[Case of late infantile form of gangliosidosis G-M1].
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Age Factors↗
Biomedical subjects
B Ryniewicz
Publications and source records attributed to B Ryniewicz.
[Epileptic seizures in children with myasthenia gravis].
In the studies material of 119 children with myasthenia epileptic seizures occurred in 8 cases (7%). They always preceded the appearance of myasthenic symptoms. The seizures were primarily generalized. The EEG tracings varied greatly in morphology and intensity. The authors discuss the relationship between myasthenia and epilepsy and the effects of the used drugs on both these diseases.
Adolescent↗
[Idiopathic juvenile osteoporosis with the symptoms suggesting nervous system damage].
10 children with final diagnosis of idiopathic juvenile osteoporosis were admitted to the Department of Neurology because of suspected lesion of nervous system. The main clinical features were gait disturbances and pain. Radiological examination was decisive for diagnosis. The authors discuss the course of disease and effect of the treatment.
Adolescent↗
[Changes in the neural conduction in spinal muscular atrophy of the Kennedy type].
Sensory conduction velocity of median and sural nerves was examined in 6 patients with spinal muscular atrophy (SMA) of Kennedy type. In all patients, except the youngest one, slight slowing of sensory conduction velocity and marked decrease of potential amplitude was observed. These changes might suggest that the Kennedy type of SMA differs from another types of SMA and, may be, does not fit into "pure" motoneuron disease.
Adult↗
[Hereditary motor and sensory neuropathy. I. Principles of classification and clinical picture].
The clinical picture was analysed in two types of hereditary motor-sensory neuropathy isolated on the ground of electrophysiological criteria. Type I comprised 34 patients with the conduction velocity in median nerve below 38 m/sec. Type II 19 patients with the conduction velocity above 38 m/sec. The age of onset was similar in both types and cases with onset below the age of 5 years prevailed. The assessment of the clinical picture using a acoring system failed to show any significant differences between type I and type II. Cases of type I shows, however, a considerable variability of the clinical picture and the course of disease process. Cases of type II were more homogeneous.
Adolescent↗
The diagnostic yield of automatic EMG analysis in neuromuscular diseases.
The aim of the study was to evaluate the diagnostic yield of automatic EMG analysis employed in differentiating normal from diseased muscle and myogenic, neural and spinal lesions. The material comprised 520 patients with neuromuscular diseases. Only diagnostically confirmed cases were included into the study. The control group comprised 51 healthy subjects. In all patients and healthy subjects routine EMG examination was performed by means of both the conventional technique and automatic method. On the basis of the statistical analysis of the material the authors concluded that the method of automated EMG using the Polish minicomputer Anops makes possible distinction of the main types of pathological processes affecting the muscles with higher than previously objectivity and reliability. They stress, however, the important role of the examiner and his experience.
Diagnosis, Computer-Assisted↗
[Evaluation of remission in childhood myasthenia gravis].
By the electrophysiological methods such as supramaximal stimulation and single-fibre EMG (SF EMG) 13 children with myasthenia were studied. In only one case the results of both tests were normal. These findings confirm the presence of subclinical disturbances of neuromuscular transmission in most cases of myasthenia during remission. This may be of practical importance in the treatment of myasthenic patients.
Action Potentials↗
[Hereditary motor-sensory neuropathy. II. Electrophysiological studies].
Electrophysiological parameters (conduction velocity, distal latency, amplitude of evoked response) were analysed in two types of sensorimotor hereditary neuropathy isolated on the ground of the values of motor conduction velocity in the median nerve which was 38 m/sec. Using this criterion the studied material of 53 cases could be divided into two groups. Group I of 34 cases in which the mean conduction velocity in the median nerve was 16.2 m/sec, and group II of 19 cases had a mean conduction velocity in the median nerve of 50.7 m/sec. The evaluation of the degree of slowing down of conduction in both types showed similar values in individual cases and in families.
