Top

Published in:

Open Access 01-12-2014 | Case report

Positivity to p-ANCA in patients with status epilepticus

Authors: Edoardo Ferlazzo, Antonio Gambardella, Marina Bellavia, Sara Gasparini, Laura Mumoli, Angelo Labate, Vittoria Cianci, Concetta Russo, Umberto Aguglia

Published in: BMC Neurology | Issue 1/2014

Abstract

Background

Status epilepticus (SE) may occur in the setting of several internal or neurologic diseases. Anti-neutrophilic cytoplasmic antibodies (ANCA) are a group of Ig that may be observed in patients with different autoimmune disorders but are particularly associated with systemic vasculitis named ANCA-associated-vasculities (AAV). We herein report 3 patients with SE and positivity to p-ANCA.

Case presentation

One patient had a catastrophic evolution and died 5 months after disease onset. The other two patients had a good outcome and remained seizure-free at 30 months and 5 years of follow-up respectively.

Conclusion

This report highlights the importance of considering ANCA dosage in patients with SE of unclear origin.

Available only for authorised users

Berden A, Göçeroglu A, Jayne D, Luqmani R, Rasmussen N, Bruijn JA, Bajema I: Diagnosis and management of ANCA associated vasculitis. BMJ. 2012, 344: e26-10.1136/bmj.e26.CrossRefPubMed

Miller KS, Miller JM: Wegener’s granulomatosis presenting as a primary seizure disorder with brain lesions demonstrated by magnetic resonance imaging. Chest. 1993, 103: 316-318. 10.1378/chest.103.1.316.CrossRefPubMed

Weinberger LM, Cohen ML, Remler BF, Naheedy MH, Leigh RJ: Intracranial Wegener’s granulomatosis. Neurology. 1993, 43: 1831-1834. 10.1212/WNL.43.9.1831.CrossRefPubMed

Bajema IM, Hagen EC, Weverling-Rijnsburger AW, van der Pijl H, van Dorp WT, van Ravenswaay Claasen HH, Bruijn JA: Cerebral involvement in two patients with Wegener’s granulomatosis. Clin Nephrol. 1997, 47: 401-406.PubMed

Nordmark G, Boquist L, Rönnblom L: Limited Wegener’s granulomatosis with central nervous system involvement and fatal outcome. J Intern Med. 1997, 242: 433-436. 10.1046/j.1365-2796.1997.00215.x.CrossRefPubMed

von Scheven E, Lee C, Berg BO: Pediatric Wegener’s granulomatosis complicated by central nervous system vasculitis. Pediatr Neurol. 1998, 19: 317-3319. 10.1016/S0887-8994(98)00071-X.CrossRefPubMed

Ulinski T, Martin H, Mac Gregor B, Dardelin R, Cochat P: Fatal neurologic involvement in pediatric Wegener’s granulomatosis. Pediatr Neurol. 2005, 32: 278-281. 10.1016/j.pediatrneurol.2004.10.008.CrossRefPubMed

Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, Segelmark M, Cohen-Tervaert JW, Scott D: Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis. 2007, 66: 222-227.CrossRefPubMed

Narbone MC, Musolino R, Granata F, Mazzù I, Abbate M, Ferlazzo E: PRES: posterior or potentially reversible encephalopathy syndrome?. Neurol Sci. 2006, 27: 187-189. 10.1007/s10072-006-0667-y.CrossRefPubMed

10.

Gambardella A, Andermann F, Shorvon S, Le Piane E, Aguglia U: Limited chronic focal encephalitis: another variant of Rasmussen syndrome?. Neurology. 2008, 70: 374-377. 10.1212/01.wnl.0000298723.96653.99.CrossRefPubMed

11.

Engelsen BA, Tzoulis C, Karlsen B, Lillebø A, Laegreid LM, Aasly J, Zeviani M, Bindoff LA: POLG1 mutations cause a syndromic epilepsy with occipital lobe predilection. Brain. 2008, 131: 818-828. 10.1093/brain/awn007.CrossRefPubMed

12.

Walton EW: Giant-cell granuloma of the respiratory tract (Wegener’s granulomatosis). Br Med J. 1958, 2: 265-270. 10.1136/bmj.2.5091.265.CrossRefPubMedPubMedCentral

13.

Wieshmann UC, Symms MR, Shorvon SD: Diffusion changes in status epilepticus. Lancet. 1997, 350: 493-494.CrossRefPubMed

14.

Nabbout R, Vezzani A, Dulac O, Chiron C: Acute encephalopathy with inflammation-mediated status epilepticus. Lancet Neurol. 2011, 10: 99-108. 10.1016/S1474-4422(10)70214-3.CrossRefPubMed

The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2377/14/148/prepub

Title: Positivity to p-ANCA in patients with status epilepticus
Authors: Edoardo Ferlazzo
Antonio Gambardella
Marina Bellavia
Sara Gasparini
Laura Mumoli
Angelo Labate
Vittoria Cianci
Concetta Russo
Umberto Aguglia
Publication date: 01-12-2014
Publisher: BioMed Central
Published in: BMC Neurology / Issue 1/2014
Electronic ISSN: 1471-2377
DOI: https://doi.org/10.1186/1471-2377-14-148

At a glance: The STEP trials

Springer Medicine

Positivity to p-ANCA in patients with status epilepticus

Abstract

Background

Case presentation

Conclusion

At a glance: The STEP trials

Springer Medicine

Abstract

Background

Case presentation

Conclusion

Please log in to get access to this content

Other articles of this Issue 1/2014

Subcortical white matter infarcts predict 1-year outcome of fatigue in stroke

Early onset frontotemporal dementia with psychiatric presentation due to the C9ORF72 hexanucleotide repeat expansion: a case report

Deterioration after corticosteroids in CIDP may be associated with pure focal demyelination pattern

Observational Dutch Young Symptomatic StrokE studY (ODYSSEY): study rationale and protocol of a multicentre prospective cohort study

Sequencing bilateral and unilateral task-oriented training versus task oriented training alone to improve arm function in individuals with chronic stroke

Recurrent posterior circulation infarction caused by anomalous occipital bony process in a young patient