[Morphology of oculomotor muscles and their nervous apparatus in lateral amyotrophic sclerosis in conditions during long-term artificial lung ventilation]. / Morfologiia glazodvigatel'nykh myshts i ikh nervnogo apparata pri bokovom amiotroficheskom skleroze, protekavshem v usloviiakh dlitel'noi iskusstvennoi ventiliatsii legkikh.

Light and electron microscopy, histochemical study of acetylcholinesterase (ACE) were used in examination of the state and innervation of ocular muscles in autopsy material from a patient who died of amyotrophic sclerosis (ATS). The patient had lived 14 years under artificial lung ventilation, ATS was diagnosed 22 years before the death. Light microscopy demonstrated the intactness of the muscle fibers and the presence of three types characteristic of the extraocular muscles: thin, granular and rough. Besides typical differences structural changes were observed in some muscle fibers of the myopathic character and inclusions not limited by membrane having filiform or granular structure. Ocular muscles had an intensive innervation. Nervous fiber terminals revealed by a reaction for ACE were represented by single motor plaques and multiple cluster-like endings. Ultrastructurally, nervous endings of two types differed by terminals and fold expression on the postsynaptic membrane. There were no pathological changes in axons and myelin. Thus, ocular muscles were not affected as well as their nervous apparatus at completion of AMS, this indicating the noninvolvement of this muscular allotype in a specific degenerative process.

[Role of multimodal evoked potentials in evaluations of persistent vegetative state of nontraumatic origin]. / Rol' mul'timodal'nykh vyzvannykh potentsialov v otsenke persistiruiushchego vegetativnogo sostoiania netravmaticheskogo geneza.

Persistent vegetative state (PVS) is a kind of release from coma, manifesting by absence of any signs of cognitive activity. Progress in reanimatology leads to an increase in the number of patients with PVS, thus creating a new medical and social problem. Study of atraumatic PVS acquires special importance because of a drastic increase in its incidence, necessitating definition of criteria of this state formation and its prognostic signs. Multimodal evoked potentials (MEP) now play an important role in diagnosis and prediction of PVS. The impact of acoustic stem (ASEP), visual (VEP), somatosensory (SSEP), and cognitive (CEP) evoked potentials is analyzed and early diagnostic and prognostic neurophysiological criteria are defined. Results of dynamic examinations of 23 patients (9 men and 14 women) aged 10-67 years with atraumatic PVS are presented. According to neurophysiological data, PVS in the majority of patients is characterized by absence of cognitive responses to a significant stimulus (wave P300) during examinations of CEP and of cortical response during examination of SSEP. VEP and long latent acoustic evoked potentials are as rule intact in atraumatic PVS. According to ASEP, stem functions were intact or slightly changed in the examined patients with PVS. Prognostically unfavorable and relatively favorable signs in the time course of MEP are defined.

Diphtheritic polyneuropathy: clinical analysis of severe forms.

BACKGROUND: Diphtheritic polyneuropathy (DP) is a dangerous complication of diphtheria, especially its severe forms with bulbar, respiratory tract, and circulatory disturbances. However, the clinical picture of severe forms of DP is practically unknown. OBJECTIVE: To investigate the clinical features and peculiarities of the course of severe forms of DP. PATIENTS: Thirty-two patients with severe forms of DP. RESULTS: The first symptoms of DP developed in most patients 3 to 5 weeks after the onset of diphtheria. The cranial nerves were involved in all patients, most frequently nerves IX and X (32 patients); VII (28 patients); III, IV, and VI (27 patients); and XI (27 patients). One third of the patients had quadriplegia. The remaining patients had quadripareses. Of the 32 patients, 24 underwent artificial ventilation. All patients had sensory signs, proprioceptive more often than superficial. Autonomic disturbances were observed also in all patients. Only 2 of the 32 patients died. CONCLUSIONS: A direct indication for tracheotomy and artificial ventilation in patients with DP is a decrease of the vital capacity of the lungs below the traditional 16 mL/kg body weight or the development of the paralytic closure of the larynx against the background of the increasing weakness of the respiratory muscles. Characteristic of severe forms of DP is the phenomenon of the oppositely directed change in the neurological symptoms in the second month of the disease: the restoration of the function of the cranial nerves against the background of the further increase of the motor disturbances in the extremities and trunk. Special attention and care should be taken of patients during the period of the appearance of the episodes of vascular collapses-between the fourth and seventh weeks of DP.

