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Biomedical subjects

B J Crain

Publications and source records attributed to B J Crain.

76 records · Page 5Linked to original sources

Degeneration of the substantia nigra in familial amyotrophic lateral sclerosis.

We have studied a large family with familial amyotrophic lateral sclerosis (FALS) and examined the brain and spinal cord of a 40-year-old male family member at autopsy three years after clinical onset of the disease. The most unusual finding was a severe degree of neuronal loss of the substantia nigra accompanied by gliosis and numerous pigment-laden macrophages. There was marked degeneration of the upper and lower motor neurons and the corticospinal tracts. In addition, there was an unusually severe degree of diffuse degeneration throughout the anterolateral columns of the spinal cord. The posterior columns and Clarke's nucleus were not involved. Nigral degeneration has rarely been reported in FALS. The findings in this case emphasize the great variability of morphologic changes encountered in FALS and raise the question of a relationship between FALS and extrapyramidal disorders such as Parkinson's disease. We conclude that careful postmortem examinations of further cases of FALS are needed to fully define the extent of degenerative changes in this disease.

Adult↗

MR imaging in patients with temporal lobe seizures: correlation of results with pathologic findings.

Thirty-nine consecutive patients with medically intractable complex partial seizures were studied with electroencephalography and MR imaging to localize an epileptogenic focus for temporal lobectomy. The patients were divided into three groups on the basis of pathologic findings after lobectomy: Group 1 comprised 13 patients with neoplasms, hamartomas, or cysts; group 2 comprised 13 patients with moderate and severe mesial temporal sclerosis (one patient was included in both groups 1 and 2); and group 3 comprised 14 patients who underwent aspiration lobectomy, which yielded limited tissue for pathologic study so no pathologic diagnosis was made. The majority of the patients in group 3 were assumed to have mesial temporal sclerosis. Abnormal MR signal in the temporal lobe on T2-weighted images was graded as minimal increase (1+), intermediate or moderate increase (2+), and very significant increase (3+). An abnormal signal was demonstrated in 26 (67%) of the 39 patients. In group 1, the tumor/cyst subgroup, an abnormal signal was seen in all 13 patients. Most had 3+ signal. There was increased signal in eight (62%) of 13 patients in group 2 and in six (43%) of 14 patients in group 3. This study suggests that MR can detect almost all tumors and a significant number of mesial temporal sclerosis lesions in individuals with complex partial seizures. On the basis of this small series, individuals who exhibit significant signal (3+) can be expected to have neoplasms, hamartomas, or cysts, and patients who exhibit minimal signal (1+) will usually have mesial temporal sclerosis.

Brain Neoplasms↗

Cerebral polygyria in the Chiari Type II (Arnold-Chiari) malformation.

Abnormalities of the cerebral cortical pattern have been observed in association with the Chiari Type II malformation and described as polymicrogyria, microgyria, stenogyria or polygyria. In order to characterize and quantify these abnormalities, the sulcal patterns of the brains of 15 patients with the Chiari Type II malformation were compared with those of 10 pediatric controls without cerebral malformations. In this analysis, sulci were grouped by anatomical characteristics into three categories. The first category, the fissures, were represented by the long, deep indentions in the cerebral cortex such as the Sylvian fissure. The second category, the principal sulci, were represented by the shorter, named, anatomically consistent sulci such as the superior frontal sulcus. The third category contained the minor sulci which were defined as those shallow grooves which depressed the cortical surface but were not recognizable as consistent anatomical landmarks. Subjectively all brains with Chiari Type II malformations demonstrated abnormal gyral patterns. These brains exhibited abnormalities in the number and distribution of principal and/or minor sulci. Sulcal density, defined as the length of sulcus per square millimeter of cortical area, was quantitated utilizing a computer assisted digitizer program. For the brains with the Chiari Type II malformations, the length of all sulci (principal and minor combined) per square mm was 0.238 +/- 0.045 mm-1 as compared with the control patients whose value was 0.182 +/ 0.021 mm-1 (t = .0015). Average length per square millimeter of principal sulci was 0.176 +/- 0.029 mm-1 as compared with 0.132 +/- 0.015 mm-1 (t = .0002) for the control brains.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Fine needle aspiration biopsy of deep cerebrum. A comparison of normal and neoplastic morphology.

Fine needle aspirates of deep white matter, basal ganglia and thalamus from necropsies of three neurologically normal men were compared with two aspirates of cerebral infarcts and with eight aspirates from neoplasms that occurred in the deep cerebrum. Normal white matter contained oligodendroglia with small, dark, round nuclei and astrocytes with larger, bland, oval nuclei and occasional cytoplasmic extensions. Basal ganglia and thalamus contained, in addition, numerous neurons with large nuclei and prominent nucleoli. Recognition of these normal components of the deep cerebral structures is necessary to avoid erroneous diagnoses of neoplasia.

Adult↗