We present here 38 patients with athetotic cerebral palsy who has trained in our clinic in recent 5 years. The mean age was 4 years and 4 months. In preterm cases, their gestational ages ranged from 27 weeks to 36 weeks, less than 31 weeks in 74%. MRI showed periventricular leukomalacia in 75%. Their clinical picture was mixed quadriplegia with athetosis and spasticity. Half of the cases were profoundly handicapped in motor ability.
Fifteen cases had been born in term. Sixty percent of them had a non-mixed type of cerebral palsy. Five cases had no remarkable risk factor during the perinatal period. Six cases were able to walk with or without support.
Hypoxic-ischemic encephalopathy was the main cause of athetotic cerebral palsy in recent years. Twin pregnancy was also an important risk factor, especially in preterm infants. MRI could detect lesions in the thalamus or the basal ganglia in only 17% of the 36 cases examined.

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We studied the effects of vitamin K₂ (menatetrenone) on the bone metabolic markers and bone mineral density (BMD) in 17 with low level of BMD cases of severely motor and intellectual disabilities (SMID).
Markers of bone formation and resorption as well as BMD were determined before, and 16, 32, and 48 weeks after administration of vitamin K₂. After 48 weeks of treatment, serum bone alkaline phosphatase and oseteocalcin levels of significantly increased (p<0.05, p<0.01, respectively), but urinary excretion of neither pyridinoline nor deoxypyridinoline decreased. BMD determined by the computed X-ray densitometer method tended to increase in cases with severe motor dysfunction (p=0.07). There were no side effects. These data demonstrate that vitamin K₂ may improve bone formation in patients with SMID.

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Blink reflexes were studied in 41 patients with severe handicaps, and were correlated with feeding problems and other lower brainstem symptoms such as drooling, an absent gag reflex and stridor. Ventrodorsal diameters of the pons and medulla oblongata on sagittal MRI were also studied in 29 of the cases. The patients were divided into three groups: tube feeding (25 cases), oral feeding (13 cases) and mixed feeding (3 cases), In the tube feeding group, all but three cases showed a prolonged or absent RI component, and all cases showed prolonged or absent R2 and R2' components. These abnormalities were significantly more frequent in the tube than in the oral feeding group. The patients with drooling, stridor or an absent gag reflex more frequently showed prolonged or absent components than the patients without these symptoms. The brainstem size on MRI was not different between the tube and oral feeding group. These results suggest that the blink reflex, particularly its late components, is a useful indicator for evaluating feeding function and other lower brainstem functions.

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Clinical symptoms and MR spectroscopic findings were studied on 4 cases of Pelizaeus-Merzbacher disease including 1 autopsy case. Common symptoms were severe mental retardation and spastic tetraplegia. These cases had nystagmus, and one had involuntary athetotic movement.
Genetical diagnosis revealed in 2 cases, duplication of proteolipid protein (PLP) and deletion in 1, whereas one case had no abnormality of PLP gene. MRI indicated the reversal of signal intensities on Tiand T₂-weighed images, a characteristic finding of PMD MR spectroscopy demonstrated a pattern of NAA in 3 cases.
This was specific to PMD because other white matter diseases show a decrease in NAA. In conclusion, MRS was useful to differentiate PMD from other white matter diseases.

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Acute measles encephalitis is a typical parainfectious encephalitis in which the brain is diffusely affected clinically and neuropathologically.
We present here a 5-year-old case of measles encephalitis with intractable seizures, bitemporal MRI abnormalities, and neurological sequelae of auditory agnosia and aphasia.
Pathophysiology of the focal MRI lesions and of expressive aphasia due to auditory agnosia was discussed. We stressed that the status convulsivus and its ischemic effect played an important role on the development of the focal lesions rather than focal encephalitis or ADEM.
In the course of auditory agnosia and acquired aphasia, the patient transiently expressed some meaningful words. The significance of this episode was discussed based on the sequential changes of symptoms and neuroimaging findings.

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We report here a pedigree of basal ganglia calcification with autosomal dominant inheritance. Following a traffic accident, the proband, a seven-year-old boy, was incidentally noted by cranial computed tomography to have calcification of the bilateral basal ganglia. Six affected members spanning three generations, aged from 5 to 57 years, also had calcification in various degree. None of them had clinical symptoms. There were neither abnormal data nor any characteristic physical symptoms associated with parathyroid disorders. There was no consanguinity. Both sexes were affected and the sex ratio was 0.5. Male-to-male transmission was documented. These findings suggested an autosomal dominant trait. The clinico-radiological findings in our pedigree were different from those of the previously reported cases of familial basal ganglia calcification, that infants were affected and that clinical symptoms were absent in elderly patients. These facts suggest our pedigree is a new type of familial basal ganglia calcification with autosomal dominant inheritance.

