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Biomedical subjects

R Korinthenberg

Publications and source records attributed to R Korinthenberg.

At least 91 records · Page 5Linked to original sources
PubMed1988

Evoked potentials and electroencephalography in adolescents with phenylketonuria.

We studied the pattern-reversal VEP, the BAEP and the EEG with conventional and computerized analysis in 41 adolescents with hyperphenylalaninemia (24 HPA type 1, 12 HPA type 2, 4 HPA type 3, 1 DHPR deficiency) and 35 control persons. A prolongation of the P100 latency in the VEP, a greater interear difference of the I-V interwave latency in the BAEP and slowing of EEG background activity were found. Five per cent of the patients demonstrated spikes and 12.5% abnormal sharp transients in the EEG. The latency increase in the VEP corresponded to the compliance with the diet during the first decade of life. No influence of the actual Phe concentration at the time of the investigation was demonstrated. The BAEP- and EEG-findings were not related to the course of treatment. Thus the VEP changes in this cross sectional study refer to alterations of brain function that occurred during the early years of life. To investigate the value of evoked potentials and EEG in monitoring brain function after discontinuing the diet longitudinal data are needed.

Adolescent↗
PubMed1987

[Mycoplasma pneumoniae encephalitis].

Clinical, CT and, in one case, autopsy findings indicated a diagnosis of a severe necrotising encephalitis in two patients. Although usually herpes simplex virus is blamed for this form of encephalitis, it was possible to prove in these two patients that Mycoplasma was the causative agent of the disease. It is concluded that this organism can produce a serious disease in the central nervous system similar to that caused by herpes simplex.

Brain↗
PubMed1987

[Computed tomography changes in children with shunted hydrocephalus and intermittent cranial pressure crises].

Seven children with drained hydrocephalus are described who showed increasingly severe and frequent episodes of intermittently raised intracranial pressure. CT examinations during symptom-free intervals, or after conservative treatment, showed collapsed slit-like ventricles. Examination during attacks of raised pressure showed relative dilatation of the ventricles as compared with earlier examinations. Children with ventricular shunts of long duration may develop a shunt-dependent syndrome; CT may show normal or narrow ventricles and this does not exclude the possibility of a rise of intracranial pressure. These findings may help in making the diagnosis by means of CT without any further invasive procedures.

Adolescent↗
PubMed1986

Severe course of glycogen storage disease type II (Pompe's disease) without development of cardiomegalia.

Glycogen storage disease type II Pompe (GSD II) is a lysosomal storage disease caused by an inherited deficiency of acid alpha-glucosidase. In addition to the classical infantile form of GSD II, several clinical variants are known. We describe an infant with the classical course of the disease. Our patient differs from the classical variant by the lack of cardiomegalia and the high residual activity of acid alpha-glucosidase in cultivated skin fibroblasts and muscle tissue. In the present case, however, glycogen storing lysosomes were found in peripheral lymphocytes and skeletal muscle cells. This finding underlines the particular value of ultrastructural investigation in the diagnosis of GSD II.

Biopsy↗
PubMed1986

[Diagnosis of hamartoma of the tuber cinereum].

Hamartomas of the tuber cinereum are tumour-like collections of normal tissue in abnormal location. They are benign lesions with slow or absent growth and without any tendency to neoplastic evolution. Due to their neurosecreting properties they usually cause precocious puberty. Further neuroendocrine disturbances, seizures, or psychoneurological symptoms may be associated in some cases. Cisternography and CT are the most conclusive radiologic procedures in all cases. The typical feature is a well circumscribed round-shaped isodense soft tissue mass without contrast enhancement. Usually the tumour is small, rarely exceeding 2 cm. in diameter. If CT diagnosis is not conclusive, examination in the coronal plane or CT cisternography are recommended. Although CT does not permit a histological diagnosis the clinical and radiological features together are sufficient to make a highly suggestive diagnosis. The treatment of choice is medical therapy. Surgery should be restricted to those tumours which damage surrounding structures by their size and cause other symptoms than precocious puberty.

