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Biomedical subjects

H Wörle

Publications and source records attributed to H Wörle.

8 recordsLinked to original sources
PubMed1993

[Aspects of long-term management of children with myelomeningocele. Analysis of the last 10 years from the neuropediatric-neurosurgical viewpoint].

BACKGROUND: Both the use of a selective policy of treatment of newborns with myelomeningocele and the right time of back closure are still controversially discussed. We report our experiences in the long-term care of children with myelomeningocele. METHODS: 103 newborns with myelomeningocele (n = 94) and meningocele (n = 9) born 1980 to 1990 and cared for regularly at the Olgahospital's pediatric clinic were followed up from the neuropediatric-neurosurgical point of view. Postpartum we predominantly practised a selective policy of treatment. RESULTS: 15 of 21 children (71.4%) not operated on died during the first days or months of life, 6 (28.6%) survived. 70 were operated selectively, of which 9 (12.9%) died, 61 (87.1%) survived with mainly good result. There were no differences in the survival rate and frequency/type of postoperative complications with respect to the time of the back closure. 31 children with myelomeningocele (33%) had CNS anomalies other than the Arnold-Chiari-Malformation, mostly being a dys- or aplasia of the corpus callosum and a deformed ventricle system. These were diagnosed almost exclusively by CT or MRT scan. CONCLUSIONS: We still predominantly practised a selective policy of treatment of newborns with myelomeningocele with mainly good results.

Cerebrospinal Fluid Shunts↗
PubMed1990

[Miller-Dieker syndrome (type I lissencephaly) with specific EEG changes].

We present the case report of a girl with a subtype of Lissencephaly syndrome, type I, "Miller-Dieker syndrome", pointing out the specific EEG features in infancy and early childhood. The following pathognomonic EEG manifestations may confirm the diagnosis of an lissencephalic syndrome: abnormally fast background activity of an extraordinary high voltage increasing with age, missing topographic structuring, no reactivity to sleep or medication, unusually high-voltaged sharp-slow-wave complexes.

Agenesis of Corpus Callosum↗
PubMed1984

[Hypoxic-ischemic encephalopathy. Clinical considerations].

The Hypoxic-Ischemic Encephalopathy (HIE) is a severe illness of the unborn, respectively of the newborn. About 90 percent of the causes occur in utero, about 10 percent after birth. The risk for HIE arises from anatomical and pathophysiological particularities: little overlapping between the great cerebral arteries, poor periventricular vascularisation, and a loss of the autoregulation of cerebral blood flow during asphyxia. Most important is the early detection of intrauterine asphyxia. After birth the general measures include: thermoneutral temperature, oxygenation, normal pCO2, regular blood pressure monitoring, glucose infusion, therapy of convulsions and of an inherent brain edema. After birth the five most common clinical settings in which HIE occurs, are: postpartum asphyxia, PFC, septic shock, pneumothorax and apneas. Therapeutic measures (e.g. volume therapy) have to be prompt but subtle, to prevent ischemia, avoiding overtherapy with its risk of intracranial hemorrhage.

Asphyxia Neonatorum↗
PubMedDate not supplied

Progressive cerebral degeneration of childhood with liver disease (Alpers Huttenlocher disease) with cytochrome oxidase deficiency presenting with epilepsia partialis continua as the first clinical manifestation.

A previously healthy and normally developed 17-year-old young female presented with a sudden onset of focal motor seizure status that proved to be refractory to anticonvulsive treatment. Severe encephalopathy with visual impairment leading to blindness, mental deterioration, and predominantly left spastic tetraparesis developed progressively. Hepatic disease evolved 4 months after onset of the first symptoms and led to death in hepatic failure 1 month later. Diagnostic studies revealed an elevated protein and lactate in the cerebrospinal fluid, slow-wave and intermittently continuous spike-wave activity in the EEG, and a complex i.v. (cytochrome-C oxidase) deficiency in the muscle biopsy. MRI scans revealed signal abnormalities in the occipital lobe, thalamus, and basal ganglia only after 3 months. Histopathological findings in liver biopsy and in postmortem brain examination displaying widespread predominantly right cortical spongiosis, neuronal loss and astrocytosis were consistent with the clinically suspected diagnosis of progressive neuronal degeneration of childhood with liver disease (PNDC) or Alpers Huttenlocher disease. This rare disorder of unknown origin is usually seen in infants and young children and is rarely reported in adolescence.

Adolescent↗