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Major central nervous system (CNS) complications such as seizures and coma, occur in about 30% of children with hemolytic uremic syndrome (HUS). Serious CNS involvement is associated with an increased mortality. The purpose of this study was to evaluate intracranial hemodynamics in HUS encephalopathy. We measured mean blood flow velocity and pulsatility index (PI) of the middle cerebral artery (MCA) with transcranial Doppler (TCD) in a two year-old girl with acute encephalopathy, and compared them to those in a one year- old girl with febrile convulsion incidentally complicating HUS (a disease control). In the patient with HUS encephalopathy TCD demonstrated abnormally low PI of 0.58, while the magnetic resonance imaging (MRI) and single-photon emission-computed tomography (SPECT) reveal no abnormal findings in the acute stage. The abnormal TCD findings disappeared with her recovery. In the HUS patient with febrile convulsion alone, TCD demonstrated normal maximal flow velocity and PI. Thus TCD may be useful in evaluating intracranial hemodynamics in HUS encephalopathy in the absence of MRI and SPECT abnormalities at the early stage of illness.

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We report three cases of symptomatic arachnoid cyst in the middle cranial fossa who presented with atypical symptoms including speech delay, vomiting and personality changes. They were improved by a cystperitoneal shunt operation.
In many patients with a small or middle sized arachnoid cyst in the middle cranial fossa, their symptom tends to be so trivial that surgery is not the choice of treatment. The present cases suggest that peculiar symptoms caused by a limbic system lesion may be improved by treating the arachnoid cyst in the middle cranial fossa.

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Anti-galactocerebroside (anti-GC) antibody has recently been recognized as a possible demyelinating agent. On the 9 th day of Mycoplasma pneumoniae (Mp) pneumonia, an 8-year-old boy had a seizure lasting three hours followed by loss of consciousness for 30 hours. Anti-GC antibody titers and myelin basic protein in the CSF were 1: 640 and 8.1ng/dl, respectively. There were no neurological sequelae. It is suggested that the degree of elevation of anti-GC antibody titers in CSF may not correlate well with the severity and neurological prognosis in Mp encephalitis.

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We encountered a 5- month- old baby with shaken baby syndrome, which had been caused by being thrown up about 50 cm in the air several times by his father. Intracranial hemorrhage, bleeding in the eye grounds in both eyes, and convulsion were noted on admission. Shaken baby syndrome was diagnosed from the history, and the patient was given necessary treatment, which led to favorable prognosis. Father was not awae of the danger of handling his baby in that way, and no sign of maltreatment was noted on the body surface of the baby. An attention should be payed that the danger leading into syndrome could be hidden among this kind of caring in the everyday life.

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The author reports a male infant with benign myoclonus of early infancy (BMEI). Series of spasms were first seen at the age of 6 months, then occurred from once to three times a day while he was awake, but never during sleep. The first examination at the age of 8 months revealed a normally developed infant with no neurological abnormality. Both interictal-and ictal-electroencephalograms (EEG) were normal. During three months of follow-up observation with no medication, the spasms completely disappeared. He was subsequently observed up to 2 years and 0 month of age and showed normal psychomotor development, normal EEG with no epileptic discharges, and absence of any types of seizures.
The spasms associated with BMEI have been considered to be a non-epileptic phenomenon. They are benign and disappear spontaneously without any complication. Therefore, it is important to differentiate BMEI from various epileptic syndromes, especially West syndrome, to avoid unnecessary administration of antiepileptic drugs.

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An 11-month-old boy developed acute polymyositis about 1 week after respiratory syncytium virus (RSV) pneumonia. He was admitted to our hospital because of interstitial pneumonia. RSV infection was confirmed by the presence of its antigen in his nasal discharge. Two weeks later, his chest X-ray findings improved and RSV antigen became negative, but severe generalized muscle weakness developed, causing respiratory failure. Muscle biopsy demonstrated inflammatory cellular infiltration with occasional fiber necrosis.
Intravenous steroid pulse therapy was remarkably effective resulting in complete recovery of his muscle power. In this patient polymyositis was preceded with RSV infection, suggesting a close relationship between polymyositis and RSV infection. Although many patients of viral myositis have been reported to be associated with coxsackie B, HCV and HTLV-1 viruses, our patient is the first infantile polymyositis secondary to RSV infection.

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We reported a fourteen years old male with subdural empyema followed by consecutive magnetic resonance imaging (MRI) examination. He was admitted to our hospital, complaining incomplete paresis of the left upper extremities, headache and high fever. The first MRI T₂-weighted image at admission revealed an abnormal high intensity in the right frontal subdural space, indicating an abscess. Antibiotics therapy and a burr hole operation were effective and he made a remarkable recovery. One month later, the third MRI T₂-weighted image showed an abnormal high intensity in the deep white matter of the right frontal lobe, which had not been detected in the subsequent MRI after the operation. On the other hand, the meningeal enhancement on the second Gd-DTPA enhanced MRI was more thickened than the previous meningeal enhancement. This abnormal high intensity on MRI had disappeared two month later, when the meningeal enhancement had grown thin. These data suggested the meningeal enhancement was relative to the transient high intensity which was considered to represent brain edema. We conculed that careful observation and serial MRI examination might be necessary in patient with subdural empyema even after disappearance of all symptoms.

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[in Japanese]

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