scholarly journals Adrenomyeloneuropathy with cerebral involvement due to a novel frameshift variant in ABCD1 gene

2021 ◽  
Vol 23 (1) ◽  
pp. 61-64
Author(s):  
Hye Weon Kim ◽  
Hyunjin Kim ◽  
Dongyoung Jeong ◽  
Kyuyoon Chung ◽  
Eun-Jae Lee ◽  
...  
BMJ ◽  
1971 ◽  
Vol 1 (5751) ◽  
pp. 714-716 ◽  
Author(s):  
H. Smitskamp ◽  
F. H. Wolthuis

Parasitology ◽  
1995 ◽  
Vol 111 (4) ◽  
pp. 443-454 ◽  
Author(s):  
A. L. Neill ◽  
N. H. Hunt

SUMMARYCBA/T6 and DBA/2J mice inoculated withPlasmodium bergheiANKA (PbA) develop cerebral involvement 6–8 days post-inoculation, from which the CBA mice almost invariably die and the DBA mice recover. Dexamethasone (DXM; 80 mg/kg) given to inoculated CBA mice twice, on day 3 and again within 48 h, reduced the cerebral symptoms and prevented death from cerebral malaria. Plasma tumour necrosis factor (TNF) levels, which increased at the time of the cerebral symptoms, were also reduced in these DXM-treated mice. Intravenously administered Evans Blue, a dye which binds to albumin, diffused extensively across the blood-brain barrier only during the period of cerebral symptoms, in proportion to the severity of the cerebral symptoms and the disease. In PbA-infected CBA mice, cerebral symptoms and the amount of Evans Blue diffusing into the brain tissue were both reduced by DXM treatment, but only if the steroid was given on day 3 and again within 48 h. Endotoxin injected intravascularly into PbA-infected DBA mice after day 5 resulted in an exaggeration of cerebral symptoms and death between days 6 and 9. Plasma TNF and the amount of Evans Blue in the brain parenchyma increased above normal levels in these mice. Endotoxin injections had only minor effects on the severity of the cerebral symptoms in PbA-infected CBA mice and did not cause the animals to die sooner.


2018 ◽  
Vol 9 ◽  
Author(s):  
Lothar Hauth ◽  
Jeroen Kerstens ◽  
Laetitia Yperzeele ◽  
François Eyskens ◽  
Paul M. Parizel ◽  
...  

1994 ◽  
Vol 11 (2) ◽  
pp. 141
Author(s):  
Umbertina C. Reed ◽  
Suely K.N. Marie ◽  
Ana M.C.B. Tsanaclis ◽  
Mary S. Carvalho ◽  
Jayme Roizenblatt ◽  
...  

2014 ◽  
Vol 14 (7) ◽  
pp. 531-533 ◽  
Author(s):  
Ana C. Matos ◽  
Luis Figueira ◽  
Maria H. Martins ◽  
Manuela Matos ◽  
Márcia Morais ◽  
...  

2014 ◽  
Vol 55 (4) ◽  
pp. 1157 ◽  
Author(s):  
Joon Won Kang ◽  
Sang Mi Lee ◽  
Kyo Yeon Koo ◽  
Young-Mock Lee ◽  
Hyo Suk Nam ◽  
...  
Keyword(s):  

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Pelin Nar Senol ◽  
Aylin Bican Demir ◽  
Ibrahim Bora ◽  
Mustafa Bakar

Hashimoto’s encephalopathy is a rare disease which is thought to be autoimmune and steroid responsive. The syndrome is characterized by cognitive impairment, encephalopathy, psychiatric symptoms, and seizures associated with increased level of anti-thyroid antibodies. The exact pathophysiology underlying cerebral involvement is still lesser known. Although symptoms suggest a nonlesional encephalopathy in most of the cases, sometimes the clinical appearance can be subtle and may not respond to immunosuppressants or immunomodulatory agents. Here we report a case who presented with drowsiness and amnestic complaints associated with paroxysmal electroencephalography (EEG) abnormalities which could be treated only with an antiepileptic drug.


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