Biomedical subjects
Publications and source records attributed to J Brismar.
The technique for orbital phlebography is described and the phlebographic anatomy of the orbit and the skull base presented. The possibilities and limitations of phlebography in the diagnosis of different intraorbital and ophthalmoneurological disorders is discussed and illustrated out of personal experiences from 200 cases.
Frontal phlebography has been used to examine the cavernous sinuses and adjacent basal venous sinuses of the skull. The method has proved to be simple and effective. The normal phlebographic anatomy of the basal sinuses and their drainage has been revised in a material of 26 cases. A normal range for the transverse diameter of the pituitary fossa has been established.
Computer assisted axial tomography was performed in 32 patients with clinically possible cerebrovascular lesions. Decreased absorption as a sign of infarction was found in 15, increased absorption as a sign of hemorrhage in 7 cases. In another 7 cases, diagnosed as hemorrhagic infarction, a simultaneous occurrence of increased and decreased absorption appeared. Increased absorption might also depend on compression effect of edema. In three cases no abnormal attenuation could be demonstrated. Infarctions could be observed years after the onset of the symptoms; intracerebral hemorrhages regressed slowly.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
A subarachnoid haemorrhage (SAH) in the rat was produced by the injection of blood via a previously implanted catheter connected to the cisterna magna. Repeated angiographical examinations of the vertebro-basilar arteries revealed a biphasic vasospasm with a maximal acute spasm at ten minutes and a maximal late spasm at two days after cisternal blood injection. Fluorescence microscopical examination of the major cerebral arteries at day two after the SAH revealed a reduction in the fluorescence intensity and in the number of histochemically visible sympathetic nerve terminals.
Intracisternal injection of blood in the rat produces an angiographically demonstrable biphasic vasospasm. Lesioning at the level of the mesencephalon of the ascending catecholamine pathways from locus coeruleus in the pons and the A1 and A2 nuclei in the medulla oblongata prior to cisternal blood injection prevents the development of both acute and late spasm. Selective lesioning in the medulla oblongata of ascending fibres from A1 and A2 also prevents development of spasm, indicating that these nuclei, which project to the hypothalamus-pituitary, are essential for the spasm syndrome. It is suggested that a substance vasospasm is produced by a substance liberated either by the hypothalamus or by the pituitary is involved in the occurrence of spasm.
In the rat an intracisternal injection of blood induces an angiographically demonstrable biphasic cerebral arterial vasospasm. Chemical destruction of the central serotoninergic and dopaminergic pathways prior to the cisternal blood injection does not affect the spasm pattern after the subarachnoid haemorrhage. It is suggested that neither system plays a role in the development of spasm.
A prospective investigation of conventional abdominal radiography, ultrasonography, computed tomography and 111In-labelled leucocyte scintigraphy was performed in 40 patients suspected of having an intra-abdominal abscess. There were 23 confirmed abscesses in the material. When conventional abdominal radiography indicated an abscess, such a lesion was usually present. The ability of abdominal radiography to exclude an abscess was, however, low. Both ultrasonography and 111In-labelled leucocyte scintigraphy detected 65 to 85 per cent of the confirmed abscesses, but both also revealed many abscess-like areas in patients where no unequivocal abscess was confirmed at follow-up. Computed tomography was, when employed as a single method, the most reliable one both to show and to exclude an abscess, However, the combination of ultrasonography and 111In-labelled leucocyte scintigraphy disclosed all the lesions demonstrated by any one of the four methods used in the investigation.
We studied 12 children who presented with a recently recognized syndrome. The salient features of this new syndrome were recurrent fever; hepatosplenomegaly; pancytopenia; blond, golden to silvery gray hair; hypopigmented skin, progressive white matter demyelination; and early death. Seven patients died, four with severe central nervous system (CNS) involvement, and three with bone marrow failure and sepsis. Cutaneous anergy to recall antigens was present in all patients. Other immunological abnormalities were poor antibody responses; deficient T-cell responses to phytohemagglutinin (PHA), concanavalin A (Con-A), and allogeneic lymphocytes; hyperresponsiveness of B lymphocytes to pokeweed mitogen; and variable phagocytic defects. Histopathologic examination of the hair and skin biopsies showed characteristic distribution of melanin with melanocytes present in normal numbers but with fewer short dendritic processes. Langerhans' cells were present in normal numbers in some patients and sparse in others. This syndrome seems to cluster into two tribes from two different geographical areas in the Arabian Peninsula. In the eight families studied, 12 other siblings and close relatives were found to be affected. The mode of inheritance in this syndrome is that of an autosomal recessive pattern. We propose the term "PAID syndrome" to identify patients with the above features.
PURPOSE: To describe our experience in infants with partial albinism and immunodeficiency (PAID), a rare, recently recognized, probably autosomal recessive disorder. PATIENTS AND METHODS: Five infants suffering from this disease were examined with CT of the brain and four of these patients also underwent MR. Four of the five children also underwent follow-up CT or MR exams. RESULTS: Three of the patients followed with serial examinations demonstrated a rapid progress of white matter changes together with a loss of brain tissue over a few months. In all four patients subjected to follow-up, the posterior fossa white matter structures were severely involved during the course of the disease. CONCLUSIONS: This syndrome should be added to the list of demyelinating diseases, and should be kept in mind when white matter changes are prominent in the posterior fossa.
In 13 patients, the GM2 gangliosidoses, Sandhoff disease and Tay-Sachs disease, were found to be constantly associated with homogeneously and symmetrically increased CT attenuation within the thalami. In the only patient examined with MR imaging, a T2-weighted sequence showed hypointense thalami. It is suggested that this finding is caused by an accumulation of calcium, associated with the intracellular storage of GM2 ganglioside. The finding of dense thalami may be useful as a specific diagnostic criterion for GM2 gangliosidoses. In a few patients with blocks in adjacent steps in the sphingolipid metabolism, this finding was not present.
A defect in biopterin synthesis not only prevents the transformation of phenylalanine to tyrosine (as in classical phenylketonuria, PKU) but also blocks the biosynthesis of the neurotransmitters dopamine, norepinephrine, and serotonin, causing severe neurologic disturbances. The brain CT and MR findings in this rare disorder have not been described. In the present series, eight patients with PKU were all examined with CT, three were also examined with MR imaging. In spite of severe clinical findings, CT was normal or almost normal in three patients; in three other children, moderate loss of brain volume was found. White matter disease was found in three patients (moderate in two and severe in one) and was also found in an additional patient with classical PKU. PKU should therefore be added to the list of possible causes for white matter disease. Furthermore, biopterin-dependent PKU should be considered when the CT examination in a child with severe neurologic manifestation only shows discrete pathology.
Canavan disease (spongy degeneration of the brain) is a rare lethal neurodegenerative disorder of infancy; fewer than 100 cases have been reported. We describe a series of nine patients with enzymatic defects and clinical features consistent with Canavan disease; in two patients the disease was proved by biopsy. All patients were examined with CT and seven with MR. In every instance, both CT and MR showed white-matter disease, sometimes sparing the external and internal capsules, the corpus callosum, and the deep cerebellar white matter. Atrophic changes were found in six patients; the changes were marked in one and slight in five. There was no obvious correlation between the severity of the white-matter disease and the clinical presentation--one patient with severe white-matter disease was clinically normal. The CT and MR findings in Canavan disease are nonspecific and somewhat nonuniform: its preautopsy diagnosis relies primarily on biochemical findings.