Adolescent↗
PubMed1984

Congenital muscular dystrophy, brain malformation and ocular problems (muscle, eye and brain disease) in two German families.

We report on two brothers and an unrelated girl with congenital muscular dystrophy (CMD), brain malformation and ocular changes (strabismus, myopia, glaucoma, cataracts, retinal dystrophy). Correlations with the inherited autosomal recessive syndromes of CMD, including the Fukuyama-type CMD with CNS malformation, and the Muscle, Eye and Brain Disease published by Santavuori are discussed.

Brain Diseases↗
PubMed1984

Primary rhabdomyosarcoma of the leptomeninx. Clinical, neuroradiological and pathological aspects.

Report of an eight-year-old boy presenting with untreatable focal epilepsy and slowly progressing hemiparesis combined with personality changes. Continuous diagnostic efforts eventually resulted in the diagnosis of a primary intracranial rhabdomyosarcoma several months later. The delay in making the correct diagnosis was due to the fact that the malignant process originated as a diffuse involvement of the leptomeninx, and only in later stages a tumor mass developed. Nosology and histogenesis of primary intracranial rhabdomyosarcomas are discussed.

Arachnoid↗
PubMed1983

[Clinical course and prognosis in medulloblastoma in infants and young children].

From 1962 until 1981 six children with histologically assured medulloblastoma younger than three years of age have been treated at Münster University Clinics. The interval from the onset of symptoms until the clinical admission lasted six weeks, a very short time compared with the admission interval of adults. The survival time after admission was only sixteen days in average. All children died because of central disregulation due to their disease.

Age Factors↗
PubMed1982

[EEG-slowing after high-dose Methotrexate with citrovorumfactor-rescue. A spectralanalytic study (author's transl)].

In 23 treatment courses of high-dose Methotrexate (MTX) with Leukovorin-rescue for osteogenic sarcoma serial EEG-examinations before and after MTX-infusion were done. In 11 course Vincristine had been administered additionally. Frequency-analysis of EEG-background activity was performed by computer-based FFT. At the end of the MTX-infusion EEGs were unchanged, compared with the findings before start of treatment. At 24 and 48 hours after start of infusion, there was a slight but statistically significant slowing, recognizable from an increase of the theta/alpha-ratio (median + 48%, range -7% to + 373%) and a drop of dominant frequency (median-8%, range +12% to -53%). There was a stronger trend towards normalisation in patients, not having received Vincristine. The greatest changes were found in a patient, whose serum-MTX-concentration at 24 hours exceeded the upper therapeutic limit. The EEG-findings are discussed as an equivalent of a subclinical MTX-encephalopathy. Acute encephalopathies with severe EEG-changes and morphological changes on CT have been observed in MTX-intoxications.

Adolescent↗
PubMed1982

[Therapy and prognosis of medulloblastoma].

Between 1962 and 1981, 37 patients with histologically proved medulloblastomas were treated at the Radiologic Hospital and the Children's Hospital of the University of Münster. Because of the inadequate treatment over this long period, the collective was divided into three groups. The first group comprised 18 patients, 15 of whom died after having been operated, so that they could not be submitted to a sufficient radiotherapy. Two patients were unconscious when admitted; they died without therapy. One patient was submitted to a primary palliative irradiation with 10 Gy. The second group comprised seven patients who received only a postoperative irradiation of the skull. One of these patients is alive since 228 months without any recurrence. The other six patients had an average survival time of 9,2 months. The third group comprises twelve patients who were submitted to an irradiation of the skull and an additional irradiation of the whole cerebrospinal axis. Nine of these twelve patients were also treated by chemotherapy according to the SIOP scheme. Their average survival time is 28,4 months. Five patients are still alive and have no recurrences or metastases. The best survival chances actually seem to be provided by a radiotherapy of the whole cerebrospinal axis combined with chemotherapy.

