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Biomedical subjects

D Wenzel

Publications and source records attributed to D Wenzel.

At least 19 recordsLinked to original sources

[Current aspects in diagnosis and therapy of pediatric facial paralysis].

Between 1986 and 1992, 60 children with facial nerve palsy (FNP) were examined at the ORL hospital at Erlangen-Nuremberg University. By using blood and cerebrospinal fluid (CSF)-serological tests acute infection with Borrelia burgdorferi (B.b.) could be proved in 16 of 40 children (40%) whose pareses could not be clarified etiologically at first examination. Whereas all idiopathic FNP patients (22) had normal CSF-results, more than half of the B.b.-infected children showed pathological findings, with pleocytosis indicating meningitis. These findings demonstrated that lumbar puncture should be performed in children with FNP of unclear etiology, particularly in cases with positive borreliosis blood serology. The children with borreliosis received penicillin G or ceftriaxone intravenously for at least 14 days and patients with idiopathic FNP were treated with cortisone either orally alone or intravenously in combination with pentoxifylline and hydroxyethylstarch. The outcome in 95% of the patients showed that complete recovery was similar in borreliosis and idiopathic FNP irrespective of treatment with cortisone. Overall, 88% of the FNP recovered completely, indicating a favorable prognosis of FNP in children.

Adolescent

Simultaneous recording of brainstem and cortical acoustic evoked potentials in children: methodical aspects and normative data.

Early and late acoustic evoked responses were simultaneously recorded from 42 children aged 3-17 years by means of a computer-based device using self-developed software. Filling the interstimulus intervals of late acoustic evoked potentials with clicks leads to an increase of latencies and a decrease of amplitudes of N1 and P2 components. Age-dependent normal values for various recording conditions are given.

Adolescent

Stimulus specificity of P300.

P300 recordings are valuable and noninvasive neurophysiological research tool to gain further insight into higher cerebral function. Due to methodological and technological restrictions the clinical applicability of these investigations for neuropediatric purposes was limited. To enhance the usefulness of this phenomenon for clinical practice in neuropediatrics a convenient, painless and short-lasting procedure was imperative. For this purpose we developed a visual priming technique to average P300 components in children. To estimate the sensitivity of this procedure two classes of modality-different stimuli were used to prime visual P300 recordings. Forty children were examined, while P300 averagings for target and related nontarget events were recorded during spatial and verbal stimulation. Topographical brain electrical activities of match/mismatch events were compared by complex statistical MANOVA methods for each period to compute modality-specific components within the surface recorded P300 waveforms. Latency statistics were correlated. Our data indicate significant differences between various topographical P300 distributions, showing a close relationship between the experimental priming period and the activity of distinct cortical regions involved in spatial-imagination and language-processing.

Adolescent

Diagnosis of moyamoya disease with additional renal artery stenosis by colour coded Doppler sonography.

Moyamoya disease is a rare vascular anomaly of the cerebral arteries. The etiology of the disease has not yet been clearly identified. We report the noninvasive diagnosis of Moyamoya disease in a patients with a very early onset of symptoms in infancy. The diagnosis was made by colour coded Doppler sonography and confirmed by angiography at the age of 6 months, following two episodes of cerebral infarction. A bilateral encephalodurosynangiosis was performed at the age of 7 months with subsequent slight improvement of the neurological deficits. Colour Doppler sonography revealed early vascularisation from the fascia temporalis graft into the arachnoid space. At the age of 10 months the patient developed arterial hypertension caused by left renal artery stenosis. Our case suggests, that in infancy Moyamoya disease can be suspected noninvasively by colour Doppler sonography of the cerebral arteries. Patients should be carefully screened for possible extracranial arterial stenoses which may develop in the course of time. Encephalodurosynangiosis seems to be a good therapeutic option for patients with severe neurological symptoms.

Cerebral Arteries

Screening of pulmonary hypertension in chronic obstructive pulmonary disease and silicosis by discriminant functions.

