Highlights
- •The prevalence of pilomotor seizures in patients affected by limbic encephalitis may be higher than expected.
- •LGI1 and GAD65 emerged as the most frequently associated antibodies to ictal piloerection.
- •SSR recording may help detecting ictal piloerection.
Abstract
Background
Ictal piloerection (IP) is a rare manifestation of focal epilepsy. Autoimmune limbic
encephalitis (LE) and malignant brain tumours are the most frequent recognized aetiologies.
Methods
We selected all patients diagnosed with LE in our Institute from 2004 to 2020 and
manifesting with IP. We performed a literature review on LE patients presenting IP.
Results
Of 15 patients diagnosed with LE (13.3%), two manifested IP as prominent ictal feature.
One of them also had stiff-limb syndrome. Video-EEG documented ictal discharges from
the right temporal regions with concomitant sympathetic skin response (SSR) recording.
Antibody testing showed elevated serum and CSF titres of GAD65 antibodies (Ab), in
both cases. Despite a combination of several anti-seizure medications and first- and
second-line immunotherapy, they showed a poor clinical outcome after 2 and 9 years
of follow-up, respectively. The literature review yielded 13 papers reporting 26 LE
cases with IP. LGI1 Ab were the most frequently associated (73.1%) followed by VGKC-complex
(7.7%), GAD65 (7.7%), NMDAr (3.8%), Ma2 (3.8%) and Hu (3.8%) Ab. Cases with LGI1 Ab
showed a good response to immunotherapy.
Discussion and conclusion
The prevalence of IP in our LE cohort was of 13.3%, higher than expected. According
to the literature review, most cases were associated with LGI1 Ab and showed a good
response to immunotherapy. With the contribution of our cases, GAD65 emerged as the
second most frequently detected Ab, showing a poor outcome. Our findings widen the
spectrum of IP-associated Ab, with the respective prognostic implications.
Keywords
Abbreviations:
IP (Ictal piloerection), LE (Limbic encephalitis), Ab (Antibodies)To read this article in full you will need to make a payment
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References
- Localising and lateralising value of ictal piloerection.J Neurol Neurosurg Psychiatry. 2004; 75: 879-883
- Autonomic auras: left hemispheric predominance of epileptic generators of cold shivers and goose bumps?.Epilepsia. 2002; 43: 41-45
- Pilomotor seizures: an autonomic semiology of limbic encephalitis?.Seizure. 2014; 23: 670-673
- Ictal piloerection is associated with high-grade glioma and autoimmune encephalitis-results from a systematic review.Seizure. 2019; 64: 1-5
- A clinical approach to diagnosis of autoimmune encephalitis.Lancet Neurol. 2016; 15: 391-404
- Searching for serum antibodies to neuronal proteins in patients with myalgic encephalopathy/chronic fatigue syndrome.Clin Ther. 2019; 41: 836-847https://doi.org/10.1016/j.clinthera.2019.04.001
- Orbitofrontal syndrome and “goosebumps”-a new manifestation of autoimmune epilepsy in anti-LGI1 encephalitis.Seizure. 2021; 86: 68-69
- Clinical features which predict neuronal surface autoantibodies in new-onset focal epilepsy: implications for immunotherapies.J Neurol Neurosurg Psychiatry. 2021; 92: 291-294
- Autoimmune epilepsy: clinical characteristics and response to immunotherapy.Arch Neurol. 2012; 69: 582-593
- Pilomotor seizures: an unusual presentation of limbic encephalitis.Acta Neurol Belg. 2016; 116: 365-367
- LGI1-antibody associated epilepsy successfully treated in the outpatient setting.J Neuroimmunol. 2020; 345577268
- Anti-LGI1 encephalitis clinical syndrome and long-term follow-up.Neurology. 2016; 4 (87): 1449-1456
- Pilomotor seizures marked by infraslow activity and acetazolamide responsiveness.Ann Clin Transl Neurol. 2019; 6: 167-173
- Pilomotor seizures and status in non-paraneoplastic limbic encephalitis.Epileptic Disord. 2005; 7: 205-211
- Pilomotor seizures in a patient with LGI1 encephalitis.Front Neurol. 2020; 11 (20): 61
- 16Evaluation of cognitive deficits and structural hippocampal damage in encephalitis with leucine-rich, glioma-inactivated 1 antibodies.JAMA Neurol. 2017; 74: 50-59
- LGI1-antibody encephalitis is characterised by frequent, multifocal clinical and subclinical seizures.Seizure. 2017; 50: 14-17
- Stop testing for autoantibodies to the VGKC-complex: only request LGI1 and CASPR2.Pract Neurol. 2020; 20: 377-384
- Intracellular and non-neuronal targets of voltage-gated potassium channel complex antibodies.J Neurol Neurosurg Psychiatry. 2017; 88: 353-361
- Refractory chronic epilepsy associated with neuronal auto-antibodies: could perisylvian semiology be a clue?.Epileptic Disord. 2017; 19: 439-449
- Neurological syndromes associated with anti-GAD antibodies.Int J Mol Sci. 2020; 21: 3701
- Sympathetic skin response Basic mechanisms and clinical applications.Clin Auton Res. 2003; 13: 256-270
- Strategies for non-EEG seizure detection and timing for alerting and interventions with tonic-clonic seizures.Epilepsia. 2018; 59: 36-41
Article info
Publication history
Published online: April 04, 2022
Accepted:
March 31,
2022
Received in revised form:
March 7,
2022
Received:
January 5,
2022
Identification
Copyright
© 2022 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.
