Potentially reversible autoimmune limbic encephalitis with neuronal potassium channel antibody

M. J. Thieben, V. A. Lennon, B. F. Boeve, A. J. Aksamit, M. Keegan, Steven Vernino

Research output: Contribution to journalArticle

335 Citations (Scopus)

Abstract

Objectives: To describe the clinical features and coexisting serum autoantibodies in seven patients with encephalitis associated with autoantibodies to α-dendrotoxin-sensitive voltage-gated potassium channels (VGKCs), and to compare this disorder with other autoimmune encephalopathies. Methods: Clinical information was obtained from a retrospective review of medical records and telephone interviews. All autoantibody testing was performed in a single laboratory. Results: The seven patients were examined for subacute cognitive and behavioral changes. Seizures, usually temporal-onset complex partial type, were documented in six patients, and all seven patients had EEG abnormalities. None had symptoms or signs of neuromuscular hyperexcitability. One described hypersalivation. Four patients had additional autoantibody markers of neurologic autoimmunity (muscle acetylcholine receptor, striational, P/Q-type calcium channel, or GAD65), and two had thyroperoxidase antibodies. Two patients had a history of cancer: one had active prostate adenocarcinoma, and the second had a remote history of tongue carcinoma. Cranial MRI demonstrated mesial temporal lobe abnormalities in all patients. One patient improved spontaneously, and six were treated with IV methylprednisolone. Three improved remarkably with treatment. At follow-up evaluation, one had no cognitive deficits, four had mild persistent short-term memory dysfunction, and two had persistent disabling behavioral deficits. Conclusions: Voltage-gated potassium channel antibodies are a valuable serologic marker of a potentially reversible autoimmune encephalopathy. The neurologic manifestations of this disorder are indistinguishable from paraneoplastic limbic encephalitis but are distinct from Morvan syndrome and Hashimoto encephalopathy.

Original languageEnglish (US)
Pages (from-to)1177-1182
Number of pages6
JournalNeurology
Volume62
Issue number7
StatePublished - Apr 13 2004

Fingerprint

Potassium Channels
Antibodies
Autoantibodies
Voltage-Gated Potassium Channels
Brain Diseases
Neuromuscular Manifestations
Q-Type Calcium Channels
P-Type Calcium Channels
Limbic Encephalitis
Sialorrhea
Autoimmune limbic encephalitis
Methylprednisolone
Cholinergic Receptors
Encephalitis
Temporal Lobe
Neurologic Manifestations
Nervous System Diseases
Autoimmunity
Short-Term Memory
Tongue

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Thieben, M. J., Lennon, V. A., Boeve, B. F., Aksamit, A. J., Keegan, M., & Vernino, S. (2004). Potentially reversible autoimmune limbic encephalitis with neuronal potassium channel antibody. Neurology, 62(7), 1177-1182.

Potentially reversible autoimmune limbic encephalitis with neuronal potassium channel antibody. / Thieben, M. J.; Lennon, V. A.; Boeve, B. F.; Aksamit, A. J.; Keegan, M.; Vernino, Steven.

In: Neurology, Vol. 62, No. 7, 13.04.2004, p. 1177-1182.

Research output: Contribution to journalArticle

Thieben, MJ, Lennon, VA, Boeve, BF, Aksamit, AJ, Keegan, M & Vernino, S 2004, 'Potentially reversible autoimmune limbic encephalitis with neuronal potassium channel antibody', Neurology, vol. 62, no. 7, pp. 1177-1182.
Thieben MJ, Lennon VA, Boeve BF, Aksamit AJ, Keegan M, Vernino S. Potentially reversible autoimmune limbic encephalitis with neuronal potassium channel antibody. Neurology. 2004 Apr 13;62(7):1177-1182.
Thieben, M. J. ; Lennon, V. A. ; Boeve, B. F. ; Aksamit, A. J. ; Keegan, M. ; Vernino, Steven. / Potentially reversible autoimmune limbic encephalitis with neuronal potassium channel antibody. In: Neurology. 2004 ; Vol. 62, No. 7. pp. 1177-1182.
@article{64100aeff14a45379f3966a77e0dba51,
title = "Potentially reversible autoimmune limbic encephalitis with neuronal potassium channel antibody",
abstract = "Objectives: To describe the clinical features and coexisting serum autoantibodies in seven patients with encephalitis associated with autoantibodies to α-dendrotoxin-sensitive voltage-gated potassium channels (VGKCs), and to compare this disorder with other autoimmune encephalopathies. Methods: Clinical information was obtained from a retrospective review of medical records and telephone interviews. All autoantibody testing was performed in a single laboratory. Results: The seven patients were examined for subacute cognitive and behavioral changes. Seizures, usually temporal-onset complex partial type, were documented in six patients, and all seven patients had EEG abnormalities. None had symptoms or signs of neuromuscular hyperexcitability. One described hypersalivation. Four patients had additional autoantibody markers of neurologic autoimmunity (muscle acetylcholine receptor, striational, P/Q-type calcium channel, or GAD65), and two had thyroperoxidase antibodies. Two patients had a history of cancer: one had active prostate adenocarcinoma, and the second had a remote history of tongue carcinoma. Cranial MRI demonstrated mesial temporal lobe abnormalities in all patients. One patient improved spontaneously, and six were treated with IV methylprednisolone. Three improved remarkably with treatment. At follow-up evaluation, one had no cognitive deficits, four had mild persistent short-term memory dysfunction, and two had persistent disabling behavioral deficits. Conclusions: Voltage-gated potassium channel antibodies are a valuable serologic marker of a potentially reversible autoimmune encephalopathy. The neurologic manifestations of this disorder are indistinguishable from paraneoplastic limbic encephalitis but are distinct from Morvan syndrome and Hashimoto encephalopathy.",
author = "Thieben, {M. J.} and Lennon, {V. A.} and Boeve, {B. F.} and Aksamit, {A. J.} and M. Keegan and Steven Vernino",
year = "2004",
month = "4",
day = "13",
language = "English (US)",
volume = "62",
pages = "1177--1182",
journal = "Neurology",
issn = "0028-3878",
publisher = "Lippincott Williams and Wilkins",
number = "7",

