Biomedical subjects

J Lütschg

Publications and source records attributed to J Lütschg.

At least 37 records · Page 2Linked to original sources

PubMed1984

Pathophysiological aspects of central and peripheral myelin lesions.

The results of nerve conduction velocities, evoked potentials and EEGs in adrenoleukodystrophy, metachromatic leukodystrophy and Pelizaeus Merzbacher's disease are discussed. As shown on the example of four own cases with metachromatic leukodystrophy, the slowing of nerve conduction velocities and the latency prolongation of evoked potentials components or loss of evoked potential components is dependent on the type of MLD (infantile and juvenile) and of the duration of the disease. The pathophysiology of the slowed peripheral and central impulse conduction is discussed.

Adolescent↗

PubMed1984

Early diagnosis and evolution of deafness in childhood bacterial meningitis: a study using brainstem auditory evoked potentials.

Fifty-one children with bacterial meningitis were studied prospectively using serial recordings of brainstem auditory evoked potential (BAEP) from the earliest phase of the disease, according to a standardized protocol. The objectives were to make an early diagnosis and follow the evolution of deafness in the course of meningitis and evaluate the prognostic value of BAEP. Thirty-five children (68.6%) always had normal recordings; 11 children (21.6%) had transient BAEP abnormalities (prolonged wave V latency or elevated threshold for wave I), and five children (9.8%) had persistent pathologic BAEP recordings from the first examination at 48 hours until discharge from the hospital and have a persistent deafness. All recordings that were normal or pathologic at discharge were confirmed by behavioral audiometry 3 months later. These results show the early occurrence of deafness in the course of meningitis with a crucial phase of possible recovery or worsening occurring during the first 2 weeks. There were no cases of "late" deafness or "late" recovery (there was sometimes slight improvement) occurring after discharge; thus BAEPs have a prognostic value. However, observation of a child with clinically proven selective high-frequency postmeningitic deficit but without a hearing handicap, a disorder that was diagnosed early with BAEP (which tests only the high frequencies), is a warning that this method alone is insufficient and that clinical auditory surveillance and conventional audiometry remain necessary.

Adolescent↗

PubMed1983

Brain-stem auditory evoked potentials and early somatosensory evoked potentials in neurointensively treated comatose children.

Forty-three comatose children treated by invasive neurointensive care were examined by brain-stem auditory evoked potentials (BAEPs) and somatosensory evoked potentials (SEPs). The evoked potential (EP) results obtained were reviewed in conjunction with the clinical outcome. As a BAEP criterion, the V-I interpeak latency and, as an SEP criterion, the central conduction time (N20-N14 latency) were measured. A loss of BAEP and SEP components portended a poor prognosis. On the other hand, latency prolongations were seen in a third of patients who made a complete recovery. However, the SEP and BAEP have proved themselves as reliable methods in the judgment of the clinical state and the prognosis of intensively treated children with a hypoxic-ischemic encephalopathy or a head injury. The measurement of both BAEPs and SEPs was a more reliable prognostic aid than either EP measurement alone.

Adolescent↗

PubMed1983

Treatment and outcome of the severely head injured child.

Twenty-four children (aged 3 months to 14 years) with severe head injuries were treated by means of invasive neurointensive care for normalizing intracranial pressure (ICP) involving hyperventilation, control of body temperature, dexamethasone, barbiturates and continuous intracranial and arterial pressure monitoring. The Glasgow Coma Scale before initiation of treatment was 3-4 in 8, 5-6 in 9 and 7 in 7 patients. Moderately to severely elevated ICP was observed in 20 patients. Seven developed acute and subacute space occupying intracranial hematomas. Nineteen children (79%) survived, most often with good recovery and 5 (21%) died. Severely elevated ICP, presence of severe consumption coagulopathy and loss of components in brain auditory evoked potentials were significantly more frequent in the fatal group. We conclude that the prognosis of the severely head injured child can be improved by prompt resuscitation and aggressive neurointensive care but probably not, however, to the extent postulated in recent literature.

Adolescent↗

PubMed1983

Hereditary motor sensory neuropathies in childhood.

Clinical data on 24 patients with hereditary motor sensory neuropathies, with onset in the paediatric period, and of their relatives, is reported. Electrophysiological studies were done in all patients and in 15 relatives. The patients were divided into two groups (Types I and II) and their hereditary trait was determined. In 11 patients a sural nerve biopsy was performed and revealed different patterns of histological alterations. The nerve biopsy always confirmed the value of conduction velocity in distinguishing between Types I and II. A genetic discordance was observed, both in regard to the phenotype and the conduction velocity, and there was increased slowing of the conduction velocity as individuals grew older. Thus the classification of these disorders in childhood can be particularly difficult. The rôle of sural nerve biopsy is discussed.

