Convulsion, disturbance of consciousness, fever
10.3760/cma.j.issn.2095-428X.2018.22.015
- VernacularTitle:抽搐,意识障碍,发热
- Author:
Xinying YANG
1
;
Tongli HAN
;
Fang FANG
;
Hua CHENG
;
Rui ZHANG
Author Information
1. 首都医科大学附属北京儿童医院神经内科
- Keywords:
Demyelinating disease of central nervous system;
Acute disseminated encephalomyelitis;
Familial haemophagocytosis syndrome
- From:
Chinese Journal of Applied Clinical Pediatrics
2018;33(22):1739-1744
- CountryChina
- Language:Chinese
-
Abstract:
The child was a preschool girl who was admitted for the first time due to " convulsions,disturbance of consciousness and fever for 2 days".The girl was diagnosed as acute disseminated encephalomyelitis (ADEM).After immunotherapy,the girl recovered.During the course of hormone reduction,the cranial magnetic resonance imaging showed aggravated,and neurological symptoms were followed.There was a transient decrease of blood cells.After repeat hormone treatment,the girl was improved again,but the intracranial lesions still showed progressive aggravation tendency.The course of central nervous system demyelinating in this patient was unknown.When her younger sibling prestentd fever,hepatosplenomegaly,blood cell decreased and was diagnosed with familial haemophagocytosis syndrome(FHPS),through case discussion and gene testing,the girl was finally diagnosis "central nervous system involvement at the onset of FHPS".The case suggests unexplained central nervous system demyelinating lesions,regardless of whether there are abnormalities of blood system,should pay attention to haemophagocytosis syndrome.Positive immunotherapy should be given after diagnosis,and stem cell transplantation should be given as soon as possible to improve prognosis.