}

TY - JOUR

T1 - Potentially reversible autoimmune limbic encephalitis with neuronal potassium channel antibody

AU - Thieben, M. J.

AU - Lennon, V. A.

AU - Boeve, B. F.

AU - Aksamit, A. J.

AU - Keegan, M.

AU - Vernino, Steven

PY - 2004/4/13

Y1 - 2004/4/13

N2 - Objectives: To describe the clinical features and coexisting serum autoantibodies in seven patients with encephalitis associated with autoantibodies to α-dendrotoxin-sensitive voltage-gated potassium channels (VGKCs), and to compare this disorder with other autoimmune encephalopathies. Methods: Clinical information was obtained from a retrospective review of medical records and telephone interviews. All autoantibody testing was performed in a single laboratory. Results: The seven patients were examined for subacute cognitive and behavioral changes. Seizures, usually temporal-onset complex partial type, were documented in six patients, and all seven patients had EEG abnormalities. None had symptoms or signs of neuromuscular hyperexcitability. One described hypersalivation. Four patients had additional autoantibody markers of neurologic autoimmunity (muscle acetylcholine receptor, striational, P/Q-type calcium channel, or GAD65), and two had thyroperoxidase antibodies. Two patients had a history of cancer: one had active prostate adenocarcinoma, and the second had a remote history of tongue carcinoma. Cranial MRI demonstrated mesial temporal lobe abnormalities in all patients. One patient improved spontaneously, and six were treated with IV methylprednisolone. Three improved remarkably with treatment. At follow-up evaluation, one had no cognitive deficits, four had mild persistent short-term memory dysfunction, and two had persistent disabling behavioral deficits. Conclusions: Voltage-gated potassium channel antibodies are a valuable serologic marker of a potentially reversible autoimmune encephalopathy. The neurologic manifestations of this disorder are indistinguishable from paraneoplastic limbic encephalitis but are distinct from Morvan syndrome and Hashimoto encephalopathy.

AB - Objectives: To describe the clinical features and coexisting serum autoantibodies in seven patients with encephalitis associated with autoantibodies to α-dendrotoxin-sensitive voltage-gated potassium channels (VGKCs), and to compare this disorder with other autoimmune encephalopathies. Methods: Clinical information was obtained from a retrospective review of medical records and telephone interviews. All autoantibody testing was performed in a single laboratory. Results: The seven patients were examined for subacute cognitive and behavioral changes. Seizures, usually temporal-onset complex partial type, were documented in six patients, and all seven patients had EEG abnormalities. None had symptoms or signs of neuromuscular hyperexcitability. One described hypersalivation. Four patients had additional autoantibody markers of neurologic autoimmunity (muscle acetylcholine receptor, striational, P/Q-type calcium channel, or GAD65), and two had thyroperoxidase antibodies. Two patients had a history of cancer: one had active prostate adenocarcinoma, and the second had a remote history of tongue carcinoma. Cranial MRI demonstrated mesial temporal lobe abnormalities in all patients. One patient improved spontaneously, and six were treated with IV methylprednisolone. Three improved remarkably with treatment. At follow-up evaluation, one had no cognitive deficits, four had mild persistent short-term memory dysfunction, and two had persistent disabling behavioral deficits. Conclusions: Voltage-gated potassium channel antibodies are a valuable serologic marker of a potentially reversible autoimmune encephalopathy. The neurologic manifestations of this disorder are indistinguishable from paraneoplastic limbic encephalitis but are distinct from Morvan syndrome and Hashimoto encephalopathy.

UR - http://www.scopus.com/inward/record.url?scp=1842423491&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=1842423491&partnerID=8YFLogxK

M3 - Article

VL - 62

SP - 1177

EP - 1182

JO - Neurology

JF - Neurology

SN - 0028-3878

IS - 7

ER -