[Moyamoya-like vascular changes 6 years after radiation therapy in a boy].
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Brain Neoplasms↗
Biomedical subjects
T Koeda
Publications and source records attributed to T Koeda.
Multiple epiphyseal dysplasia with small head, congenital nystagmus, hypoplasia of corpus callosum, and leukonychia totalis: a variant of Lowry-Wood syndrome?
We report on a boy with multiple epiphyseal dysplasia (MED), mild short stature, small head, mental retardation and congenital nystagmus associated with other visual problems. These manifestations were similar to those seen in Lowry-Wood syndrome (LWS). He also had hypoplasia of the corpus callosum and leukonychia totalis, which were not described in the previous cases.
Abnormalities, Multiple↗
The EEG in acallosal children. Coherence values in the resting state: left hemisphere compensatory mechanism?
Resting EEG interhemispheric and intrahemispheric coherences (ICoh and HCoh) in the theta, alpha and beta bands were studied in 7 patients with agenesis of the corpus callosum (5 children, aged 10-14 years, and 2 adults) and 2 groups of sex- and age-matched normal children and adults (42 subjects). In patients the ICohs (F3/F4, C3/C4, P3/P4, O1/O2) were lower than in the normal sample. The ICoh decrease, corresponding with the completeness of commissural agenesis, showed the essential role of the corpus callosum in interhemispheric EEG synchronization. A remarkable new fact was found, namely lower right hemisphere HCoh in the acallosal patients in comparison to the normals, suggesting reduced connectivity of the right hemisphere. It is assumed that the deviant HCoh patterns in the patients, most pronounced in the beta band, are indicative of compensatory left hemisphere mechanisms, accounting for a specific brain plasticity phenomenon in acallosal subjects.
Adolescent↗
Effects of mazindol in two patients with Prader-Willi syndrome.
Two patients with Prader-Willi syndrome, including gross obesity with food-related behavior and mild mental retardation, are presented. One patient was an 11-year-old girl with the diagnosis of development delay, hypoactivity, and waxy skin with normal female karyotype. The other patient was a 15-year-old girl with the diagnosis of abnormal chromosome 15. Obesity had been present since early childhood, and it was difficult for them to manage their weight control by means of diet and exercise therapy. With 24-week mazindol administration, they demonstrated marked improvement in weight control during the early period and improvement in pathologic behavior without side effects. Mazindol was given orally, 1.0-2.0 mg/day, in one or two daily doses. Mazindol may prove to be useful in the treatment of patients with Prader-Willi syndrome.
Adolescent↗
Protoporphyric disorder in livers of broiler chickens.
Two cases of protoporphyrin (PP) disorder detected in a 60- and 65-day old female chicken were described. The gross lesions were restricted to the livers which were enlarged and dark green to black in color. Histologically, dark brown granules were found in hepatocytes, Kupffer cells, macrophages, sinusoids, bile canaliculi, and bile ducts. These granules, as seen in smears and sections of livers, were red under a fluorescence microscope and exhibited bright birefringence with a centrally located dark Maltese-cross by polarized light. Ultrastructurally, these granules consisted of aggregates of needle-like crystals in a radial arrangement. Fluorometrically, extracted level of PP in the affected liver was determined to be 390.6 micrograms per gram of wet tissue. Spectrofluorometric scans of liver extract and PP standard were almost identical.
Animals↗
[Two cases of post-encephalitic epilepsy characterized by auditory cognitive dysfunction; comparison with "a peculiar type of post-encephalitic/encephalopathic epilepsy"].
Two cases of post-encephalitic epilepsy mainly characterized by auditory cognitive dysfunction were reported. In acute phase they only showed slight pleocytosis of CSF, and serum antiviral antibodies were all negative. Although their seizures were partial seizures with secondary generalization, their EEG and radiographic imaging did not show any lesions. Their waking state EEG continuously showed slowing with decrease of alpha activities. After clusters of convulsions, they showed delirium and aggressiveness. Both of them were thought to have post-encephalitic epilepsy with pathogen unknown and they were compatible with "a peculiar type of post-encephalitic/encephalopathic epilepsy" reported by Fukuyama and Awaya. The presented two cases were characterized by auditory cognitive dysfunction and intractable epilepsy with secondary generalization.
Acute Disease↗
[Clinical studies of learning disability. Part I: Two-axial diagnosis of learning disability using the Pupil Rating Scale Revised (PRS) and WISC-R].