Adolescent↗

PubMed1983

The evolution of cerebral hemispheric lesions due to pre- or perinatal asphyxia (clinical and neuroradiological correlation).

The clinical, neurophysiological and neuroradiological data of 10 babies who, at the age of three months showed a severe leucomalacia usually combined with cortical atrophy, are presented. All had shown signs of intrauterine asphyxia and were severely hypotonic and suffered from seizures in the neonatal period. At the age of 3 months, all patients were hypo- or hypertonic and had infantile spasms. Computed tomography (CT) in the neonatal period showed unspecific hypodensities and at the age of 3 months the complete CT picture of leucomalacia. Thereafter, CT remained unchanged. An angiographic examination in two cases showed a very marked rarefication of the leptomeningeal vessels and circulatory delay in the medial cerebral artery. The risk factors leading to this clinical and neuroradiological picture are discussed.

Asphyxia Neonatorum↗

PubMed1982

Longitudinal differences of susceptibility to ischaemia in human sensory nerve fibres.

The effect on the median nerve of ischaemia induced by a tourniquet (at a pressure of 250 mm Hg) placed on the upper arm was investigated in 12 young volunteers. In 6 of these 12 persons a second cuff, applying the same pressure as the first, was placed on the mid-forearm. In both groups the decrease in sensory action potentials and in nerve conduction velocity was more rapid in the proximal than in the distal segments. No other difference was detected between the two groups. It is concluded that the pressure induced by the cuff was not responsible for the changes and it is suggested that this phenomenon is due to internodal lengths being greater in the proximal than in the distal segments.

Adult↗

PubMed1982

Cefuroxime in bacterial meningitis.

In order to find an alternative antimicrobial treatment for childhood bacterial meningitis 30 infants and children with meningitis, due to Haemophilus influenzae (n = 13), Neisseria meningitis (n = 9), Streptococcus pneumoniae (n = 5), or meningitis of unknown aetiology (n = 3), were treated with cefuroxime, 200 mg/kg a day, as the only antibiotic. Prompt clinical and bacteriological responses were noted and every patient was cured. Cefuroxime concentrations in cerebrospinal fluid ranged from 1.1 to 18.8 (mean 7.0) mg/l at the beginning and from 0.5 to 4.1 (mean 1.6) mg/l at the end of treatment. Three infants developed symptomatic sterile subdural effusions which were managed by repeated subdural aspirations while still on antibiotics. Cefuroxime concentrations in the subdural fluid ranged from 17.4 to 32.4 mg/l. At follow-up 2 patients had moderate unilateral hearing loss and one had mild ataxia. We conclude that cefuroxime is effective and safe for the treatment of childhood bacterial meningitis caused by any of these common organisms.

Cefuroxime↗

PubMed1982

Retinoblastoma and chromosome 13 deletion.

Two cases of association of unilateral and bilateral retinoblastoma, respectively, with interstitial deletions of long arms of chromosome 13 are presented. The clinical pictures of both children corresponded to the moderate extent of the deletions, with both somatic and mental retardation in both children, and mild phenotypic manifestations (hypertelorism, slight epicanthi, mild facial hirsutism, and partial syndactyly in the child with unilateral retinoblastoma). Opinions concerning the association of chromosome 13 deletion with retinoblastoma are discussed.

Chromosome Deletion↗

PubMed1981

Electromyographic findings in patients after recovery from peripheral nerve lesions and poliomyelitis.

Electromyography was performed in 10 patients 14 to 50 years after acute poliomyelitis and in 12 patients 6 to 16 years after a peripheral nerve lesion. All patients had made an almost complete recovery from initially severe paresis. In the poliomyelitis group the main finding was an increased mean action potential duration in all patients. In the patients with peripheral nerve lesions an increased frequency of spontaneous activity could be seen in 8 out of 10 and an increased mean action potential duration in 7 out of 9 patients during the initial phase. Six to 16 years later the main pathological results were a prolonged mean action potential duration (9 out of 12 patients) whilst increased spontaneous activity was detectable only in 3 out of 12 patients. In two patients all EMG findings were normal.

Adolescent↗

PubMed1981

Neurological complications in hemophilia.

The clinical data of 59 patients with hemophilia A or B are reviewed. Intracranial bleeding was observed in 6 patients and a minor bleeding episode was assumed in a further 8 patients. Neurosurgical evacuation of the hematoma was necessary in 2 cases and the remaining patients were treated solely with factor VIII or IX. In 10 patients a peripheral nerve lesion was observed, paresis of the femoral nerve being the most frequent (5 cases). Two patients showed a lesion of the lumbar and sacral plexus, 2 patients a lesion of the radial nerve and one patient a lesion of the cutaneous femoris lateralis nerve.

