Hirayama disease. Clinical cases

Hirayama disease, or monomelic amyotrophy, is a rare neurological pathology manifested by unilateral or bilateral asymmetric paresis with atrophy of the distal upper extremities. The development of this disease is associated with the forward displacement of the posterior dural sac during neck flexion, which leads to compression of the spinal cord and venous stasis. The diagnosis of monomelic amyotrophy is based on the clinical picture and the results of magnetic resonance imaging of the cervical spine with flexion, showing segmental atrophy of the anterior horns of the spinal cord at C7 — Th1, detachment of the posterior dura mater and venous stasis. Most of the cases described in the literature are from India and Japan, with isolated cases diagnosed in North America and Europe. This article presents two clinical cases of progressive hand weakness in young men. The clinical picture of the disease and the results of additional examination methods are presented, which is necessary to establish a diagnosis of Hirayama’s disease.

New association between idiopathic scoliosis and Luckenschadel skull (lacunar skull)

Objective: Luckenschadel skull is a skull that is radiologically characterized by lacunae in the cranial vault. To date, although the association between neurological abnormalities and scoliosis is well-recognized, no relationship between idiopathic scoliosis and a lacunar skull has been defined. We explored the incidence and time courses of lacunar skulls in patients with idiopathic scoliosis. Materials and Methods: Spinal X-rays of 3,170 children aged 6 to 16 years with idiopathic scoliosis evaluated from October 2010 to August 2020 were examined for the presence of an irregular inner calvarial table indicative of a Luckenschadel skull. A total of 1,760 (55.5%) of the 3,170 images included the skull. We also explored the frequency of intraspinal abnormalities in children with lacunar skull images who underwent spinal magnetic resonance imaging. Results: The study population consisted of 1,760 children, 1,203 girls (68.4%) and 557 (31.6%) boys. A total of 268 (15.2%) clearly evidenced lacunar skulls in their radiographs; 186 (69.4%) girls(11.3±4.3 years) and 82 (30.6%) boys(12.6±3.3 years). In total, 2 of 56 patients (3.6%) who underwent spinal MRI had intraspinal abnormalities (isolated Chiari malformation-I). No additional neurological problems were detected in children with lacunar skulls. Conclusion: We are the first to report that the lacunar skull is very common in children with idiopathic scoliosis who lack any other neurological pathology. The lacunar skull does not disappear even in adolescence. Although previous publications have stated that lacunar skull disappears over time in radiographic images, we found that it became more noticeable over time in children with scoliosis.

Neonatal Seizures: Core Concepts

Seizures are the abnormal, excessive, synchronous discharge of cortical neurons that results in injury to the brain. Seizures presenting in the neonatal period may be the first and only clue to underlying neurological pathology. Despite advances in care, the mortality rate for infants experiencing neonatal seizures is still as high as 20 percent, with up to 65 percent of infants with seizures demonstrating significant morbidity. Early identification and treatment of the seizure or modifiable underlying etiology greatly reduces the extent of morbidity associated with neonatal seizures. Literature, including journal articles and relevant textbooks, was reviewed and condensed into a practical guide to neonatal seizures which includes the pathophysiology of injury associated with neonatal seizures, clinical manifestations, methods of diagnosis, and various options available for treatment.

Algorithm for the diagnosis of breathing disorders during sleep in children with neurological pathology

Introduction. Sleep disorders in children with nervous system pathology are more pronounced than in the general population. One of the most common causes of sleep disorders in children is sleep-disordered breathing. The aim of our study was to create an algorithm for the diagnosis of sleep breathing disorders in children with neurological pathology. Materials and methods. The results of night studies (160 polysomnographies, 98 cardiorespiratory monitorings) carried out in the department of instrumental diagnostics at the National Medical Research Center for Children’s Health in 258 children (154 boys and 104 girls, aged one month to 17 years) with neurological pathology were analyzed. Questionnaires were used to assess the quality of sleep. An essential criterion for dividing children into groups was the presence of maxillofacial anomalies (MFA). The results were compared with complaints according to questionnaires, age, BMI and ENT-status of patients. Results. In the general sample, a significant (p < 0.001) relationship between respiratory disorders and complaints and the presence of ENT pathology was obtained. The most severe breathing disorders were recorded in children with MFA (p < 0.001). The result was the creation of an algorithm for examining children with neurological pathology. Conclusion. Children with neurological pathology in sleep-breathing disorders require a personalized approach to correcting the causes of these disturbances, depending on the nosology. Chronic pathology of the ENT organs aggravates respiratory disorders during sleep. Regular monitoring of sleep disorders using questionnaires should be included in the algorithm for managing children with neurological diseases of all ages for early detection of these disorders.

