Recent advances in the experimental study on the production, migration and differentiation of neurons were introduced. All neurons in the central nervous system are produced either at the matrix layer, the subependymal layer or the external granular layer. The neuroblasts, produced at these places, migrate to the respective regions to occupy their fiaal position. Each groups of neuro_is are pro 1 aced at a definite time in embryonic or early postnatal life. Granule cells are the last group of the neurons which are produced in the central nervous system. Production of the granule cells in the human cerebellar cortex is known to last until eight to twenty months of age. In the cerebral cortex, neurons in the layer VI are produced first and those in the layer II are last. Thus the neurons occupying the outer part of the cortex are phylogenetically younger than those in the deeper.
Various problems concerning modern therapy of convulsive seizures in children have been reviewed on the basis of our own clinical experiences during the past 10-old years as well as on the findings reported by various investigatons both in Japan and abroad. In the treatment of epilepsy an emphasis has been placed on the importance in mobilizing all the knowledge at command as to the pathophysiology and neurophysiology of epilepsy aside from trial-anderror basis. As to epilepsy with an adequate therapy it has now become possible to suppress completely the seizure generally in 50 to 60 per cent of the cases and obtain a remarkable improvement in about 30 per cent of the cases. Especially with epilepsy in children we can restore most of these cases to normal from clinical and electroencephalographic aspects by antiepileptic treatment over a long period of time. Therefore, it is a great responsibility on the part of pediatricians or neurologists to assure the child a happy and unmolested life in the days to come by diagnosing at an early stage and formulating a systematic therapy regimen. In view of a remarkable advance in epileptology made possible by the development of antiepileptics coupled with the progress of clinical neurophysiology, further studies on the treatment of epilepsy are to be expected.
One half of all the cranial nerves subserbed the eye. Thirty eight percent of all fibers entering the central nervous system were concerned with the visual function. There were some specific entities in pediatric neuro-ophthalmology. In this paper I selected for discussion a number of diagnostic procedures and a few specific subjects which present unique problems in neuro-ophthalmologic examination of pediatric eye patients. Part II The normal visual stimulation was essential in the development and maintenance of the visual acuity or binocular function during early infancy and childhood. If the adequate stimulation was disturbed by such diseases as congenital cataracts, corneal opacities and strabismus at or soon after birth, the normal vision and binocular function never developed or very maldeveloped. The theoretic and practical implications of amblyopia were discussed by the neuro physiologic experiments of Wiesel and Hubel. Furthermore, some cases of congenital gustolacrimal syndrome and congenital cyclic oculomotor paralysis were reported.
The author discribed mainly the position of the supratentorial larger vessels in relation to fixed points of the skull during the different periods of age growth. Our material of 100 carotid angiographies of normal children of up to the 15 years of age was compared with the data of other foreign authors. If the Neuropediatrician do not have any such accurate knowledge of the position according processes, they will give often false diagnosis angiographically. This might be serious problem for patients. First of all, the most important thing is to get the accurate angiogram. This means the correct head fixation and accurate projection of the central x-ray to the children. Therefore, general anesthesia are almost necessary for the younger children. This thing might seems to be troublesome for Neuropediatrician, but they will get much informative data from these angiograms, if they performe these method above mentioned. At this time, technical problem, the indication and side-effects had not been mentioned here. And also infratentorial vessel will discribe on the next time.
For the diagnosis of neurological diseases in children, skilled history-taking and observation of children in the natural state are utmost important. In the differential diagnosis of convulsive disorders the most important information is obtained by careful history-taking. Spontaneous movement and posture of the extremities, expression of the face, attention to the surround, position and movement of the eyes, behavior, reaction to the sound etc. should be observed at the beginning. Abnormal extension, flexion or rotation of the joints of the extremities in the supine position may indicate a motoric disturbance. Evaluation of motor function is made by observing spontaneous movement and posture at the supine, prone, upright position on the bed, ground or sustained in the air. Physicians should be accustomed to observe the skin. Several spots of pigmentation or depigmentation on the trunk may be a symptom of neurocutaneous syndroms, such as tuberous sclerosis, Recklinghausen's disease, and radiation embryopathy. Presence of minor structural anomalies and rare palmar print pattern may suggest that the disease is developed in the embryonal stage. Because a child neurologist encounters many kinds of congenital diseases, knowledge on developmental biology, embryology and genetics is very necessary.
