Gastroenteritis associated encephalopathy with reversible splenial lesion: three cases

2012 ◽  
Vol 43 (02) ◽  
Author(s):  
S Leiz ◽  
U Hiener ◽  
J Peters ◽  
M Baethmann
Keyword(s):  
2021 ◽  
Vol 41 (4) ◽  
pp. 815-820
Author(s):  
Xu-fang Li ◽  
Bin Ai ◽  
Jia-wei Ye ◽  
Li-mei Tan ◽  
Hua-mei Yang ◽  
...  

Author(s):  
J. Chauffier ◽  
N. Poey ◽  
M. Husain ◽  
T. de Broucker ◽  
A. Khalil ◽  
...  
Keyword(s):  

2021 ◽  
Author(s):  
Jiao Xue ◽  
Ying Zhang ◽  
Jie Kang ◽  
Chongfeng Duan ◽  
Zhi Yi ◽  
...  
Keyword(s):  

Neurocase ◽  
2018 ◽  
Vol 24 (4) ◽  
pp. 220-226 ◽  
Author(s):  
Tigran Kesayan ◽  
Hamlet Gasoyan ◽  
Kenneth M. Heilman

Author(s):  
Rena Okada ◽  
Yuri Sakaguchi ◽  
Takeshi Matsushige ◽  
Isamu Kamimaki ◽  
Toshiki Takenouchi ◽  
...  

Background: Acute encephalopathy during childhood represents a highly heterogeneous group of infectious and non-infectious pathologies. According to a recent nationwide survey on acute childhood encephalopathy in Japan, the combination of clinical and radiographic features left approximately half of the affected children unclassified, mainly because of the lack of disease-specific biomarkers. Case: Herein, we document a school-aged boy who manifested with acute encephalopathy that was characterized by a prolonged fever, altered mental status, urinary retention, and intention tremor lasting for more than a month. Accompanying features included syndrome of inappropriate secretion of antidiuretic hormone, pleocytosis with elevated interleukin-6 and interferon-gamma levels in the cerebrospinal fluid, and a transient splenial lesion on neuroimaging. No pathogens were identified, and C-reactive protein was negative throughout his clinical course. This constellation of clinical features was not compatible with any of the existing entities of acute pediatric encephalopathy. Discussion: Our retrospective literature review identified two additional school-aged male patients who exhibited highly similar clinical courses. The prolonged altered mental status with pleocytosis in the cerebrospinal fluid and a transient splenial lesion in the absence of serum inflammatory markers suggest a primary central nervous system pathology. Conclusion: This combination of features defines this presumably new group of acute childhood febrile encephalopathy with prolonged fever and ataxia in school-aged boys.


2020 ◽  
Author(s):  
xiaoqing li ◽  
fei han ◽  
qianlong chen ◽  
tienan zhu ◽  
yongqiang zhao ◽  
...  

Abstract Background: Reversible splenial lesion syndrome (RESLES) is a clinico-radiological syndrome characterized by the presence of reversible lesions specifically involving the splenium of the corpus callosum (SCC). The cause of RESLES is unknown. However, infectious-related mild encephalitis/encephalopathy (MERS) with a reversible splenial lesion remains the most common cause of reversible splenial lesions. Acute intermittent porphyria (AIP) is an autosomal dominant disorder caused by a partial deficiency of porphobilinogen deaminase (PBGD), the third enzyme in the heme biosynthetic pathway. It can affect the autonomic, peripheral, and central nervous system. Result: In this study, we report a 20-year-old woman with AIP who presented with MRI manifestations suggestive of RESLES, she had a novel HMBS nonsense mutation, a G to A mutation in base 594, which changed tryptophan to a stop codon (W198*). Conclusion: To the best of our knowledge, this is only one published case of RELES associated with AIP.


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