Volume selective proton MR spectroscopy (¹H-MRS) of the brain was performed on a 1.5T magnet infive patients with congenital muscular dystrophy (CMyD), 46 healthy children, and one healthy adult. Peaks of N-acetylaspartate (NAA), choline (Cho) and creatine (Cr) were observed in both patients and control groups on ¹H-MRS, but lactate was not observed.¹H-MRS revealed an increase in the ratio of NAA/Cho and NAA/Cr, and a decrease in Cho/Cr ratio with age in control subjects. In patients with CMyD, the values of NAA/Cho ratio did not increase with age. The values of NAA/ Cr ratio decreased with age. The values of Cho/Cr ratio decreased with age in both patient and control groups. At age more than 4 years, the values of NAA/Cho and NAA/Cr ratios were lower in patients with CMyD than in controls. The values of Cho/Cr ratio did not differ between CMyD and controls. These results suggest that in patients with CMyD, there is an NAA decrease and a developmental disorder of neurons in the brain.2

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We analyzed the carotid artery blood flow waveform (CABFW) through multivariate autoregressive analysis in a case with multicystic encephalomalacia (MCE) after neonatal asphyxia and compared the result with those of 35 healthy newborns. The total power of CABFW was at the-2 SD level of the value for 35 healthy newborns, and the power, % power, bio-informing amounts and damping time of component 3 (damping frequency 11.15 Hz) were less than-2 SD of the values in 35 healthy newborns. The Pulsatility Index (PI) of anterior cerebral artery (ACA) was high (0.76). These results suggest that cerebral blood flow decreases because of cerebral vasoconstriction in MCE after neonatal asphyxia

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Chorea minor is the most curious manifestation of rheumatic fever, first described by Sydenham in 1686. Subsequent evidence showed that chorea could be a late manifestation of rheumatic fever, often occurring several months after a streptococcal infection in contrast to other major manifestations. During the ten-year period between 1984 and 1993, 11 children with rheumatic fever were seen at our hospital, two cases of these being accompanied with chorea minor. Case 1, a male aged 12, presented with involuntary movements. He was diagnosed as having rheumatic fever because of chorea and systolic ejection murmur at the apex of the heart. Plain cranial CT was normal. However, positron emission computed tomography revealed an increased ¹¹C-glucose uptake in the caudate nucleus as compared with the cerebral cortex. Case 2, a female aged 14, presented with involuntary movements. Plain cranial CT was normal, but single photon emission CT showed a difference between the right and left brain. These two patients were given penicillin G (PCG), predonisolone (PSL) and haloperidol. Haloperidol was administered, because PCG and PSL had no effect to improve the clinical manifestations. After administer decreased to a half with a clinical improvement, but the level of GABA did not change. Haloperidol seemed to be an effective and useful agent for motor manifestations of the disease.

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Somatosensory evoked potentials (SSEPs) were investigated to evaluate the function of the central nervous system in 53 severely handicapped patients. P₉-P₁₃ interpeak latencies of these patients were within normal limits in comparisonw ith those of normal controls. But P₁₃-N₂₀ interpeak latencies showed various abnormalities including prolongation or absence. On etiological study, prenatal onset cases, especially in migration disorders, h ad markedly prolonged P₁₃-N₂₀ and postnatal onset cases frequently showed the absence of N₂₀. Most of the patients showing diffuse brain destruction on CT scan revealed the absence of N2₀.

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Total body and leg bone mineral densities (BMD) were measured in 42 severely handicapped children and adults using a dual energy X-ray absorptiometry (DEXA) (LUNAR Radiation Corp., DPX). Despite the differences in motor ability and nursing history, about 95% of patients, except for 2 cases, were diagnosed as having osteopenia. The degree of osteopenia was dependent on the motor disabilities of their original disease. Therefore we should bear in mind a precaution and therapy from early period. Because BMD of the legs in females were less than-SD of the age-matched control values, we should be careful for a possible fracture of femur.
Since X-ray hazard of DEXA for patients is considerable to be negligible, this will provide an effective means for quantifying bone mineral in severely handicapped patients

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There have been many reports concerning cases with basal ganglionic and thalamic lesions due to neonatal hypoxic-ischemice ncephalopathy (HIE), which are considered as a clinicopathologicasly ndrome. But the clinical symptoms and extension of the brain damage varied from case to case. We previously reported seven cases with bilateral basal ganglionic and thalamic lesions (BBTL) due to neonatal HIE. The affected lesions were posterior putamen, lateral thalami, hippocampus and perirolandic cortex. Now, we have experienced two cases who presented symmetrical thalamic lesions (STL) with involvement of the brainstem and periventricular white matter due to neonatal HIE. Of these lesions of the basal ganglia and thalamus, STL comprisedc alcificationa nd atrophy of the bilateralt halami, a nd BBTLc omprisedc ystic lesionso f the bilateral putamen and thalami. The cerebral lesion in the cases of BBTL were observed in the perirolandic cortex, which is a watershed area of the main vascular territories. While the cerebral lesion in the cases of STL were found in the periventricularw hite matter, which is a watersheda rea in prematureb abies. The brainstemw as more severely injured in the case of STL than BBTL. The distribution of the affected lesions suggested that the cases with STL had a different clinicopathologicasly ndromef rom those with BBTL. We discussedt he developmentaml echanismo f the cases.

