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Perovskite-type La0.5Sr0.5Fe0.9Mo0.1O3-δ synthesized via glycine nitrate combustion and sintered at 1350 °C was found to have an orthorhombic lattice, which transforms upon heating into a rhombohedral and then a cubic one. The oxygen content and electrical conductivity in this oxide were measured in the range of oxygen partial pressures from 10-20 to 0.5 atm at 750-950 °C by coulometric titration and four-probe dc techniques, respectively. The oxygen content data were used to model the defect equilibrium in the oxide. Oxidation, charge disproportionation and electron exchange reactions between iron and molybdenum were assumed by the model to be involved in the formation of defects. The experimental data were well approximated with the model and the concentrations of charge carriers in La0.5Sr0.5Fe0.9Mo0.1O3-δ were determined to be used for the electrical conductivity analysis. The average mobility of oxygen ions and n- and p-type charge carriers was determined to be about 10-5, 0.007, and 0.07 cm2 V-1 s-1 with an activation energy of 0.80 ± 0.02, 0.34 ± 0.01, and 0.23 ± 0.01 eV, respectively. Comparison with La0.5Sr0.5FeO3-δ shows that 10% Mo substitution provides a substantial increase in both the concentration and mobility of n-type carriers, which results in an almost threefold increase in electron conductivity under reducing conditions, while maintaining a high level of ionic conductivity.
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The article presents the work of a multidisciplinary team of experts from various fields of medicine to optimize the «Questionnaire for assessing chronic pelvic pain and pelvic organ dysfunction (QCPPD) of the Ryzhikh National Medical Research Centre for Coloproctology¼ for use in clinical practice. The survey of respondents was conducted from June 28 to September 28, 2021. As a result of this survey, by repeatedly making edits and clarifications during communication with respondents, the final version was obtained, which allows assessing the patient's subjective sensations by the nature and localization of pelvic pain, sensitivity disorders and pelvic organ function. The main objective of this Questionnaire is to differentiate patients with neurogenic pain from a huge number of patients with chronic pelvic pain. This aspect will allow a more targeted approach to the diagnosis and pathogenetically justified treatment of patients, including after appropriate instrumental examinations. The work of a multidisciplinary team implies a higher degree of objectification and terminological accuracy of the Questionnaire under discussion. The presented version of the «Questionnaire for assessing chronic pelvic pain and pelvic organ dysfunction (QCPPD) of the Ryzhikh National Medical Research Centre for Coloproctology¼ will be primarily used in coloproctological patients with pelvic pain problems and anal incontinence and obstructive defecation. Further studies will be directed to the clinical evaluation of the results of the work carried out.
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Incontinencia Fecal , Insuficiencia Multiorgánica , Humanos , Dolor Pélvico/diagnóstico , Dolor Pélvico/etiología , Estreñimiento , Encuestas y CuestionariosRESUMEN
Pathogenic variants in the HINT1 gene lead to hereditary axonopathy with neuromyotonia. However, many studies show that neuromyotonia may remain undiagnosed, while axonopathy is the major clinical finding. The most common cause of neuromyotonia and axonopathy, especially in patients of Slavic origin, is a c.110G>C (p.Arg37Pro) pathogenic variant in homozygous or compound heterozygous state. In this study, we analyzed a peripheral neuropathy caused by pathogenic variants in the HINT1 gene and evaluated its contribution to the hereditary neuropathy structure. The studied group included 1596 non-related families diagnosed with hereditary motor and sensory neuropathy (HMSN). The results show that HINT1 gene pathogenic variants make a significant contribution to the hereditary neuropathy epidemiology in Russian patients. They account for at least 1.9% of all HMSN cases and 9% of axonopathy cases. The most common HINT1 pathogenic variant in Russian patients is the c.110G>C (p.Arg37Pro) substitution. Its allelic frequency is 0.2% (95% CI 0.19-0.21%), carrier frequency is 1 in 250 people in Russian Federation, and the estimated disease incidence is 1 in 234,000 individuals. It was determined that the cause of this pathogenic variant's prevalence is the founder effect.
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Genes Recesivos , Neuropatía Hereditaria Motora y Sensorial/genética , Mutación/genética , Proteínas del Tejido Nervioso/genética , Frecuencia de los Genes/genética , Haplotipos/genética , Neuropatía Hereditaria Motora y Sensorial/epidemiología , Heterocigoto , Humanos , Desequilibrio de Ligamiento/genética , Repeticiones de Microsatélite/genética , Federación de RusiaRESUMEN
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.