The aim of our prospective multicentric study was to develop a screening method for pulmonary hypertension in patients with chronic lung diseases. We investigated 710 patients in 10 hospitals: 315 males and 109 females with chronic obstructive pulmonary disease, and 286 males with silicosis. Manifest pulmonary hypertension was defined as pulmonary artery pressure greater than 20 mmHg (2.7 kPa) at rest. The multivariate statistical method used was a stepwise discriminant analysis. In males with chronic obstructive pulmonary disease, the diameter of the right descending pulmonary artery, forced expiratory volume in one second (FEV1) arterial oxygen tension (PaO2) at rest, and age turned out to be relevant for discrimination of groups with and without manifest pulmonary hypertension. For females the FEV1/FVC (forced vital capacity) ratio replaced the absolute value of FEV1 in the calculated discriminant function. In females, sensitivity and specificity were below 80%. In males, both were distinctly above 80%. In silicosis, the diameter of the right descending pulmonary artery was much less important, since it could frequently not be measured precisely. In these cases, precision of the prediction of about 80% could only be obtained by combined evaluation of spirometry, PaO2 during exercise, and body plethysmography. The calculated discriminant functions are appropriate for screening patients with risk of pulmonary hypertension. For different chronic lung diseases, and for both sexes, different combinations of parameters are relevant. The method is recommended to select patients who should undergo an invasive examination of pulmonary haemodynamics.

Discriminant Analysis

[Life threatening menorrhagia in thrombasthenia. (Glanzmann-Naegeli) thrombasthenia].

Glanzmann's Thrombasthenia is a rare inherited disorder of platelet aggregation with normal platelet count and humoral coagulation. It is caused by the deficiency or functional disorder of platelet membrane glycoproteins IIb und IIIa. This complex is considered to be a receptor for fibrinogen. Menorrhagia often occurs as a clinical manifestation of affected females. We report a case of severe menorrhagia in a 13-year-old girl during her third menstrual cycle. She needed several red blood cell transfusions. The bleeding could only be stopped by administration of Lynestrenol.

Adolescent

[Effects of laparotomy on respiratory function in patients without and with pre-existing respiratory compromise].

The direct effect of the upper laparotomy on the ventilation were examined in 23 patients without and in 22 patients with preexisting disorders of breathing. During the first 3 postoperative days the parameters FVC and FEV1 were decreased for 50-20% in an inverse relation to the preexisting degree of respiratory dysfunction and the arterial PO2 decreased about 2 kPa (15 mm Hg). Clear hypoxaemia (without hypercapnia) were only found in the group with a high degree of preexisting respiratory dysfunction (FEV1 less than 1.4 l). Especially pretreatment and aftercare are recommended for this patients in the case of laparotomy.

Adult

Reversible visual loss after shunt malfunction.

Report on three hydrocephalic, shunt dependent children, who became blind due to shunt malfunction and rapidly decreased intracranial pressure. They regained vision several days (one case) or several months (2 cases) after shunt revision. The related literature is reviewed and possible pathophysiological mechanisms are discussed.

Adolescent

Brainstem acoustic evoked responses: maturational aspects from cochlea to midbrain.

BAEPs were recorded from 92 healthy children with a gestational age of 35 weeks up to 16 years. The maturation kinetic of I-III and I-V interpeak latency can well be approximated by the exponential regressions I-V : y = 0.9588 x e -0.9215 x x + 3.9728; r = 0.87; I-III : y = 0.6182 x e -1.1737 x x + 2.1759; r = 0.81; Adult values are reached by about two years of life. In contrast to this immaturity of the central auditory pathways the slopes of latency--intensity functions of waves I and V show no significant differences between premature infants and neonates versus older children over three years. Caused by the prolonged I-V IPL wave V latency is about 1 ms longer in the first group, whereas wave I is only slightly prolonged (0.2 ms) within the first three months of life.

Acoustic Stimulation

[Bronchospasmolytic test].

Bronchodilation test using metered aerosols is a simple but extraordinary reliable method of respiratory functional diagnostics. It is highly sensitive (92%) and absolutely specific (100%) for demonstration of a pathologic elevated tonus of the bronchial musculature. The importance for the choice of therapy is limited. The main advantage concerns the diagnostic classification of the obstructive bronchopulmonary diseases.

Aerosols

Congenital brain tumours: diagnostic and therapeutic approach. With a report of 3 cases.