Evoked Potentials↗
[Motor-sensory hereditary neuropathy. III. Histological changes].
The authors describe the results of histological examinations of the sural nerve in 40 cases of sensorimotor hereditary neuropathy. A comparison of the morphological findings with the values of conduction velocity showed that all cases with "primary demyelination" belonged to the I type of this neuropathy (with conduction velocity under 38 m/sec) while those with axonal changes (and conduction velocity over 38 m/sec) belonged to type II. In 2 cases the degree of demyelination and axonal changes was similar, but the electrophysiological criteria failed to correspond to those of the "intermediate" type. These observations confirmed the validity of the classification of Harding and Thomas, but give no basis for isolation of an "intermediate" group as suggested in the classification of Bradley et al. A progression of demyelination changes was observed with increasing intensity of the pathological process, and frequent coexistence of axonal changes in type I, and possibility of greater damage to the thin myelinated fibres in relation to thick fibres in type II.
Axons↗
[Sensorimotor hereditary neuropathy. IV. Clinical, electrophysiologic and histologic correlations. Summary of studies].
On the basis of a material comprising 53 cases of sensorimotor hereditary neuropathy from 40 families the authors discuss the results of studies on the clinico-electrophysiological-histological correlations. The electrophysiological and histological studies demonstrated the validity of separation of this disease into two types according to the criteria given by Harding and Thomas. No significant differences were found in the clinical manifestations between type I and type II of the disease. In type I the clinical and histological findings were more varied than in type II. No basis was found for isolation of an intermediate type of peroneal muscular atrophy.
Arm↗
[Isaacs-Mertens syndrome: continuous activity of motor units].
The syndrome of Isaacs-Mertens is a rare neuromuscular disease in which the motor unit is continuously active. The diagnosis is based on electrophysiological methods and pharmacological tests. The involvement of laryngeal muscles observed in the reported female patient has been as yet rarely described in this syndrome. In the treatment long-term administration of carbamazepine 600 mg daily gave a considerable clinical improvement. The pathological mechanism of the syndrome is discussed in the light of the performed examinations and a review of the pertinent literature.
Adult↗
The subclinical facial nerve involvement in generalized neuropathies.
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Facial Nerve↗
Motor and sensory conduction velocity in spinal muscular atrophy. Follow-up study.
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Adolescent↗
[Electromyographic findings in children with dermatomyositis].
The author analysed electromyographic records in 14 children wit dermatomyositis. The findings included primary muscular changes of great intensity and presence of activity at rest in 50% of cases. The intensity of primary muscular changes resembled that observed in Duchenne's dystrophy and the distribution of changes was similar to that in non-progressive myopathies. Electromyographic changes occur in all cases of dermatomyositis and confirm the clinical diagnosis.
Action Potentials↗
Correlative morphological and biochemical studies in a case of subacute progressive panencephalomyelitis caused by paramyxovirus.
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Brain↗
Diagnostic value of satellite potentials in clinical EMG.
Complex motor unit potentials (CMUPs) with satellites were recorded and analysed in 231 electromyograms of patients with spinal muscular atrophy (SMA), amyotrophic lateral sclerosis (ALS), Duchenne muscular dystrophy (DMD) and a healthy control group. In the control adult group only few CMUPs with satellites were found; no CMUP were found in children. In SMA and DMD patients the CMUPs were present with similar frequency but their morphology, i.e. shape, duration, amplitude of the main and satellite spike components and number of satellites was different. In ALS patients a significant difference between severely involved interosseous muscle and proximal muscles was found. The pathomechanism of CMUPs with satellites is different in myopathic and neurogenic processes, in both however they reflect remodelling of the motor unit. In myopathy they reflect muscle fiber diameter variability and distribution of preserved muscle fibers within the motor unit under study. In neurogenic lesion the CMUPs with satellites are the result of increasing desynchronisation during progressive de- and reinnervation.
Adolescent↗