The purpose of this study is to determine infants' preliminary linguistic ability. For this a pre-speech language test method has been developed. The test method is as follows. First, a definition of language is given. Then the development process in infancy is explained according to the definition. Subsequently, the test items are decided according to the above process.
(1) Definition of language: It is dificult to define language because of its many aspects. Here, for the language definition, the aspects are restricted to the following based on the properties of natural language.
§-1 Situation of language
i) Formation of communication
ii) Understanding and grasping of situation circumstance
§-2 Development of symbolizing function
§-3 Representing the function of language (generally, articulation of babbling and functional change)
(2) Development of language: the above three aspects develop and change from preterm infancy to late infancy. The development process is as follows.
§-1 i) The formation of communication is confirmed by action situations between baby and mother. The actions include a steady gaze in the early stage and talking baby talk later. Requests for instruction assistance and emotional communication appear with gradual differentiation of communication.
§-1 ii) Understanding and grasping of the situation circumstance is confirmed with response to action from mother to baby in the early stage. These are then confirmed with the ability to understand the action and situation of surroundings as time and space extensity, and to give appropriate appeal to the surroundings. Subsequently, understanging with language only is acquired by daily experience, including language and understanding of the surrounding.
§-2 For the development of the symbolizing function, symbolizing and action in the early stage are confirmed as recognition and operation of the surroundings. The recognition is confirmed with visual and auditory recognition, and development from an attitude of contemplation and that of listening to language. Operation is confirmed with development from the stage of grasping the subject to that of operating it.
§-3 Representation is confirmed with the articulation change of babbling and that of the communication function. The articulation change is confirmed with different types of pronunciation and long-syllabic babbling The functional change is confirmed with that of calling, proto- language, monoreme, and popularly accepted pronounciation.
(3) Test items: forty-seven concrete indicators of development are established based on preliminary language ability. The test is conducted on these 47 items.
Results: The test was conducted with 281 healthy babies. A test profile was given based on the results (these will be reported in a second paper) as shown in Fig. 1. The horizontal axis represents three aspects of language definition, and the vertical axis represents the age (month). The position of the test item on the profile shows items included in each aspect and development stage (proportion of right answers 50%) on the horizontal axis, and the development order on the vertical axis. The language ability could be divided into five stages and is shown in the second paper.

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The tests as reported in the pevious paper were conducted on 281 healthy babies. The test included 47 items. An accomplished item was accorded one point, and the total points were counted.
Results: descriptive statistics: 1) Table 4 shows the proportion of right answers of each item by age (day). The day when the item was accomplished was definite and showed the properties of development.ii) Fig. 1 and table 1 showed the relationship between the total points and the age (day). The total points increased linearly with age (day).
Discussion of test properties: i) Reliability: special attention was devoted to maintain consistentconditions on item formation to increase reliability. Consequently, the confidence coefficient was 0.989 in all cases. ii) Validity: predictive validity was tested. The changes in language ability were investigated in 90 cases for observation over the passage of time (including cases other than those for standardization). The cases were divided into three groups by age (0-200, 201-400, 401-day) (Table 7). For an estimated value, the PPV was 0.60-0.67 and the NPV was 0.94-1.00 (Table 8). The NPV was higher than the PPV. Hi) Test construction and figure of test construction: it was found that the test construction was composed of five factors and had stages of development as follows.
(1) Initial call, communication
(2) Positive appeal to the surrounding
(3) Stage just before speech
(4) Initial language acquirement and differentiation of language function
(5) Speech-increasing stage
The items included in each factor (each stage) are shown in the Fig. 1. profile in the previous paper.The factor loading in a factor analysis is shown in Fig. 7.
It is concluded that this test is a valid determinant of language ability in infancy and is valuable in establishing a “diagnostic criteria for speech disorder”.

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Carotid blood flow was measured with a ultrasonic volume flow meter in 38 infants. The carotid blood flow was 1.50±0.55 ml/sec. At the same time peak systolic flow velocity, mean blood velocity, end diastolic velocity, pulsatility index (PI) and resistance index (RI) in the middle and anterior cerebral arteries were measured by duplex Doppler scanning. There was a correlation between the carotid blood flow and the mean blood velocity in middle and anterior cerebral arteries. However, PI and RI were not well correlated with carotid bood flow.

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Single photon emission computed tomography (SPECT) of the brain using N-isopropyl-p-[¹²³I] iodoamphetamine (¹²³I-IMP) was performed in 11 children with port wine stain on the face or head, aged 1.0-14.2 years at investigation.
Four cases without neurologic symptoms had no specific abnormality on SPECT and X-ray computed tomography (CT), In 4 cases of so-called Sturge-Weber syndrome with developmental quotients (DQ) or intelligence quotients (IQ) more than 80 and the neurologic symptoms consisting of seizures and hemiplegia, SPECT showed localized reduction of IMP accumulation, and CT exhibited calcification, atrophy and enhancement in 2 cases of 3 with contrast medium infusion in the same areas. In 3 cases with DQ of 50-60 and severer neurologic symptoms, SPECT showed diffuse reduction or defect of IMP accumulation in the ipsilateral hemisphere, and CT exhibited remarkable atrophy, calcifications and enhancement in 2 cases with contrast medium infusion in the same hemisphere. In one case with severe neurologic symptoms, SPECT performed at an early stage showed high IMP accumulation in the ipsilateral hemisphere.