Clinical and electroencephalographic studies were made on 16 infants and children in the age range of 23 days old to 3 years old with subdural hematoma verified surgically for the purpose to elucidate the etiology, symptomatology and prognosis, with a special emphasis on the significance and usefulness of electroencephalography in early diagnosis. The results are briefly summarized as follows. Their EEG revealed abnormalities in everyone of the cases, especially in 15 cases (93. 8%) out of the 16 there was observed localized suppression of the electrical activity (focal low voltage), indicating that it is very important for the diagnosis. In infancy the important points to be noted are that most of the symptoms at the acute stage may disappear gradually while the EEG abnormalities mentioned above persist and aggravate. Therefore, these EEG findings are valuable for the clinical diagnosis. The facts that localized spikes can be observed in 68. 8% of the cases in the acute stage and also even after removal of the hematoma often there appear epileptic discharge, suggest the coexistence of parenchymal cerebral damage. Hence it is essential to follow up the patient electroencephalographically for the purpose to predict the onset of clinical epilepsy. In addition, it is rare to observe such a localized suppression of the electrical activity in infancy, but in Hemiconvulsion-hemiplegia syndrome it is characteristic to see unilateral hemispheric suppression of the EEG. For this reason, we measured the mean visual evoked potential (MVEP) of the cases with subdural hematoma and that of the cases of Hemiconvulsion-hemiplegia syndrome, and found there can be observed asymmetry in MVEP in the two groups, but no change can be seen in the peak latency of MVEP of the former group while a prolongation of the peak latency in the latter. The EEG is of aid in establishing the diagnosis of subdural hematoma in infancy and early childhood.
Two sibling cases of Kugelberg-Welander's disease were presented. Both patients developed slight muscular weakness in the lower extremities at the age of 3 years. When these patients visited our clinic, the elder patient was 13 years old and the younger one was 6 years old. The degree of muscular involvement in both patients was apparently proportional to the time course from the onset of the disease, i. e., functional disability was severer in the elder than in the younger, muscular fasciculations in the shoulder girdle and the tongue were noted in the elder but not in the younger, and knee jerk was absent in the elder, but was present in the younger. Electromyogram showed decrease of NMU and the presence of high amplitude polyphasic action potentials in both patients but more obviously in the elder. The muscle fiber degeneration seen in the biopsied specimen had the characteristics of both neurogenic and myogenic origins. Groups of fiber atrophy and targetform fibers were scattered among the fibers with relatively well-preserved structure indicating the nature of neurogenic degenerations. In addition to deformation of some fibers and increase of connective tissue in some areas, prominent vacuolar formation was noted in almost all muscle fibers. All of these were considered as signs of myogenic degeneration. Histochemical study revealed decreased activities of phosphorylase, succinic dehydrogenase and aldolase. Deformity of end plate was noted in the cholineesterase stained preparation. All these laboratory findings were less dominant in the younger patient.
A case of infantile subacute necrotizing encephalomyelopathy with mammillary bodies involvement was reported. The patient was 3-year-10 month old boy. His parents had consanguinity of cousins, and his elder sister was blind. He developed normally, until the gait disturbance began at 2 years 7 months of age. His gait was spastic and became impossible at 2 years 9 months. On examination the patient had general hypotonia, spastic diplegia, bilateral optic atrophy and eczema in the face and head. He died at 3 years 10 months of age. In necropsy, the gross examination of the CNS revealed stagnant meninges. On section of the brain the focal colored lesions were present bilaterally in the periventricular or periaqueductal regions of thalamus, hypothalamus including mammillary bodies, midbrain, pons and medulla oblongata, and in the cingulate gyrus and hypocampal gyrus of the cerebral cortex. Sections through various levels of the spinal cord showed symmetrical lesions involving the posterior, anterior and lateral columns. Histological examination revealed in lesions the following 1. spongy degeneration or tissue necrosis, 2. intense vascular dilation and capillary proliferation with thick walls of fibrous tissue, 3. little degeneration of nerve cells in the grey matter, 4. demyelination in the spinal cord. The spongy changes of ground substance were spread more widely than prominent capillary vascularity with thick walls. Therefore the capillary proliferation and fibrous proliferation of the capillary walls may develop in the spongy state after breakdown of ground substance.
Case 1: The patient was a girl, aged 26 months. Gestation, delivery, and development until 16 months of age were normal. Soon after beginning to walk, she had a gait of progressive stiffness and ataxia. Eight months after onset of the illness, she was unable to sit alone and speak, and mentally deteriorated. Her parents were in cousinship, and her elder sister died of clinically suspected leukodystrophy at the age of 4 years. Microscopic examination revealed some metachromatic substances in the urine sediment. The right frontal lobe was biopsied. The white matter included abundant quantities of the metachromatic, granular material demonstrated by Hirsch-Peiffer and toluidine blue stains. The lipid content of the biopsied specimen showed a pronounced increase in suifatide. Case 2: The patient was a 33-month-old boy. His parents were also in cousinship. Although he could stand with a support at 12 months, he was never able to walk. At the age of 21 months, the child could no longer sit alone and speak. General weakness and hypotonia noted at the same time. During about one month after the admission, he had spasticity of limbs with hyperactive tendon reflexes and Babinski sign. No metachromatic substance was observed in the urine sediment, but arylsulfatase-A activity in urine was not detected. In frozen sections of the biopsied ulnar nerve, stained with cresyl violet acetic acid, the metachromasia characteristic of metachromatic leukodystrophy was readily identified. Electron microscopical examination revealed cytoplasmic inclusions of variable density, measuring up to 2 microns in diameter, in Schwann cells of the myelinated nerves.
