paroxysmal dysarthria-ataxia in multiple sclerosis
TRANSCRIPT
Neurology Publish Ahead of PrintDOI: 10.1212/WNL.0000000000011498
Paroxysmal Dysarthria-Ataxia in Multiple Sclerosis
Shailee Shah, MD1, Bryan T. Klassen, MD1, Eoin P. Flanagan, MD.1
1Departments of Neurology, Mayo Clinic College of Medicine, Rochester, MN, USA.
Summary Word Count: 105 words
Figure Legend Word Count: 53 words
Title Character Count: 50 characters
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Published Ahead of Print on January 5, 2021 as 10.1212/WNL.0000000000011498
Keywords: multiple sclerosis, ataxia, demyelination
Correspondence to:
Eoin P. Flanagan
E-mail: [email protected]
Study funding: No targeted funding reported.
Disclosures: The authors report no disclosures relevant to the manuscript.
Case Summary
A twenty-year-old man first presented with vertigo followed by hourly episodes of dysarthria and
incoordination lasting 5-20 seconds (video,http://links.lww.com/WNL/B304). Neurologic
examination was normal between episodes. The only brain/spine MRI lesion was in the right
superior cerebellar peduncle (Figure, A). Cerebrospinal fluid analysis revealed elevated white
blood cells (6/µL[normal, 0-5]) and oligoclonal bands(≥4). Paroxysmal dysarthria-ataxia
associated with CNS demyelination was diagnosed and oral acetazolamide 250mg twice-daily
resolved episodes without recurrence. Subsequent MRI brain (Figure, B) confirmed multiple
sclerosis (MS). Paroxysmal dysarthria-ataxia arises from ephaptic transmission within midbrain
or cerebellar MS lesions and additionally responds to carbamazepine.1 It should be
distinguished from genetic or contactin-associated-protein-like-2[CASPR2]-IgG-associated
episodic ataxia.2
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Figure 1 MRI brain demonstrates a T2-hyperintense right superior cerebellar peduncle lesion
(A.a, arrow) with ring enhancement (A.b, arrow). MRI two years later reveals new brain (B.a,
arrow) and spinal (B.b, arrow) demyelinating lesions.
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Figure 2 (Video,http://links.lww.com/WNL/B304) An episode of right upper extremity
dysmetria and ataxic dysarthria occurs upon reading at 15 seconds and resolves within 10
seconds.
Video - http://links.lww.com/WNL/B304 Teaching Slides - http://links.lww.com/WNL/B305
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References 1. Marcel C, Anheim M, Flamand-Rouviere C, et al. Symptomatic paroxysmal dysarthria-ataxia in demyelinating diseases. J Neurol 2010;257:1369-1372. 2. Joubert B, Gobert F, Thomas L, et al. Autoimmune episodic ataxia in patients with anti-CASPR2 antibody-associated encephalitis. Neurol Neuroimmunol Neuroinflamm 2017;4.
Acknowledgements: None
Appendix 1: Authors
Name Location Contribution
Shailee Shah, MD Mayo Clinic, Rochester Design and conceptualization of study
Bryan T. Klassen Mayo Clinic, Rochester Design and conceptualization of study
Eoin P. Flanagan, MD Mayo Clinic, Rochester Design and conceptualization of study, study supervision
ACCEPTED
Copyright © 2021 American Academy of Neurology. Unauthorized reproduction of this article is prohibited
DOI 10.1212/WNL.0000000000011498 published online January 5, 2021Neurology
Shailee Shah, Bryan T. Klassen and Eoin P. FlanaganParoxysmal Dysarthria-Ataxia in Multiple Sclerosis
This information is current as of January 5, 2021
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