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April 12, 2004

Potentially reversible autoimmune limbic encephalitis with neuronal potassium channel antibody

April 13, 2004 issue
62 (7) 1177-1182

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.

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References

1.
Buckley C, Oger J, Clover L, et al. Potassium channel antibodies in two patients with reversible limbic encephalitis. Ann Neurol. 2001; 50: 73–78.
2.
Liguori R, Vincent A, Clover L, et al. Morvan’s syndrome: peripheral and central nervous system and cardiac involvement with antibodies to voltage-gated potassium channels. Brain. 2001; 124: 2417–2426.
3.
Vernino S, Lennon VA. Ion channel and striational antibodies define a continuum of autoimmune neuromuscular hyperexcitability. Muscle Nerve. 2002; 26: 702–707.
4.
Hart IK, Maddison P, Newsom-Davis J, Vincent A, Mills KR. Phenotypic variants of autoimmune peripheral nerve hyperexcitability. Brain. 2002; 125: 1887–1895.
5.
Schott JM, Harkness K, Barnes J, et al. Amnesia, cerebral atrophy, and autoimmunity. Lancet. 2003; 361: 1266.
6.
Pozo-Rosich P, Clover L, Saiz A, Vincent A, Graus F. Voltage-gated potassium channel antibodies in limbic encephalitis. Ann Neurol. 2003; 54: 530–533.
7.
Brierly J, Corsellis J. Subacute encephalitis of later adult life mainly affecting the limbic areas. Brain. 1960; 83: 356–368.
8.
Gultekin SH, Rosenfeld MR, Voltz R, et al. Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain. 2000; 123: 1481–1494.
9.
Lawn ND, Westmoreland BF, Kiely MJ, Lennon VA, Vernino S. Clinical, magnetic resonance imaging, and electroencephalographic findings in paraneoplastic limbic encephalitis. Mayo Clin Proc. 2003; 78: 1363–1368.
10.
Cunningham JD, Burt ME. Limbic encephalitis secondary to malignant thymoma. Ann Thorac Surg. 1994; 58: 250–251.
11.
McArdle JP, Millingen KS. Limbic encephalitis associated with malignant thymoma. Pathology. 1988; 20: 292–295.
12.
Voltz R, Gultekin SH, Rosenfeld MR, et al. A serologic marker of paraneoplastic limbic and brain-stem encephalitis in patients with testicular cancer. N Engl J Med. 1999; 340: 1788–1795.
13.
Wingerchuk DM, Noseworthy JH, Kimmel DW. Paraneoplastic encephalomyelitis and seminoma: importance of testicular ultrasonography. Neurology. 1998; 51: 1504–1507.
14.
Caselli RJ, Boeve BF, Scheithauer BW, O’Duffy JD, Hunder GG. Nonvasculitic autoimmune inflammatory meningoencephalitis (NAIM): a reversible form of encephalopathy. Neurology. 1999; 53: 1579–1581.
15.
Castillo P, Boeve B, Schäuble B, et al. Steroid-responsive encephalopathy associated with thyroid autoimmunity: clinical and laboratory findings. Neurology. 2002; 58: A248. Abstract.
16.
Shaw PJ, Walls TJ, Newman PK, Cleland PG, Cartlidge NE. Hashimoto’s encephalopathy: a steroid-responsive disorder associated with high anti-thyroid antibody titers—report of 5 cases. Neurology. 1991; 41: 228–233.
17.
Chong JY, Rowland LP, Utiger RD. Hashimoto encephalopathy: syndrome or myth? Arch Neurol. 2003; 60: 164–171.
18.
Lennon VA, Lambert EH, Whittingham S, Fairbanks V. Autoimmunity in the Lambert-Eaton myasthenic syndrome. Muscle Nerve. 1982; 5: S21–S25.
19.
Walikonis JE, Lennon VA. Radioimmunoassay for glutamic acid decarboxylase (GAD65) autoantibodies as a diagnostic aid for stiff-man syndrome and a correlate of susceptibility to type 1 diabetes mellitus. Mayo Clin Proc. 1998; 73: 1161–1166.
20.
Lee EK, Maselli RA, Ellis WG, Agius MA. Morvan’s fibrillary chorea: a paraneoplastic manifestation of thymoma. J Neurol Neurosurg Psychiatry. 1998; 65: 857–862.
21.
Hart IK, Waters C, Vincent A, et al. Autoantibodies detected to expressed potassium channels are implicated in neuromyotonia. Ann Neurol. 1997; 41: 238–246.
22.
Griesmann GE, Lennon VA. Detection of autoantibodies in myasthenia gravis and Lambert-Eaton myasthenic syndrome. In: Rose N, DeMacario C, Folds J, Lane H, Nakamura R, eds. Clinical and Laboratory Immunology, 5th ed. Washington, DC: ASM Press, 1997: 983–988.
23.
Vernino S, Low PA, Fealey RD, et al. Autoantibodies to ganglionic acetylcholine receptors in autoimmune autonomic neuropathies. N Engl J Med. 2000; 343: 847–855.
24.
Lennon VA. The case for a descriptive generic nomenclature: clarification of immunostaining criteria for PCA-1, ANNA-1, and ANNA-2 autoantibodies. Neurology. 1994; 44: 2412–2415.
25.
Vernino S, Lennon VA. New Purkinje cell antibody (PCA-2): marker of lung cancer-related neurological autoimmunity. Ann Neurol. 2000; 47: 297–305.
26.
Yu Z, Kryzer T, Griesmann G, et al. CRMP-5 neuronal autoantibody: marker of lung cancer and thymoma-related autoimmunity. Ann Neurol. 2001; 49: 146–154.
27.
Kokmen E, Naessens JM, Offord KP. A short test of mental status: description and preliminary results. Mayo Clin Proc. 1987; 62: 281–288.
28.
Brandt J, Spencer M, Folstein M. The telephone interview of cognitive status. Neuropsychiatry Neuropsychol Behav Neurol. 1988; 1: 111–117.
29.
Vernino S, Lennon V. Muscle and neuronal autoantibody markers of thymoma: neurological correlations. Ann NY Acad Sci. 2003; 998: 359–361.
30.
Bohnen NI, Parnell KJ, Harper CM. Reversible MRI findings in a patient with Hashimoto’s encephalopathy. Neurology. 1997; 49: 246–247.

