No abstract

No abstract

Pick's disease is a rare cause of dementia. We studied two clinical cases of Pick's disease in its early stage. A computed tomography (CT) demonstrated lobar atrophy affecting primarily the anterior portions of the frontal lobes and the inferior portions of the temporal lobes. The fact that the characteristic pattern of lobar atrophy is already apparent in the early stage of the disease was demonstrated.

SUMMARY The authors reported five cases of here‐ditary ataxia (Marie). The same symptoms of the disease have been observed through four generations in ten members of this family. Clinical and pathological features of one of five cases were as follows:  Clinical course: A male, aged thirty‐eight at the time of death, complained of spastic‐ataxic gait at the age of twenty‐five, which was followed gradually by double vision dysarthria. At the age of thirty‐six, the patient became bedridden, demented and depressed, accompanied with confusional‐oneiroid state in the night. Neurologically he showed accomodation paralysis, horizontal nystagumus, slight nerve deafness, logoclonia and cerebellar ataxia. Deep tendon reflexes were exaggerated, there was a Babinski unilaterally. Muscle atrophy as well as rigidity of the lower limbs was noted. He died after the clinical course of thirteen years.  Autopsy findings: Macroscopically pons, medulla oblongata, spinal cord and cerebellum were small. In frontal sections, moderate enlargement of lateral ventricles was noted. On cross‐section, transverse fibers of pons were diminished in number and gray matter of spinal cord was unsharply‐defined. White matter of cerebellar hemispheres was narrower than normal.Microscopically, ventral and dorsal spinocerebellar tracts were degenerated from lower thoracic segments to lateral sides of corpus restiforme. In addition, moderate demyelination of fasciculus gracilis (Goll) was found from thoracic segments to refgion of pyramidal decussation. Marked diminution of anterior horn cells was found. Chronic cell change of nerve cells of pontine nucleus, severe disparation of nerve cells and scattering of melanin pigments in substantia nigra were also found. Cerebellum was almost normal.  This case belongs to hereditary spastic ataxia (Greenfield). In this case, degeneration of anterior horn cells and substantia nigra was also found. Clinical and pathological comparison with nine cases reported in the literature was made.

In brain perfusion experiments conducted with blood containing [U-14C]glucose the relative specific activity (RSA) of blood glucose carbon incorporated in brain intermediate metabolites was measured. It was demonstrated that the so-called metabolic pattern of Geiger is not constant, but it bears a close relation to the function of the brain. The results of the study may be summarized briefly as follows. (1) In a group (A) of cats with a high level of brain function, the RSA of lactic acid was 75 per cent; that of glutamic acid 80 per cent; aspartic acid 75 per cent; glutamine 61 per cent; GABA 43 per cent; and respiratory CO2 55 per cent. It was observed that the major part of the carbon of amino acids, such as glutamic acid and aspartic acid, which are directly associated with the tricarboxylic acid cycle are derived from blood glucose. (2) In a group (B) showing a low level of brain function, the RSA of each amino acid was considerably lowered. The RSA of glutamic acid and aspartic acid was about 50 per cent and that of respiratory CO2 was 27 per cent. (3) In a group (C) with a still lower level of brain function, each amino acid as well as the respiratory CO2 had still lower RSA values. (4) The metabolic pattern of Geiger corresponds to values obtained during low functional activity of the brain in our experiment.