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Biomedical subjects

H G Lenard

Publications and source records attributed to H G Lenard.

At least 55 records · Page 3Linked to original sources
PubMed1992

Immunotherapy with stimulated autologous lymphocytes in a case of a juvenile anaplastic glioma.

The effect of immunotherapy with stimulated autologous lymphocytes (SAL) in malignant gliomas is documented and discussed in a bioptical and autoptical case study. A five-year-old child with a recurrently operated and radiated right hemispheric anaplastic astrocytoma died six weeks after immunotherapy with mitogen-activated killer cells and recombinant Interleukin-2. The autopsy revealed a large butterfly glioma with partially necrotic gelatinous tissue at the site of the SAL reservoir. The tumor cell density on the right was less than on the left hemisphere, and T-lymphocyte content was higher on the right hemisphere. These results demonstrate a local effect of SAL therapy in vivo, although the tumor progression as a whole could not be stopped. They also demonstrate the need of a detailed neuropathological examination in all cases of immunotherapy of malignant gliomas.

Brain Neoplasms↗
PubMed1992

Sudden loss of hearing and vestibular function, muscular weakness, and multiple white matter lesions in preschool children.

Sudden cochlear hearing loss, occurring successively or simultaneously in both ears, was observed in four unrelated preschool children. Vestibular testing could be carried out in three patients and showed complete bilateral loss of function. All patients had a mildly retarded motor development due to nonprogressive muscular weakness. On MR imaging all patients showed multiple periventricular and subcortical white matter lesions. These lesions were not clearly progressive in one patient examined repeatedly over 6 years. Virological, bacteriological, immunological, and metabolic examinations were normal in all patients. Muscle biopsy showed morphologically abnormal mitochondria in two and lipid storage in one patient. No indications have been found for a disturbed functioning of the muscle mitochondria. The identical pattern and course of the disease in these patients suggests a new nosological entity, the aetiology of which can only speculatively be attributed to a vascular process associated with a mitochondriopathy.

Biopsy↗
PubMed1992

Severe adverse reaction to carbamazepine: significance of humoral and cellular reactions to the drug.

Hypersensitivity to carbamazepine is a well-known phenomenon. The involvement of several organ systems including liver, kidney, bone marrow and other organs have been described. We have observed a 7-year-old boy who had been treated with carbamazepine for seizures. After 10 days of treatment he developed a severe illness with skin rash, high fever, lymphadenopathy, hepatosplenomegaly and lymphopenia. Only slightly decreased complement components and increased complement split products but no circulating immune complexes were demonstrable on admission. Anti-carbamazepine antibodies, T-cell-activation and a significant T-cell reactivity against carbamazepine were found, indicating specific hypersensitivity. Complete recovery was observed after discontinuation of the drug and steroid treatment.

Carbamazepine↗
PubMed1991

Maturation of lower extremity EMG responses to postural perturbations: relationship of response-latencies to development of fastest central and peripheral efferents.

EMG responses to toe-up tilt perturbations on a movable platform system were analysed in 86 children between the age of 12 months and 13 years. To assess the relative contribution of peripheral and central nerve conduction properties, a concomitant recording of the fastest efferent pathways in the central and peripheral motor system was made using non-invasive transcranial magnetic stimulation of motor cortex and peripheral nerve roots. This allowed the determination of the fastest downstream efferent connection times from motor cortex to lumbar motor neuron pools and to measure the fastest efferent conduction from these motor neuron pools to effector muscles in the lower leg. The sequence observed for stance stabilizing EMG responses was similar to that obtained in earlier studies with short latency (SL) and middle latency (ML) companents occurring in the stretched triceps surae muscle and long latency (LL) responses occurring in the non-stretched tibialis anterior muscle. Homologous responses were also obtained in upper leg muscles, being recruited consistently later than those in lower leg muscles across all age groups. In the short latency range two different SL1- and SL2-responses were obtained in children of all age groups as well as in adult controls. Both the SL1- and the SL2-responses showed a flat developmental profile, reaching adult values between 20 and 30 months of age which correlated with that of the fastest efferents from lumbar motor neuron pools to leg muscles, i.e. the final motor path. ML-responses showed a steeper developmental profile.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1991

Spontaneous regression of a temporal arachnoid cyst.

Surgery is considered to be the standard therapy for arachnoid cysts (ACs). We report the case of a 13-year-old boy in whom a right temporal AC disappeared spontaneously over a period of 10 years. Bulging of the right temporal skull led to the detection of the cyst by computed tomography (CT) scan at the age of 3 years. There were no other clinical symptoms. Subsequent CT scans showed spontaneous regression of the cyst without surgical intervention. The question as to how ACs should be treated is discussed.

