Epileptic temporal and parietal cortices, removed from 6 patients with therapy-resistant (intractable) partial epilepsy (TRPE) during neurosurgery, were studied. Neurons (40-50 in each slice) in laminae I-VI and white matter were injected with Lucifer Yellow (LY). Samples were examined in a confocal laser scanning microscope (BioRad [Richmond, CA] MRC 600), and individual cells were scanned at 0.1-2 microns incremental levels. 2D maximal linear projection was used for overview. Frames (50-60) of scanned neurons were transformed into 3D volumes, using VoxelView software on a Silicone Graphics workstation, and rotated. All samples contained pyramidal neurons with duplicated apical dendrites, additional basal dendrites, or were misplaced in a horizontal position in the white matter. Rarely were such cells observed in normal cases. The relation between the observations and the disease is discussed. The attempt to simultaneously apply immunofluorescence was successful concerning synaptic vesicle antigens. This approach will be used for a detailed study of the synaptology of this disease.

Adult↗

PubMedDate not supplied

Effects of training on skeletal muscle in protein-deprived rats.

The effects of exercise on atrophy of muscle fibres and loss of mitochondria in the extensor digitorum longus (EDL) and soleus muscles were studied in protein deprived rats. They had smaller muscle fibres than aged-matched control rats, the difference being more evident in type 2 than type 1 fibres both in the fast EDL and slow soleus muscles. The loss of weight was more pronounced in the EDL muscle which is composed mainly of type 2 fibres than in the soleus muscle which is composed mainly of type 1 fibres. Protein deprived rats subjected to a programme of periodic running on a treadmill for 12 weeks showed less muscle atrophy than sedentary, protein deprived rats. This effect of exercise in diminishing the degree of atrophy was more pronounced in the type 2 than type 1 fibres. The protein deprived rats which had been sedentary showed a marked loss of subsarcolemmal mitochondria, which was not seen in protein deprived rats undergoing exercise.

Adenosine Triphosphatases↗

PubMedDate not supplied

Rett syndrome: spinal cord neuropathology.

The morphologic changes of the spinal cord in Rett syndrome are described in 2 young women who died at 20 and 30 years of age. Both patients had been in a severely disabled state for many years with tetraparesis and extreme muscle wasting. Degeneration and loss of spinal ganglion nerve cells, in addition to gliosis of both the white and gray matter of the spinal cord, were evident. The number of motor neurons appeared to be reduced and axonal changes suggestive of degeneration were observed in both the ascending and descending tracts.

Adult↗

PubMedDate not supplied

Rett syndrome: cerebellar pathology.

The cerebellar pathology at autopsy of 5 patients with Rett syndrome is described. The patients ranged in age from 7-30 years. All had markedly reduced brain weights with proportionately small cerebella. Microscopic examination revealed loss of Purkinje cells, atrophy, astrocytic gliosis of the molecular and granular cell layers, and gliosis and loss of myelin in the white matter. Cortical atrophy occurred focally along the folia and was often more marked in the tips of the folia. The 2 oldest patients had been treated with phenytoin which may have contributed to the morphologic changes. Atrophy and gliosis increased with age or in patients without phenytoin treatment; the youngest patient demonstrated only minor microscopic changes. In addition to the generalized alterations, 1 patient had several adjacent folia with severe atrophy. The results indicate that cerebellar changes in Rett syndrome consist of general hypoplasia with the addition of atrophy beginning in childhood and progressing over many years.

Adolescent↗

PubMedDate not supplied

Infantile globoid cell leucodystrophy (Krabbe's disease). A clinical and genetic study of 32 Swedish cases 1953--1967.

Explore the source record for details and available documents.

Biopsy↗

PubMedDate not supplied

Structural and vasoactive factors influencing intracerebral arterioles in cases of vascular dementia and other cerebrovascular disease: a review. Immunohistochemical studies on expression of collagens, basal lamina components and endothelin-1.

The condition of the brain parenchyma in cases of vascular dementia and other cerebrovascular conditions may be influenced by structural and functional changes of the terminal intracerebral blood vessels. Arterioles can develop obliterative lesions, capillaries and postcapillary venules can be altered, causing edema. The first part of this review is focused on expression of different types of collagens and other components of the extracellular matrix in intracerebral arterioles. The changes present in hereditary multi-infarct disease of the brain are compared with those occurring in the Binswanger type of encephalopathy and cases presenting hyalinosis of intracerebral vessels. Deposition of collagens in degenerated parts of the media and adventitia of the arterioles may contribute to impaired blood flow regulation in the brain parenchyma. Fibrillary collagens and basal laminae are probably the most important components of the hyaline material in vessels showing 'hyalinosis'. The second part of our review concerns the possibility that the vasoactive peptide, endothelin-1, released from reactive astrocytes, can influence the function of intracerebral arterioles. Normal astrocytes do not show endothelin-1-like immunoreactivity, but in cases of infarcts, lacunes, hereditary multi-infarct disease, Binswanger's encephalopathy and Alzheimer's disease numerous reactive astrocytes express such immunoreactivity. If endothelin-1 is produced and released from reactive astrocytes it may reach intracerebral arterioles and induce long-lasting vasoconstriction. Endothelin-1 is the most powerful vasoconstrictor peptide known to date and has mitogenic capacity. It may promote cellular mechanisms leading to astrocytic gliosis and neovascularization.

Arterioles↗