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Biomedical subjects

A Jaspert

Publications and source records attributed to A Jaspert.

11 recordsLinked to original sources

Multifocal motor neuropathy: clinical and electrophysiological findings.

Multifocal motor neuropathy (MMN) can be differentiated from motor neuron disease by electrophysiological evidence of conduction block. To increase the probability of recording conduction block, we studied the whole nerve length including proximal segments in 84 patients with pure motor syndromes, using a special stimulation technique. In 8 patients, the diagnosis of MMN was confirmed by electrophysiological evidence of conduction block or temporal dispersion. The typical clinical picture of MMN with chronic progressive, asymmetrical, marked distal weakness was observed in our patients. Electrophysiological routine tests of distal nerves were usually normal except in nerve segments with conduction block. In 4 patients, conduction block could be recorded only in proximal nerve segments or spinal roots. All patients showed rapid improvement of clinical features and parallel reduction of conduction block during or after high-dose intravenous immunoglobulin (ivIG) therapy, supporting the diagnosis of an immune-mediated neuropathy. Three of them are now in remission without any therapy, whereas 5 still receive a regular ivIG course every 2-12 weeks as long-term treatment. In all patients with pure or predominantly motor syndromes and normal findings in electrophysiological routine tests of distal nerve segments, there should be proximal conduction block studies to avoid overlooking a treatable disorder such as MMN.

Adolescent↗

[Immunoglobulin therapy of chronic inflammatory neuropathies].

High-dose intravenous immunoglobulins (ivIg) are an effective therapy in chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN). In both diseases, data regarding ivIg long-term treatment are sparse. Therapy with ivIg was performed in 18 patients with CIDP or MMN. Sixteen patients responded to ivIg therapy; they were treated for more than 6 months. Two of them had not previously shown any positive response to other immunosuppressive treatments. Response to ivIg therapy could be observed even after a long disease duration (maximum of 19.5 years). All 16 therapy responders now have no or only mild clinical symptoms. Treatment could eventually be completely stopped in 6 patients; they have now been in complete remission without therapy for a maximum of 63 months. Ten patients still receive regular ivIg infusions in different dosages. No severe side effects were observed in the whole group. IvIg therapy is an effective, safe and easily applicable treatment regimen even in the long-term course of CIDP and MMN.

Adolescent↗

Focal upper limb demyelinating neuropathy.

Observations are presented on nine selected patients with chronic upper limb demyelinating neuropathy to illustrate the range of manifestations that may be observed. In three, the involvement was purely motor, in five, mixed motor and sensory and, in one, virtually purely sensory; in seven the symptoms were unilateral and in two bilateral. The presence of reduced nerve conduction velocity and conduction block and the response to treatment in seven of the cases indicate that they represented examples of chronic inflammatory demyelinating polyneuropathy (CIDP) with focal involvement. This was confirmed by nerve biopsy in two cases. The presentation in one patient was accompanied by forearm swelling initially suspected of being a tumour but shown to be due to muscle hypertrophy. This was probably the consequence of recurrent muscle cramps and fasciculation and possibly neuromyotonia. The patient with predominant sensory involvement restricted to the upper limbs demonstrates that sensory CIDP can present focally. In one patient with monomelic motor and sensory involvement, nerve biopsy showed multifocal areas of hypertrophic demyelinating neuropathy distally in the ulnar nerve without inflammatory infiltration. This patient failed to respond to therapy. Response in the others was satisfactory, although one patient with a monomelic motor neuropathy showed a severe deterioration after being given corticosteroids; he subsequently improved with intravenous human immunoglobulin therapy.

Adolescent↗

Myotonic dystrophy: correlation of clinical symptoms with the size of the CTG trinucleotide repeat.

An unstable DNA sequence of a gene encoding a protein kinase has been identified as the molecular basis of myotonic dystrophy. The correlation between different symptoms of myotonic dystrophy and the size of this unstable base triplet (CTG)n repeat was investigated in 14 patients. DNA was prepared from whole blood by standard procedures. Detailed clinical, psychological, electrophysiological (quantified measurement of myotonia, electrocardiography) and other laboratory examinations (muscle biopsy in 4 patients, slit lamp examination) were performed. Triplet size correlated significantly with muscular disability and inversely with age at onset of the disease. A greater frequency of mental and gonadal dysfunction could be observed in patients with a larger repeat size. Other symptoms, however, such as cataract, myotonia, gastrointestinal dysfunction and cardiac abnormalities were not correlated with repeat size. Somatic mosaicism with different amplification rates in various tissues might be one possible explanation for the variable phenotypes. Furthermore, other factors such as different expression of the myotonic dystrophy gene might contribute to the clinical variability of the disease at a given triplet size.

Adult↗

Initial lithium augmentation improves the antidepressant effects of standard TCA treatment in non-resistant depressed patients.

The hypothesis was tested that an initial lithium-tricyclic antidepressant (TCA) combination has a better antidepressant effect than standard TCA treatment in non-refractory depression at the beginning of an episode. Twenty bipolar melancholic type depressed inpatients under lithium-TCA treatment were compared with 20 patients with the same diagnosis and TCA-placebo treatment for 5 weeks under double-blind conditions. All patients were male. Initial lithium-TCA treatment reduced depressive symptoms significantly more than antidepressant treatment with TCA and placebo after 5 weeks, but not in weeks 1 or 2. It can be concluded that lithium augmentation of TCA treatment should be started even at the beginning of antidepressant TCA treatment to provide a better treatment response in those patients who will profit from long-term lithium prophylaxis, e.g. bipolar patients with melancholic type depression.