Adolescent↗
PubMed1991

The Floating-Harbor syndrome.

We describe the seventh patient with the Floating-Harbor syndrome. Similar to previous cases in the literature this girl presented with proportionate intrauterine and postnatal growth retardation, normocephaly, triangular face with bulbous nose, long eyelashes, short upper lip, small vermilion border of upper lip, dorsally rotated ears, deep nuchal hair line, hirsutism, and clinodactyly of little fingers. She exhibited mental retardation and retarded speech development. Clinical symptoms and differential diagnosis of this rare syndrome are briefly discussed.

Abnormalities, Multiple↗
PubMed1991

Myopathy in Williams-Beuren syndrome.

Williams-Beuren syndrome (WBS) is a disorder of unknown aetiology. The classical features of the syndrome include a typical ('elfin') facies, mental retardation and heart defects. Myopathy has not so far been part of the spectrum of WBS. We studied six patients with WBS aged 3-25 years, five of whom showed clinical and morphological evidence of myopathy. The clinical manifestations of myopathy included hypotonia in infancy, walking delay, joint contractures, scoliosis, and increased exhaustion on exertion. These symptoms were present in variable expression but part of a typical postural pattern. Examination of muscle biopsies showed lipid storage in four patients and increased variability of fibre size in three. In one patient a muscle biopsy gave normal results. Biochemical investigation in four patients with morphological evidence of lipid storage in muscle revealed muscle carnitine deficiency in three. In addition, enzyme activities of fatty acid beta-oxidation were low in one of two specimens tested. It is concluded that a clinically relevant myopathy is part of the multi-system manifestation of WBS and a clinical trial of carnitine supplementation is justified.

Adult↗
PubMed1991

Magnetic stimulation of motor cortex and nerve roots in children. Maturation of cortico-motoneuronal projections.

Magnetoelectrical stimulation of motor cortex and peripheral nerve roots was used to establish the maturational profiles of central and peripheral nervous system conduction times in normal children in upper and lower extremity muscles. To obtain true central conduction times, not contaminated by variation due to different preinnervational levels, a protocol using stimulation under full muscular relaxation was used. With this protocol clear responses of the upper extremity could be obtained after the first year of life, whereas in the lower extremity they could not be obtained before the fourth year of life. The developmental profile of central conduction times to upper and lower extremity muscles showed an age-dependent acceleration with adult values not being reached before the age of about 10 years. In contrast, peripheral conduction times obtained after cervical or lumbar root stimulation to upper and lower extremities showed a much faster maturational profile, with adult values being reached at about the age of 3 years. The data are discussed in relation to the available literature on neuromorphological development and provide normative values for the understanding of normal development of motor control in children as well as for application of this technique in children with motor disturbances.

Adolescent↗
PubMed1991

Congenital muscular dystrophies--problems of classification.

The classification of congenital muscular dystrophies (CMD), based on perceived clinical and morphological similarities or differences, is controversial. CMD without cerebral involvement has sometimes been divided into a mild and a severe form. This distinction is, however, arbitrary and not uncontested. Whether Ullrich's disease, formerly called atonic-sclerotic dystrophy, is a disease entity and if so, whether it is a primary muscle disorder, is uncertain. CMD without cerebral involvement is inherited in an autosomal recessive fashion in the great majority of cases. CMDs with cerebral involvement are usually classified into at least three forms: the Fukuyama type of CMD, occurring almost exclusively in Japanese patients; CMD with hypomyelination, sometimes also called the occidental type of cerebromuscular dystrophy; and Walker-Warburg syndrome. Muscle-eye-brain disease, described in a number of Finnish patients, may or may not belong in this last category. In CMD with cerebral involvement inheritance is also autosomal recessive. It is possible that single sporadic cases are phenocopies due to infectious or other exogenous causes. Reports of clinical and morphological findings from an increasing number of patients show a high degree of variability within and, on the other hand, certain similarities between the forms of CMD with cerebral involvement. In addition, neuroradiological changes are also found with increasing frequency in CMD patients without clinical neuropsychological abnormalities. It is not unreasonable to speculate that molecular genetic techniques will reveal in the near future a variable defect in one gene locus or defects in a few gene loci as the cause of the various clinical forms of CMDs.

Humans↗
PubMed1991

[Linear IgA dermatosis in childhood].

In a six-year-old girl with linear IgA dermatosis of childhood there were lesions with typical rosette like configuration, crusted erosions and isolated bullae on apparently normal skin. The histopathology revealed subepidermal blister and superficial perivascular lymphohistiocytic infiltrate with many neurophils and some eosinophils. Direct immunofluorescence examination showed linear deposits of IgA along the basal membrane zone. Circulating antibodies were absent. Therapy with dapsone in a dose of 25 mg/day was effective.

