Kazuhiko Cho

Muscle histopathology in spastic cerebral palsy

We studied the histopathology of spastic muscles in patients with spastic cerebral palsy using specimens obtained from the gastrocnemius muscles during orthopedic operations. Though there was no disease-specific abnormality, we found changes in fiber type distribution, i.e., type-1 fiber predominance and type-2B fiber deficiency. These changes were not found in the patient with brain infarction at age 1 year 5 months. Variation in fiber size, especially in type-1 fibers, was also detected in older patients and at the more severely affected sides. We speculated that the influence on the spinal motor unit from upper motor cortex might be applicable to the muscle histological findings and the time of the onset of the brain damage might be important.

ASSESSMENT OF VISUOPERCEPTUAL DISTURBANCE IN CHILDREN WITH SPASTIC DIPLEGIA USING MEASUREMENTS OF THE LATERAL VENTRICLES ON CEREBRAL MRI

The authors estimated perceptual disturbance in children with spastic diplegia from the difference between the visual and performance IQ scores (VIQ‐PIQ) on the Wechsler Intelligence Scale for Children‐Revised (WISC‐R), having found a strong negative correlation between this score and the PQ obtained on the Frostig Developmental Test of Visual Perception (DTVP). The ratio of the areas of the posterior horns to the anterior horns (P/A) correlated negatively with visuoperceptual disturbance. This ratio can therefore be used to assess perceptual disturbance at an early age in children with spastic diplegia.

Neuroradiological assessment of visuoperceptual disturbance in children with spina bifida and hydrocephalus

The intellectual status of twelve children with spina bifida and hydrocephalus was evaluated. Seven children were considered to have verbal and non‐verbal skill discrepancies. It was considered that the Verbal IQ‐Performance IQ score by WISC‐R could be used as an index of their visuoperceptual disturbance. The disturbance was closely related to the morphological characteristics of the lateral ventricles on cerebral MRI. The ratio of the areas of the posterior horns to the anterior horns (P:A) showed a negative correlation with visuoperceptual ability. The visual pathway, visual cortex, and the ventral system were thus considered to be the sites of the affected lesions. From P.A changes with time, it was evident that adequate shunting would prevent the perceptual disturbance.

Transfer of cefazolin into human milk

phate load will be rapidly excreted by the kidney in the presence of normal glomerular and tubular function, but only in conjunction with a cation to preserve electroneutrality. This is a possible explanation for the large concentration of cations (sodium and potassium) noted in the random urine specimen obtained at the time of the initial admission. In the presence of extracellular volume contraction and increased aldosterone production, a significant proportion of this cation loss could be potassium. The use of enemas, especially hypertonic phosphate enemas, has become routine in pediatrics in preparation for roentgen studies and in the treatment of chronic constipation. Although enemas given to normal individuals usually do not cause electrolyte imbalance, children with abnormal colons or impaired renal function are especially prone to fluid and electrolyte disturbances following the administration of enemas. If the chronic use of enemas cannot be avoided, such patients should be monitored with periodic determinations of serum electrolyte, phosphorus, and calcium concentrations.

Hirschsprung disease, unusual face, mental retardation, epilepsy, and congenital heart disease: Goldberg-Shprintzen syndrome

A 5-year-old girl with Hirschsprung disease, unusual facial appearance, psychomotor retardation, epilepsy, and congenital heart disease is reported. Patients with similar clinical features have been reported and they appear to exhibit the recently identified Goldberg-Shprintzen syndrome. It is believed that this girl also exhibits this new syndrome. Cranial computed tomography demonstrated abnormal findings that may suggest defective neuronal migration and/or dysgenesis of the brain. These findings were considered to cause psychomotor retardation and epilepsy in this patient.