Adult↗

[Value of proximal conduction block study in diagnosis of inflammatory neuropathies].

Conduction block is a common finding in inflammatory neuropathies, indicating circumscribed demyelination. Since demyelination and conduction block are often localized proximally, the whole ulnar nerve including its proximal segments was studied fractionally in 31 patients with inflammatory neuropathies. In 5 of 15 patients with Guillain-Barré syndrome, conduction block at the spinal roots was the first electrophysiological finding indicating demyelinating neuropathy. Segmental conduction blocks were also found in 8 of 9 patients with chronic inflammatory demyelinating polyneuropathies (CIDP) and 4 of 4 patients with atypical neuropathies. In 3 patients, multifocal motor neuropathy (MMN) could only be differentiated from motor neuron disease by recording proximal conduction block. Corresponding to clinical recovery, conduction block improved with immunoglobulin therapy in CIDP and MMN patients. The technique of proximal conduction block studies clearly improves the diagnosis of focal demyelination in inflammatory, treatable neuropathies. They should be performed particularly in patients with atypical neuropathies in whom electrophysiological tests of distal nerve segments show normal results.

Adolescent↗

[Benzodiazepine monotherapy in acute schizophrenia].

Two patients with acute schizophrenic or schizo-affective psychosis were treated with benzodiazepine-monotherapy. In the first patient with paranoid-hallucinatory psychosis, catatonic symptoms disappeared completely after application of Lorazepam. Side effects of neuroleptic medication (neuroleptic turbulences) were the reason for benzodiazepine treatment in the second patient. In neither patients were psychotic symptoms observed during several weeks on benzodiazepine medication. Subsequently, no further neuroleptic treatment was necessary in one patient. Benzodiazepine effects on schizophrenia are probably caused by an activation of inhibitory GABA-ergic neurons. Besides stupor and catatonia, severe side effects of neuroleptic treatment or even contra-indications of neuroleptic medication may be an indication for benzodiazepine treatment in acute schizophrenia.

Acute Disease↗

Follow-up investigations of peripheral facial palsy by transcranial magnetic stimulation.

Twenty-three patients (10 men, 13 women; age: from 23 to 81 years, mean 52 years) with Bell's palsy were investigated by magneto-electrical stimulation in order to evaluate the usefulness of this method for prognostication. At each examination the facial nerve was electrically stimulated, and an orbicularis-oculi-reflex was elicited. Follow-up investigations were possible in 13 patients. All patients showed pathological long- and short-latency responses at the first examination. The blink reflex examination revealed a pathological result in all cases. The responses to electrical stimulation were pathological only in 35%. The follow-up examinations revealed that the analysis of the short-latency response did not give a prognostic information. In contrast, maintained elicitation of the long-latency response pointed to a favourable prognosis. In follow-up investigations, the increase of amplitude and the decrease of latency of the long-latency response correlated with a complete recovery, while the decrease of amplitude and the increase of latency correlated with a partial recovery. The improvement of the responses in magnetic stimulation preceded the clinical improvement. By using magnetic stimulation, an early registration and evaluation of the course of Bell's palsy are possible.

Action Potentials↗

[Transcranial magnetic stimulation in the cranial nerve region in cranio-cerebral injuries].

By means of transcranial magnetic stimulation bilateral compound muscle action potentials of long latency and ipsilateral potentials of short latency can be obtained in muscles supplied by cranial nerves. In this study motor evoked potentials were recorded from the mentalis muscle of 14 patients with traumatic brain lesions. These results were compared with clinical and radiological as well as other neurophysiological examinations. In spite of the absence of paralysis in muscles supplied by the facial nerve, 9 patients showed pathological long-latency responses, which correlated with the localisation of the paralysis of the limbs. In contrast to this, the degree of pathological latencies of motor evoked potentials did not correspond with the degree of paresis in different patients. A prediction concerning the clinical outcome could not be made by the results of motor evoked potentials. The clinical data showed a poor correlation with the structural brain lesions seen in the CT-scan and the results of EEG, BAEP and orbicularis oculi reflex examination. In conclusion, transcranial magnetic stimulation of cranial nerves proved to be a sensitive method for the evaluation of degree and localization of motor deficits following traumatic brain lesion and seems to be superior to the other examinations compared.

Adult↗

[Acute spinal epidural abscess].

An acute spinal epidural abscess is a rare cause of paraplegia, seen in seven patients over a period of ten years. All patients had fever and severe localized back-pain. Unless treated, within hours or a few days, there will be root defects and rapidly progressive paraplegia. Staphylococcus is the most frequent causative organism and clinically manifest septicaemia is common. Rapid diagnosis and treatment are essential in deciding the patient's fate. Myelography is an important additional examination as it demonstrates the abscess in 96% of cases. Non-contrast radiology is of little value. High-dosage antibiotics and surgical spinal decompression are the cardinal treatment procedures. Antibiotics alone are justified only so long as there are no neurological deficits and neurosurgical intervention, if needed, is immediately available.

Abscess↗