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Serum triiodothyronine (T3), free T3, thyroxine (T4) and free T4 levels were measured before and im -mediately after daily ACTH-Z therapy (0.01 mg/kg/day, 1-2 weeks) for patients with 9 cases of infantile spasms and one case of myoclonus epilepsy. In some of them, serum reverse T3 (rT3) and thyroxine binding globulin (TBG) levels were also measured. All patients became seizure free after ACTH-Z therapy. Before ACTH-Z therapy, all of the hormone levels were within normal limits. Serum T3 and free T3 levels were markedly decreased after daily ACTH-Z therapy, and serum T4, free T4 and TBG levels were moderately decreased after the therapy. 1-6 weeks after the daily treatment, all of these hormone levels returned to pretreatment levels. Serum rT3 levels did not change after ACTH-Z therapy. These results suggest that the effect of long-term daily ACTH-Z therapy is very critical to the immature brain, and conventional ACTH-Z therapy has to be reconsiderd.

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Among 848 cases of profound mental retardation with motor disturbance admitted to Metropolitan Medical Center of Severely Handicapped in the last 20 years, 98 died. The 94 cases whose cause of death was determined were clinically investigated.
There was no difference in sex, and72% of the patients died before the age of 15 years. Half of the patients died ofpneumonia; sudden death occurred in 9, and ileus in 8. These three were thought to be the most important and characteristic causes of death in severely handicapped patients. In recent years, deaths due to pneumonia have decreased and those due to ileus have disappeared, but deaths due to malignantneoplasm have begun to be recognized. There were also some deaths from intracranial hemorrhage in young children, and some deaths from tracheal bleeding in those who had tracheal tubes. These two were also important causes of death in the patients. Sudden death had certain characteristics: most cases were adolescent or young patients with mixed quadriplegia who were sensitive to environmental changes and often showed marked hypertonia by athetosis, and in addition, all of their acute changes occurred between 5 and 8 a.m. or between 6 and 9 p.m.

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We reported two cases of brain infarction. They were cousins. Case 1 was a 12-year-old girl, who complained of aphasia, dyscalculia, right-left disorientation and right homonymous hemianopsia. CT showed low density areas in left superior and middle temporal gyri. Case 2 was a 15-year-old boy, who had left hemiplegia and hypesthesia to pain, temperature and touch on the left side of the body. CT showed low density areas from the genu of the internal capsule to the corona radiata, and from the posterior portion of putamen to the posterior limb of the internal capsule on the right side. Both cases had hypertriglyceridemia which might be associated with the etiology of infarction.

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A 7-year-old girl with normal psychomotor development during infancy began to have easy fatigability about 3 years of age. At the age of 5 years, she developed respiratory distress and became unconscious when the serum lactate and pyruvate levels were markedly elevated and a blood gas analysis showed respiratory and metabolic acidosis. Thereafter, she had similar episodic respiratory problems with lactic acidosis. Her muscle biopsy showed a myopathic pattern and numerous ragged-red fibers in an approximately half of muscle fibers. Lipid droplets were slightly to moderately increased in amount mostly in the raggedred fibers. A biochemical analysis on the isolated mitochondria from the biopsied sample showed markedly decreased NADH cytochrome c reductase activity with no specific but rather uniformly decreased subunits of complex I by the immunoblotting method. She was diagnosed as having the myopathic form of complex I deficiency because she and her relatives with similar muscle symptoms had no central nervous system symptoms such as progressive mental deterioration, convulsions and stroke-like episodes. Diagnosis of complex I deficiency was further confirmed by an oxograph study; the oxygen consumption was not detectable when malate and pyruvate were added as the substrates in the isolated mitochondria. Although stroke-like episodes and convulsions are commonly seen in complex I deficiency, episodic respiratory distress as seen in the present patient has not been described in the literature.

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A five-year-old girl of Aicardi syndrome showed moderate psychomotor retardation. She could walk and communicate. At six months of age, she developed salaam convulsion with series. Convulsions disappeared immediatly after ACTH treatment. At eighteen months of age, she developed myoclonic seizures. From three years of age, head- nodding seizures with series relapsed and could not be controlled. But she could walk alone at two years old and speak a word at one and half years old.
A degree of her psychomotor retardation was more slightly than others published cases of Aicardi syndrome. There were eleven reports, included our case, with mild retardation in Aicardi syndrome. The features of these are 1) late onset of seizures, 2) good response to ACTH therapy, 3) no significant abnormalities of brain except for agenesis of corpus callosum.

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We diagnosed a 3-year-old boy as type C Niemann- Pick disease by studies on cultured fibroblasts; sphingomyelinase activity was almost normal, but esterification of exogenously administered cholesterol was deficient. Also sparse cultures of his fibroblasts developed a relatively intense fluorescence with filipin that was not observed either in normal or type B Niemann-Pick fibroblasts. We tried to treat him with dimethylsulfoxide (DMSO), 100 mg-120 mg/day for a year, but it had clinically insufficient effect on our case.

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We presented a 3-year-old boy, a product of consanguineous parents, with periodic apnea during waking and sleep states, severe psychomotor retardation and hypotonia. According to polysomnographical recordings, he exhibited frequent central apneas which decreased in frequency and regularity in the stage REM. He showed abnormal background EEG, undifferentiated sleep stage and very short duration of stage REM. The initiation of breathing after apnea was often accompanied with generalized muscles contraction like a startle response. In the waking state the apnea induced generalized muscular hypotonicity and the decline of wakefulness. Arterial CO2 and O2 saturation was within normal limits. It was suggested that the malfunction of the brain stem responsible for the control of breathing, sleep-wakefulness cycle and determination of sleep stages was closely connected with the pathogenesis of abnormal breathing patterns.

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