nach oben

Erschienen in:

01.12.2015 | Diagnostic Neuroradiology

Supratentorial white matter blurring associated with voltage-gated potassium channel-complex limbic encephalitis

verfasst von: H. Urbach, S. Rauer, I. Mader, S. Paus, J. Wagner, M. P. Malter, H. Prüss, J. Lewerenz, J. Kassubek, H. Hegen, M. Auer, F. Deisenhammer, F. Ufer, C. G. Bien, A. Baumgartner

Erschienen in: Neuroradiology | Ausgabe 12/2015

Einloggen, um Zugang zu erhalten

Abstract

Introduction

Limbic encephalitis (LE) associated with voltage-gated potassium channel-complex antibodies (VGKC-LE) is frequently non-paraneoplastic and associated with marked improvement following corticosteroid therapy. Mesial temporal lobe abnormalities are present in around 80 % of patients. If associated or preceded by faciobrachial dystonic seizures, basal ganglia signal changes may occur. In some patients, blurring of the supratentorial white matter on T2-weighted images (SWMB) may be seen. The purpose of this study was to evaluate the incidence of SWMB and whether it is specific for VGKC-LE.

Methods

Two experienced neuroradiologists independently evaluated signal abnormalities on FLAIR MRI in 79 patients with LE while unaware on the antibody type.

Results

SWMB was independently assessed as present in 10 of 36 (28 %) compared to 2 (5 %) of 43 non-VGKC patients (p = 0.009). It was not related to the presence of LGI1 or CASPR2 proteins of VGKC antibodies. MRI showed increased temporomesial FLAIR signal in 22 (61 %) VGKC compared to 14 (33 %) non-VGKC patients (p = 0.013), and extratemporomesial structures were affected in one VGKC (3 %) compared to 11 (26 %) non-VGKC patients (p = 0.005).

Conclusion

SWMB is a newly described MRI sign rather specific for VGKC-LE.

Brierley JB, Corsellis JAN, Hierons R, Nevin S (1960) Subacute encephalitis of later adult life. Mainly affecting the limbic areas. Brain 83:357–368CrossRef

Corsellis JAN, Goldberg GJ, Norton AR (1968) Limbic encephalitis and its association with carcinoma. Brain 91:481–496CrossRefPubMed

Dalmau J, Rosenfeld MR (2014) Autoimmune encephalitis update. Neuro Oncol 16:771–778CrossRefPubMedCentralPubMed

Irani SR, Gelfand JM, Bettcher BM, Singhal NS, Geschwind MD (2014) Effect of rituximab in patients with leucine-rich, glioma-inactivated 1 antibody- associated encephalopathy. JAMA Neurol 71:896–900CrossRefPubMedCentralPubMed

Urbach H, Soeder BM, Jeub M, Klockgether T, Meyer B, Bien CG (2006) Serial MRI of limbic encephalitis. Neuroradiology 48:380–386CrossRefPubMed

Wagner J, Witt JA, Helmstaedter C, Malter MP, Weber B, Elger CE (2015) Automated volumetry of the mesiotemporal structures in antibody-associated limbic encephalitis. J Neurol Neurosurg Psychiatry 86:735–742CrossRefPubMed

Wagner J, Schoene-Bake J-C, Malter MP, Urbach H, Huppertz HJ, Elger CE, Weber B (2013) Quantitative FLAIR analysis indicates predominant affection of the amygdala in antibody-associated limbic encephalitis. Epilepsia 54:1679–1687CrossRefPubMed

Irani SR, Alexander S, Waters P, Kleopa KA, Pettingill P, Zuliani L, Peles E, Buckley C, Lang B, Vincent A (2010) Antibodies to Kv1 potassium channel-complex proteins leucine-rich, glioma inactivated 1 protein and contactin-associated protein-2 in limbic encephalitis, Morvan’s syndrome and acquired neuromyotonia. Brain 133:2734–2748CrossRefPubMedCentralPubMed

Soeder BM, Urbach H, Elger CE, Bien CG, Beyenburg S (2005) VGKC antibodies associated with limbic encephalitis. Nervenarzt 76:760–762CrossRefPubMed

10.

