ABSTRACT
Between 1978 and 1980, 10 patients with myasthenia gravis underwent treatment by plasmapheresis. Of these, 7 responded to plasma exchange. Pre- and post-exchange anti-acetylcholine receptor antibody concentrations were found to be a useful parameter for intraindividual comparisons, but failed to correlate with the stage of the disease. Anti-acetylcholine receptor antibody assays cannot be replaced by determinations of IgG and globulin concentrations. In view of the potential risks and of the high cost factor, plasmapheresis should be reserved for particularly severe cases for obtaining transient clinical improvement in life threatening situations.
Subject(s)
Myasthenia Gravis/therapy , Plasmapheresis , Adult , Aged , Antibodies, Antinuclear/analysis , Autoantibodies/analysis , Female , Humans , Immunoglobulin G/analysis , Male , Middle Aged , Muscles/immunology , Plasma Exchange , Receptors, Cholinergic/immunologyABSTRACT
The fibre density of the extensor digitorum communis muscle was examined with a monopolar single fiber electrode in 2 definite, one probable and 15 possible Duchenne-carriers. In addition serum-CK was evaluated three times in intervals of a least one week. The fibre density was increased in one definite, one probable and 4 possible carriers (greater than 1.6). The highest value was 2.0. Excepting one case the increase of fibre density was accompanied by an increase of serum-CK (greater than 60 mg%). The highest value of CK was 1626 mg%. The conventional EMG was pathological in 2 cases. In no case with an increase of CK or with pathological EMG the fibre density was normal. The fibre density is an unspecific but rather sensible indicator of Duchenne-carriers but as we could observe in a definite carrier, negative results do not exclude carriers.
Subject(s)
Creatine Kinase/blood , Electromyography/methods , Genetic Carrier Screening , Muscular Dystrophies/genetics , Adolescent , Adult , Child , Female , Genetic Counseling , Humans , Middle Aged , Muscular Dystrophies/diagnosisABSTRACT
In a placebo-controlled double blind study at random with 72 healthy persons the effect of an active TBE-vaccination (purified TBE vaccine) on EEG was investigated. 49 out of 72 examined persons could be examined twice, before and 3 to 5 days after vaccination respectively. The EEG was registered according to the international 10-20 electrode system in bipolar, unipolar and source derivations, using a 16 channel Elema-Schoenander electroencephalograph. The registration was made at rest, with hyperventilation and photo-stimulation. The EEG was registered on a tape-recorder and, using a computer, an automatic analysis (Fourier-Analysis) of the CZ-O2 derivations was performed, in order to get more exact results and a quantification of the basic-rhythmus. The visual evaluation and automatic analysis showed no significant changes of the EEG after TBE vaccination. There results were statistically determined by T-Tests and by discriminance analysis.
Subject(s)
Electroencephalography , Encephalomyelitis, Acute Disseminated/etiology , Meningoencephalitis/prevention & control , Adolescent , Adult , Double-Blind Method , Female , Humans , Male , Middle AgedABSTRACT
The etiology, pathogenesis, diagnosis and treatment of myasthenia gravis are discussed. We treated 16 patients, 13 of them with conventional X-rays and three by megavoltage irradiation. Our results show that radiotherapy has a definite role in the management of patients with myasthenia gravis and should be considered more frequently in future.
