Biomedical subjects
J Peiffer
Publications and source records attributed to J Peiffer.
Alcohol embryo- and fetopathy. Neuropathology of 3 children and 3 fetuses.
Maternal chronic ethanol abuse during pregnancy causes malformations of the offspring. Three children (aged 6 months, 9 months, 4 1/2 years) and 3 fetuses (17th, 18th, and 20th gestational week) showed a wide spectrum of disorders ranging from severe dysraphic state, arhinencephaly, porencephaly, agenesis of corpus callosum, a range from hydranencephaly to microdysplasias (p.e. reduced gyration of dentate nucleus and inferior olives), and a range from gastrochisis or congenital heart defects to craniofacial dysmorphogenesis and palmar crease anomalies. The patterns of the cerebral malformations were not as uniform as the clinical phenotype of the alcohol embryopathy. The observations did not support the assumption that there exists a specific period for alcohol teratogenicity.
[The current status of neuropathology in the Federal Republic of Germany 1979 (author's transl)].
This article describes changes in neuropathologic work towards a more intravital diagnostic aid, the increase in university neuropathologic departments, and the increased amount of neuropathology in teaching and examinations. These positive developments contrast with some genuine problems: the clinically oriented departments of neuropathology in some big research centers have been closed or altered and the personal staff of the university departments has been reduced. Subspeciality training in neuropathology within pathology should be supplemented by the possibility of entering neuropathology by way of neurology and psychiatry. Neuropathology should be connected to the neurosciences since this is the prerequisite to attain and maintain international research standards. However, the conditions necessary to achieve this aim personally and institutionally should be made available to all pathologists, clinicians, and administrators concerned with this subject.
[Reversible calcification of skeletal muscles in acute renal failure (author's transl)].
The clinical course in two patients with acute renal failure and focal calcifications of skeletal muscle are reported. In the first case renal failure was due to a hypovolemia or shock combined with supercooling and alcoholic intoxication. In the second case a rhabdomyolytic crisis with myoglobinuria occurred. This patient was known to have a McArdle disease. Dialysis treatment was necessary in both cases in order to overcome the oligoanuric phase. Biopsy specimens from biceps muscle showed intense calcium deposits within the necrotic muscle fibres. In the beginning of oliguria remarkable hypocalcemia occurred followed by hypercalcemia. During the polyuric phase which was accompanied by hypercalcemia and hypercalcuria the calcium deposits disappeared completely. This could be demonstrated in our first case by a control biopsy.
Neurovisceral lipidosis compatible with Niemann-Pick disease type C: morphological and biochemical studies of a late infantile case and enzyme and lipid assays in a prenatal case of the same family.
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[Interruption of pregnancy in alcoholic women (author's transl)].
Basing on previous experience, with alcohol embryopathy the authors recommended interruption of pregnancy in three chronic alcoholics, two of whom were in the chronic phase and one in the critical phase of alcohol addiction. All the three fetuses were hypertrophic, two severely malformed. In the authors' opinion there is eugenically speaking an absolute indication of interruption of pregnancy in alcoholics in the chronic phase of addiction. In women who are in the critical phase of addiction, each case requires close scrutiny, whereas interruption is not indicated from the eugenic aspect in women in the prodromal stage.
[Basilar or vertebral artery aneurysm as a cause of presumed cervical spine injury (author's transl)].
The characteristic clinical features and morphological findings of five cases of (clinically not diagnosed) fusiform aneurysm of the basilar or vertebral arteries were recurring attacks of positional occipital headache, pain and stiffness in the neck, cranial nerve disturbances, expecially oculomotor palsies and anisokoria, nystagmus, attacks of nausea, vomiting and sweating, tachycardia, pyramidal tract symptoms, and pareses. Severe hypertension had been present in four instances. The aneurysm, which is usually thrombosed, pressed against the pons and medulla oblongata as a space-occupying mass. In addition to hypertension and atheromatosis, congenital defect in the arterial wall are probably significant causative factor. To mistake an aneurysm for a cervical syndrome may be fatal to the patient.
Generalized giant axonal neuropathy: a filament-forming disease of neuronal, endothelial, glial, and schwann cells in a patient without kinky hair.
The process of Giant Axonal Neuropathy (GAN) is not restricted to the peripheral nerves, but also involves the central nervous system. In a 25 year old man with normal hair, abundant axon swellings and spheroids were observed in the spinal cord, brain system, and cerebral cortex. The findings in the sural nerve have already been published by Boltshauser et al. (1977). Accumulations of filaments in the axons and in the perineural cells were accompanied by Rosenthal fibres. The ultrastructural pattern of GAN differs clearly from that of Neuroaxonal Dystrophies.
[Hypoplasia ponto-neocerebellaris (author's transl)].
In an otherwise healthy family three male infants fell ill with microcephaly, hypotonus of the muscles, non-specific hyperkineses, seizures, and rapid mental deterioration. In addition to microdysplasia in the cerebral cortex the main neuropathological findings were the separation of the dentate nucleus into many islets, an atypical band of the inferior olives, and deficiency of the pontocerebellar fibers in the basal part of the pons. The hypoplasia of the areas involved can be distinguished from the degenerative process in the dégénérescence systematisé optico-cochleo-dentelé (Nyssen-van Bogaert).
[Generalized infantile neuroaxonal dystrophies with pigmentation and lipophanerosis of the pallidum in concordant twins (author's transl)].
Monozygotic male twins died at the age of 6 1/2 and 7 1/2 years respectively after a progressive course of mental deterioration, hypotonia, spasticity, optic atrophy and seizures that had commenced at the age of 2 years. Both patients showed generalized neuroaxonal dystrophy (NAD), marked by numerous spheroids, iron-positive pigment and lipophanerosis of the pallidum. NAD can be classified as a generalized form without pigmentation of the pallidum (infantile type of Seitelberger), a juvenile type of Rozdilsky, a generalized form with pigmentation (cases described here), and localized forms (infantile, late infantile, juvenile = classic Hallervorden-Spatz disease, adult types).