Pilomotor seizures: An autonomic semiology of limbic encephalitis?

Rocamora, Rodrigo; Becerra, Juan Luís; Fossas, P; Gómez, M.J. Gil De; Vivanco-Hidalgo, Rosa María; Mauri, J.A.; Molins, A

doi:10.1016/j.seizure.2014.04.013

Cited by 90 publications

(61 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…FBDS seems to be related with LGI1 antibodies, since no FBDS was reported in 5 Korean patients with CASPR2 antibodies [Sunwoo et al, 2015]. Even though ictal and peri-ictal dysautonomic features such as tachychardia, blood pressure abnormalities, or hypersalivation are primarily observed in anti-NMDAR encephalitis (see above, Part 1) or Caspr2 encephalitis [Baysal-Kirac et al, 2016], pilomotor seizures [Rocamora et al, 2014] and bradycardia [Naasan et al, 2014] were respectively described in 3/5 and in 3/14 patients in association with LGI1 antibodies.…”

Section: Limbic Encephalitis Associated With Anti-mentioning

confidence: 96%

Seizures and Epilepsies due to Channelopathies and Neurotransmitter Receptor Dysfunction: A Parallel between Genetic and Immune Aspects

Lascano¹,

Korff

Picard³

2016

Mol Syndromol

View full text Add to dashboard Cite

Despite intensive research activity leading to many important discoveries, the pathophysiological mechanisms underlying seizures and epilepsy remain poorly understood. An important number of specific gene defects have been related to various forms of epilepsies, and autoimmunity and epilepsy have been associated for a long time. Certain central nervous system proteins have been involved in epilepsy or acute neurological diseases with seizures either due to underlying gene defects or immune dysfunction. Here, we focus on 2 of them that have been the object of particular attention and in-depth research over the past years: the N-methyl-D-aspartate receptor and the leucin-rich glioma-inactivated protein 1 (LGI1). We also describe illustrative examples of situations in which genetics and immunology meet in the complex pathways that underlie seizures and epilepsy.

show abstract

Section: Limbic Encephalitis Associated With Anti-mentioning

confidence: 96%

Seizures and Epilepsies due to Channelopathies and Neurotransmitter Receptor Dysfunction: A Parallel between Genetic and Immune Aspects

Lascano¹,

Korff

Picard³

2016

Mol Syndromol

View full text Add to dashboard Cite

show abstract

“…In particular, the strong association of FBDS with LGI1 antibodies or the unusual type of seizure semiology including autonomic or pilomotor seizures in LGI1 patients23 suggests that bizarre, isolated phenotypes of movement or cognition can occur that may have led to consideration of a psychosomatic disease in several previous cases. However, there are difficulties in attributing cognitive impairment to VGKC antibodies in some patients 24.…”

Section: Cognitive Impairment and Vgkc Complex Antibodiesmentioning

confidence: 99%

Emerging psychiatric syndromes associated with antivoltage-gated potassium channel complex antibodies

Prüß

Lennox

2016

J Neurol Neurosurg Psychiatry

View full text Add to dashboard Cite

Antibodies against the voltage-gated potassium channel (VGKC) were first recognised as having a potential pathogenic role in disorders of the central nervous system in 2001, with VGKC antibodies described in patients with limbic encephalitis, and the subsequent seminal paper describing the clinical phenotype and immunotherapy treatment responsiveness in 13 patients with VGKC antibodies and limbic encephalitis in 2004. These initial case descriptions were of a progressive neuropsychiatric syndrome with abnormalities of mood, sleep and cognition recognised alongside the neurological symptoms of seizures and autonomic instability. The clinical syndromes associated with VGKC complex (VGKCC) antibodies have broadened considerably over the last 15 years, with multiple cases of more restricted 'formes fruste' presentations associated with VGKCC antibodies being described. However, the relevance of antibodies in these cases has remained controversial. The understanding of the pathogenic nature of VGKC antibodies has further advanced since 2010 with the discovery that VGKC antibodies are not usually antibodies against the VGKC subunits themselves, but instead to proteins that are complexed with the potassium channel, in particular leucine-rich, glioma-inactivated protein 1 (LGI1) and contactin-associated protein 2 (Caspr2). Antibodies against these proteins have been associated with particular, although overlapping, clinical phenotypes, each also including neuropsychiatric features. Our aim is to critically review the association between VGKCC, LGI1 and Caspr2 antibodies with isolated psychiatric presentations-with a focus on cognitive impairment, mood disorders and psychosis. We recommend that screening for VGKCC, LGI1 and Caspr2 antibodies be considered for those with neuropsychiatric presentations.

show abstract

“…[12] About 106 cases of pilomotor seizures have been reported in literature. [12345678910111213] Out of these, 31 had isolated pilomotor phenomenon without any other aura or dialeptic features. The majority of patients reported in literature were noted to have ictal onset over the temporal region.…”

mentioning

confidence: 99%

“…The majority of patients reported in literature were noted to have ictal onset over the temporal region. [126710] Piloerection is mostly unilateral or initially unilateral with later contralateral spread, and at times it can be bilateral at onset. Although majority had left temporal localization, one-third (35 cases) had right temporal localization.…”

mentioning

confidence: 99%

See 1 more Smart Citation

Piloerection: A rare ictal phenomenon – Case report and review of literature

Panda

Agarwal

Sharma

2017

Ann Indian Acad Neurol

View full text Add to dashboard Cite

Pilomotor seizures: An autonomic semiology of limbic encephalitis?

Abstract: Our series describes a so far not well-recognized autoimmune association of pilomotor seizures to limbic encephalitis. This etiology should be ruled out through a comprehensive diagnostic work-up even in cases of long-lasting TLE with typical hippocampal atrophy on MRI.

Cited by 90 publications

References 10 publications

Seizures and Epilepsies due to Channelopathies and Neurotransmitter Receptor Dysfunction: A Parallel between Genetic and Immune Aspects

Seizures and Epilepsies due to Channelopathies and Neurotransmitter Receptor Dysfunction: A Parallel between Genetic and Immune Aspects

Emerging psychiatric syndromes associated with antivoltage-gated potassium channel complex antibodies

Piloerection: A rare ictal phenomenon – Case report and review of literature

Contact Info

Product

Resources

About