[Experimental data from a study of the etiology of amyotrophic lateral sclerosis].
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Biomedical subjects
Publications and source records attributed to L M Popova.
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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 (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.
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.
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.
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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.
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.