Soeder BM, Gleissner U, Urbach H, Clusmann H, Elger CE, Vincent A, Bien CG (2009) Causes, presentation and outcome of lesional adult onset mediotemporal lobe epilepsy. J Neurol Neurosurg Psychiatry 80:894–899CrossRefPubMed

11.

Malter MP, Frisch C, Schoene-Bake JC, Helmstaedter C, Wandinger KP, Stoecker W, Urbach H, Surges R, Elger CE, Vincent AV, Bien CG (2014) Outcome of limbic encephalitis with VGKC-complex antibodies: relation to antigenic specificity. J Neurol 261:1695–1705CrossRefPubMed

12.

Malter MP, Helmstaedter C, Urbach H, Vincent A, Bien CG (2010) Antibodies to glutamic acid decarboxylase define a form of limbic encephalitis. Ann Neurol 67:470–478CrossRefPubMed

13.

Lai M, Huijbers MGM, Lancaster E, Graus F, Bataller L, Balice-Gordon R, Cowell JK, Dalmau J (2010) Investigation of LGI1 as the antigen in limbic encephalitis previously attributed to potassium channels: a case series. Lancet Neurol 9:776–785CrossRefPubMedCentralPubMed

14.

Irani SR, Michell AW, Lang B, Pettingill P, Waters P, Johnson MR, Schott JM, Armstrong RJ, Zagami S, Bleasel A, Somerville ER, Smith SM, Vincent A (2011) Faciobrachial dystonic seizures precede Lgi1 antibody limbic encephalitis. Ann Neurol 69:892–900CrossRefPubMed

15.

Irani SR, Stagg CJ, Schott JM, Rosenthal CR, Schneider SA, Pettingill P, Pettingill R, Waters P, Thomas A, Voets NL, Cardoso MJ, Cash DM, Manning EN, Lang B, Smith SJM, Vincent A, Johnson MR (2013) Faciobrachial dystonic seizures: the influence of immunotherapy on seizure control and prevention of cognitive impairment in a broadening phenotype. Brain 136:3151–3162CrossRefPubMed

16.

Plantone D, Renna R, Grossi D, Plantone F, Iorio R (2013) Teaching NeuroImages: basal ganglia involvement in facio-brachial dystonic seizures associated with LGI1 antibodies. Neurology 80:183–184CrossRef

17.

Boesebeck F, Schwarz O, Dohmen B, Graef U, Vestring T, Kramme C, Bien CG (2013) Faciobrachial dystonic seizures arise from cortico-subcortical abnormal brain areas. J Neurol 260:1684–1686CrossRefPubMed

18.

Fidzinski P, Jarius S, Gaebler C, Boegner F, Nohr R, Ruprecht K (2014) Faciobrachial dystonic seizures and antibodies to Lgi1 in a 92-year-old patient: a case report. J Neurol Sci 347:404–405CrossRefPubMed

19.

Andrade D, Tai P, Dalmau J, Wennberg R (2011) Tonic seizures: a diagnostic clue of anti-LGI1 encephalitis? Neurology 77:2140–2143CrossRef

20.

Lancaster E, Huijbers MG, Bar V, Boronat A, Wong A, Martinez-Hernandez E, Wilson C, Jacobs D, Lai M, Walker RW, Graus F, Bataller L, Illa I, Markx S, Strauss KA, Peles E, Scherer SS, Dalmau J (2011) Investigations of Caspr2, an autoantigen of encephalitis and neuromyotonia. Ann Neurol 69:303–311CrossRefPubMedCentralPubMed

21.

Kotsenas AL, Watson RE, Pittock SJ, Britton JW, Hoye SL, Quek AML, Shin C, Klein CJ (2014) MRI findings in autoimmune voltage-gated potassium channel complex encephalitis with seizures: one potential etiology for mesial temporal sclerosis. Am J Neuroradiol 35:84–89CrossRefPubMed

22.