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Neoplasias de los Nervios Craneales , Neoplasias de la Vaina del Nervio , Neoplasias del Sistema Nervioso Periférico , Humanos , Neoplasias de la Vaina del Nervio/diagnóstico por imagen , Neoplasias de la Vaina del Nervio/cirugía , Neoplasias del Sistema Nervioso Periférico/diagnóstico por imagen , Neoplasias del Sistema Nervioso Periférico/cirugía , Estudios Retrospectivos , UltrasonografíaRESUMEN
Differential high-resolution ECG (V1-V2) and pelvic electric potential measured between the coccyx and perineum were recorded simultaneously in resting supine position in men with autonomic nervous system disorders (N=37). In healthy volunteers (N=23), the effective (rms) value of PEP presented by median and interdecile range was 30 (20-80) µV within the frequency band of 0.03-80 Hz. In patients, the corresponding value was significantly higher: 140 (80-280) µV. In both groups, the amplitude harmonic spectrum of pelvic electric potential decreased monotonically with frequency according to 1/f1.6 law. In some patients (N=16), rare single or grouped high-amplitude impulses (up to 1 mV) of pelvic electric potential with total duration of about 1 sec were observed; of them, some persons (N=7) demonstrated practically one-to-one synchronous relations between these impulses and arrhythmia episodes indicating abnormal activity of the autonomic nervous system as their most probable common cause. The high-amplitude pelvic electric potential impulses were also observed in ECG records as interference signals with an amplitude attaining 50 µV. Thus, high-resolution ECG and pelvic electric potential can reveal the risk of abnormal neurogenic influences on the heart. The data obtained are discussed in relation to diagnostics of the autonomic nervous system disorders, neurogenic arrhythmias, and risk of sudden cardiac death.
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Arritmias Cardíacas/diagnóstico por imagen , Enfermedades del Sistema Nervioso Autónomo/diagnóstico por imagen , Electrocardiografía/métodos , Corazón/diagnóstico por imagen , Potenciales de la Membrana/fisiología , Pelvis/diagnóstico por imagen , Adulto , Arritmias Cardíacas/fisiopatología , Enfermedades del Sistema Nervioso Autónomo/fisiopatología , Biomarcadores/análisis , Estudios de Casos y Controles , Cóccix/diagnóstico por imagen , Cóccix/inervación , Cóccix/fisiopatología , Muerte Súbita Cardíaca/prevención & control , Corazón/inervación , Corazón/fisiopatología , Frecuencia Cardíaca/fisiología , Humanos , Masculino , Persona de Mediana Edad , Pelvis/inervación , Pelvis/fisiopatología , Perineo/diagnóstico por imagen , Perineo/inervación , Perineo/fisiopatología , RiesgoRESUMEN
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.
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Mononeuropatías , Síndromes de Compresión Nerviosa , Neuropatía Radial , Humanos , Arteria Radial , Nervio RadialRESUMEN
The expert consensus is aimed to develop an algorithm for the diagnosis and treatment of mononeuropathies for outpatient neurologists. Leading experts in the field of neurology have suggested workup options for certain types of tunnel mononeuropathies based on current data on the effectiveness and safety of various types of conservative and surgical treatment.
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Algoritmos , Humanos , Consenso , Mononeuropatías/diagnóstico , Mononeuropatías/terapia , Síndrome del Túnel Carpiano/diagnóstico , Síndrome del Túnel Carpiano/terapia , Síndrome del Túnel Carpiano/cirugíaRESUMEN
During examination of 165 children aged 5 to 15 years (primarily identified during planned monitoring in Petrozavodsk children's institutions) with dysfunctional urination and encopresis without organic lesion of the central nervous system, autonomic dysfunction syndrome (ADS) was revealed. According to the results of urological examination, which was supplemented with the registration of spontaneous voiding rate and counting the radial pulse, overactive bladder syndrome and insufficient relaxation of the pelvic floor muscles during urination and defecation were detected; relationship between the number of heart rate (as a marker of sympathetic nervous system activity) and the effective volume was identified. It was revealed that the children with ADS in the presence of tachycardia show intermittent decrease of effective amounts of urination, and have residual urine. The standard course of treatment using colon hydrotherapy and biofeedback to activate cystic and obturator reflex caused a positive but short-term therapeutic effect; clinically and statistically significant increase in the effective volume of the bladder was not achieved, despite the reduction in residual urine volume. During the course of treatment using methods of biofeedback, bladder volume remained almost unchanged and tachycardia persisted, indicating the continued oppression of the sympathetic activity. The course of treatment using nootropic drug picamilon and alpha-adrenoblocker doxazosin with peripheral actions allowed to restore the reservoir and evacuation functions of the bladder, to achieve a regular bowel movement without encopresis. It was revealed that the combined dysfunction of pelvic organs occur in children with high activity of the sympathetic division of the ANS, which has a direct impact on the accumulation phase of voiding cycle and relaxation of the pelvic floor muscles.
