No to hattatsu. Brain and development
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We reported a surviving case of 6-year-old boy with pontine hematoma. He complained of headache as an initial symptom and developed progressively Foville's syndrome with impairments of the IX-XII cranial nerves. Although brainstem tumor was suspected initially using CT scan, MRI revealed the existence of hematoma in the ventromedial pons. ⋯ However, hemangiomatous lesion was suspected by subsequent serial MRIs with positive Gd-DTPA enhancement. Using conservative treatment including oral corticosteroids, all the neurological deficits disappeared in several months and he did not show any recurrence of clinical signs for 3 years. It was suggested that MRI was very useful in the differential diagnosis and the follow-up of hematoma in the posterior fossa.
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In order to reveal whether or not brain impairment occurs in the human fetus during the intrauterine period, we described three term fetuses, having successful prenatal diagnosis of localized brain lesion, through the real-time ultrasound observation of in utero behavioral patterns. Movement of extremities, breathing movement, alternation of REM (rapid eye movement) and NREM (non-rapid eye movement) periods, and the concurrence of regular mouthing movement with the NREM period were used as parameters. Despite normal breathing movement patterns, in case 1, there were no movements observed in any of the four extremities, thereby suggesting impairment of the spinal cord at and below C5. ⋯ As for case 3, although movement of extremities, breathing movement and REM/NREM period alternation were all found to be within the normal range, there was no concurrence between regular mouthing and the NREM period. This suggested impairment of brain function responsible for NREM sleep, located from the pons through the thalamocortical connection to the cerebral hemisphere. Neonatal CT were found to be in agreement, by indicating a lesion, diffusely involving the cerebral white matter.
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Case Reports
[Carnitine palmitoyltransferase deficiency in a patient with severe psychomotor retardation].
A 13-year-old girl who had severe brain damage due to unknown prenatal cause presented rhabdomyolysis triggered by a mild viral infection. Her muscle biopsy revealed mild variation in fiber size and type 2 fiber atrophy without excess lipid storage. Biochemical analysis of the biopsied material showed decreased carnitine palmitoyltransferase (CPT) activity (15% of the control). ⋯ She recovered from rhabdomyolysis without renal failure after a month with conservative therapy. CPT deficiency is usually found in young healthy persons. This is the first case report of CPT deficiency which presented severe psychomotor retardation since neonatal period.
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Intracranial hemorrhage is a fatal complication associated with aplastic anemia. We have encountered four patients who experienced six episodes of intracranial hemorrhage (intracerebral hemorrhage; 3, subarachnoid hemorrhage; 2, and subdural hematoma; 1,). Intracranial hemorrhage occurred without traumatic episodes, and was followed by frequent hemorrhagic episodes in other parts of about one year duration. ⋯ Continuous spinal drainage for subarachnoid hemorrhage was a relatively safe and useful means of management. In the patients who showed precipitous deterioration, the CT scan findings indicated that the high-density-area was diffusely spread with an unclear border, just like "contusional hemorrhage". For such severe cases, blood evacuation with craniotomy and emergency splenectomy are considered to be valuable.