Search PubMed⌕ Search

Biomedical subjects

A Ieshima

Publications and source records attributed to A Ieshima.

At least 37 records · Page 2Linked to original sources

Neuroleptic malignant syndrome in striatonigral degeneration.

A 6-year-old boy with striatonigral degeneration had an episode of neuroleptic malignant syndrome after an upper respiratory infection. Dantrolene treatment was successful. Some reports have demonstrated that anesthetic and antipsychotic agents lead to neuroleptic malignant syndrome in disorders of the basal ganglia. However, neuroleptic malignant syndrome attributable to a respiratory infection has not previously been reported. Our patient illustrates the potential morbidity of neuroleptic malignant syndrome in patients with striatonigral degeneration.

Child↗

A case of distal 9q trisomy syndrome associated with an unusual inheritance of ABO blood type.

A male infant with distal 9q trisomy syndrome associated with an unusual inheritance of ABO blood type is reported. His clinical features were concordant with those of distal 9q trisomy syndrome. His karyotype was 46,XY, -10, +der (10) t(9;10) (q22.3;q24.3) confirmed by G-banding and high resolution methods. His father had the balanced translocation t(9;10) (q22.3;924.3). He had a blood type of AB, despite his father's blood type of AB and his mother's blood type of O. The gene of ABO blood type is located at 9q34.1-q34.2. Therefore, he would have received A and B type alleles from his father. 9q trisomy syndrome should be carefully investigated with ABO blood type.

ABO Blood-Group System↗

Juvenile variant of Schimke immunoosseous dysplasia.

We report on a 16-year-old girl with spondyloepiphyseal dysplasia, nephrotic syndrome, lymphopenia, and signs of defective cellular immunity. The manifestations are very similar to those reported by Spranger et al. [1991: J. Pediatr 119: 64-72] as Schimke immunoosseous dysplasia, except for age of onset. In Schimke immunoosseous dysplasia, growth retardations as an initial symptom is noted in early childhood and about 1 year after onset of progressive proteinuria. In our case the skeletal abnormality was noted at age 10 years as dislocation of the hip joints and the diagnosis of nephrotic syndrome was made at age 16 years. The findings strongly suggest that our patient has a juvenile variant of Schimke immunoosseous dysplasia.

Adolescent↗

[CT findings and prognoses of anoxic brain damage due to near-drowning in children].

We investigated the relationship between serial cranial CT findings and prognoses in 11 children after near-drowning. These patients were rescued after heart arrest for more than 10 minutes and all comatose on admission. CT scans were performed within 2 weeks, at 3 weeks-1 month, 2-4 months and more than 5 months after admission. Characteristics of CT findings and prognoses were classified into four groups. Group 1: low density areas in thalami, basal ganglia and cortical white matters within 2 weeks (three cases; one died, two became vegetative). Group 2: enlargement of the third ventricle at 3 weeks-1 month, and atrophy of pons at 2-4 months (three cases; severe quadriplegia and mental retardation). Group 3: enlargement of the third ventricle at 3 weeks-1 month, but atrophy of pons not observed at 2-4 months (three cases; mild motor disabilities and mild mental retardation). Group 4: enlargement of third ventricle not observed at 3 weeks-1 month (two cases; neither paralysis nor mental retardation).

Child↗

Dendrites, dementia and the Down syndrome.

Findings from a Golgi study of the visual cortex in patients with the Down syndrome were compared with those from neurologically normal, age-matched control subjects. The dendritic atrophy seen in childhood continued into adulthood, with a marked decrease in dendritic branching, dendritic length, and spine frequency in elderly adults with the Down syndrome. Subject more than 30 years old occasionally had degenerating pyramidal neurons in the cerebral cortex and degenerated pyramidal neurons and aspiny stellate cells, particularly in the temporal cortex. These dendritic abnormalities may be related to mental retardation in children and early dementia in older adults who have the Down syndrome. The genetic and extrinsic factors may be important determinants of Alzheimer type dementia in the Down syndrome.

Adult↗

Comparison of brain imaging and neuropathology in cases of trisomy 18 and 13.

A comparative study of intracranial imaging and brain pathology in cases of trisomy 18 and 13 was performed. Computed tomography (CT) and ultrasonography (US) revealed disproportional dilatation of the lateral ventricles, a wide Sylvian fissure and a large extracerebellar space with a small cerebellum in each case. In addition, it was characteristic that the occipital poles of the cerebrum protruded in the infero-posterior direction in trisomy 18, and the pontine basis was relatively wide in trisomy 13. The brain pathology in trisomy 18 and 13 demonstrated that the large extracerebellar space is due to the cerebellar dysplasia and protruding occipital poles, the wide Sylvian fissures due to the temporal lobes or external capsular dysplasia, and the relatively wide pontine basis due to meningeal glioneuronal heterotopia. Thus, the characteristic intracranial image in trisomy 18 and 13 suggest microdysgenesis of the brain and might be useful for understanding the pathological structure of the central nervous system in these conditions.

Abnormalities, Multiple↗

Focal vulnerability of developing brain: CT studies on disproportionally dilated lateral ventricles.

Cranial computed tomography (CT) of 108 cases with dilated lateral ventricles was reviewed to elucidate the relationship between focal vulnerability of developing brain and disproportional dilatation of lateral ventricles. CT findings of 108 cases with symmetrical dilatation of lateral ventricles were classified into three types by morphometry of lateral ventricles: anterior horn predominant type (31 cases), diffuse type (36 cases), posterior horn predominant type (41 cases). Posterior horn predominant type has a tendency to occur in congenital anomalies and premature brain damage, and anterior horn predominant type in infantile brain damage. This disproportional dilatation of anterior or posterior horns suggests a vulnerability of periventricular structure in developing brain.

Adolescent↗

Neuropathological and Golgi study on a case of thanatophotoric dysplasia.

Neuropathological examination of a 3-day-old female with thanatophoric dysplasia revealed abnormal sulci with polymicrogyria and neuronal heterotopia in the temporal lobes and cerebellar hypoplasia. Rapid Golgi stainings showed immature or maldeveloped neurons with short dendrites and a reduced number of spines in the cerebral cortex, and abnormal neurons with peculiar dendrites in the subcortical heterotopia. These abnormalities were predominant at the base of the large brain which occupied the hypoplastic middle and posterior cranial fossas.

Brain↗

A morphometric CT study of Down's syndrome showing small posterior fossa and calcification of basal ganglia.

We report characteristic and morphometric changes of cranial computed tomography (CT) with increasing age in 56 patients with Down's syndrome aged from 0 month to 37 years. Patients were compared with 142 normal controls aged 0 to 59 years. Width of ventricles, Sylvian fissures, posterior fossa, pons and cisterna magna were measured on CT. The incidences of the cavum septi pellucidi, cavum vergae and cavum veli interpositi and high density in the basal ganglia were examined. There was high incidence (10.7%) of bilateral calcification of basal ganglia in Down's syndrome, although that of pineal body and choroid plexus calcification was similar in Down's syndrome and controls. Basal ganglia calcification is more frequently seen in young Down's syndrome and may be related to the premature aging characteristic of Down's syndrome. The CT in Down's syndrome showed relatively small posterior fossa, small cerebellum, small brain stem and relatively large Sylvian fissures in those under one year of age. There was a high frequency of midline cava and large cisterna magna. There were no significant atrophic changes on CT except after the fifth decade comparing with controls.

Adolescent↗