Subject(s)
Myasthenia Gravis/radiotherapy , Aged , Female , Humans , Male , Middle Aged , Radiotherapy/methods , Radiotherapy Dosage , Radiotherapy, High-EnergyABSTRACT
Comparative bioptic and electrophysiological investigations were performed in 30 cases of polyneuropathy of varying aetiology. Axonal and demyelinating neuropathies were distinguishable concordantly on the basis of both investigations. Maximum motor nerve conduction velocity (NCV) and compound potential (CP) were regularly examined in several nerves of each patient. Low (NCV) values (critical limit 25 m/sec) and long-standing high CP values were characteristic of demyelinating processes, whereas axonal neuropathies showed a slight to moderate decrease in NCV with early decrease in CP, often rapidly followed by loss of nerve excitability. Nonclassifiable stages and diseases are discussed. In biopsies, ultrastructural examination in particular, not only allowed the differentiation of primary axonal and primary demyelinating lesions and the respective neuropathic syndromes, but permitted moreover, further classification of these heterogenous groups of neuropathies. According to the sequence of lesions of the nerve fibre classes on the one hand, and to the nature of the primary lesion of the nerve fibres on the other hand, the following types of neuropathies were distinguished: in axonal neuropathies of the myelinated fibres, dystrophic and disintegrative forms were differentiated; axonal neuropathies of unmyelinated fibres correlated with sensory neuropathies. Demyelinating neuropathies were represented by hypertrophic, leucodystrophic and paraproteinaemic forms. The correlation of these types of neuropathies to the different clinical and aetiological forms of polyneuropathies is diagrammatically shown. The connection between the electrophysiological data and the basic nerve fibre alterations of the different neuropathies is discussed.
Subject(s)
Nervous System Diseases/pathology , Demyelinating Diseases/pathology , Electrophysiology , Neural Conduction , Sural Nerve/pathology , Sural Nerve/ultrastructureSubject(s)
Akinetic Mutism/physiopathology , Brain Injuries/physiopathology , Cerebral Cortex/physiopathology , Electroencephalography , Adult , Consciousness/physiology , Female , Humans , Longitudinal Studies , Male , Mesencephalon/physiopathology , Middle Aged , Prognosis , Remission, Spontaneous , Time Factors , Wakefulness/physiologyABSTRACT
The following parameters have been examined in twenty-one patients suffering from chronic renal failure (creatinine level between 4.5 and 18.8 mg/100 ml serum): maximum motor nerve conduction of the peroneal nerve, amplitude of the compound muscle action potential of the extensor digitorum brevis muscle, serum creatiine, total protein, serum globulins, serum albumins, alkali reserve, time of increase of serum creatinine above 4 mg/100 ml up to time of determination of the maximum motor nerve conduction, daily urinary excretion, mean blood pressure, (p less than 0.01) was found between maximum motor nerve conduction, as well as amplitude of the compound muscle action potential, and the serum albumin level only. Decreased levels of serum albumin, is correlated with diminished nerve conduction and a lower amplitude. The relationship between the electrophysiological data and serum albumin levels maybe explained on the basis of progression of a pre-existing polyneuropathy due to additional dietary malnutrition. A different interpretation is the assumption of an inactivation of neurotoxin on binding by albumins. A decrease in the albumin level would, therefore, result in an increased amount of unbound toxic agent. The values of the maximum motor nerve conduction were between 16 m/sec and 51 m/sec (mean value 42.2 m/sec), pointing to a polyneuropathy of primary axonal type rather that to primary demyelinization. The amplitudes of the compound muscle action potentials were not greatly reduced and thus the uraemic polyneuropathy seems to be of mixed type. In uraemic polyneuropathy different aetiological factors have to assumed. According to the prevalent factor a polyneuropathy of predominantly axonal or predominantly demyelinizing type may result.
