Annals of Indian Academy of Neurology
  Users Online: 1754 Home | About the Journal | InstructionsCurrent Issue | Back IssuesLogin      Print this page Email this page  Small font size Default font size Increase font size

Table of Contents
Year : 2021  |  Volume : 24  |  Issue : 4  |  Page : 589

A case of paroxysmal kinesigenic myoclonus with episodic piloerection

Department of Neurology, Kerala Institute of Medical Sciences, Thiruvananthapuram, Kerala, India

Date of Submission24-Oct-2020
Date of Acceptance04-Dec-2020
Date of Web Publication19-Jan-2021

Correspondence Address:
Dr. C J Suresh Chandran
Department of Neurology, Kerala Institute of Medical Sciences, Thiruvananthapuram, Kerala
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/aian.AIAN_1124_20

Rights and Permissions


How to cite this article:
Suresh Chandran C J, Sugunan A, Shibu J. A case of paroxysmal kinesigenic myoclonus with episodic piloerection. Ann Indian Acad Neurol 2021;24:589

How to cite this URL:
Suresh Chandran C J, Sugunan A, Shibu J. A case of paroxysmal kinesigenic myoclonus with episodic piloerection. Ann Indian Acad Neurol [serial online] 2021 [cited 2022 Oct 5];24:589. Available from:

A 45-year-old lady presented with 3 weeks history of very brief episodic involuntary posturing on initiating movements. The episodes consisted of mild neck flexion with outstretching of both upper limbs lasting 1–2 s. She was fully conscious and there were no facial movements. These occurred exclusively on movement and had several episodes per day. They were absent in sleep. She also had short-lasting episodes of goosebumps all over body. She was fully conscious during these episodes of goosebumps which use to last 10-20 seconds. Clinical examination revealed episodic piloerection and mild episodic memory decline. A video EEG showed paroxysmal Kinesigenic myoclonus [Video 1]. Interictal EEG was normal and ictal EEG showed EMG artifacts only. MRI brain showed limbic encephalitis [Figure 1]. Serum and CSF anti LGI1 antibody was positive. She was treated with pulse methylprednisolone followed by oral prednisolone, azathioprine and oxcarbazepine. She responded in 3 weeks, both piloerection and involuntary movements disappeared. Medications were tapered off after 1 year and she is in remission at 2-year follow-up.
Figure 1: MRI Brain FLAIR axial image showing limbic encephalitis

Click here to view

Prototypical paroxysmal dyskinesia in LGI1 encephalitis is facio brachial dystonic seizure (FBDS).[1] Paroxysmal kinesogenic dyskinesia has been rarely described in anti-LGI1 encephalitis and other autoimmune encephalitis.[1],[2] Pilomotor seizures are unusual autonomic seizures usually associated with temporal lobe epilepsy (TLE). Rarely frontal and hypothalamic origin also have been reported. Unilateral piloerection may indicate an ipsilateral focus, whereas bilateral piloerection does not have any clear lateralizing value.[3] Association of Piloerection to autoimmune encephalitis is recently recognized, anti-LGI1 encephalitis being the commonest.[1],[4]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Balint B, Vincent A, Meinck H-M, Irani SR, Bhatia KP. Movement disorders with neuronal antibodies: Syndromic approach, genetic parallels and pathophysiology. Brain 2018;141:13-36.  Back to cited text no. 1
Rahangdale R, Scott T, Leichliter T, Baser S, Valeriano J. A case of paroxysmal dystonia associated with LGI-1 antibody encephalitis. Clin Neurol Neurosurg 2019;186:105508.  Back to cited text no. 2
Loddenkemper T, Kellinghaus C, Gandjour J, Nair DR, Najm IM, Bingaman W, et al. Localising and lateralising value of ictal piloerection. J Neurol Neurosurg Psychiatry 2004;75:879-83.  Back to cited text no. 3
Rocamora R, Becerra JL, Fossas P, Gomez M, Vivanco-Hidalgo RM, Mauri JA, et al. Pilomotor seizures: An autonomic semiology of limbic encephalitis? Seizure 2014;23:670-3.  Back to cited text no. 4


  [Figure 1]


Print this article  Email this article


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Article in PDF (408 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

    Article Figures

 Article Access Statistics
    PDF Downloaded70    
    Comments [Add]    

Recommend this journal