Biomedical subjects
Publications and source records attributed to P Fehlow.
Among 28 autopsy-cases with EMC had been none diagnosed clinically before. Typically we found an interstitial myocarditis and perivenous encephalitis with a preferred participation of the brain stem, eventually resulting in a central failure. There were only 5 cases with meningitis. Myelitis was not found. Former cardiac, less often cerebral lesions are likely risks for the lethal event.
Report of a boy aged 15 years with ADAMS-OLIVER-Syndrome, which is associated with dysraphia and cardiomegalia. The differential diagnosis is regarded specially.
At first estimations of turricephaly from antiquity till modern times are exhibited and at last also results of own examination of over an average disturbed social behaviour and constitutional biological aspects of patients with turricephaly. The dominant inheritance of turricephaly and also peristatic and influences of races in the genesis of premature craniosyntosis are viewed by current literature.
Report of a 17-year-old boy with oculo-auricolo-vertebral dysplasia. He exhibited microphthalmos on the left and auricular deformity at hemifacial microsomia, scoliosis, dysraphia and other malformations of vertebras and ribs further other minor anomalies of face and hands. The disfiguring microphthalmia with ptosis was an essential cause of a social maldevelopment with temporary important aggressivity. Patients with oculoauriculovertebral dysplasia should be examined for associating cardial and renal dysplasias and cases of psychosocial disorders treated psychotherapeutically in time.
It is reported on a 14-year-old girl with a hirsutism gingival fibromatosis syndrome, which is combined with moderate generalised muscle hypotonia, inhibition of physical maturation, severe scoliosis, diastasis recti abdominis and aplasia of a rib. Psychically there are idioty, autism and severe selfinjurious behaviour.
A weak minded man aged 31 years exhibited with moderate acrobrachycephalia, basal cutaneous syndactylies of the 2nd and 3rd fingers, marking symptoms of the Saethre-Chotzen syndrome (SCS), with dominant inheritance in three generations. Additionally there were symptoms of Ascher's syndrome, blepharochalasis, goitre and broad lower lip. The psychic maldevelopment is considered in association with a syndrome of the frontal lobe. Severe craniostenosis is rare at SCS; cosmetic handicap and obstruction of the nasal airway can be corrected by craniofacial surgeon.
It is reported on a mental retarded female, observed from 3-33 years of age, which demonstrated even in adulthood with long lasting shrill crying the clinical leading symptom of Lejeune syndrome and further typical symptoms with inhibition of physical maturation, important delayed motoric and mental development, microcephaly resp. characteristic craniofacial dysmorphia, anomalies of extremities, muscular hypotonia and susceptibility to infection. Psychically there were a striking affective instability and irritability, neuropathologically pachygyria and multiple cortical heterotopias.
Among 100 patients with premature synostosis of the coronal suture or of several cranial sutures there were 37 patients with problems of social adaptation. 54% of them had committed punishable offences and 22% were even recidivists. Sexual offenders with mental retardation predominated. Frontal lobe dysfunctions with increased susceptibility to environmental damages are assumed to be the basic disorder.
It is reported on a 26 year-old man with a paedophilic-voyeuristic behavior in the frame of a sexual abnormal development on the basis of a primary sexual pathological personality structure (Szewczyk). The maldevelopment has be expressed monosymptomaticly in the sphere of sexuality.
Based on 100 turriecephalous patients 5 types of personality disorders are exhibited, which were found in association with turricephaly: I. the psychosocial disturbed, sociopathic type, II. the neurasthenic type, III. the vascular type, IV. the psychosis type, V. the craniostenosis type, strictly speaking.
Without regard to early childhood self-injurious behaviour is as a rule therapeutic difficulty to influence. Therefore analysis of the joint of causes is relevant for prevention. Among 87 hospitalized oligophrenic patients there were 10 with self injury, their sensibility for pain was considerably diminished at least. Early hospitalization resp. missing or very early deficit of maternal affection and of familiar contacts on the whole are considered to be a risk for later severe self injury.
Report of a boy aged 16 years with LEBER's congenital amaurosis, which is associated with typical nystagmus and further severe general retardation, including ossification, imbecillity, acrocephaly, dysraphia and cardiomyopathia.
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