[Respiratory failure in disseminated sclerosis]. / Dykhatel'naia nedostatochnost' pri rasseiannom skleroze.

The development and patterns of respiratory failure (RF) are analyzed in 9 patients with disseminated sclerosis (DS). Forced ventilation of the lungs was carried out with consideration for main location of the process. Relationship between patterns of respiratory disorders and neuroanatomy of respiratory regulation is discussed. Involvement of the corticospinal routes is paralleled by dissociation during functional pulmonary tests: spontaneous volumes are less than controlled inspirations. The most severe symptom complexes were observed in RF of predominantly bulbar localization: respiratory anarchy, blocking of airways caused by impaired swallowing, impaired mechanism of coughing reflex, loss of spontaneous respiration, sometimes apnea during sleeping. Involvement of the respiratory nuclei of medullary respiratory center and airways and of the corticonuclear routes of caudal cranial nerves causes the development of a triad of symptoms: glossopharyngolaryngeal paralysis, dysfunction of respiratory nuclei of medulla oblongata, and decreased sensitivity of respiratory center to CO2. Aspiration complications caused by dysphagia are characteristic of bulbar DS. Respiratory function in 5 patients without clinical picture of RF are specially discussed. The authors emphasize unfavorable prognostic significance of signs of extracorporeal obstruction indicating the probability of RF long before its manifestation. Special attention is paid to early diagnosis of symptoms of coming RF when evaluating the status of patients with DS during treatment. Timely use of respiratory resuscitation methods reduces the mortality and ensures a good chance for remissions with recovery of respiratory function, which are characteristic of RF.

[Videofluoroscopy study of swallowing in neurogenic dysphagia]. / Videofliuoroskopicheskoe issledovanie glotaniia pri neirogennoi disfagii.

Videofluoroscopy (VFS) was for the first time used for examining swallowing in 49 patients with nervous diseases. Disturbances in each phase of swallowing act are analyzed with evaluation of the time parameters and defects, causes of aspiration in neurogenic dysphagia are discussed, and cricopharyngeal insufficiency is described. Neurogenic dysphagia is characterized by a combination of disorders which determine the degree of dysphagia. The most severe swallowing disorders were observed in patients with multiple foci in the brain stem and in diphtheritic polyneuropathies. The authors conclude that VFS is the optimal method for the diagnosis of neurogenic dysphagia.

[Pathology of the brain in Creutzfeldt-Jakob disease]. / Patologiia golovnogo mozga pri bolezni Kreittsfel'dta-Iakoba.

The autopsy cases of Creitsfeldt-Jacob disease (a sporadic form) are reported which were diagnosed clinically and supported by the data of biopsy and autopsy of the brain (classic triad: death of neurons, astrogliosis and spongiform degeneration of the gray substance of the cortex of brain hemispheres, preferentially), followed by clinical morphologic comparisons. The focal character of the disease was observed on the early stages of the disease, while diffuse alterations were found on the late stages.

[The treatment of demyelinating polyneuropathies of different origins with small doses of immunoglobulin G]. / Lechenie demieliniziruiushchikh polineiropatii razlichnogo geneza malymi dozami immunoglobulina G.

Application of small doses of IgG-Sandoglobulin (0.16 to 0.98 g per kg of body weight) was quite effective in 2 patients with Guillain-Barré syndrome and in 2 cases of diphtheritic polyneuropathy. These patients had a severe form of the disease with disorders of essential functions. An international course of the disease in one case of IgG treatment was observed. Meanwhile preparation was uneffective in 2 patients with chronic inflammatory demyelinating polyneuropathy caused by lymphoproliferative process. It is worth while to accumulate experience to define optimal doses of the drug.

[Respiratory insufficiency in amyotrophic lateral sclerosis and methods of treatment]. / Dykhatel'naia nedostatochnost' pri amiotroficheskom bokovom skleroze i metody ee lecheniia.

Respiratory failure in amyotrophic lateral sclerosis is caused by paralysis and paresis of the respiratory and glossopharyngeolaryngeal muscles obstructing and restricting ventilation of the lungs. Examination of the external respiratory function helps early diagnose the condition. Monitoring of vital capacity of the lungs and respiratory rate is the most informative method. Analyzing the problem of progressive respiratory failure, we cannot neglect its ethical, social, and financial aspects. Informed consent of patients, families, and sponsors to prolonged forced ventilation of the lungs is to be obtained before the condition progresses to critical.