Adolescent↗

PubMed1980

Lipoprotein metabolism in nephrotic syndrome in childhood.

In nine patients with proteinuric nephrotic syndrome (NS), significant negative correlations were shown between plasma albumin and cholesterol (r=-0.85, p less than 0.005) and between plasma albumin and low density lipoprotein (LDL)-apoprotein B (ApoB) (r=-0.84, p less than 0.005), whereas there was no such correlation in ten matched ntrol patients. LDL-ApoB (182.8 +/141.2 MG/DL), cholesterol (249.3 +/-190.2 mg/dl) and triglycerides (40.8 +/- 23.5 mg/dl) were all elevated above controls. Control values: LDL-ApoB: 75.4 +/-25.0 mg/dl, -cholesterol: ll9.3 +/-27.6 mg/dl and -triglycerides: 29.2-17.9 mg/dl. The patients with proteinuric NS also exhibited a negative correlation between plasma triglyceride levels and plasma lipoprotein lipaseactiveiy (r=-0.79, p less than 0.02), which was not observed in controls. The hepatic triglycerice lipase (H-TGL) showed a mean activity of 22.2 +/- 10.7 mumol/ml FFA, which was not different form the control value of 21.2 +/-3.9 mumol/ml FFA. Not only LDL-cholesterol values were elevated in the patients with proteinuric NS, but high density lipoprotein (HDL)-cholesterol as well. HDL-cholesterol in proteinuric NS: 68.6 +/- 29.0 mg/dl, controls: 45.7 +/- 8.5 mg/dl. Twelve patients with NS in remission showed no correlation between plasma albumin and cholesterol and plasma albumin and LDL-ApoB respectively. The concentrations of LDL-ApoB (92.2 +/- 24.5 mg/dl),-cholesterol (121.7 +/- 37.1 mg/dl) and -triglycerides (28.9 +/-18.9 mg/dl) were within the normal range. No significan correlation was found between plasma triglyceride levels and plasma lipoprotein lipase activity (r=0.38, p greater than 0.2), and H-TGL activity, at 24.1 +/-9.3 mumol/ml FFA, was not different from control. In contrast to the complete normalization of LDL-cholesterol level, the HDL-cholesterol level remained slightly above control (HDL-cholesterol: 57.0 +/- 17.6 mg/dl). The hyperlipoproteinaemia in NS represents a combination of an increasents a combination of an increased hepatic lipoprotein synthesis and a decreased catabolism of triglyceride rich lipoproteins as indicated by an insufficiency of the lipoprotein lipase. The simultaneous elevation of LDL- and HDL-cholesterol suggests at most a moderate degree of atherogeneity of the hyperlipoproteinemia in NS.

Adolescent↗

PubMed1979

[Clinical results of total endoprosthesis of the knee--follow-up of 51 cases (author's transl)].

After a short historic introduction, the article describes the total endoprotheses of the knee used in our hospital. This is followed by a description of the case material. The follow-ups of the 51 total endoprotheses of the knee applied between April 1971 and October 1975, covered the following criteria: Pain, extent of mobility, extension deficit, stability and walking performance. The article subsequently reviews the complications occurring with the hinged and hingeless total endoprostheses of the knee. Pain, extent of mobility and stability are evaluated via a system of points, thus enabling an evaluation of the functional results of the individual prostheses. During the following discussion, our results are compared with those of three other follow-ups. Comparisons are not always possible, because the evaluation standards may differ. However, we believe that we can say that our results do not differ significantly from those of the studies against which they were compared. In both cases, the complications are mainly the same. With particular reference to a comparision of the results obtained with the Freeman-Swanson prostheses, the results obtained by Freeman during both periods of time were superior to ours. Our experiences with the hingeless Freeman-Swanson prosthesis are rather better than those obtained with the hinged prostheses according to Shiers ans Guépar, especially with regard to the frequency of loosening and infection. However, it must be borne in mind that the follow-up control times are shorter for the Freeman-Swanson prostheses. We believe that implantation of a hinged prosthesis in joints with severe malpositioning of the axis, severe deformities, and instability, continues to be indicated.

Aged↗

PubMed1978

The syndrome of 'continuous muscle fiber activity.'.

A 7-year-old boy who suffered from increasing stiffness and contractures of the extremities had distally pronounced atrophy and absent tendon reflexes. Electromyography showed continuous electrical activity during rest, sleep, after intravenous injection of diazepam, and after peripheral nerve block. The H reflex was elicitable; the silent period after the reflex was absent. Histopathological examination of the peroneus muscle disclosed a marked preponderance of type I fibers and slight atrophy of the type II fibers. Electron microscopic examination of the endplates demonstrated a marked atrophy of the postsynaptic regions and widened synaptic clefts. After one year's treatment with phenytoin, 200 mg daily, the patient showed an almost normal muscle tone. As not all of these electrophysiological phenomena can be fully explained by disturbances of the nerve terminals or the endplates, a further anomaly proximal from the peripheral nerve block seems to have been present.

