[Pulmonary hypertension associated with refractory hyperthyroidism: a case report].

A 25-year-old woman was admitted to our hospital with goiter. The diagnosis was Grave's disease. Diagnostic transthoracic echocardiography revealed a hyperdynamic stage of the heart with right ventricular dilation. Doppler echocardiography showed mild to moderate tricuspid regurgitation and elevated systolic right ventricular pressure. Right heart catheterization revealed high cardiac output (9.49 l/min) and pulmonary hypertension (57 mmHg) with increased pulmonary vascular resistance and total pulmonary resistance. No intracardiac shunts were detected. Since neither thiomazole nor propylthiouracil was effective and both caused side effects, she underwent subtotal thyroidectomy. After the surgery, pulmonary hypertension improved and cardiac output normalized, but without normalization of pulmonary vascular resistance and total pulmonary resistance. Reversible pulmonary hypertension may occur in patients with hyperthyroidism. Increased pulmonary blood flow and sustained high pulmonary artery resistance were suspected as the causes of pulmonary hypertension. In addition, pulmonary endothelial dysfunction as a result of sustained increased pulmonary blood flow could be another cause of pulmonary hypertension.

Synthesis and structure of 4-O,6-O-glycosylidene glycosides.

Interglycosidic spiro ortho esters (9-20) were efficiently prepared from methyl 2,6-di-O-benzylgluco- or galactopyranoside and various sugar lactones in the presence of methoxytrimethylsilane and a catalytic amount of trimethylsilyl triflate. All of the prepared sugar ortho esters possess perhydrospiro[2H-pyran-2,2'-pyrano[3,2-d][1,3]dioxin] ring systems commonly in their molecules and, remarkably, were afforded as single isomers. The configurations of the spiro centers in their molecules were determined or estimated by X-ray single crystallographic analysis and molecular modeling studies. By comparing the conformations of prepared ortho esters, we revealed that the difference in the stability between two possible isomers was principally caused from that between the spiro ring systems in their molecules in each case.

A highly stereoselective construction of beta-glycosyl linkages by reductive cleavage of cyclic sugar ortho esters.

The preparation of beta-glycosides by the reductive cleavage of spiro sugar ortho esters is described in this report. This procedure is based on a concept completely different from those of other methods for glycosylation. Twelve sugar ortho esters that commonly possess perhydrospiro[2H-pyran-2,2'-pyrano[3,2-d][1,3]dioxin] ring systems in their molecules were reduced by LiAlH(4)/AlCl(3) or NaCNBH(3)/AlCl(3). Among these ortho esters, those (9a-12a) prepared from the D-sugar lactones (1-4) and 2, 3-di-O-benzyl-alpha-D-glucopyranoside (7) or those (19a, 20a) prepared from the L-sugar lactones (5, 6) and 2, 3-di-O-benzyl-alpha-D-galactopyranoside (8) were selectively converted into beta-(1 --> 4)-glycosides (9b-12b or 19b, 20b) in excellent yields by the treatment of LiAlH(4)/AlCl(3). In contrast, the ortho esters (13a-16a or 17a, 18a) that were prepared from combinations of the D-sugar lactones and 8 or those of the L-sugar lactones and 7 were efficiently reduced with NaBH(3)CN/AlCl(3) to afford beta-(1 --> 6)-glycosides (13b-16b or 17b, 18b) selectively. It was remarkable that the resulting disaccharides were obtained with extremely high beta-selectivity even in the cases with mannosyl or rhamnosyl glycosides. Moreover, these products would be useful units for the construction of branched saccharides, because the newly formed hydroxy groups could be again glycosylated without further deprotection procedures. The high regio- and stereoselectivity was totally explained by considering the structures and the conformations of these ortho ester molecules and the stereoelectoronic effects of their spiro ring systems. In addition, the preparation of the sugar ortho esters with glucosamine derivatives and the reactivity of these ortho esters are described in this report. N-Phthaloyl glucosamine derivatives (21, 22) were efficiently reacted with the benzyl-protected gluconolactone (1) in the presence of TMSOMe and TMSOTf to afford ortho esters (23a-c). After the conversion of the phthalimido functionality to the dibenzyl amino group, glucosylideneglucosamine (25) was reduced with LiAlH(4)/AlCl(3) to afford beta-(1 --> 4)-glycoside (26) selectively.

Clinical evaluation of 99mTc-Technegas SPECT in thoracoscopic lung volume reduction surgery in patients with pulmonary emphysema.

