We report 10 cases of pulmonary atelectasis diagnosed by chest computed tomography in patients with neurological or muscular disease. Atelectasis was frequently seen in hypotonic patients who could not roll over on their own. The atelectases located mostly in the dorsal bronchopulmonary segments, adjacent to the heart or diaphragm. Atelectasis diminished in two patients after they became able to roll themselves over. Gravity-related lung compression by the heart and intra-abdominal organs on persistent supine position cancause pulmonary atelectasis in patients with neurological or muscular disease who can not roll over by their own power.
To confirm that the prone position reduces compression of the lungs, chest computed tomography was performed in both the supine and the prone position in three patients. Sagittal images with three-dimensional computed tomographic reconstruction revealed significant sternad displacements of the heart and caudal displacements of the dorsal portion of the diaphragm on prone position compared with supine position. The prone position, motor exercises for rolling over, and biphasic cuirass ventilation are effective in reducing gravity-related lung compression. Some patients with intellectual disabilities were also able to cooperate in chest physiotherapy. Chest physiotherapy is useful in preventing atelectasis in patients with neurological or muscular disease.

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We introduced an intramuscular injection of botulinum toxin type A (hereafter, BTX-A) in 5 cases of cervical dystonia (CD) with generalized muscular hypertonia (GMH). The patients all had severe motor and intellectual disabilities (SMID). The initial injections (first and second) of BTX-A exerted a focal muscular relaxant effect in all 5 cases. It is interesting that they decreased hypertonia in the lower extremities, which resulted in an improved activity of daily living (ADL) in 4 cases (80%). This fact reveals theinvolvement of many muscles in regard to CD and GMH, especially in patients with severe cerebral palsy. As for adverse effects, a mild increase of saliva and cough indicated transient misswallowing in 2 cases (40%) and changes in the pattern of head movement and posture in 2 cases (40%). These effects were all mild and did not disturb ADL in any patient. BTX-A is considered to be safer and worthier of trying to decrease complaints related to CD and GMH than we expected. Furthermore, a modified Tsui's score cannot explain the many benefits from BTX-A in the treatment of SMID. For a reliable assessment of BTX-A therapy used to treat CD and GMH, especially in persons with SMID, more expressible and specific assessment methods will need to be established.

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We investigated modality-specific changes in N400 event related potential using a semantic category decision task in 38 control subjects and 8 patients with developmental dyslexia. In control children under 10 years old, auditory N400 showed a negative deflection over the fronto-centro-parietal areas with substantial amplitude. Control children over 10 years old showed a similar pattern of N400 waves in a visual and an auditory-visual modality, suggesting that the visual modality becomes dominant in the late teens. Dyslexic children showed more errors on a visual than auditory modality task with poorer N400 waves for visual stimuli. However, peak latencies of N400 in an auditoryvisual modality were almost the same for auditory stimuli in control children. Differences in the N400 pattern in children might reflect the fragility and reversibility of the semantic processes through stimulus modalities.

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Children with sequelae of perinatal hypoxic-ischemic encephalopathy (HIE) occasionally suffer from cytokine-related disease.We investigated 12 children with perinatal HIE sequelae, who died in childhood, concerning (1) the incidence of cytokine-related disease as the cause of death, and (2) the characteristics of the cytokine-related disease. Six (50%) of the 12 patients died from cytokine-related disease: two had virus-associated hemophagocytic syndrome (VAHS); one had acute encephalopathy; one had systemic inflammatory response syndrome (SIRS); and two had severe pneumonia/acute respiratory distress syndrome (ARDS). These six patients presented with increased liver transaminase, LDH, and CK, and decreased platelet count and albumin. This study shows the high incidence of cytokinerelated disease as the cause of death in children with perinatal HIE sequelae. Further investigation is needed to clarify the pathogenesis of this disease.