Information & Authors

Information

Published In

Neurology®
Volume 62Number 7April 13, 2004
Pages: 1177-1182
PubMed: 15079019

Publication History

Received: October 8, 2003
Accepted: January 29, 2004
Published online: April 12, 2004
Published in print: April 13, 2004

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Authors

Affiliations & Disclosures

M. J. Thieben, MBBS FRACP
From the Departments of Neurology (Drs. Theiben, Lennon, Boeve, Aksamit, Keegan, and Vernino), Immunology (Dr. Lennon), and Laboratory Medicine and Pathology (Dr. Lennon), Mayo Clinic and Mayo Clinic College of Medicine, Rochester, MN.
V. A. Lennon, MD PhD
From the Departments of Neurology (Drs. Theiben, Lennon, Boeve, Aksamit, Keegan, and Vernino), Immunology (Dr. Lennon), and Laboratory Medicine and Pathology (Dr. Lennon), Mayo Clinic and Mayo Clinic College of Medicine, Rochester, MN.
B. F. Boeve, MD
From the Departments of Neurology (Drs. Theiben, Lennon, Boeve, Aksamit, Keegan, and Vernino), Immunology (Dr. Lennon), and Laboratory Medicine and Pathology (Dr. Lennon), Mayo Clinic and Mayo Clinic College of Medicine, Rochester, MN.
A. J. Aksamit, MD
From the Departments of Neurology (Drs. Theiben, Lennon, Boeve, Aksamit, Keegan, and Vernino), Immunology (Dr. Lennon), and Laboratory Medicine and Pathology (Dr. Lennon), Mayo Clinic and Mayo Clinic College of Medicine, Rochester, MN.
M. Keegan, MD FRCP(C)
From the Departments of Neurology (Drs. Theiben, Lennon, Boeve, Aksamit, Keegan, and Vernino), Immunology (Dr. Lennon), and Laboratory Medicine and Pathology (Dr. Lennon), Mayo Clinic and Mayo Clinic College of Medicine, Rochester, MN.
S. Vernino, MD PhD
From the Departments of Neurology (Drs. Theiben, Lennon, Boeve, Aksamit, Keegan, and Vernino), Immunology (Dr. Lennon), and Laboratory Medicine and Pathology (Dr. Lennon), Mayo Clinic and Mayo Clinic College of Medicine, Rochester, MN.

Notes

Address correspondence and reprint requests to Dr. Steven Vernino, Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905; e-mail: [email protected]

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