clinical recommendations.

[Rare variant of median nerve compression: compression by brachial artery false aneurysm combined with carpal tunnel syndrome. Case report and literature review]. / Redkii variant kompressii sredinnogo nerva: kompressiya lozhnoi anevrizmoi plechevoi arterii v sochetanii s sindromom karpal'nogo kanala. Sluchai iz praktiki i obzor literatury.

BACKGROUND: The authors present severe compression of the median nerve by iatrogenic false aneurysm of the brachial artery combined with carpal tunnel syndrome. MATERIAL AND METHODS: An 81-year-old woman developed acute anesthesia of fingers I-III of the left hand, impaired flexion of the thumb and forefinger, swelling of the hand and forearm, local pain in postoperative period after angiography. The patient was previously followed-up for transient numbness in both hands for 2 years with a diagnosis of carpal tunnel syndrome. Electroneuromyography and ultrasound of the median nerve at the level of shoulder and forearm were carried out. We visualized a pulsatile lesion with Tinel's sign within the elbow (false aneurysm of the brachial artery). RESULTS: Resection of brachial artery aneurysm and neurolysis of the left median nerve were followed by regression of pain syndrome and improvement of motor function of the hand. CONCLUSION: This case demonstrates a rare variant of acute high compression of the median nerve after diagnostic angiography. This situation should be considered in differential diagnosis with classical carpal tunnel syndrome.

[Neuralgic amyotrophy]. / Nevralgicheskaya amiotrofiya.

A review of the literature covering the issues of etiology, pathogenesis, variety of atypical forms, diagnosis and management of patients with neuralgic amyotrophy is presented.

[Informative value of ultrasound in the diagnosis of intraneural perineurioma]. / Informativnost' UZI v diagnostike intranevral'noi perinevriomy.

OBJECTIVE: To describe clinical and sonographic features of confirmed intraneural perineurioma in 5 patients. MATERIAL AND METHODS: We report clinical and sonographic features and retrospective follow-up data in 5 patients with intraneural perineurioma verified by biopsy. RESULTS: In all cases, symptoms were represented by a slowly progressive painless mononeuropathy with muscle atrophy and impaired tendon reflexes. Ultrasound examination revealed locally enlarged cross-sectional area of the nerve that was confirmed by intraoperative visualization. CONCLUSION: Intraneural perineurioma should be obligatory suspected in case of fusiform thickening of the nerve with locally changed echogenicity. Pooled multiple-center studies with large samples are needed to determine sensitivity and specificity of the main ultrasound parameters including changes in cross-sectional area and echogenicity of the nerve.

[Mononeuropathy of dorsal interosseous nerve: compression by return radial arteries]. / Mononeiropatiia zadnego mezhkostnogo nerva: kompressiia vozvratnymi luchevymi arteriiami.

Among many causes of compression neuropathies involving the radial nerve, compression of the posterior interosseous nerve (PIN) by the returning radial arteries is rare. OBJECTIVE: To describe the clinical instrumental characteristics of cases of mononeuropathy caused by compression of the return radial arteries. MATERIAL AND METHODS: Two observations with neuropathy of the deep branch of the radial nerve, manifested by weakness in the corresponding muscles, inconsistent pain syndrome with previous stereotypic (recurring) physical activity are presented. RESULTS: According to the results of ultrasound examination of posterior interosseous nerve, a local hypoechogenic increase in the cross-sectional area of nerve of different lengths was revealed. During surgical decompression of the nerve, in the first case, a tight arterial loop was found around the nerve, in the second case, expanded arterial trunks, one of which, of small diameter, covered the nerve in the 'loop', and the other, larger, pierced the nerve. After the operation, regression of motor deficiency and relief of pain were noted.

[Chronic dysimmune hypertrophic plexopathy as a variant of atypical chronic inflammatory demyelinating polyneuropathy]. / Khronicheskaia dizimmunnaia gipertroficheskaia pleksopatiia kak variant atipichnoi khronicheskoi vospalitel'noi demieliniziruiushchei polineiropatii.

The authors report the clinical case of a 7-year-old child with gradual development in left leg weakness considering by the neurologist as the unknown lesion of the sciatic nerve. According to the results of clinical and instrumental examinations, the diagnosis was established as a focal form of chronic inflammatory demyelinating polyneuropathy (CIDP). The dysimmune cause of the disease was confirmed by MRI of the lumbosacral plexus with contrast enhancement of neural structures and response to therapy with high-dose intravenous immunoglobulin in combination with corticosteroids.

[An attempt to study the psychological sequelae of the accident at the Chernobyl Atomic Electric Power Station]. / Opyt izucheniia psikhologicheskikh posledstvii avarii na Chermobyl'skoi AES.

The paper describes the image of radiation menace. Basic differences in image parameters are revealed for some population groups. The psychological levels of the image are regarded as psychosocial phenomena. Some specific psychological consequences of mental regression are outlined in the paper.