[Prolonged artificial ventilation of the lungs in nervous system diseases]. / Mnogoletnaia IVL pri zabolevaniiakh nervnoi sistemy.

Artificial ventilation of the lungs (AVL) has been carried out for many years in 25 patients with nervous diseases. Spontaneous respiration ceased because of peripheral neuron involvement in malignant myasthenia (5 cases), chronic polyneuropathy (1), paralysis of motor muscles after poliomyelitis (1), amyotrophic lateral sclerosis (11), spinopontocerebellar atrophy (1), and because of the central neuron involvement in 5 patients, 3 of these with the apnea syndrome during sleeping caused by syringobulbomyelia, atrophic process in the cerebellum and stem, and Arnold-Chiari's syndrome, and 2 with an extensive infarction in the pons (deefferentiation or locked-in syndrome). AVL was the longest in patients with paralysis of the motor muscles following acute poliomyelitis (23 years) and with amyotrophic lateral sclerosis (about 14 years). Prolonged AVL is possible in malignant myasthenia, but it may be discontinued during obligatory specific treatment. Specific features of many-year AVL, somatic functions and metabolic disorders associated with it are described.

[Intensive care in myasthenic and cholinergic crises]. / Intensivnaia terapiia miastenicheskikh i kholinergicheskikh krizov.

Presents the epidemiology, possible etiology, and pathogenesis of myasthenia, methods of diagnosis of the disease, and clinical picture of critical states. Describes the present-day methods of treating myasthenic and cholinergic crises: programmed plasmapheresis combined with hormone therapy, pulsed therapy with superhigh doses of corticosteroids, and the most novel method for the treatment of autoimmune diseases, intravenous infusions of immunoglobulins. Draws attention to indications for artificial ventilation of the lungs and tracheostomy. Enumerates the drugs which are not recommended for patients with myasthenia. Emphasizes that the use of the whole complex of modern therapeutic methods for the treatment of grave myasthenia helps reduce the mortality to virtually null.

[Respiratory insufficiency in adults with diphtheric polyneuropathy]. / Dykhatel'naia nedostatochnost' pri difteriinoi polineiropatii u vzroslykh.

Twenty-five adult patients with grave diphtheric polyneuropathy after toxic diphtheria were followed up. Two symptom complexes of neurologic disorders leading to the development of peripheral respiratory failure of different severity were distinguished: 1) predominating glossopharyngeal paralysis and 2) combination of glossopharyngeal paralysis with grave generalized sensorimotor polyneuropathy (with pareses and paralyses of the respiratory muscles). The major quantitative parameters of pulmonary functions associated with various manifestations of respiratory failure are characterized. The significance of information on the type of dysfunction of vocal cords is emphasized. A high efficacy of a complex of respiratory reanimation used in the treatment of patients with peripheral respiratory failure is demonstrated.

[Prognostic significance of neurophysiological parameters in nontraumatic apallic syndrome]. / Prognosticheskoe znachenie neirofiziologicheskikh pokazatelei pri netravmaticheskom apallicheskom sindrome.

The time course of neurophysiological parameters (EEG, evoked potentials of various modalities, including P300, and polysomnography) was followed up in 10 patients with apallic syndrome (AS) of nontraumatic origin (heart arrest, stroke, neuroinfection, etc.) during the formation of the syndrome and the condition of various severity: incomplete formation of AS eventuating in gross psychoneurological defects and complete formation of AS (a stable vegetative state). A dynamic follow-up of not only EEG of awaken and sleeping patients, but of their evoked potentials, specifically somatosensory and P300, were shown to be significant for assessment of the course of AS and prediction of its outcome.

[The development of the science of neural resuscitation]. / Razvitie neiroreanimatologii.

The complex approach to both restoration and maintenance of vital functions in patients with severe nervous system diseases is described in this article. The respiratory reanimation is thought to be the main problem of neuroreanimatology. The long-term experience of the Institute of Neurology of Rus. Acad. Med. Sci. in this field is presented. It was concerned with the arbitrary respiratory control disorders, automatic respiratory regulation disturbances, the damages of peripheral centers associated with respiratory function.