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Enfermedades del Sistema Nervioso Autónomo , Vejiga Urinaria Hiperactiva , Incontinencia Urinaria , Adolescente , Antagonistas de Receptores Adrenérgicos alfa 1/administración & dosificación , Enfermedades del Sistema Nervioso Autónomo/patología , Enfermedades del Sistema Nervioso Autónomo/fisiopatología , Enfermedades del Sistema Nervioso Autónomo/terapia , Niño , Preescolar , Doxazosina/administración & dosificación , Femenino , Humanos , Hidroterapia , Masculino , Nootrópicos/administración & dosificación , Vejiga Urinaria Hiperactiva/patología , Vejiga Urinaria Hiperactiva/fisiopatología , Vejiga Urinaria Hiperactiva/terapia , Incontinencia Urinaria/patología , Incontinencia Urinaria/fisiopatología , Incontinencia Urinaria/terapia , Ácido gamma-Aminobutírico/administración & dosificación , Ácido gamma-Aminobutírico/análogos & derivadosRESUMEN
Men aged over 40 years more commonly develop NK/T-cell lymphomas (NK/T-CL). The paper describes a case of NK/T-CL in a 20-year-old man. Despite the fact that the disease (nasal septum perforation, hard palate bone destruction, recurrent nasopharyngeal bleeding, considerable weight loss, and high erythrocyte sedimentation rate,) progressed rapidly for 5 months, the patient was found to be diagnosed as having Wegener' granulomatosis (WG). Repeated incisional biopsies showed massive necrotic changes with no clear histological verification of the diagnosis. The absence of lung and kidney lesions typical of WG, the lack of antineutrophil antibodies, and the detection of Epstein-Barr virus DNA in blood could presume NK/T-CL and confirm it by extended biopsy to have materials sufficient for morphological and immunomorphological studies. This observation shows that the disease may occur at a young age and rapidly progress; only early diagnosis can improve prognosis in patients with this type of lymphomas.
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Granulomatosis con Poliangitis/diagnóstico , Linfoma Extranodal de Células NK-T/diagnóstico , Neoplasias Nasales/diagnóstico , Factores de Edad , Biopsia , Diagnóstico Diferencial , Progresión de la Enfermedad , Diagnóstico Precoz , Granulomatosis con Poliangitis/patología , Humanos , Linfoma Extranodal de Células NK-T/patología , Masculino , Neoplasias Nasales/patología , Pronóstico , Adulto JovenRESUMEN
The authors present a review of literature focusing on state-of-art of surgical management of cervical spondylogenic myelopathy (CSM). Pathophysiology of CSM, evaluation and differential diagnosis are also described. Special attention is given to the value of neurophysiological aspects in pre- and postoperative examination.
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Compresión de la Médula Espinal , Espondilosis , Diagnóstico Diferencial , Fenómenos Electrofisiológicos , Humanos , Monitoreo Intraoperatorio , Examen Neurológico/métodos , Procedimientos Neuroquirúrgicos/métodos , Pronóstico , Índice de Severidad de la Enfermedad , Compresión de la Médula Espinal/diagnóstico , Compresión de la Médula Espinal/etiología , Compresión de la Médula Espinal/cirugía , Espondilosis/complicaciones , Espondilosis/diagnóstico , Espondilosis/cirugía , Estimulación Magnética TranscranealRESUMEN
Dorsalgia's is an actual medical and social problem. It gains prominent significance among railway workers, connected with railway communication. Pain syndromes among this group of patients have different complex pathogenetic mechanisms of the development. In a great number of cases it is usually seen the worsening of the condition in connection to concomitant anxiety and depression. There are also concomitant osteoporosis and autonomic trophical disturbances.