Szots M, Marton A, Kover F, Kiss T, Berki T, Nagy F, Illes Z (2014) Natural course of LGI1 encephalitis: 3–5 years of follow-up without immunotherapy. J Neurol Sci 343:198–202CrossRefPubMed

23.

Irani SR, Pettingill P, Kleopa KA, Schiza N, Waters P, Mazia C, Zuliani L, Watanabe O, Lang B, Buckley C, Vincent A (2012) Morvan syndrome: clinical and serological observations in 29 cases. Ann Neurol 72:241–255CrossRefPubMed

24.

Iorio R, Lennon VA (2012) Neural antigen-specific autoimmune disorders. Immunol Rev 248:104–121CrossRefPubMed

25.

Wellmer J, Quesada CM, Rothe L, Elger CE, Bien CG, Urbach H (2013) Proposal for a magnetic resonance imaging protocol for the detection of epileptogenic lesions at early outpatient stages. Epilepsia 44:1977–1987CrossRef

26.

Urbach H, Mast H, Egger K, Mader I (2015) Presurgical MR imaging in epilepsy. Clin Neuroradiol

27.

Hacohen Y, Absoud M, Woodhall M, Cummins C, De Goede CG, Hemingway C, Jardine PE, Kneen R, Pike MG, Whitehouse WP, Wassmer E, Waters P, Vincent A, Lim M, UK & Ireland Childhood CNS Inflammatory Demyelination Working Group (2014) Autoantibody biomarkers in childhood-acquired demyelinating syndromes: results from a national surveillance cohort. J Neurol Neurosurg Psychiatry 85:456–461CrossRefPubMed

28.

Silva J, Sharma S, Hughes B, Yu YE, Cowell JK (2010) Homozygous inactivation of the LGI1 gene results in hypomyelination in the peripheral and central nervous systems. J Neurosci Res 88:3328–3336CrossRefPubMed

29.

Faivre-Sarrailh C, Devaux JJ (2013) Neuro-glial interactions at the nodes of Ranvier: implication in health and diseases. Front Cell Neurosci 7:196

30.

Buckley C, Oger J, Clover L, Tüzün E, Carpenter K, Jackson M, Vincent A (2001) Potassium channel antibodies in two patients with reversible limbic encephalitis. Ann Neurol 50:73–78CrossRefPubMed

31.

Schott JM, Harkness K, Barnes J, della Rocchetta AI, Vincent A, Rossor MN (2003) Amnesia, cerebral atrophy, and autoimmunity. Lancet 361:1266CrossRefPubMed

32.

Vincent A, Buckley C, Schott JM, Baker I, Dewar BK, Detert N, Clover L, Parkinson A, Bien CG, Omer S, Lang B, Rossor MN, Palace J (2004) Potassium channel antibody-associated encephalopathy: a potentially immunotherapy-responsive form of limbic encephalitis. Brain 127:701–712CrossRefPubMed

33.

Thieben MJ, Lennon VA, Boeve BF, Aksamit AJ, Keegan M, Vernino S (2004) Potentially reversible autoimmune limbic encephalitis with neuronal potassium channel antibody. Neurology 62:1177–1182CrossRefPubMed

34.

Kröll-Seger J, Bien CG, Huppertz H (2009) Non-paraneoplastic limbic encephalitis associated with antibodies to potassium channels leading to bilateral hippocampal sclerosis in a pre-pubertal girl. Epileptic Disord 11:54–59PubMed

35.

Baumgartner A, Rauer S, Mader I, Meyer PT (2013) Cerebral FDG-PET and MRI findings in autoimmune limbic encephalitis: correlation with autoantibody types. J Neurol 260:2744–2753CrossRefPubMed

36.

Shin YW, Lee ST, Shin JW, Moon J, Lim JA, Byun JI, Kim TJ, Lee KJ, Kim YS, Park KI, Jung KH, Lee SK, Chu K (2013) VGKC-complex/LGI1-antibody encephalitis: clinical manifestations and response to immunotherapy. J Neuroimmunol 265:75–81CrossRefPubMed

37.