UNLABELLED: 99mTc-Technegas (Tcgas) SPECT is useful for evaluating the patency of the airway and highly sensitive in detecting regional pulmonary function in pulmonary emphysema. The aim of this study is to evaluate regional ventilation impairment by this method pre and post thoracoscopic lung volume reduction surgery (LVRS) in patients with pulmonary emphysema. METHODS: There were 11 patients with pulmonary emphysema. The mean age of patients was 64.1 years. All patients were males. LVRS was performed bilaterally in 8 patients and unilaterally in 3 patients. Post inhalation of Tcgas in the sitting position, the subjects were placed in the supine position and SPECT was performed. Distribution of Tcgas on axial images was classified into 4 types, A: homogeneous, B: inhomogeneous, C: hot spot, D: defect. Three slices of axial SPECT images, the upper, middle and lower fields were selected, and changes in deposition patterns post LVRS were scored (Tcgas score). RESULTS: Post LVRS, dyspnea on exertion and pulmonary function tests were improved. Pre LVRS, inhomogeneous distribution, hot spots and defects were observed in all patients. Post LVRS, improvement in distribution was obtained not only in the surgical field and other fields, but also in the contralateral lung of unilaterally operated patients. In 5 patients some fields showed deterioration. The Tcgas score correlated with improvements in FEV1.0, FEV1.0% and %FEV1.0. CONCLUSION: Tcgas SPECT is useful for evaluating changes in regional pulmonary function post LVRS.

[Mental deterioration in childhood epilepsy. II. Investigation of intelligence of right-handed epileptic children with right-hemispheric dominance of language].

To clarify the pathogenesis of mental deterioration in epilepsy, we investigated the intelligence of right-handed epileptic children with right hemispheric dominance of language. On dichotic listening test, all 13 patients with right-handedness, 7 to 20 years of age, showed a left ear dominance suggesting right hemispheric dominance of language. Among 13 patients, 9 showed mental retardation, and 6 patients of them showed mental deterioration during the clinical course of epilepsy. Eight patients showed findings suggesting brain damage in the left hemisphere either in clinical feature, EEG or CT. A relationship was suspected between the right-hemispheric dominance of language and the epileptic focus in the left hemisphere. The usefulness of dichotic listening test was suggested both in disclosure of the brain damage and the investigation of mental retardation in childhood epilepsy.

[Two cases whose epileptic attacks disappeared at the time of infection].

Two cases with secondary generalized epilepsy whose epileptic attacks disappeared abruptly were reported. Permanent improvement was observed in case 1 with residual state of West syndrome, aged 4 years, after viral pneumonia, both epileptic attacks and epileptic discharges on EEG disappeared more than 3 years. Transient improvement was observed in case 2 with Lennox-Gastaut syndrome, aged 6 years, after an episode with insomnia, poor appetite and confusion for three days. After this episode epileptic attacks disappeared for 9 months and epileptic discharges on EEG were also markedly decreased. However, epileptic attacks relapsed following the reduction of phenytoin after 9 months. An effect resembling ketogenic diet therapy and a change of pharmacokinetics of antiepileptic drugs may be involved in the transient improvement of case 2. However, any effects involving an immunological effect and secretions of adreno-cortico-hormone following after stress may be not involved in the permanent improvement of case 1.

[A study on functional plasticity of the brain in childhood. II. Speech development and intelligence after the damage of cerebral hemisphere under 1 year of age].

To investigate the functional plasticity of the brain in childhood, the speech development, the intelligence test and dichotic listening test were performed on 27 patients who had suffered from hemiplegia under 1 year of age. Among 13 patients with right hemiplegia, 7 to 24 years old, 11 patients showed a left ear dominance suggesting the lateralization of language in the right hemisphere. All 14 patients with left hemiplegia, 5 to 37 years old, showed a right ear dominance suggesting the lateralization of language in the left hemisphere. All 27 patients acquired speech function enough to converse with other people during daily life. There were no differences in speech development or intelligence scores between both groups of hemiplegia. Although there was no correlation between the speech development and the age of onset of hemiplegia, there was a correlation between the speech development and the intelligence score in both groups of hemiplegia.

[A study of functional plasticity of the brain in childhood. I. Critical period dislodging lateralization of language in the brain].

To investigate the functional plasticity of the brain in childhood, the intelligence and language tests, brain CT and dichotic listening test were performed on 34 patients with hemiplegia. All 13 patients with left hemiplegia, 8 to 37 years old, showed a right ear dominance suggesting the lateralization of language in the left hemisphere. Among 21 patients with right hemiplegia, 7 to 22 years old, 16 patients who had suffered from the left hemispheric damage before 6 years and 1 month of age, showed the left ear dominance suggesting the lateralization of language in right hemisphere except 2 patients. On the contrary, 3 patients with right hemiplegia who had suffered from the left hemispheric damage after 6 years and 6 months of age showed a right ear dominance. It was concluded that the critical period for the dislodging of language lateralization in the brain from the damaged left hemisphere to the intact right hemisphere is the first half of 6 years after birth. Five patients out of 8 patients with right hemiplegia showed significantly lower scores in performance than in verbal WISC IQ. The compensation of language function rather than performance function was considered to be characteristic for the left-hemispheric damage in childhood.

West syndrome associated with hyperlexia.