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Enfermedades Profesionales/fisiopatología , Dolor/fisiopatología , Vías Férreas , Ansiedad/patología , Ansiedad/fisiopatología , Depresión/complicaciones , Depresión/patología , Depresión/fisiopatología , Femenino , Humanos , Masculino , Enfermedades Profesionales/etiología , Enfermedades Profesionales/patología , Osteoporosis/etiología , Osteoporosis/patología , Osteoporosis/fisiopatología , Dolor/etiología , Dolor/patologíaRESUMEN
Small variations of electric impedance (bioimpedance) of human penis were examined in healthy volunteers and in patients with vascular erectile dysfunction (ED). The harmonic analysis revealed rhythmic oscillations of penile bioimpedance at frequencies corresponding to the heart and respiration rates and Mayer wave (0.1 Hz) and to multiple frequencies (harmonics) of the respiratory and cardiac oscillations. In normal penile bioimpedance spectrum, the Mayer and respiratory peaks were several times higher than the first cardiac (pulsatile) harmonic indicating neurogenic origin of rhythmic bioimpedance variations in the whole penis. The most of healthy individuals (78%) demonstrated the cardiac harmonics at the frequency range of 4-7 Hz that violated the monotone decrement of the pulsatile harmonic series suggesting the resonant character of oscillations of the penile arteries at this "near" frequency range. In contrast to stable 1-4 cardiac harmonics, the amplitudes of the near-range resonant harmonics could vary during few minutes suggesting a causal relation of the corresponding bioimpedance oscillations with the varying vascular tone in penile arteries. The most patients (89%) with vascular ED demonstrated not only the first 1-4 monotonically decrementing harmonics and the near-resonant ones, but also the stable cardiac harmonics at the "far" frequency range of 8-14 Hz that also disturbed the monotonic character of the cardiac harmonic series indicating the sclerotic alterations in regional arteries. In ED patients, insignificant decrease of the initial cardiac harmonics C1-C3 in comparison with the norm was accompanied by pronounced and significant decrease of the respiratory R1 and Mayer M1 peaks. The study showed that the far-frequency bioimpedance resonances at the range of 8-14 Hz and dramatic drop of Mayer and respiratory peaks are the diagnostic signs of vascular ED independent on the accompanying hormonal or neurogenic disorders.
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Impedancia Eléctrica , Disfunción Eréctil/fisiopatología , Pene/fisiología , Periodicidad , Adolescente , Adulto , Anciano , Estudios de Casos y Controles , Humanos , Masculino , Persona de Mediana Edad , Pene/irrigación sanguínea , Pene/fisiopatología , Adulto JovenRESUMEN
The article analyses problems of diagnosis and treatment of neurogenic disorders of urination in the group of patients operated for prostatic pathology, the role of the conduction system of the corticospinal tract and conductive potential of perineal muscles in the above patients. Main standard electromyographic parameters were established by the data of examination of 7 controls--latency of the cortical and segmental evoked motor response, time of central motor conduction, amplitude of potentials of motor unities. Fifteen patients with prostatic diseases showed severe disorders of both corticospinal conduction and perineal muscular contraction. Patients with prostatic cancer or adenoma treated with different methods had denervation changes in pelvic inferior muscle in unaffected corticospinal conduction. Denervation changes after surgical treatment for prostatic cancer and delay of reinnervation, recovery of nervous control may be determined not only by the fact of operation but also by the course of the underling disease. Examination of the pelvic inferior muscles demonstrated that HIFU is a traumatic treatment of prostatic cancer because of extention of the physical impact beyond the prostate on muscular-nervous structures. These denervation muscular changes should be taken into consideration in assessment of the patient's condition and choice of treatment policy.
