Abstract：
UNLABELLED:A 2-year-old Japanese boy with a haemophagocytic lymphohistiocytosis (HLH) associated encephalopathy which developed after rotavirus infection is described. The neurological symptoms consisted of coma, seizures and spastic quadriplegia. On therapy with steroids, etoposide and cyclosporin A, the patient recovered without any neurological deficits. The interferon-gamma levels in serum and CSF were elevated at onset of the disease but had returned to normal at the time of clinical remission. Brain MRI revealed diffuse white matter abnormalities and parenchymal volume loss. Proton magnetic resonance spectroscopy revealed elevated lactate in the abnormal lesions observed on MRI, indicating that macrophages not exhibiting aerobic metabolism had infiltrated the CNS. At the time of clinical remission, the white matter abnormalities and brain lactate had disappeared. These findings suggested that the neurological symptoms resulted from the overproduction of cytokines by activated T-cells and macrophages. The pathophysiology of a HLH associated encephalopathy was considered to be a local immune response within the CNS, because interferon-gamma can induce the expression of major histocompatibility complex class I and II antigens on glial cells in the CNS. CONCLUSION:Haemophagocytic lymphohistiocytosis associated encephalopathy should be considered early in the differential diagnosis of cases with acute onset neuropathy.
journal_name
Eur J Pediatrjournal_title
European journal of pediatricsauthors
Takahashi S,Oki J,Miyamoto A,Koyano S,Ito K,Azuma H,Okuno Adoi
10.1007/s004310051033keywords:
subject
Has Abstractpub_date
1999-02-01 00:00:00pages
133-7issue
2eissn
0340-6199issn
1432-1076journal_volume
158pub_type
杂志文章,评审abstract：UNLABELLED:We describe a rare case of acute acalculous cholecystitis (AC) due to Salmonella group D infection in a previously healthy child who developed gall bladder empyema and bacteraemia. Salmonella group D was recovered from blood culture, as well as cultures of stool, bile and gall bladder wall samples. The patie...
journal_title：European journal of pediatrics
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