PMID- 32662046
OWN - NLM
STAT- MEDLINE
DCOM- 20211005
LR  - 20211005
IS  - 1559-1166 (Electronic)
IS  - 0895-8696 (Linking)
VI  - 71
IP  - 2
DP  - 2021 Feb
TI  - Movement Disorder in Wilson Disease: Correlation with MRI and Biomarkers of Cell 
      Injury.
PG  - 338-346
LID - 10.1007/s12031-020-01654-0 [doi]
AB  - To report the phenomenology of movement disorder (MD) in neurological Wilson 
      disease (NWD), and correlate these with MRI, and biomarkers of oxidative stress, 
      excitotoxicity, and inflammation. Eighty-two patients were included, and their 
      phenomenology of MD was categorized. The severity of dystonia was assessed using 
      the Burke-Fahn-Marsden score, and chorea, athetosis, myoclonus, and tremor on a 
      0-4 scale. The MRI changes were noted. Serum glutamate, cytokines, and oxidative 
      stress markers were measured. Movement disorders were noted in 78/82 (95.1%) 
      patients and included dystonia in 69 (84.1%), chorea in 31 (37.8%), tremor in 24 
      (29.3%), parkinsonism in 19 (23.2%), athetosis in 13 (15.9%), and myoclonus in 9 
      (11.0%) patients. Dystonia was more frequently observed in the patients with 
      thalamic (76.8 vs 23.2%), globus pallidus (72.0 vs 28.0%), putamen (69.5 vs 
      30.5%), caudate (68.3 vs 31.7%) and brainstem (61.0 vs 39.0%) involvement, and 
      tremor with cerebellar involvement (37.5 vs 5.2%). The median age of onset of 
      neurological symptoms was 12 (5-50) years. WD patients had higher levels of 
      malondialdehyde (MDA), glutamate, and cytokines (IL-6, IL-8, IL-10, and TNFalpha) and 
      lower levels of glutathione and total antioxidant capacity (TAC) compared with 
      the controls. Serum glutamate, IL-6, IL-8, and plasma MDA levels were increased 
      with increasing neurological severity, while glutathione and TAC levels 
      decreased. The severity of dystonia related to the number of MRI lesions. MD is 
      the commonest neurological symptoms in WD. Oxidative stress, glutamate, and 
      cytokine levels are increased in WD and correlate with neurological severity.
FAU - Kalita, Jayantee
AU  - Kalita J
AUID- ORCID: 0000-0002-6141-3592
AD  - Department of Neurology, Sanjay Gandhi Post Graduate Medical Sciences, Raebareily 
      Road, Lucknow, 226014, India. jayanteek@yahoo.com.
FAU - Kumar, Vijay
AU  - Kumar V
AD  - Department of Biotechnology, Yeungnam University, Gyeongsan, Gyeongbuk, 38541, 
      Republic of Korea.
FAU - Misra, Usha K
AU  - Misra UK
AD  - Department of Neurology, Sanjay Gandhi Post Graduate Medical Sciences, Raebareily 
      Road, Lucknow, 226014, India.
FAU - Kumar, Sunil
AU  - Kumar S
AD  - Department of Radiology, Sanjay Gandhi Post Graduate Medical Sciences, Lucknow, 
      226014, India.
LA  - eng
GR  - 39:287/Sanjay Gandhi Post Graduate Medical Sciences, Lucknow, India/
PT  - Journal Article
DEP - 20200713
PL  - United States
TA  - J Mol Neurosci
JT  - Journal of molecular neuroscience : MN
JID - 9002991
RN  - 0 (Biomarkers)
RN  - 0 (Cytokines)
RN  - 0 (Thiobarbituric Acid Reactive Substances)
RN  - 3KX376GY7L (Glutamic Acid)
RN  - 4Y8F71G49Q (Malondialdehyde)
RN  - GAN16C9B8O (Glutathione)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Age of Onset
MH  - Biomarkers/blood
MH  - Child
MH  - Cytokines/blood
MH  - Disease Progression
MH  - Dyskinesias/blood/diagnostic imaging/etiology
MH  - Female
MH  - Glutamic Acid/blood
MH  - Glutathione/blood
MH  - Hepatolenticular Degeneration/*physiopathology
MH  - Humans
MH  - *Magnetic Resonance Imaging
MH  - Male
MH  - Malondialdehyde/blood
MH  - Movement Disorders/blood/diagnostic imaging/*etiology
MH  - *Neuroimaging
MH  - Oxidative Stress
MH  - Severity of Illness Index
MH  - Thiobarbituric Acid Reactive Substances/analysis
MH  - Young Adult
OTO - NOTNLM
OT  - Dystonia
OT  - Glutamate
OT  - Myoclonus
OT  - Oxidative stress
OT  - Parkinsonism
OT  - Wilson disease
EDAT- 2020/07/15 06:00
MHDA- 2021/10/06 06:00
CRDT- 2020/07/15 06:00
PHST- 2020/05/19 00:00 [received]
PHST- 2020/06/30 00:00 [accepted]
PHST- 2020/07/15 06:00 [pubmed]
PHST- 2021/10/06 06:00 [medline]
PHST- 2020/07/15 06:00 [entrez]
AID - 10.1007/s12031-020-01654-0 [pii]
AID - 10.1007/s12031-020-01654-0 [doi]
PST - ppublish
SO  - J Mol Neurosci. 2021 Feb;71(2):338-346. doi: 10.1007/s12031-020-01654-0. Epub 
      2020 Jul 13.