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Desnervación Muscular , Músculo Esquelético/fisiopatología , Hiperplasia Prostática/fisiopatología , Tractos Piramidales/fisiopatología , Trastornos Urinarios/fisiopatología , Anciano , Electromiografía/métodos , Humanos , Masculino , Persona de Mediana Edad , Músculo Esquelético/inervación , Hiperplasia Prostática/cirugíaRESUMEN
The oxygen content and electrical conductivity in Nd0.25Sr0.75FeO3-δ were measured in the range of oxygen partial pressure from 10-19 to 0.5 atm at 750-950 °C by coulometric titration and four-probe dc techniques. The thermodynamic analysis of defect equilibrium in the oxide allowed successful simulation of the oxygen content data and calculation of charge carrier concentrations that were used for the analysis of electrical conductivity. The electrical conductivity data were accurately described in the models, which implied that the hole mobility increased upon an increase in the oxygen content in the oxide. The results suggest that only some of the Fe3+ sites are available for hole transport, and their fraction increases with an increase in the oxygen content. The migration energy for oxygen ions, electrons and holes was found to be 0.89 ± 0.02, 0.62 ± 0.01 and 0.230 ± 0.006 eV, respectively. Nd0.25Sr0.75FeO3-δ was shown to have a considerable oxygen conductivity (0.12 S cm-1 at 950 °C) and fairly good stability under reducing conditions, which is a good recommendation for using this oxide as a functional material in high-temperature electrochemical applications.
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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.
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Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante , Corticoesteroides , Niño , Humanos , Hipertrofia , Inmunoglobulinas Intravenosas , Imagen por Resonancia MagnéticaRESUMEN
We studied the excitability of the visual and motor cortex in 36 patients with frequent migraine without aura (30 women, mean age 38.6 +/- 10.0 years) before and after treatment with topiramate (100 mg/day) using transcranial magnetic stimulation. Treatment with topiramate resulted in reduction of both headache frequency (12.0 +/- 1.3 to 5.8 +/- 3.2 migraine days per month; P = 0.004) and cortical excitability: motor cortex thresholds increased on the right side from 43.8 +/- 7.5% to 47.7 +/- 9.2% (P = 0.049) and on the left side from 43.4 +/- 7.0% to 47.2 +/- 9.6% (P = 0.047), and phosphene thresholds increased from 58.9 +/- 11.1% to 71.2 +/- 11.2% (P = 0.0001). Reduction of headache frequency correlated inversely with an increase of visual thresholds and did not correlate with motor thresholds. The effect of topiramate in migraine prevention is complex and can not be explained simply by inhibition of cortical excitability.
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Corteza Cerebral/efectos de los fármacos , Fructosa/análogos & derivados , Trastornos Migrañosos/tratamiento farmacológico , Trastornos Migrañosos/fisiopatología , Adolescente , Adulto , Anciano , Corteza Cerebral/fisiología , Femenino , Fructosa/farmacología , Fructosa/uso terapéutico , Humanos , Masculino , Persona de Mediana Edad , Trastornos Migrañosos/epidemiología , Corteza Motora/efectos de los fármacos , Corteza Motora/fisiología , Estudios Prospectivos , Factores de Tiempo , Topiramato , Estimulación Magnética Transcraneal/métodos , Corteza Visual/efectos de los fármacos , Corteza Visual/fisiologíaRESUMEN
AIM: To describe the sonographic phenomenon of the focal 'hourglass-like constriction' of the peripheral nerves (FCPN). MATERIAL AND METHODS: The authors described 7 patients meeting the criteria for the diagnosis of neuralgic amyotrophy with unilateral FCPN identified with ultrasound in 4 cases and detected intraoperatively in 3 cases (preliminary ultrasound was not performed). The US scanner Sonoscape Pro mode gray scale in the transverse and longitudinal scanning, linear probe 8-15 MHz and Logiq9 scanner with elastography were used. RESULTS: FCPN was detected in the single nerve in 4 patients, in two nerves in 2 patients and in 3 nerves in one patient. Among all the nerves involved in the pathological process, the radial nerve and its branches were affected in 73% (8 nerves); the ulnar nerve was involved in 18% (2 nerves) and the musculo-cutaneous nerve in 9%. The length of the constriction of the peripheral nerve did not exceed 1.7 mm. The deformation coefficient (DC) of constriction area was 3.8 to .,9; the change in the elasticity in the form of an increase of DC to 5.9 when compared to the intact portion of the nerve and a decrease in echogenicity were observed in one patient. CONCLUSION: High-resolution ultrasound of the nerve can be an informative method for the diagnosis of idiopathic non-traumatic FCPN mononeuropathy.