Wegner F, Wilke F, Raab P, Tayeb SB, Boeck A, Haense C, Trebst C, Voss E, Schrader C, Logemann F, Ahrens J, Leffler A, Rodriguez-Raecke R, Dengler R, Geworski L, Bengel FM, Berding G, Stangel M, Nabavi E (2014) Anti-leucine rich glioma inactivated 1 protein and anti-N-methyl-d-aspartate receptor encephalitis show distinct patterns of brain glucose metabolism in ¹⁸F-fluoro-2-deoxy-d-glucose positron emission tomography. BMC Neurol 14:136CrossRefPubMedCentralPubMed

Titel: Supratentorial white matter blurring associated with voltage-gated potassium channel-complex limbic encephalitis
verfasst von: H. Urbach
S. Rauer
I. Mader
S. Paus
J. Wagner
M. P. Malter
H. Prüss
J. Lewerenz
J. Kassubek
H. Hegen
M. Auer
F. Deisenhammer
F. Ufer
C. G. Bien
A. Baumgartner
Publikationsdatum: 01.12.2015
Verlag: Springer Berlin Heidelberg
Erschienen in: Neuroradiology / Ausgabe 12/2015
Print ISSN: 0028-3940
Elektronische ISSN: 1432-1920
DOI: https://doi.org/10.1007/s00234-015-1581-x

Leitlinien kompakt für die Neurologie

Mit medbee Pocketcards sicher entscheiden.

^{Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag}

Kostenlos registrieren

Neu im Fachgebiet Neurologie

Hirnblutung unter DOAK und VKA ähnlich bedrohlich

17.05.2024 Direkte orale Antikoagulanzien Nachrichten

Kommt es zu einer nichttraumatischen Hirnblutung, spielt es keine große Rolle, ob die Betroffenen zuvor direkt wirksame orale Antikoagulanzien oder Marcumar bekommen haben: Die Prognose ist ähnlich schlecht.

Thrombektomie auch bei großen Infarkten von Vorteil

16.05.2024 Ischämischer Schlaganfall Nachrichten

Auch ein sehr ausgedehnter ischämischer Schlaganfall scheint an sich kein Grund zu sein, von einer mechanischen Thrombektomie abzusehen. Dafür spricht die LASTE-Studie, an der Patienten und Patientinnen mit einem ASPECTS von maximal 5 beteiligt waren.

Schwindelursache: Massagepistole lässt Otholiten tanzen

14.05.2024 Benigner Lagerungsschwindel Nachrichten

Wenn jüngere Menschen über ständig rezidivierenden Lagerungsschwindel klagen, könnte eine Massagepistole der Auslöser sein. In JAMA Otolaryngology warnt ein Team vor der Anwendung hochpotenter Geräte im Bereich des Nackens.

Schützt Olivenöl vor dem Tod durch Demenz?

10.05.2024 Morbus Alzheimer Nachrichten

Konsumieren Menschen täglich 7 Gramm Olivenöl, ist ihr Risiko, an einer Demenz zu sterben, um mehr als ein Viertel reduziert – und dies weitgehend unabhängig von ihrer sonstigen Ernährung. Dafür sprechen Auswertungen zweier großer US-Studien.

Update Neurologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.

Newsletter bestellen

Die Highlights vom Kongress des American College of Cardiology 2024

Springer Medizin

Abstract

Introduction

Methods

Results

Conclusion

Bitte loggen Sie sich ein, um Zugang zu diesem Inhalt zu erhalten

Weitere Artikel der Ausgabe 12/2015

Somatic mutations associated with MRI-derived volumetric features in glioblastoma

A neuroradiologist’s guide to arterial spin labeling MRI in clinical practice

Mapping of cerebral metabolic rate of oxygen using dynamic susceptibility contrast and blood oxygen level dependent MR imaging in acute ischemic stroke

Early biomarkers from dynamic contrast-enhanced magnetic resonance imaging to predict the response to antiangiogenic therapy in high-grade gliomas

Reduced-dose CT protocol for the assessment of cerebral vasospasm