Three cases of congenital brain tumours are reported, which presented with signs of increased intracranial pressure soon after birth, giving rise to the suspicion of a cerebral haemorrhage or of hydrocephalus. Correct diagnosis of tumour was established by computerised tomography, which additionally demonstrated a concomitant haematoma in each case. Two of the newborns had a primarily fatal course, with no specific treatment of the tumours being feasible. The remaining child underwent two operations, experiencing an unhindered neurologic and mental development thereafter. The histologic diagnoses were spongioblastoma and medulloblastoma in the first two cases, and ganglioneuroblastoma in the last. A conspicuous clinicopathologic feature of this neuroblastoma was the marked change in its growth pattern, revealing a higher degree of histologic differentiation and less malignant biological behaviour when tumour regrowth occurred. The diagnostic, therapeutic, and prognostic implications for this special clinicopathologic condition are discussed, with a review of the relevant literature.

Astrocytoma

Visual evoked potentials in tumors from orbita to occipital lobe in childhood.

68 infants and children with proven tumours along the visual pathway visually evoked potentials with pattern reversal and flash were investigated. Subdivided in 5 locational groups (orbital, optic nerve, chiasmatic, retrochiasmatic and occipital lobe) best results were obtained by stimulation with pattern reversal using variable checksizes (91.2%) versus 61.8% by using flash evoked responses. Furthermore, using patterned stimuli not only latency shifts are best detected but also hints for visual function correlating to visual acuity could be given also in nonverbal or disabled children. For long term follow-up studies in known space-occupying lesions along the visual pathway, the use of pattern VEP is extraordinary because of its high sensitivity and complete harmlessness. Pattern VEP are even more sensitive than visual acuity testing in cooperative patients. The close interdisciplinary follow-up controls in these children between pediatric neurophysiologists and neurosurgeons are, therefore, strongly recommended.

Adolescent

[Acute recurrent pancreatitis as the main symptom of lupus erythematosus disseminatus in childhood].

A 17 year old girl with chronic relapsing pancreatitis since the age of 11 developed focal seizures, leukopenia, anemia and thrombozytopenia, signs suggesting lupus erythematodes. All immunologic investigations, including tests for Anti-DNS-Antibodies were negative, however. Therapy with prednisone and azathioprine resulted in clinical remission. Reduction of the immunosuppression lead to a severe crisis, which was controlled by plasmaseparation and cyclophosphamide. Subsequent analysis of circulating immune complexes revealed bound anti-DNS-antibodies.

Acute Disease

Evoked potentials in infantile spasms.

In infants and toddlers (N = 8) with infantile spasms, visual and somatosensory evoked potentials (VEP, SEP) were recorded during the course of the illness. During hypsarrhythmia the pattern evoked potential was missing (7/8) or grossly abnormal (1/8), indicating functional amaurosis. Flash evoked responses were normal in 50% even during the EEG-status, showing the superiority of pattern versus flash evoked potentials for visual functions in infancy. The alterations were reversible after successful treatment. Early SEP-components remained normal even during the bioelectrical status but later cortical components were altered in a reversible manner. The results show the objective state of functional sensory block during the EEG-status and underline the necessity of quick and effective treatment to avoid delayed visual maturation.

Child, Preschool

[Legionellosis in a sample of pneumonia patients at a lung clinic].

The Legionellaceae as agents causing pneumonias are widespread distributed and lead to partially very serious diseases, particularly in predisposed persons with impaired resistance. Two of 12 cases observed are reported casuistically. Erythromycin as the drug of choice should be given already at suspicion. Since Legionellosis cannot be differentiated from pneumonias of other etiology on the basis of clinical findings alone, the authors refer to the importance of the relevant microbiologic methods.

Adult

Progressive cerebellar and extrapyramidal motor disturbances in galactosaemic twins.

Progressive cerebellar and extrapyramidal motor disturbances are described in two 16-year-old female twins with classical galctosaemia. The neurological disturbances, characterized by hyper- and dysmetric movements and bilateral intention tremor with choreatic, atactic and even ballistic motor storms, appeared at 12 years of age. Computerized tomography demonstrates cerebral atrophy in cerebellar, brain stem and basal ganglia structures. The central conduction times, determined by somatosensible evoked potentials, are grossly prolonged; the peripheral nerve conduction velocities are normal. The neurological sequelae described are considered a distinct entity in the course of galactosaemia.

Adolescent