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Neuritis del Plexo Braquial , Neuritis del Plexo Braquial/diagnóstico por imagen , Constricción Patológica/diagnóstico por imagen , Humanos , Nervios Periféricos , UltrasonografíaRESUMEN
Pompe disease (PD) is a rare autosomal recessive muscle lysosomal glycogenosis caused by a deficiency of acid-α-glucosidase. There are two main forms of the disease: aggressive infantile PD started within the first year of life with a severe enzyme deficiency and multiorgan involvement, and late onset PD (LOPD) with progressive signs and symptoms including predominant proximal, axial muscle weakness and respiratory insufficiency started at any time from 1 till 75 years and older. Usually due to physician's unawareness, most adults with PD are diagnosed with great delay. The typical features and early nonspecific signs in four patients, aged between 35 and 72 years, with confirmed LOPD are delineated and discussed in correspondence with the age of first signs, age development of muscle weakness, distribution and age of final diagnosis. The disorders for differential diagnosis and spectrum of conditions that expanded the possibility of PB are listed. The fluorometrically analyzed level of acid α-glucosidase from dried blood spots is considered to be the first choice diagnostic method for clinically suspected cases of LOPD.
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Enfermedad del Almacenamiento de Glucógeno Tipo II/diagnóstico , Enfermedad del Almacenamiento de Glucógeno Tipo II/enzimología , Enfermedades de Inicio Tardío/diagnóstico , Enfermedades de Inicio Tardío/enzimología , alfa-Glucosidasas/sangre , Adulto , Anciano , Diagnóstico Diferencial , Diagnóstico Precoz , Femenino , Fluorometría , Enfermedad del Almacenamiento de Glucógeno Tipo II/complicaciones , Humanos , Lactante , Enfermedades de Inicio Tardío/complicaciones , Masculino , Persona de Mediana Edad , Debilidad Muscular/diagnóstico , Debilidad Muscular/etiología , Músculo Esquelético/fisiopatología , alfa-Glucosidasas/deficienciaRESUMEN
The clinical data and findings of thorough electrophysiological and morphohistochemical examinations of the muscles of 13 patients with the myasthenic syndrome associated with terminal polyneuropathy are presented. Correlation of the clinical and electrophysiological data to the clinical manifestations of the disease and the magnitude of the block of the neuromuscular transmission disclosed a number of characteristic features of the clinical course of the disease (a predominantly oculodistal type of damage, a diminution or the absence of tendon and periosteal reflexes, the presence of atrophies of distal muscular groups) and of the EMG pattern (the presence of enlarged PDDE, an increase in residual latency, the inverse ratio of the decrement of electric and mechanical responses upon indirect stimulation of the muscle at a frequency of 3/sec) and in the histochemical picture (the presence of groupings of type I and II muscular fibrils, which are not characteristic of myasthenia). On the basis of the evidence obtained, a conclusion is drawn that the described syndrome is an independent clinical form rather than a myasthenia variant.
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Miastenia Gravis/diagnóstico , Enfermedades del Sistema Nervioso Periférico/diagnóstico , Adenosina Trifosfatasas/metabolismo , Adulto , Diagnóstico Diferencial , Electromiografía , Humanos , Persona de Mediana Edad , Músculos/patología , Miofibrillas/enzimología , Conducción Nerviosa , Nervios Periféricos/fisiopatología , SíndromeRESUMEN
Changes of the activity potentials of motor units and muscular fibres registered with the aid of needle electrodes are compared with the results of morphological examinations of biopsy specimens of 1,096 muscles taken from 137 patients with myasthenia, combinations of myasthenia with thymoma and polymyositis, as well as from patients with terminal polyneuropathy with the myasthenic syndrome. It has been shown that characteristic of myasthenia are differentiated changes of the motor unit activity potentials: in half of the muscles examined the parameters of those potentials were found to be normal, no polyphasic or protracted potentials were observed. In the patient with combinations of myasthenia with thymoma, polymyositis, and in terminal polyneuropathy with the myasthenic syndrome pronounced changes of all the activity potentials of the motor units were observed in the form of both a sharp shortening and a prolongation of their duration; a great number of polyphasic potentials was registered and spontaneous activity of various degree was observed. Morphological examinations of 55 biopsy specimens of the muscles of the same patients revealed a correlation between the degree of the dystrophic and atrophic changes of the muscular fibres and depth of the affection as determined on the basis of electromyographic findings.