Autoimmune Diseases↗
PubMed1990

Pain in newborns and prematures: current practice and knowledge.

No unambiguous answer can be given as to whether newborns are able to feel pain similar to that experienced by older children and adults. However, there are several lines of evidence--anatomical, physiological and behavioral--which substantiate the possible presence of distressing nociceptive activity in the full-term and preterm neonate. Although the efficacy and safety of anesthesia in newborns and prematures has repeatedly been demonstrated, there are still numerous recommendations and current practices, based on antiquated theories, that withhold adequate medications from neonates during surgery. Even if the emotional and cognitive aspects of nociception in the newborn remain a subject of speculation giving rise to philosophical discussions as to the correct terminology, it is the mandate of newborns' physicians to provide the best possible therapy to their patients and to protect them from distress, unease and presumptive pain.

Humans↗
PubMed1989

[Space-occupying lesions in the pineal region--MRT using Gd-DTPA].

The results obtained from contrast-enhanced MRI in 21 patients with masses in the pineal region are reported. The use of gadolinium-DTPA results in intensive contrast uptake by the tumour; this produces better definition of the tumour and more exact delineation from neighbouring structures. This is of importance in judging resectability and in choosing the operative approach. During chemotherapy or radiotherapy, it provides reliable demonstration of the success of treatment and accurately demonstrates the size of the tumour and the presence of necrosis. On the other hand, histologic classification of pineal tumours is possible only occasionally.

Adolescent↗
PubMed1989

[Contrast medium enhanced MRT of tumors of the posterior fossa in children and adolescents].

The results of contrast-enhanced MR in 24 children and adolescents with tumours of the posterior cranial fossa are presented. During initial diagnosis, the majority of tumours showed intensive enhancement with improved demarcation from neighbouring structures. The solid component of a cystic tumour, even when small and not visible on the original scan, could be demonstrated in all cases by means of gadolinium-DTPA. Post-operatively, T2-weighted scans regularly showed areas of high signal strength at the margin of the resection; in the absence of a mass or demonstrable progression, the significance of this is uncertain. In these cases gadolinium-DTPA, because of the function of the blood-brain barrier, greatly increases accuracy in demonstrating residual or recurrent tumours.

Adolescent↗
PubMed1989

[MRT using gadolinium-DTPA in spinal intradural space-occupying lesions].

The results of contrast enhanced MRI in 36 patients with suspected spinal intradural tumours are described. All intramedullary tumors showed distinctive enhancement and solid tumors could be delineated clearly, even if they were not clearly visible on unenhanced scans. The differentiation between neoplasm and non-neoplastic syrinx was markedly improved. The sensitivity of MRI for demonstrating intradural extramedullary tumours was greatly improved by gadolinium DTPA and even small lesions or flat meningeal infiltrates could be visualised. In addition, gadolinium DTPA improved the delineation and localisation of larger lesions, even if they had already been seen on unenhanced images.

Adolescent↗
PubMed1989

Motor control in childhood onset dopa-responsive dystonia (Segawa syndrome).

To elucidate the pathophysiological features of childhood onset dopa-responsive dystonia (DRD) we used a variety of quantitative analysis techniques to evaluate aspects of reflex- and voluntary motor control in two brothers with this disorder. The observed patterns were compared with those obtained in patients with adult onset Parkinson's disease (PD) and Huntington's disease (HD). In both brothers onset of the disease was in the first decade. Both responded either to treatment with L-Dopa or a combination of L-Dopa with trihexiphenidyl. Neurophysiological studies revealed slowing of different upper extremity voluntary motor activities and a low frequency postural tremor similar to results in other basal ganglia disorders including PD. In contrast to adult onset PD, fastest isometric voluntary index finger contractions did not show the typical kinetic tremor oscillations superimposed on the force trajectories. Also, different to adult PD, no impairment of stance regulating reflexes or saccadic and smooth pursuit eye movements was found in DRD. Magnetoelectrical stimulation of motor cortex showed normal efferent cortical spinal activity. Data indicate that like in other basal ganglia diseases slowing of voluntary motor activity is also a constant feature in DRD. DRD patients show, however, a clear difference to the pattern of motor abnormalities obtainable in PD. Both the pattern of motor control abnormalities is different, and the longlasting effect. In contrast to PD the pathophysiological mechanism in DRD possibly involves a lack of maturation of dopaminergic substantia nigra neurons rather than a degenerative process.

Adolescent↗