We investigated the correlation between intelligence quotient and school performance evaluated by PRS (the Pupil Rating Scale Revised) to assess an adequacy of two-axial diagnosis of learning disabilities (LD). The subjects were 37 children, including 31 cases with developmental or school problems and 6 normal children. The Japanese Wechsler Intelligence Scale for Children-Revised (WISC-R) was employed for an estimation of intelligence quotient (IQ) and, PRS was used to evaluate school performance. We defined 'LD risk child' as a child whose IQ was no less than 70 and PRS score was no more than 65. A statistical evaluation of IQ and PRS was done in this group separately. Ten children were diagnosed as 'LD risk' using this criterion. Fourteen children had normal IQ, and thirteen children were mentally retarded. There was a significant correlation (r = 0.68, P < 0.001) between IQ and the PRS score in these 37 children. On the other hand, no significant correlation was found between IQ and the PRS score of the 'LD risk child'. This result suggests that we can expect high PRS score in proportion to their IQ level. Because LD children generally tend to demonstrate deviated IQ-school performance correlation, we conclude that the WISC-R and PRS are appropriate for two-axial screening of LD.
Adolescent↗
[Clinical studies of learning disability. Part II: A prospective study of children with risk factors for learning disability at three year-old screening].
We followed children with the risk factors for learning disability (LD) at the three year-old screening prospectively. The five risk factors were speech delay, hyperkinesia, delayed social skill, delayed comparative conception (big and small, long and short) and mutistic behaviour. We evaluated seventeen elementary school children using WISC-R and the Pupil Rating Scale Revised. Six of them were diagnosed as normal, six were learning-disabled, and five were mentally retarded children. We proposed that the screening of LD at three years by the risk factors were effective but only partially.
Child↗
Cerebral oxygenation in childhood moyamoya disease investigated with near-infrared spectrophotometry.
In children with moyamoya disease, the time courses of hemoglobin concentrations before and after hyperventilation were examined using near-infrared spectrophotometry (NIRS) to determine brain tissue oxygenation. Five patients were studied: 1 was studied both before and after surgery and 4 were studied only after surgery. Nine hemispheres of these 5 patients were examined. The oxygenated hemoglobin concentration increased immediately after beginning hyperventilation. This parameter later decreased sharply to below the prehyperventilation level and remained at the reduced level for a prolonged period. These changes in oxygenated hemoglobin concentration were similar to those previously reported in arterial blood oxygen and CO2 tension. The magnitude of variance in oxygenated hemoglobin concentration (C-HV) was significantly greater in moyamoya disease patients than in healthy controls (P < .003, Wilcoxon test). The changes of NIRS after operation were markedly more sensitive than those of electroencephalography or single-photon emission computed tomography. NIRS could be applied to cerebral oxygenation as well as cerebral hemodynamics, which may be used to better define the time sequence and pathologic processes of various childhood encephalopathies.
Adolescent↗
Handedness and spastic diplegia from premature birth.
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Adolescent↗
[Hypoxic brain damage and higher cortical dysfunction in children].
1) Twenty-one children with right hemiplegia and 17 with left hemiplegia were studied as to emotional differences using the Toddler Temperament Test. Right hemiplegic children were less adaptable than controls or left hemiplegic, while left hemiplegic children were found to be more approachable than controls or right hemiplegic ones. These emotional features were similar to those of adult hemiplegics. We speculate that these characteristics emotions are associated with the damage to each hemisphere. 2) Eighteen patients with spastic diplegia (SD) after preterm birth were studied to clarify the relationship between visuo-perceptual impairment (VPI) and their cerebral lesions. The VPI was significantly correlated with the volume of peritrigonal white matter. In another psychological study, SD with preterm birth also showed impairment of stereoscopic vision. We suggest that the VPI might be caused by the impairment of stereoscopic vision.
Brain Diseases↗
Intracranial calcification in siblings with nephrogenic diabetes insipidus: CT and MRI.
Computed tomography and magnetic resonance imaging (MRI) were used to examine three male siblings with nephrogenic diabetes insipidus (NDI). The two elder brothers had varying degrees of unusual intracranial calcification; the eldest also showed involvement of the cerebral white matter on MRI. The severity of intracranial calcification was related to the time before initiation of treatment and inversely to mental ability. Brain damage and mental retardation in NDI may be caused by a delay in initiating treatment; early detection and treatment are important to prevent brain damage.
Adolescent↗
Tactile naming disorder of the left hand in two cases with corpus callosum agenesis.
The authors report two girls with corpus callosum agenesis, with unilateral tactile naming disorder in the left hand. Neuropsychological examinations were performed and tactile perception and naming were studied. The results suggested that tactile naming disorder in both cases could be regarded as a disconnection deficit. Both had daily epileptic attacks and mild or moderate mental deficiency. In these severely impaired children, brain capacity and neural plasticity seem inferior, so classical disconnection deficits might be demonstrated.
Adolescent↗
Progressive encephalopathy associated with cytomegalovirus infection without immune deficiency.
Slowly progressive encephalopathy caused by cytomegalovirus is an unusual disorder, and its pathogenesis remains unknown except for cases associated with the acquired immune deficiency syndrome and organ transplantation. We report a case who showed clinical features of progressive encephalopathy. Cytomegalovirus was repeatedly isolated from urine, and cytomegalovirus-infected cells were detected in bone marrow. Serial computed tomographic head scan revealed periventricular calcification and its progression to the thalamus, cerebellum, and brain stem. On autopsy, there were multiple calcifications and diffuse glial proliferation in the gray and white matter. Perivascular inflammation was only minimal. There was no evidence of immune deficiency. This case suggests that progressive encephalopathy can be caused by cytomegalovirus infection without immune deficiency. This type of cytomegalovirus infection may be unusual, but its serious outcome should remind us to detect it accurately.