Child↗

PubMed1977

The Landry-Guillain-barré syndrome. Complications, prognosis and natural history in 123 cases.

One hundred and twenty-three patients, 68 males and 55 females in whom acute polyradiculitis Guillain-Barré appeared at an age of between 1,6 and 76 years were re-examined after 0.9 to 12.3 years. The initial symptoms, the signs during the acute phase, in particular the cranial nerve signs and central nervous signs, as well as findings in the cerebrospinal fluid and complications are described. Three patients died during the acute phase. At follow-up only 57% of the patients were completely cured. In 22% motor signs, mainly distal in the lower extremities were found. Only 6 of these patients however were handicapped. Twenty-two patients had loss of one or more tendon reflexes, in general the ankle jerk. Only 6 showed very slight central nervous system signs. Eleven of 55 follow-up cases had pathological findings in needle electromyography. A disturbance of conduction velocity or distal latency was also found in several adults and children without residual clinical signs. The time span between the maximum of the initial signs and the beginning of recovery seemed to be particularly long in patients who showed residual signs on follow-up. These patients also seemed frequently to have had a severe tetraparesis in the initial phase. We could not confirm the therapeutic effect of cortisone or ACTH in our patients: amongst the 30 adults treated with cortisone 6 (20%) had residual signs at follow-up, whilst 5 (25%) of the 22 untreated ones had similar findings.

Acute Disease↗

PubMed1977

[The Landry-Guillain-Barré-Strohl-syndrome. Prognosis in adults (author's transl)].

The clinical aspect of acute polyradiculitis (Landry-Guillain-Barré-Strohl syndrome) of 85 patients is resumed. 52 of these 85 patients were seen after an average of 5, 1 years, 8 were dead at the time of control and 25 could not be traced. Two patients died during the acute phase of polyradiculitis corresponding to a letality of 2%. 24 of the subjects (46%) had recovered completely, 28 (54%) showed some residual symptoms. Eleven patients (21%) had some residual weakness of hands and/or feet. Out of these eleven six (11% of total) were so much disabled that they received a disablement pension. 17 patients with slight sensory disturbances and/or some loss of reflexes were not handicapped in their every-day life. 20 patients were examined electrophysiologically during the follow-up. All the five patients with a pathological EMG also showed some clinical residual signs. A positive correlation between pathological conduction velocities in the median and/or deep peroneal nerve and clinical residua was only inconstantly found. Patients with severe tetraparesis during the acute phase had more often residual symptoms. The time from the maximum motor deficiency to the beginning of recovery was longer in the latter group than in patients without residual symptoms (23 respectively 8 days) while there was no difference of the time between the beginning of motor weakness and the maximum motor deficiency in the two groups. A treatment with ACTH and/or corticosteroids seemed not to influence the long term prognosis of the illness.

Acute Disease↗

PubMed1977

[Comparison of orthodromic and antidromic sensory nerve conduction. 1. Normals and patients with carpal tunnel syndrome (author's syndrome)].

In 32 healthy volunteers aged between 10 and 72 years the orthodromic and the antidromic sensory nerve conduction in the median nerve have been studied. Normal values for conduction velocities and for the configuration of the orthodromic nerve action potentials are described in detail. 26 patients suffering from a carpal tunnel syndrome have been examined both with the orthodromic and the antidromic method. It could be shown that the diagnostic yield of both methods is about the same for this special problem.

Action Potentials↗

PubMed1977

[Comparison of orthodromic and antidromic sensory nerve conduction. 2. Patients with polyneuropathies and polyradiculitis (author's transl)].

The results of the orthodromically and the antidromically recorded nerve action potentials from a median nerve in 30 patients with clinically slight or beginning polyneuropathies have been compared. Antidromic conduction velocity was slowed in 8 cases, in 8 patients it was normal and in 14 patients no definite antidromic nerve action potential could be recorded. This last finding is not considered as being definitely abnormal. In all but one patient orthodromic nerve action potentials could be elicited. In 28 out of 30 patients the orthodromic method gave pathological findings. Duration of the nerve action potentials and the number of their potential component were the most sensitive single parameters, which were abnormal in more cases than conduction velocity of the fastest fibres. For the detection of slight or beginning polyneuropathies the diagnostic yield of the orthodromic method turned out to be much greater than the antidromic. In contrast, in a group of 19 young patients, which previously had suffered from a polyradiculitis, no significant difference between the two methods was found.

Action Potentials↗