Biomedical subjects
Publications and source records attributed to P Fehlow.
Among 100 patients with turricephalus we found 37 with disorders of social adaption, 54% of them has been punishable, 22% even relapsing. Sexual offenders were dominating. Dysplasia of front cranial fossa with dominating exhibition of radiologic signs of craniostenosis frontal and frontotemporal let consider frontal cortex dysfunctions as basic disorder of psychic maldevelopment and delinquency of turricephalus persons.
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In case of an indication for an operation on craniosynostosis not only an atrophy of the optical nerve, mental retardation and epileptic fits should be taken into consideration, but also the danger of faulty psychic developments, criminality of male patients, increased occurrence of psychoses and early cerebral blood flow disturbances as well as, especially in girls with scapho- or oxycephaly, cosmetic impairments with resulting psychosocial disturbances. Convulsions in craniosynostosis are not always an expression of a generally increased intracranial pressure, sometimes genetic couplings should be taken into consideration in case of an indication for craniotomy. Disturbances of social adaptation are related to a psychic fronto-cerebral syndrome due to frontal dysplasia of the base of the skull. For carrying out a craniotomy it is advisable to employ a combination of orbito-fronto-sphenoidal osteotomy for an extension of the anterior cranial fossa.
Among 2000 dead we found a frequency of death by bolus of about 1% in neuropsychiatric patients in the course of 17 years. Severe oligophrenia mostly combined with epilepsy was leading with almost 50% followed by depressive syndrome, schizophrenia and dementia. Organic cerebral lesion, disorders of activity and vigilance, longterm psychopharmacotherapy, alteration of condition by acute internal disease and perhaps disorders of the liver are considered to be risks of death by bolus.
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It is reported on four girls aged 8 to 20 with Incontinentia pigmenti (Ip) The ectodermal dysplasia was associated with oligophrenia, therapy resistant epilepsy with severe psychorganic syndromes and dementia, Lennox syndrome, lability of emotion with severe disorders of social adaptations, spasticity, hypo- and areflexia, hemiathetosis, choreiform unrest, pathological EEG findings, including spike-potentials; relations to cleidocranial dysostosis, dysraphia, Poland's, Greig's and Chotzen's syndrome were found. Ip means unfavourable prognosis and progression of neuropsychiatric disorders.
It is reported on a girl with a chronic encephalitis leading to death after 8 years. The course had first been typical for subacute sclerosing panencephalitis, but autopsy showed lesions of the brain typical for herpes simplex encephalitis.
The case of a boy, observed from 9-17 years of age, with de Lange syndrome is described. The typical symptoms of the de Lange syndrome (brachymicrocephalie, characteristic face, hypertrichosis, typical form of the hand, debility and proportioned shortening) were combined with intensivity to pain and psychomotoric epilepsy. He showed a strikingly aggressive behaviour and simultaneous masochism with self-mutilation as it is typical for analgesia congenita. The autopsy showed cortical heterotopias of the brain and slight microgyria.
It is reported on a 19 year-old female patient with a mild mental retardation and a neurocutaneous syndrome. A tumour of a lateral ventricle had caused a partial blockade of the Foramen of Monro without symptoms of brain pressure. The patient shows lesions on the skin corresponding to a neurofibromatosis. Neurologically there was a choreatic syndrome, which can occur in transitional forms from neurofibromatosis to tuberous sclerosis (TS) of the brain. Psychologically a psychorganic syndrome, typical for TS, with disturbances of affectivity and motive developed. There was no essential improvement of the neurological and psychical symptoms by surgical removal of the brain tumour nor were there changes in the EEG except for an acceleration of the basic rhythm.
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The case of a 15-year-old boy with Ehlers-Danlos-Syndrome (EDS) is described. Clinically the symptoms of considerable hyperextension of joints, abnormal extensibility of skin, moderate bleeding tendencies and slight vulnerability of the skin, deformity of the thorax are corresponding with type I of EDS. Ocular symptoms are missing. Histologically the picture is that of mitis type resp. type II of EDS. An autosomal dominant inheritance is to be supposed. Psychically an imbecillity likely as a result of perinatally acquired brain damage, and an autistic syndrome of broken home situation are present. Differential diagnosis and genetic significance of EDS are discussed.
As a result of an one time electroencephalographic transversal examination of 130 patients with enuresis aged from 3 to 18 years a back ground activity that was too slow for their age was found as the most frequent abnormal finding (31,5%). Focal sharp waves and spikes were observed in 20 per cent; they seem to be more frequent in enuretics than in other children and adolescents with disorders of behaviour. Their frequency is dependent on age. They are of small use for proving an epilepsy as possible cause of enuresis.
In comparison with the literature causes and circumstances of juvenile delinquency of sodomy are examined by 5 cases. All of them were light weak-minded and badly educated, showed disorders of behaviour and personality. In two cases by violation and killing of animals as the result of sodomy a considerable economical loss was caused.
A genetically remarkable case of the Vogt syndrome (combination of the Apert and Crouzon syndromes) associated with a dysraphia syndrome is described. Clinically, malformations corresponding to the Apert syndrome were prominent, and radiological examination of the skull revealed a prominent Crouzon syndrome. Investigation of the familial background revealed the presence of malformations in other members of the family, such as malformation of the extremities and acrocephalus. On the other hand, the father's age of 63 years was considerably higher than that of fathers in the average population, so that the possibility of a new mutation had to be considered. The fact that the mother suffered an attack of influenza during pregnancy, finally, brought the possibility of peristatic influences being involved in the complex malformations.
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