[Course of apallic syndrome after clinical death during anesthesia]. / Evoliutsiia apallicheskogo sindroma posle klinicheskoi smerti v narkoze.

Anesthesiological errors after clinical death during narcosis for strumectomy are considered. These errors were continuation of surgery after systole, inadequate management of the systemic arterial pressure and cerebral circulation after global ischemia, early switch-off of artificial ventilation of the lungs. All these steps led to prolonged coma, development of the apallic syndrome, and invalidism of the patient.

[Activation of free-radical processes as a factor of ionizing radiation-induced changes in contractile activity of a vascular wall]. / Aktyvatsiia vilnoradykalnykh protseciv iak faktor porushennia skorochuvalnoi aktyvnosti sudynnoi stinky pid diieiu ionizuiuchoi radiatsii.

The influence of ionizing irradiation (1, 2 and 4 Gy 137Cs) on both the activity of free-radical processes in plasma, formed elements and aorta wall as well as on the character of contractile vascular reactions of isolated rings of thoracic aorta and carotid artery in rabbits has been studied. The experiments were carried out on the 7th day after the whole-body irradiation. The results indicate that simultaneously with the weakening of antioxidant mechanisms both endothelium-dependent and endothelium-independent vascular wall relaxation slightly decreases after 1 Gy exposure. Noradrenaline and KCI-induced contraction is shown to increase. However, these changes are not statistically significant. Irradiation in dose of 2 and 4 Gy considerably decreases endothelium-dependent relaxation. Nitroglycerin-induced relaxation greatly diminishes, KCI- and noradrenaline-induced constriction considerably increases in these conditions. The level of activation of free-radical processes considerably increases too. Thus, already on the 7th day after irradiation significant changes in reactivity of vascular wall are developed. Radiation injures both endothelium and vascular smooth muscle cells. The free-radical processes seem to be the main cause of radiation vascular damage, so there is a pronounced correlation between the changes of vascular contractile properties and the degree of activation of these processes.

[The clinical picture of non-traumatic apallic syndrome]. / Klinika netravmaticheskogo apallicheskogo sindroma.

Clinical signs of non-traumatic apallic syndrome (AS) are caused by universal necrosis of the brain cortex, with the brain stem remaining intact. The reasons of AS are heart and respiration arrest, stable collapses of various genesis, vascular brain diseases. Management was ineffective. Strict AS prevention is necessary, i.e. timely and expert cardiorespiratory resuscitation. Intensive care and cardiorespiratory resuscitation are not recommended in patients with vast brain infarction secondary to brain vascular diseases.

[Clinical course of amyotrophic lateral sclerosis during life support care]. / Klinika bokovogo amiotroficheskogo skleroza v usloviiakh prodlennoi zhizni.

The authors provide the data on lateral amyotrophic sclerosis (LAS) in 11 patients whose life was extended during respiratory failure by respiratory resuscitation and intensive care. The disease was established to run a cyclic course. The following clinical disease periods were distinguished: period of precursors, local motor disorders period, and period of initial and marked generalization. De-efferentation was the final period. The latter one was characterized by the arrest of all motor functions, namely by total paralysis with the exception of the function of oculomotor nerves. Motoneurons of the truncus cerebri nuclei disintegrated much more slowly than those of the spinal cord. The phase of de-efferentation lasted 8 years whereas the total disease period reached 23 years. Intellectual functions were preserved whatever the disease periods. Slow progress of motoneuronal disintegration enables using the respiratory systems both inpatiently and at home. Respiratory resuscitation and intensive care can be employed, if the patient and his relatives give their consent.

[Apnea during sleep in syringobulbomyelia and Arnold-Chiari deformity]. / Apnoé vo sne pri siringobul'bomielii i mal'formatsii Arnol'da--Kiari.

Apnea in sleeping patients with syringobulbomyelia can be accounted for by damages in afferent and efferent impulsation from the respiratory centre due to cysts in medulla oblongata and spinal channel distension. Controlled lung ventilation has been applied to such patients in sleep for more than 8 years. In Arnold-Chiari syndrome apnea in sleep is associated with paroxysms of occlusive hydrocephalus. Periodic controlled lung ventilation during sleep is obligatory in these patients. Both nosological forms are characterized by lower sensitivity of the respiratory centre to hypoxia and hypercapnia. The nature of the pathological process is confirmed by magnetic resonance tomography.