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Biomedical subjects

J Sperner

Publications and source records attributed to J Sperner.

28 records · Page 2Linked to original sources
PubMed1990

[Magnetic resonance tomography of laminar heterotopia].

In one baby and 2 infants who presented with psychomotor retardation and epilepsy laminar heterotopic grey matter was demonstrated via magnetic resonance imaging. Laminar heterotopia is a rare migrational disorder with bilateral symmetric ribbons of grey matter within the centrum semiovale, separated from ventricular walls and from obviously normal-sized cortex by broad layers of white matter. The heterotopic grey matter has a signal intensity which is isointense compared with that of normal cortex irrespective of image weighting. On account of this signal behaviour differentiation against other white matter diseases is easy. The knowledge of these pathognomonic findings facilitates correct diagnosis, especially during the first and the second year of life, when signal intensities of white and grey matter differ from normal findings because of the occasionally delayed myelination process. Therefore, further diagnostic procedures can be avoided and early counseling of parents is possible.

Brain↗
PubMed1990

MR-imaging findings in children with Sturge-Weber syndrome.

Intracranial extent and distribution of leptomeningeal angiomatosis, visualized by magnetic resonance imaging (MRI) with Gadolinium-DTPA (Gd-DTPA) enhancement, is demonstrated in four children with Sturge-Weber syndrome (SWS). Aged 7, 9, 11 and 19 months, they presented with cutaneous, neurologic and ocular symptoms at the time of MRI examination. Angiomatous alteration of the skull, atypically located and congested intracerebral and basal veins as well as intracerebral changes secondary to the leptomeningeal angiomatosis are demonstrated with T2 weighted images. Gd-DTPA enhanced T1 weighted images exhibit clearly the regional distribution of angiomatosis in the skull, meninges and within the brain. Before calcifications in children with SWS are detectable by CT, MRI is the method of choice to detect intracranial involvement. Enhancement with Gd-DTPA improves the diagnostic value of MRI, before neurological symptoms appear. Follow-up studies with Gd-DTPA enhanced MRI can be applied to recognize thrombotic changes of leptomeningeal angiomatosis as well as subsequent intracerebral impairment.

Atrophy↗
PubMed1990

[Magnetic resonance tomography of adrenoleukodystrophy].

Adrenoleucodystrophy (ALD) is an uncommon, sex-linked disease, characterised by the accumulation of very long-chain fatty acids in various tissues. There is a great predilection for young boys between the age of three and ten years. The disease ends fatally after a clinical course of several years. The clinical and MR findings of a child with typical ALD are presented. T2-weighted MR demonstrates confluent, symmetrical, hyperintense lesions of the occipital white matter bilaterally. After the application of Gd-DTPA, contrast enhancement is seen in the margins of the lesions indicating active demyelination. MR is recommended as imaging modality of choice in patients with suspected or confirmed diagnosis of adrenoleucodystrophy.

Adrenoleukodystrophy↗
PubMed1988

Lissencephaly: diagnosis by computed tomography and magnetic resonance imaging.

A patient with Miller-Dieker-Syndrome, which is associated with lissencephaly, was examined by Computed Tomography (CT) and Magnetic Resonance Imaging (MRI). While CT demonstrated the main features of lissencephaly, MRI detected disturbed myelination and cell migration in the cerebral hemispheres and a normal appearance of the cerebellum. MRI provides more accurate information of the pathomorphologic changes of lissencephaly and is thus superior to CT.

Abnormalities, Multiple↗
PubMed1978

The spreading of focal brain edema induced by ultraviolet irradiation.

Focal brain edema limited to one cerebral hemisphere was produced by ultraviolet irradiation of the exposed cortex. Tissue water content was determined by the gravimetric method which allows microsampling. Therefore, the spread of edema around the small necrotic area could be mapped more precisely than by determination of dry weight which calls for larger samples. As early as 30 min following irradiation, hyperemia and swelling of the brain are observed under the operating microscope. This correlates with venous stasis, hyperemia, and broadened perivascular spaces around venules and large capillaries accompanied by a marked rise in the specific weight of the tissue. After 4 h an edema front can be observed spreading from the perinecrotic zone in which there is a marked rise in endothelial cell vesicular activity. Edema reaches maximum levels in the deep white matter at 48 h post irradiation with normalisation of the tissue water content after 96 h. The velocity at which the edema front spreads from the cortex to the periventricular area lies in the range of 0.25 mm/hr. Edema reabsorption coincides with signs of retrograde micropinocytosis in endothelial cells.

Animals↗
PubMed1976

Ultrastructure of the neuroglial fatty metamorphosis (Virchow) in the perinatal period.

The ultrastructure of neuroglial fatty metamorphosis (GFM) has been investigated in the telencephalic white matter of 12 premature and mature infants (gestational age 22-40 weeks; survival 0-96 days). GFM was found in all cases apart from a 22-week-old fetus, and involves predominantly astrocytic cells (68.8%), then glioblasts (43.5%), but only 7.4% of oligodendrocytes. GFM, therefore, seems to be independent of the myelination process and indicates the vulnerability of the immature neuroglial population in the metabolic and circulatory disorders of the perinatal period. Since GFM is found in almost all children dying within the early postnatal period, this subtle alteration reflects a special form of minimal brain damage. The relationship between GFM, astrocytic hypertrophy and periventricular leucomalacia and their role in the telencephalic leucoencephalopathy are discussed.

Astrocytes↗