A case of slowly progressive aphasia accompanied with auditory agnosia]

A 68-year-old right-handed woman was admitted to our hospital because of difficulty to speak and understand conversation over 10 years. She was able to make herself by writing. No change in her personality or behavioral abnormality was observed so that she could live without help. Although her WAIS score and auditory brain stem response were normal, she could not understand the speech or distinguish the sound. She also spoke plenty of words fluently, resulting in undifferentiated jargon. She did not make any effort in speaking. Her speech was, however, unclear and hard to understand. Brain MRI scan disclosed a moderate atrophy of bilateral temporal lobe and enlargement of Sylvius fissure. A three-dimension reconstructed brain surface image showed enlargement of the perisylvian fissure, and atrophy of the gyrus frontalis inferior, operculum, gyrus temporal superior, bilaterally. Reduced cerebral blood flow was demonstrated on 99mTc-ECD SPECT in the left thalamus and bilateral fronto-temporal lobe. A diagnosis of slowly progressive aphasia with auditory agnosia was made. Our case suggests that bilateral disturbance of neuronal network between the primary auditory area and the secondary auditory area is responsible to the consequence of auditory agnosia.     

X-Linked recessive bulbospinal neuronopathy: Clinical phenotypes and cag repeat size in androgen receptor gene

Clinical phenotypes and the CAG repeat size of the androgen receptor gene were assessed in 95 Japanese patients with X-linked recessive bulbospinal neuronopathy. There was an age- and duration-dependent deterioration of muscle strength, ADL scores, and plasma creatine kinase levels. However, there was no correlation between the presence or absence of gynecomastia or diabetes mellitus and the age at onset or duration of illness. Correlations were present between the CAG repeat size and the age at onset (P < 0.0001) as well as the presence or absence of gynecomastea (P < 0.05). Muscular weakness and ADL scores were also correlated with the CAG repeat size only when they were adjusted by the age at examination not by the duration of illness. These findings suggest that CAG repeat size is one of the determinant factors of disease progression. However, extensive variation in phenotypic severity in patients with the same size of CAG repeat was present even among the siblings, suggesting that other factors than CAG repeat size influence the phenotypic manifestation. The average gain of CAG repeat size expansion was a 1.4 repeat in paternal transmission which was more unstable than that in maternal transmission, but the magnitude of the expansion in paternal transmission was much smaller than is presently known for other diseases in which CAG repeat expansion is the responsible gene defect. © 1995 John Wiley & Sons, Inc.     

An autopsy case of parotid clear cell carcinoma with cerebellar invasion and metastasis to the lung

An autopsy case of a clear cell carcinoma of the salivary gland with a cerebellum invasion and a metastasis to the lung is reported. This case has been classified as a monomorphic variant, although we were unable to demonstrate the presence of glycogens in the cytoplasm of the tumor cells. Ultrastructurally, most of the proliferating cells were immature cells with few organelles. The tumor cells were unable to be differentiated into ductal cells and myoepithelial cells. Recently, the tumor has been determined to be a low grade malignancy. Obviously, this tumor had a malignant character, with a cerebellum invasion and a metastasis to the lung.     

The effects of idazoxan combined with 30% nitrous oxide on the jaw-opening reflex in the rat.

In rats, the jaw-opening reflex is elicited by activation of a nociceptive receptor by the electric stimulation of the tooth pulp. This study was undertaken to assess the effects of 30% nitrous oxide and 30% nitrous oxide with idazoxan, an alpha 2-adrenergic antagonist, on this reflex. Each rat received electric stimulation for the jaw-opening reflex at 3, 5, 7, 10, 15, and 20 min after both the start of inhalation and the withdrawal of 100% oxygen or 30% nitrous oxide in oxygen. Idazoxan, 400 micrograms/ kg, was administered intravenously at the start of the inhalation period. Amplitudes significantly decreased during inhalation of nitrous oxide, but they returned gradually to control levels after cessation of nitrous oxide inhalation. In the cases of 100% oxygen, 100% oxygen with idazoxan, and 30% nitrous oxide in oxygen with idazoxan, amplitudes did not change from controls during and after 30% nitrous oxide inhalation. The latency remained unchanged irrespective of the treatment. Since in rats the degree of inhibition by 30% nitrous oxide in oxygen is partially diminished by administration of idazoxan, we conclude that nitrous oxide affects an alpha 2-adrenergic receptor in the central nervous system.     