Adult↗
PubMed1980

[Serial EEG-examinations in children undergoing chemotherapy of ALL according to the Berlin-West-protocol (author's transl)].

55 children suffering from acute lymphoblastic leukemia were treated wih prednisone, vincristin-sulfate, daunorubicine and L-asparaginase (1st month), 6-mercapto-purine, cytosine-arabinoside. cyclophospamide, methotrexate-i.th. and cranial irradiation (2nd month). Maintenance-therapy comprised 6-mercapto-purine, cyclophosphamide and methotrexate-i.v. Serial EEG-examination were done in most of them during the course of treatment. 4 children presented with meningeal leukemia at the time of diagnosis. Their EEGs showed moderate slowing before treatment and at the end of the first month. One of them suffered a focal convulsion two days after the last VCR/daunorubine-injection. All EEG-changes were completely reversible later on. 3 patients showed severe focal EEG-disturbances and convulsions during serious neurological complications (intracranial bleeding, rubella-encephalitis). The remaining patients were free of complications at the time of the EEG-examination. Before therapy 24% of them presented normal findings, 57% slightly and 19% moderately abnormal EEGs. At the end of the first month there was an increase of moderate and severe EEG-slowing to 37% and 23%, only 11% of findings were normal. Improvement during the second month of therapy led to 37% normal and 57% slightly abnormal EEG-findings at its end. After maintenance-therapy of 1/2--1 year duration there were 65% normal EEGs and no moderate and severe disturbances any longer. Quantitative spectral analysis of the EEG in 6 additional children revealed similar changes.

Adolescent↗
PubMed1979

EEG-changes during intensified induction-therapy of acute lymphoblastic leukemia.

In 49 children with acute lymphoblastic leukemia serial EEGs were performed during the course of treatment. Therapy in the first four weeks consisted of: Prednisone, vincristine, daunorubicine and L-asparaginase. In the second month 6-mercaptopurine, cytosin-arabinoside, cyclophosphamide, methotrexate-i. th. and cranial irradiation were administered. Maintenance-therapy consisted of 6-mercaptopurine, cyclophosphamide and methotrexate i.v. Before treatment only 24% of patients showed normal EEG-findings, whereas 57% presented sly induced by leukemic infiltrations and partly due to the impaired clinical state. At the end of the first phase of therapy, the combined toxicity of vincristine and L-asparaginase led to the finding of 23% severely and 37% moderately slowed EEGs. Slightly disturbed EEGs were found in 29% and normal ones in 11% of children. Regression occurred duirng the phase of CNS-prophylaxis. At its end 37% of recordings were normal and 57% slightly abnormal. After maintenance-therapy of 1/2 to 1 year duration, there were 65% normal findings. Moderate and severe disorders were no longer demonstrated. Paroxysmal activity developed twice, each during the first phase of therapy and accompnaying convulsions. In both cases we saw slowing of background-activity and signs of increased excitability still months after. In one of these patients, the probable cause was a vincristin-encephalopathy, the cause of the second case remained unknown. EEGs of two furtehr patients with rubella-encephalitis and subarachnoideal hemorrhage exhibited severe unspecific changes.

Adolescent↗
PubMed1977

[Acute hemorrhagic meningoencephalitis due to rubella-infection in acute lymphatic leukemia (author's transl)].

We observed a fatal meningoencephalitis in a patient who suffered from acute lymphatic leukemia. The infection occurred after complete bone marrow remission. As the cause of this complication we discovered a rubella-infection, whose beginning had been several weeks ago. Relations to several forms of rubella-encephalitis in otherwise healthy patients are discussed and pathogenetical conclusions are made.

Acute Disease↗
PubMedDate not supplied

Echocardiographic and electrocardiographic findings of cardiomyopathy in Duchenne and Becker-Kiener muscular dystrophies.