Brain↗
[A case of tyrosinemia type II with convulsion and EEG abnormality].
A 10-year-old boy with palmoplantar hyperkeratosis and keratitis was reported. His physical development was normal and mental development was lower limit. He had also convulsions with low grade fever several times, and his EEG showed paroxysmal discharges. The plasma levels of phenylalanine and tyrosine were 5 to 10 times higher than those of controls. Tyrosinemia II was diagnosed on the low level of cytosol tyrosine aminotransferase in biopsied liver. The cases of tyrosinemia II were reviewed on the symptoms of the central nervous system. Two of twelve cases had convulsions. Adult cases demonstrated nystagmus, tremor, ataxia, and convulsion. Hyperkeratosis and corneal lesions were characteristic in symptoms of tyrosinemia II, but attention should be paid to the symptoms of the central nervous system.
Amino Acid Metabolism, Inborn Errors↗
[A case of asymptomatic myocardial infarction with multiple coronary aneurysms].
A 36-year-old man was hospitalized in December, 1990 because a small R wave was observed on his ECG in V1-3. The patient had suffered from a cold with fever for a month when he was a child. He had no symptoms or signs of myocardial infarction. The patient underwent selective right and left coronary angiography, which revealed specific coronary aneurysms in the left circumflex coronary artery and left anterior descending coronary artery. The aneurysm in the distal lesion of the left circumflex coronary artery was embolized by a thrombus and filled with many capillary arteries. The proximal lesion of the right coronary artery was slightly dilated. Good collateral flow from the right coronary artery to the left coronary artery was revealed by coronary angiography. Left ventriculograms were performed, and the anterior wall motion of the left ventricle was shown to the hypokinetic. A perfusion defect of the anterior wall of the left ventricle was revealed by Thallium-201 imaging with single photoemission computed tomography. Based on these findings, we diagnosed the patients' illness as old myocardial infarction caused by Kawasaki disease.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
[Two cases of theophylline-associated encephalopathy in childhood: clinical and CT findings].
Two asthmatic children who developed status epilepticus during theophylline treatment followed by semi-coma were reported. They suffered from severe neurological sequelae. A two-year-old male received oral maintenance theophylline therapy and a four-year-old male received intravenous theophylline therapy at the time of seizures. Theophylline blood levels measured several hours after the onset of seizures were within or below the therapeutic level; 12.7 and 8.8 micrograms/ml. They had febrile convulsions prior to the onset of episode and one of them was mildly delayed in psychomotor development. Brain CT scans showed diffuse cortical low-density in the acute period. Follow up CT scans revealed progressive cortical low-density in the subacute period and subsequently reached to the peak in the 10th day and 19th day of illness respectively. We consider that the progressive and long-lasting severe cortical edema on brain CT scan is characteristic of theophylline-associated encephalopathy.
Asthma↗
Relationship between plasma atrial and brain natriuretic peptide concentration and hemodynamic parameters during percutaneous transvenous mitral valvulotomy in patients with mitral stenosis.
Brain natriuretic peptide (BNP), a family of peptides with structural and biologic homologies to previously identified atrial natriuretic peptide (ANP), has been found in human cardiac tissue and plasma. To examine the secretion mechanism of these peptides, we have studied the relationship between their plasma concentrations and hemodynamic parameters before and at 0.5 and 24 hours after percutaneous transvenous mitral commissurotomy (PTMC) in 14 patients with mitral stenosis. We have also investigated the validity of measuring plasma natriuretic peptides as a means for estimating changes in hemodynamic parameters after PTMC. The procedure decreased left atrial pressure (p < 0.01) with an elevation in left ventricular end-diastolic pressure (p < 0.05). Plasma ANP levels decreased significantly after PTMC (before, 64.1 +/- 33.7 fmol/ml; at 0.5 hour, 58.9 +/- 27.7 fmol/ml; at 24 hours, 45.7 +/- 18.3 fmol/ml; p < 0.01), whereas plasma BNP levels remained unchanged after the procedure (before, 5.3 +/- 1.5 fmol/ml; at 0.5 hour, 5.6 +/- 1.9 fmol/ml; at 24 hours, 5.0 +/- 1.9 fmol/ml; p = NS). There was a significant relationship between basal plasma ANP and left atrial pressure (r = 0.88; p < 0.001), and changes in plasma ANP were correlated with those in left atrial pressure (r = 0.69; p < 0.01). Basal plasma BNP was significantly correlated with basal left ventricular end-diastolic pressure (r = 0.65; p < 0.05) but not with the other measured hemodynamic parameters or with plasma volume.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