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Neurologic morbidity is observed more frequently in twins than in singletons. Low birthweight and premature birth are observed more frequently in twins but cannot always explain the cause of cerebral palsy, although circumstances related to twinning may cause brain damage. We attempted to select the patients whose brain damages might be caused by circumstances related to twinning and discussed the developmental mechanisms of their brain damages. We divided the cases into 3 groups. One comprised the monozygotic (MZ) twins whose co-twins had died. The second comprised the MZ twins with the complication of the twin-twin transfusion syndrome or discordant twins. The third comprised the MZ twins with concordant cotwins. We postulated that group I and II had complication of the disturbance of fetal circulation, but group III did not.
Hydranencephaly (group I) and polymicrogyria (group II) might develop when the disruption occurs at an intermediate stage of morphogenesis (before the 6th month of gestation). In the cases with cerebral infarction (group I), the distribution of the lesions were related to the main vascular territories of the mature form, might be caused by occulusion of blood vessels associated with intrauterine disseminated intravascular coagulation. In the cases of MCE (group I), multiple cavities are present mainly in the white matter, the gray matter having a tendency to be spared. As the distribution of the cavities is not related to the main vascular territories, it was less likely that the lesions was produced by occluded vessels. The death of one fetus might cause severe blood deprivation, leading to hypoxic-ischemic brain insults in the surviving fetus. The cases having ventriculomegaly predominant in occipital horn (group II) accompanied with compatible findings of periventricular leukomalacia. Therefore, the brain insults might occurred at the stage of immature brain. Among the cases with normal CT or mild brain atrophy (group II and III), the cases who presented ataxic diplegia and mental retardation with behavioral disturbance might have microscopic abnormality, such as synaptic dysplasia or abnormal structure of the cortex.

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We reported a case of acute disseminated encephalomyelitis (ADEM) after Streptococcus infection. Brain MRI (T₂-weighted image) showed high intensity lesion in the gray matter in the acute phase. The high intensity pattern of the lesion was different from those in previously reported cases. The boy, aged 14, had fever late in August 1993. He had lumbago and back pain since September 3 and also leg weakness developed since September 7. He became unable to urinate on September 10 and was admitted on September 12. His consciousness became indistinct. We considered ADEM on the basis of high CSF level of myelin basic protein, clinical course, symptoms and MRI findings and began to administer steroid hormone on the second day after admission. He rapidly recovered. We reported here an atypical case of ADEM as to the antecedent infection and MRI lesion.

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Recent effective treatments for subacute sclerosing panencephalitis (SSPE) are oral inosiplex and intrathecal interferon therapy. The former seems to be effective for life expectancy but not for neurological disability, and the latter is a debating antiviral treatment although favorable reports are increasing.
A 12 year-10 month-old boy with SSPE was presented as a successful case of prolonged effectiveness in both neurological symptoms and life expectancy. There are no significant side effects by the 200 weeks treatment of intrathecal a -interferon of large dose (6 million unit/dose/week) and ordinary dose of inosiplex (100mg/kg/day).
Neurological disability index (Dyken) improved from 70% to 10%; speech and higher cerebral functions improved from aphasia and almost vegetative states to verbal communicable level; motor dysfunction from bedridden to wheel chair level; and myoclonic and other seizures were controlled.
Laboratory data also improved; periodic synchronized discharge (PSD) and other paroxysmal discharges disappeared and alpha activity that had once disappeared in the background activity of active stages reappeared in the EEGs. Measles antibodies in serum and CSF improved, and oligoclonal bands disappeared. However diffuse brain atrophy remained on neuroimaging.
Long-term intrathecal large dose treatment with α-interferon is effective and safe. It should be started as soon as the diagnosis is made, and a trial of large-dose and long-term therapy may be worthy even for more advanced cases irrespective of progress of neuroimages.

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We reported a patient with choreic movements, emotional lability, and compulsive-obsessive behavior that developed 4 weeks after onset of fever and lasted for several years. There was no evidence of streptococcal infection or rheumatic fever. T2-weighted MRI showed hyperintense lesions in the bilateral caudate nuclei and putamina. A diagnosis of focal encephalitis was made according to initial fever, convulsion, and CSF pleocytosis. Treatments with haloperidol and prednisolone were effective in some degree.
The neurobehavioral syndrome as well as the involuntary movements in this patient can be attributed to the striatal damage, which may disrupt the basal ganglia-limbic-frontal lobe tracts. A symptomatic similarity between the syndrome in this patient and chorea minor suggests a striatal damage in chorea minor, where the causative lesions remain unknown.

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We present an autopsy case of a severely handicapped 21-year-old woman. A subependymoma that was pathologically confirmed by biopsy was found at the age of 6 months, and radiation therapy was administered at 1 year. Thereafter, she developed severe growth retardation, spastic quadriplegia, profound mental retardation and intractable epilepsy. The patient died of acute cardiovascular failure at the age of 21. Unexpectedly, at autopsy more than 20 multiple meningiomas were found intracranially. Such a large number of meningiomas is rare, even though they might be induced by radiation. This case warns us of the use of radiotherapy for brain tumors in infancy, and points to the necessity of following such patients with radioimaging to detect radiation-induced brain tumors in an early stage.

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[in Japanese]

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[in Japanese]

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