Two patients are reported with West syndrome associated with hyperlexia. They had peculiar linguistic and psychosocial development and autistic behavior, yet began to read Japanese and Chinese characters, numbers, Roman alphabet letters, and trademark letters at 3 years of age. Neuropsychologic, electroencephalographic, and radiologic studies were performed at 11 years of age in Patient 1 and at 10 years of age in Patient 2. Although they were able to read fluently with correct intonation, their comprehension was significantly impaired. The neuropsychologic evaluation of both patients demonstrated selective, excellent ability in auditory memory in contrast to impairment of associational abilities, visual retention, and visual constructional abilities. These findings suggest that the functions in the nondominant hemisphere were also impaired. Although children with West syndrome frequently have associated mental retardation, patients with West syndrome associated with hyperlexia have never been reported. A neuropsychologic investigation of epileptic children with mental retardation is necessary for the detection of hyperlexia.

[Mental deterioration in childhood epilepsy. I. Neuropsychological and electroencephalographic studies].

To clarify the pathogenesis of mental deterioration in epilepsy, neuropsychological and electroencephalographic studies were performed. Thirty-seven children were classified into two groups: group I consisted of 24 children with mental deterioration during the clinical course of their epilepsy, and group II consisted of 13 children without mental deterioration. Mental deterioration was assessed on the basis of the intelligence test scores during follow-up studies. The mean intelligence quotient of 24 children in group I decreased from 91.8 +/- 11.5 to 64.3 +/- 15.4 during a mean period of 4.4 years. Significant differences were found between the two groups in the levels of both representation (association function) and automation (auditory closure, visual recognition and visual-motor function). There were also significant differences between the two groups in the continuation of epileptic discharges on EEG, in the persistence of epileptic attacks at the final visit, and in the numbers of antiepileptic drugs at the neuropsychological examination. The degree of mental deterioration was correlated with the defect in the association function and, eleven children in group I showed mental deterioration even after their attacks had been suppressed.

[Visual perception in normal children and adults I. Developmental changes in dominance of visual field in normal children].

Developmental changes of dominance of visual field was evaluated using a tachistoscope in 70 normal subjects from 4 years of age up to adulthood (35 males and 35 females). The time of exposure was measured under two experimental conditions; simultaneous stimulation of different types to both left and right fields, or a single stimulus only to one visual field. One of the following stimuli was exposed to the visual field; one, two or three letters of Japanese characters ("hirakana"), one of Chinese characters ("kanji"), figures, and Roman alphabets. Simultaneous bilateral stimulation revealed frequent dominance in the right visual field, which was established by 4 years of age. The average time of exposure become shorter with age up to 6-7 years for one "hirakana" letter and one "kanji", and up to 10 years for two and three "hirakana" letters. All four left-handed subjects in this study showed dominance of the right visual field. Dominance of the left visual field was observed in 3 of 52 subjects with dominance of the right eye (5.8%), and in 5 of 15 with dominance of the left eye (33.3%). This difference was statistically significant (chi 2 = 8.41, p less than 0.01). It was suggested that dominance of the visual field was related with dominance of the eye, and not with handedness.

[Visual perception in normal children and adults II. Correlation between dominance of visual field and dominance of the eye].

To clarify more precisely about the correlation between the dominance of visual field and dominance of the eye, tachistoscopic examination was performed on 48 normal adults; 24 males and 24 females, and 24 right-handed and 24 left-handed subjects. The mean of the time of exposure was less than 4.5 msec. The time of exposure was independent of handedness, dominance of the eye, sex, or the type of stimulus. Four right-handed and four left-handed subjects showed dominance of the left visual field for the A value, suggesting no correlation between handedness and dominance of the visual field. However, six of those eight subjects were found to have dominance of the left eye for the A value, and all seven subjects with dominance of the left visual field for the D value showed dominance of the left eye (chi 2 = 16.39, p less than 0.001). It was concluded that dominance of the eye was significantly correlated with visual field. It is therefore important to examine dominance of the eye as well as dominance of the visual field for evaluation of cerebral dominance with regard to visual perception.

Mumps-induced opsoclonus-myoclonus and ataxia.

A 9-year-old boy manifested acute cerebellar ataxia associated with mumps infection. He developed opsoclonus, myoclonus, tremor, and truncal ataxia 7 days after mumps infection. Lumbar puncture revealed pleocytosis without elevation of protein; ELISA demonstrated an increased IgM titer of cerebrospinal fluid against mumps virus. From these results it was determined that acute cerebellar ataxia was induced by a direct invasion of mumps virus. Electroencephalography demonstrated normal background activity, although alpha-like activity appeared bilaterally in the frontal regions which was induced by eye closure and decreased by eye opening. Polygraphic electroencephalography revealed that the alpha-like activity corresponded to the ocular movement recorded above or lateral to the eyelids by electro-oculography; therefore, the alpha-like activity was considered to be derived from the opsoclonus which was secondary to cerebellar involvement. His neurologic symptoms improved gradually and resolved completely within 3 months after the onset of acute cerebellar ataxia.