Intellectual status of children with cerebral palsy after elementary education

Using Wechsler Intelligence Tests, we compared the intellectual status of children with cerebral palsy (CP) immediately before and 2 years after entering school. Verbal and performance IQs could be assessed for 23 children with spastic diplegia but only verbal IQs in six children with spastic quadriplegia. Performance IQs were much lower than verbal IQs in both the Wechsler Preschool and Primary Scale of Intelligence (WPPSI) and the Wechsler Intelligence Scale for Children-Revised (WISC-R) in children with spastic diplegia. After schooling, verbal IQ was significantly increased but there was no change in performance IQ, thus, the difference between these IQs became more pronounced. When comparing the school class types, the verbal IQs of children studying in ordinary classes became statistically higher than those of children in special classes. In contrast, since the increases in mean performance IQs were greater in children in special classes, the differences between these IQs became more pronounced in children studying in ordinary classes. We also found that although verbal IQs were lower for quadriplegic children than for diplegic children at preschool age, there was no difference after 2 years of schooling. These results underline the importance and benefits of appropriate education for children with CP.

Serial imaging in MELAS

Abstract We report two patients with fatal mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). Single-photon emission computed tomography (SPECT) with 123I-N-isopropyl-p-iodoamphetamine was more sensitive to the lesions than CT or MRI. SPECT showed focal hyperperfusion before or during the stroke and diffuse hypoperfusion of the brain, sparing the basal ganglia in the terminal stages. These findings support the theory that metabolic disturbance in the brain causes the “stroke” in MELAS.

Improvement of hypertonus after treatment for sleep disturbances in three patients with severe brain damage

Treatment for sleep disturbance was given to three patients with severe brain damage (a 14-year-old boy, an 8-year-old girl and a 9-year-old boy), and changes in their muscle tone were estimated using F-wave analysis. In all patients, F-wave analysis was performed in the ulnar nerve before and 2 weeks after treatment with flunitrazepam or melatonin. From 16 recordings of F-waves, the mean amplitude and latency, the ratio of F-wave amplitude to M-wave amplitude (F/M ratio), the mean F-wave conduction velocity and the F-wave occurrence were evaluated. All patients showed at least one significant decrement of mean F-wave amplitude, the F-wave occurrence and mean F/M ratio, which suggests a reduction in muscle tone after treatment for sleep disturbance. It is concluded that treatment for sleep disturbance occurring in brain damage is important in view of the improvement of increased muscle tone.

Effects of neonatal hypoxia on brainstem cholinergic neurons-pedunculopontine nucleus and laterodorsal tegmental nucleus

Hypoxic changes in the cholinergic neurons of the pedunculopontine nucleus (PPN) and the laterodorsal tegmental nucleus (LDT) were studied morphologically using immunohistochemistry for choline acetyltransferase (ChAT). Fifty-three postnatal day (PND) 7 Sprague-Dawley rats were subjected to a hypoxic load of 8% oxygen for 5 h. The rats which survived were later sacrificed at PND 14 or 28 for histological analysis. The results were compared with those obtained from control rats. Three weeks after hypoxic load, a decrease in the number of ChAT immunoreactive cells, especially in the caudal PPN, was found, although no remarkable changes were detected in cell morphology. Since several studies support the possibility that the cholinergic system from PPN/LDT is responsible for both REM generation and the general motor inhibition during REM sleep, our results may account, in part, for the clinical features of hypoxic brain damage such as sleep disorders and abnormal muscle tonus.

Hypocalcemic focal seizures in a one-month-old infant of a mother with a low circulating level of vitamin D

We present a case of a one-month-old infant with hypocalcemia and rickets, with symptoms of focal seizures. The ictal EEG showed left occipital spikes spreading over all of the left hemisphere. From the laboratory studies, we concluded that a low maternal circulating level of vitamin D would cause infantile hypocalcemia and rickets, while immature renal response to parathyroid hormone and transient hypoparathyroidism in infancy would induce hyperphosphatemia. Hypocalcemia may be an important factor in the cause of focal seizures which start even after the age of one month. Further, investigation of maternal vitamin D levels should be done in infantile hypocalcemia.