A 23-year-old Japanese woman experienced sudden severe shoulder pain, and subsequently died of cardiorespiratory arrest after an 8-day course of illness. Autopsy revealed a venous malformation in the spinal cord at the C6 level with fresh massive hemorrhage showing widespread rostrocaudal extension. The upper limit of the hemorrhage was in the left gracile nucleus of the medulla oblongata, and the lower limit was in the left posterior horn of the spinal cord at L4. The hemorrhage extended through the spinal cord, involving the left posterior horn and intermediate zone of the gray matter and the ventral part of the posterior funiculus. The mechanism of the extension of the hemorrhage and the pathogenesis of the spinal venous malformation are discussed.

Adult↗

PubMedDate not supplied

A morphometric reevaluation of Huntington's chorea with special reference to the large neurons in the neostriatum.

In order to reevaluate the quantitative changes in the neostriatum of Huntington's chorea (HC), sections of the caudate head and putamen from 4 HC and 5 control cases were treated with Klüver-Barrera stain and the nuclear area of the neurons was measured by a digitizer. The number of neurons of different sizes was evaluated statistically. This study revealed a significant decrease in the number of large neurons (nuclear area; greater than 121 microns 2) as well as a severe decrease in the number of small neurons (nuclear area; less than 80 microns 2) in the neostriatum of HC. There was a parallel degree of neuronal loss in the upper and lower portions of both caudate head and putamen. The degree of neuronal loss was slightly more severe in the putamen than in the caudate head. The depletion of the large neurons was not correlated with the loss of small neurons. The number of medium-sized neurons (nuclear area; 81-120 microns 2) showed no statistically significant change.

Adult↗

PubMedDate not supplied

Rod-like intracytoplasmic inclusions in large neurons of the caudate nucleus: frequent appearance in myotonic dystrophy.

Brain autopsy materials from 5 patients diagnosed clinicopathologically as having myotonic dystrophy (MyD) were studied. All of the cases showed many rod-like intracytoplasmic inclusions, which have never been reported in the disease, in the large neurons of the caudate nucleus. The rod-like inclusions were observed in the cytoplasm near the nucleus, and showed eosinophilic staining with HE, blue with PTAH, red with Masson's trichrome, and blue with toluidine blue. Electron microscopically, the inclusions had an internal structure composed of parallel beaded dark filaments and clear spaces. In transverse section, the inclusions showed tetragonal arrays of filaments. These histological characteristics of the inclusions were similar to those of inclusions reported previously in the same location in various disorders, showing increasing incidence with aging. Quantitative investigation on the 5 patients and 24 control subjects revealed that the number of neurons containing the inclusion in the caudate nucleus of MyD patients was about twice as many as the control subjects, and that the occurrence rate of the inclusion body to the large neurons in the caudate nucleus in MyD was far higher than that of the controls. The finding indicates that the inclusion body is important for the diagnosis of the disease, and may have a close relationship with the neurological symptoms and pathogenesis of the disease.

Adult↗

PubMedDate not supplied

Primary central nervous system lymphoma presenting as diffuse cerebral infiltration.

We report a case of primary central nervous system lymphoma presenting as diffuse cerebral infiltration. On T2-weighted spin echo MR imaging using a 1.0 Tesla unit, high signal intensity lesions were presented mainly in the bilateral pyramidal tracts and also diffusely extended to white matter with brain swelling. No enhancing lesion was demonstrated after the intravenous injection of Gadolinium-DTPA, during the course of her disease. Radiological diagnosis of gliomatosis cerebri was made because of the characteristic MR appearance. Stereotactic biopsy was performed, and diffusely scattered lymphocytes, probably neoplastic, in the cerebral white matter were demonstrated histopathologically. Oral corticosteroids, radiation therapy, and chemotherapy were applied soon after her admission. The patient's condition improved dramatically. MR imaging using a 1.5 Tesla unit after the treatments showed marked regression and reduction of high intensity lesions on T2-weighted images and improvement of brain swelling. It should be emphasized that brain biopsy is important method for correct diagnosis in case of diffuse non-enhancing brain lesions like our case.

Biopsy↗