Thermosemiotics of hands. Neuropathic disorders in thermotopography of hands

Central and peripheral neurological pathology, which affects the thermal pattern and thermoreactions of the hands, is diverse both in etiology and pathogenesis, and in the nature and severity of thermal imaging signs, depending on the localization, severity and duration of the disease, individual adaptive and compensatory features, and a number of other reasons. The variants of the temperature distribution on the surface of the hands and its changes under the influence of specialized functional tests in injuries and diseases of the peripheral nerves of the upper limb, as well as in disorders of segmental and suprasegmental genesis, studied with the help of thermal imaging, are the subject of this article.

Immune Response in Neurological Pathology: Emerging Role of Central and Peripheral Immune Crosstalk

Neuroinflammation is a key component of neurological disorders and is an important therapeutic target; however, immunotherapies have been largely unsuccessful. In cases where these therapies have succeeded, particularly multiple sclerosis, they have primarily focused on one aspect of the disease and leave room for improvement. More recently, the impact of the peripheral immune system is being recognized, since it has become evident that the central nervous system is not immune-privileged, as once thought. In this review, we highlight key interactions between central and peripheral immune cells in neurological disorders. While traditional approaches have examined these systems separately, the immune responses and processes in neurological disorders consist of substantial crosstalk between cells of the central and peripheral immune systems. Here, we provide an overview of major immune effector cells and the role of the blood-brain barrier in regard to neurological disorders and provide examples of this crosstalk in various disorders, including stroke and traumatic brain injury, multiple sclerosis, neurodegenerative diseases, and brain cancer. Finally, we propose targeting central-peripheral immune interactions as a potential improved therapeutic strategy to overcome failures in clinical translation.

Vibrations and spasticity

This experimental study aims to evaluate and validate the effectiveness of the proposed application method in the use of "focused mechanical vibrations" for the treatment of muscle spasticity. The basic concept of this study is that in spasticity, by specifically stimulating some clearly identifiable trigger points of the body, better results are obtained; in fact, with the localized mechanical-sound vibrations, by positioning the handpiece for the treatment on those specific points, immediate generalized relaxation of the entire spastic muscle is obtained through reflex pathways. To evaluate this operative reality we treated 5 subjects affected by neurological pathology with spasticity and at T0 time (before treatment) and at T1 time (immediately after treatment), they were assessed with Ashworth Scale, Passive joint evaluation, Evaluation of active motility, Test of Bahkta (for non-functioning hand), surface electromyography The subjects were treated with a 120Hz vibratory therapy handpiece for a total time of 15 minutes The data showed us a clear improvement of the parameters lost in the exam, in the less serious patients and a good improvement also in the most serious subjects.

About the Conditions, Issues and Problems of Children Palliative Care Using the Example of the Children’s Hospice Activity in Saint Petersburg

The topicality of reforming the children palliative care (CPC) system due to the increase of the number of patients with cancer, congenital disorders, neurological damages, injuries consequences, genetically determined pathologies is discussed. The patterns of CPC service development, the importance to track those in need, to determine the required volume, forms, and methods of palliative care, and to develop human capacity were analyzed. The results of “Children's Hospice” (Saint Petersburg) activities in 2014–2019 years are presented alongside with the data on CPC development in Russia. This example shows the advantages of clear routing, establishment of indications for patients’ hospitalization, and availability of high-technology medical care and medications. All innovations have led to the significant change in medical conditions structure (over 80% of patients are children with neurological pathology), to the decrease in the number of hospitalizations, to the increase in the number of children who can get required respiratory support at home. The issues of high employees’ turnover and low staffing level in CPC institutions are discussed.