Pathological examination of the intracranial complications of low birth weight infants and retros pective clinical examination of the central nervous system damages of prematurely born children were performed. Intracranial pathologies were found in 133 cases of the 238 autopsied low birth weight infants. Those cases consisted of the intracranial hemorrhage (96cases), kernicterus (7cases), purulent meni ngitis (11cases), non-bacterial meningoencephalitis (2cases), periventricular leukomalacia (45cases) and encephalomalacia (1case). These pathological findings overlapped in a half of the cases. The incidence of intraventricular hemorrhage, subependymal hemorrhage and hemorrhage in perivent ricular white matter was increased, as birth weight decreased. Periventricular leukomalacia was found very often in the group with birth weights from 1, 001 to 2, 000g. Those pathological changes were recog nized in the periventricular regions of the cerebrum. In 675 prematurely born children with various brain damages, cerebral palcy (326 cases), epilepsy (124 cases) and mental retardation (103 cases) showed high incidence. These diseases overlapped often in each case. There was a large percentage of children with cerebral palsy, as birth weight decreased, while convulsion and mental retardation did not reveal such a tendency. In the group of the children with birth abnormalities and with no perinatal abnormalities, hypertonic type or spastic type of the cerebral palsy was dominant. This tendency was eminent in lower birth weight children. Those two results showed similar features. Therefore the periventricular pathological changes of the cerebrum in the autopsied newborn cases may correlate to hypertonic type or spastic type of cerebral palsy in the prematurely born children.
Two cases with seizures induced by movement were presented and the nomenclature of this entity was discussed. The clinical manifestations of the cases were characteristic and the peculiar seizures were induced by sudden voluntary movement without loss of consciousness, often associated with emotional excitement. Age of onset was six and eight years respectively. One case showed the athetoid-dystonia and the other the torsion spasm. Duration of the attacks was several seconds to one minute. Good response to anti convulsant medication was observed. In addtion to the seizures induced by movement, one case had a past history of afebrile grand mal attacks and another case showed psychomotor seizures. The titles hitherto reported of this entity are divided into two groups: one is the name showing the form of the paroxysmal involuntary movement and the other is that showing the pathogenesis of the reflex epilepsy. The pathogenesis of the reflex epilepsy appeared to be confirmed through the exper ience of the cases with two types of seizures in one individual. Since this is a sort of reflex epilepsy, the name of “kinesigenic epilepsy” is preferable to “paroxysmal choreoathetosis”.
Sixty nine children who had spike or sharp wave foci on the temporal area were clinico-electroenc ephalographically studied. Fifty seven patients manifested the various kinds of seizure clinically such as psychomotor seizure (20 cases), grand mal seizure (22 cases), focal motor seizure (13 cases) and autonomic seizure (2 cases). Autonomic symptoms were observed in 70% and generalized seizure were observed in 40% of the patients who had psychomotor seizure. Etiologic factors were perinatal brain damage (24 cases), head trauma (5 cases), and unknown (33 cases). The high incidence of cerebral palsy was also proved in 12 patients who had no manifestation of seizure clinically. The occurrence of temporal foci did not differed in frequency difference between right and left hemispheres. The incidence of abnormal discharge on the midtemporal region was slightly higher than that on the anterio-temporal except in psychomotor epileptics. The abnormal discharge recorded in the psychomotor epileptics usually localized to the temporal area, whereas spreading tendency were obs erved in the other. There was no difference between the incidence of sporadic spikes and of spike & waves in our patients, but the spike & waves (usually 2. 5-3 c/s) were more frequently observed in psychomotor epileptics. Rhythmic temporal discharge was observed in 12 patients.
A 5-year-old boy developed somnambulistic episodes while he had been hyperthermic (40-41.0°C) from pneumonia of about two weeks duration. After recovery from pneumonia, typical somnambulistic episodes recurred about once a week. Attacks usually occurred one to two hours after falling asleep and lasted for about ten minutes. No other epileptic manifestation like convulsive disorder had been observed. At the age of 7 years, when he visited our clinic for the first time, the day time EEG during natural sleep showed 14c/s positive spikes at the anterior temporal lead. Since then the patient has been kept to take diphenylhydantoin and mephobarbital. This anticonvulsive medication has been apparently effective because the occurrence of somnambulistic episodes has been reduced to only once a year, although the day time EEG has consistently shown the same type of spikes up to the present time when he is 10 years old. Literature on EEG findings in somnambulism was reviewed, and EEG examination at the early stage of the disease is recommended for even a typical somnambulism without convulsive disorder.