Subject(s)
Neural Conduction , Polyneuropathies/etiology , Uremia/complications , Action Potentials , Adolescent , Adult , Aged , Creatinine/blood , Female , Humans , Male , Middle Aged , Motor Neurons/metabolism , Polyneuropathies/blood , Polyneuropathies/metabolism , Serum Albumin/analysis , Uremia/bloodABSTRACT
The anti-convulsive action of dipropyl acetic acid (Convulex) was tested in 21 patients with grandmal seizures (GM) and/or temporal lobe attacks (TL), or with Jacksonian epilepsy. Patients were chosen according to a negative selection system. Results showed that in GM and TL seizures, Convulex constitutes an important addition to the battery of anticonvulsives available today. Action was more potent in GM than in TL attacks. Toxic effects are slight. Treatment had to be discontinued in two patients -- once because of cerebellar symptoms and once because of crowded incidence of seizures. In two cases the patients gained weight. Occasional gastrointestinal complaints disappeared when antacids were administered. No changes were observed with reference to blood count, or to hepatic or renal function. Fatigue set in only when treatment was combined with barbiturates and disappeared again when pre-medication was reduced. Five patients showed an improved state of mind (activation and better communication with others); this psychological action may be considered an additional advantage. Since the action of barbiturates and hydantoins is potentiated by Convulex, pre-medication may be reduced --provided Convulex therapy alone does not seem adequate. Hence, trial treatment with Convulex may also be recommended in those patients who are successfully controlled with barbiturates and hydantoins,but whose daily work schedule is impaired due to fatigue.
Subject(s)
Valerates/therapeutic use , Valproic Acid/therapeutic use , Adult , Aged , Barbiturates/therapeutic use , Epilepsies, Partial/drug therapy , Epilepsy/drug therapy , Epilepsy, Temporal Lobe/drug therapy , Epilepsy, Tonic-Clonic/drug therapy , Female , Humans , Hydantoins/therapeutic use , Male , Middle Aged , Premedication , Valproic Acid/adverse effectsABSTRACT
Two cases are presented of the paraneoplastic syndrome in patients with a malignant tumour of the thyroid gland. The first case presented with a pseudomyasthenic syndrome (in conjunction with a haemangio endothelioma) and the second case with a form of neuromyopathy (in conjunction with a partly solid and partly hormone-producing thyroid carcinoma). The pathogenesis and classification of the so-called carcinomatous neuropathies is briefly outlined and the initially atypical course taken by the first case is discussed.
Subject(s)
Muscular Diseases/diagnosis , Nervous System Diseases/diagnosis , Paraneoplastic Endocrine Syndromes/diagnosis , Thyroid Neoplasms/complications , Aged , Deglutition Disorders/diagnosis , Diagnosis, Differential , Eye Manifestations , Female , Humans , Male , Middle Aged , Myasthenia Gravis/diagnosis , Neurologic Examination , Neurologic ManifestationsABSTRACT
A review is given of the present state of knowledge and current concepts of the causative factors involved in, and the pathological mechanisms underlying status epilepticus. A synopsis of the commonly-applied therapeutic methods is presented. A step-wise therapeutic procedure is advised for the different stages of status epilepticus. Treatment is directed towards a lowering of cerebral excitability (hydantoin, diazepam), the prevention of brain oedema (osmotherapy, positive pressure respiration) and the avoidance of the development of secondary complications (antibiotics, intensive care). In every case the requisite investigations should be undertaken in order to clarify the aetiology.
Subject(s)
Epilepsy/therapy , Anticonvulsants/therapeutic use , Brain Edema/etiology , Brain Edema/prevention & control , Epilepsy/complications , Epilepsy/drug therapy , Humans , Intensive Care Units , Positive-Pressure Respiration , Serum Albumin/therapeutic useABSTRACT
A short historical outline of electric treatment of spasticity is given. A specially developed management with a "gait-stimulator" is described. Four muscles of each lower extremity being mainly engaged in walk were electrically stimulated in the physiological sequences according to the normal gait. The used electric impulses were of a duration of 0,25 msec and an intensity up to 700 V. Using such a "gain-stimulator" in spastic-paraparetic patients a reduced spasticity has been achieved. Positive effect of this treatment has been mostly pronounced, when the programming of impulses was adjusted to the end of the expected physiological contraction of the corresponding muscles. Physiological and pathological data of the "Silent period" is proposed to be mostly involved. The application of impulses in physiological sequences seems to reactivate normal reflex - mechanisms which are disturbed by supraspinal laesion. The results indicate that the electric impulses activates muscle-sensory - organs and that impulses on these organs produce a pace-making function on the spinal cord, which lessens spasticity.