Apolipoprotein AI levels in cord blood]

Lipid is known to increase during pregnancy but the factors responsible for the change have not been established. In addition, the lipid concentration in preeclamptic pregnancy is significantly higher than in normal pregnancy. The apolipoproteins are an important determinant of metabolism and the structure of plasma lipoproteins. The 26 healthy pregnant women, the levels of cord apolipoprotein AI were determined by TIA methods. The cord and plasma apolipoprotein AI were 76.12 +/- 20.04 mg/dl (n = 26, mean +/- S.D.) and 190.50 +/- 18.84 mg/dl, respectively. Cord apolipoprotein levels correlated to maternal age (r = -0.12, p less than 0.05), maternal weight (r = -0.11, p less than 0.01), the gestational week (r = +0.42, p less than 0.01), infant weight (r = -0.01, p less than 0.05), placental weight (r = -0.03, p less than 0.05), and diastotlic blood pressure (r = +0.06, p less than 0.05). These data suggest that the measurement of cord apolipoprotein AI may be a useful factor in evaluating preeclamptic pregnancy.     

Effect of dosing schedule on aminoglycoside ototoxicity: comparative cochlear ototoxicity of amikacin and isepamicin

The effect of the dosing schedule on aminoglycoside ototoxicity was investigated in the guinea pig. The animals were given amikacin or isepamicin either by once-daily intramuscular injection of 200 mg/kg or by twice-daily injections of 100 mg/kg for 28 days. The once-daily treatment induced a lesser degree of ototoxicity than the twice-daily injections, which may indicate that the once-daily treatment therapy has a potential value in the clinical use of aminoglycoside antibiotics. Isepamicin sulfate is a new aminoglycoside antibiotic currently undergoing clinical investigation. This study revealed that isepamicin also has ototoxic properties as has been observed in all other aminoglycosides. The degree of ototoxicity, however, was markedly less than that of amikacin.     

Effect of thiamylal on superoxide generation in the cells and on lipid peroxidation of biological membranes

The effect of thiamylal on the cellular superoxide generation and on membrane lipid peroxidation was examined. Active oxygens may play an important role in lung oxygen toxicity, since they attack the biological membranes and cause their lipid peroxidation. These active oxygens are generated in intra- and extra-cellular spaces in vivo. Under a certain condition, main part of intracellular superoxide generation is dependent on mitochondrial electron transport system. In this study, this superoxide generation was inhibited by the treatment with a low concentration of thiamylal concomitant with the uncoupling of mitochondrial oxidative phosphorylation. On the other hand, most part of extracellular superoxide is generated by the stimulation of phagocytotic cells such as neutrophils. This superoxide generation was also inhibited by the modification of cell membrane with thiamylal. Furthermore, the lipid peroxidation of mitochondria induced by Fe2+ was inhibited by the drug. Similar inhibitory action of the drug was observed on the superoxide dependent lipid peroxidation of Vitamin E deficient rat erythrocyte ghost or of phosphatidylinositol containing liposomal membranes. From these results, it was concluded that the thiamylal might act as a protective agent against the lung oxygen toxicity.     

Intraventricular craniopharyngioma: its characteristics in magnetic resonance imaging and successful total removal

We experienced a craniopharyngioma in the third ventricle which was removed totally by a staged operation through the lamina terminalis and then through the foramen of Monro using a transventricular approach. In magnetic resonance imaging, chiasmal thickening with pot-belly expansion and bilobed shape of the mass were found, and postoperative magnetic resonance imaging showed indentation of the chiasm and flattening of the floor of the third ventricle.