We assessed the cardiac findings in Duchenne muscular dystrophy (DMD) and Becker-Kiener muscular dystrophy (BMD) patients in the large outpatient group of our single-center institution. The analysis included the revision of patient records (retrospectively) and current echocardiography, electrocardiogram (ECG), and Holter ECG data (prospectively). Reduced left ventricular fraction shortening (FS) < 25% was found in 24% of all patients (50 DMD, 12.1 +/- 4.7 years: 20 BMD, 17.1 +/- 8.5 years). Median age of onset of FS < 25% was 16.8 +/- 1.0 in DMD and 30.4 +/- 3.4 in BMD; (p < 0.05). Holter ECG in DMD demonstrated sinus tachycardia in 26% deprivation of circadian rhythm in 31%, and reduced heart rate variability in 51%. For these reasons, we recommend carrying out echocardiography annually in DMD and BMD > 10 years. Because the rate of disturbances in the cardiac autonomic nervous system is high in DMD, we also recommend Holter ECG annually. Further investigation should be conducted to determine if treatment with ss-blockers and ACE inhibitors in combination reduces cardiac autonomic nervous imbalance, thus improving patient outcome in DMD.

Adolescent↗
PubMedDate not supplied

Obesity after childhood craniopharyngioma--German multicenter study on pre-operative risk factors and quality of life.

BACKGROUND: Craniopharyngiomas are tumorous embryogenic malformations. As the survival rate after craniopharyngioma is high (92 %), prognosis and quality of life (QoL) in survivors mainly depend on adverse late effects such as obesity. PATIENTS AND METHODS: We analyzed 214 children and adolescents with craniopharyngioma. The records of 185 patients (86 %) were available for retrospective analysis of weight profiles and risk factors for obesity. Quality of life (QoL) was measured in 145 patients by the Fertigkeitenskala Münster/Heidelberg score (FMH) and in 77 patients by PEDQOL questionnaire. RESULTS: Eighty-two of 185 patients (44 %) developed severe obesity (body mass index [BMI] > 3 SD). Obese patients were compared with 79 patients (43 %) who kept normal weight (BMI < 2 SD). No differences between obese and normal weight patients were found in terms of gender distribution, age at diagnosis and follow-up period. However, the BMI SDS at the time of diagnosis was higher (p < 0.0001) in patients who developed obesity than in those who did not. Furthermore, obese patients presented with bigger tumors (p < 0.05) and a higher rate of a hydrocephalus requiring a shunt (p < 0.05) and hypothalamic involvement (p < 0.05). The mothers of patients who developed severe obesity had a higher BMI (p < 0.001) at the time of diagnosis. Obese patients had a higher height-SDS at diagnosis (p < 0.05) and at the time of last follow-up (p < 0.05) when compared with normal weight patients. A prediction model for severe obesity after craniopharyngioma was calculated by logistic regression based on the risk factors: patient's BMI > 2 SD at diagnosis (p < 0.05; odds ratio: 16.4), hypothalamic involvement (p < 0.05; odds ratio: 3.4) and maternal BMI > 25 kg/m(2) (p < 0.05; odds ratio: 4.6). Significant increases in BMI (p < 0.001) occurred during the early post-operative period especially during the first three years after diagnosis. FMH percentiles correlated negatively with BMI SDS (Spearman r: - 0.37; p < 0.001). Children with craniopharyngioma rated their QoL more negative (p < 0.05) in regard to physical abilities, cognitive functioning and social functioning when compared with healthy children of the same age group. Severely obese patients with craniopharyngioma estimated their QoL lower (p < 0.05) for all domains except for autonomy, cognition and familial integration in comparison with non-obese patients. CONCLUSION: Hypothalamic tumor involvement and familial disposition for obesity are risk factors for the development of severe obesity in patients with craniopharyngioma. As weight gain starts early after diagnosis and severe obesity causes a reduction in QoL, early therapeutic efforts should be considered in patients at risk. To confirm our results the prospective multicenter study Kraniopharyngeom 2000 on children and adolescents with craniopharyngioma was initiated (www.kraniopharyngeom.com).

Adolescent↗