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We experienced an 8-year-old-boy with non-herpetic acute limbic encephalitis (NHALE), who developed headache, convulsion, consciousness disturbance, and ataxia following cold like symptoms. Disturbance of short term memory and a change of character were recognized. Myoclonic seizures and generalized tonic clonic convulsions developed, that responded to antiepileptic agents. Although other symptoms resolved spontaneously, short term memory disturbance persisted. Brain MRI demonstrated the lesion involving the bilateral claustrum and right hippocampus. Three months later, the lesion in the claustrum disappeared, but the hippocampus still showed slight hyperintensity on FLAIR image of MRI. Autoantibodies against glutamine receptor were detected in the cerebrospinal fluid and plasma, which suggested the involvement of immunologic disturbances in this disease. In NHALE, many cases have been reported in adults but not in children, and the further attentions should be paid to childhood-onset NHALE.

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A boy with bilateral hearing impairment developed pneumococcal meningitis at 14-month-old. Further examination revealed cerebrospinal fluid leakage due to bilateral Mondini dysplasia. He was cured by treatment with panipenem/betamiprone and dexamethasone, and then, he was performed an operation to fill the inner ear on day 30. He did not have bacterial meningitis 19 months after the operation. Children with congenital hearing impairment should be examined for malformation of the inner ear because the inner ear malformation has cerebrospinal fluid leakage and bacterial meningitis frequently.

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Tracheo-innominate artery fistula (TIF) is one of the most important complications of tracheostomy in that it is often life threatening. We experienced two cases with TIF and tracheo-innominate artery allovascularity, in which three-dimensional helical CT (3DCT) was useful in making the diagnosis of TIF and tracheo-innominate artery allovascularity. In one case, acute hemorrhage occurred during the procedure of routine tube exchange, and hemostasis was achieved by inflating the cuff of the tracheostomy tube. After TIF was confirmed by 3DCT, surgical ligation of the innominate artery was performed. In the other case, the recognition of a pulsating movement of the tracheostomy tube prompted us to perform 3DCT, which demonstrated a collateral branch from the innominate artery to the trachea. Laryngotracheal separation was performed to prevent hemorrhagic events in the future. For patients with severe neurological disorders, 3DCT has the advantage that only a short time is required to produce a digtal image demonstrating the structure surrounding the trachea, which is necessary for the management of the patients'airway.

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We report here two boys who presented with atrophy of the right calf muscle. The onset was insidious and the symptom was found in infancy in case 2. They were followed for more than 5 years and no progression was seen. No sensory disturbances or autonomic nervous system symptoms were observed. Although needle electric myograph and muscle biopsy findings showed a neurogenic pattern, no cause was confirmed.
Recently, a new disease concept of “benign monomelic amyotrophy of lower limb” was established and this could be applied to these patients. This disorder is rare and it is seen in young adults. It is characterized by non-progressive unilateral calf muscle atrophy. There is almost no possibility that this disorder is a variant of spinal muscular atrophy.
When a patient presents with unilateral calf muscle atrophy, the treatable causes of the atrophy, including spinal cord disorders or peripheral nerve disorders, should be excluded first. If a confirmed cause cannot be found, then there should be a careful follow-up of the patient, even if the symptoms are stable.

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A 1-year-old girl with influenza-associated encephalopathy initially exhibited prolonged febrile convulsions and subsequent drowsiness. She became unconsciousness and experienced a cluster of seizures 4 days later. Diffusion-weighted magnetic resonance imaging (DWI) showed high signal intensity in the bilateral frontal white matter. This signal change migrated to the bifrontal cortical areas and the caudate nuclei within 10 days, when T, elongation appeared over the gray and white matter of frontal lobes. Choreoathetosis and oculogyric crisis were transiently noted at this period. Frontal lobe signs, including the forded mouth opening response, after diminution of these movement disorders. The DWI signal change subsequently resolved and frontal cortical atrophy appeared thereafter. Levels of inflammatory cytokines in the cerebrospinal fluid were normal during the acute phase of clinical course. The biphasic clinical course with initial prolonged seizure, involvement of the frontal lobes, and the progression of signal change on DWI from white to gray matter, meets the characteristics of “status epilepticus-type acute encephalopathy” suggested by Shiomi et al. Although pentobarbital infusion, steroid pulse therapy and mild hypothermia did not show any apparent effects on the clinical course of this patient, further trial of these therapies may be warranted for the treatment of this type of encephalopathy.

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[in Japanese]

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