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NEUROLOGY 2008;71:1973-1980
© 2008 American Academy of Neurology

Altered language processing in autosomal dominant partial epilepsy with auditory features

R. Ottman, PhD, L. Rosenberger, BA, A. Bagic, MD, K. Kamberakis, MPH, E. K. Ritzl, MD, A. M. Wohlschlager, MD, S. Shamim, MD, S. Sato, MD, C. Liew, MD, W. D. Gaillard, MD, E. Wiggs, PhD, M. M. Berl, PhD, P. Reeves-Tyer, BA, E. H. Baker, MD, PhD, J. A. Butman, MD, PhD and W. H. Theodore, MD

From the G.H. Sergievsky Center (R.O., K.K.) and Departments of Epidemiology (R.O., K.K.) and Neurology (R.O.), Columbia University, New York; New York State Psychiatric Institute (R.O.), New York; Clinical Epilepsy Section (L.R., A.B., E.K.R., A.M.W., S. Shamim, C.L., W.D.G., E.W., P.R.-T., W.H.T.) and EEG Section (A.B., S. Sato), National Institute of Neurological Disorders and Stroke, Bethesda, MD; Department of Neurosciences (M.M.B.), Children’s National Medical Center, George Washington University School of Medicine, Washington, DC; and Diagnostic Radiology Department (E.H.B., J.A.B.), National Institutes of Health, Bethesda, MD is currently affiliated with the Department of Neurology, Center for Advanced Brain Magnetic Source Imaging (CABMSI), University of Pittsburgh Medical School, PA.

Address correspondence and reprint requests to Dr. Ruth Ottman, G.H. Sergievsky Center, Columbia University, 630 W. 168th Street, P&S Box 16, New York, NY 10032 ro6{at}columbia.edu

Background: Autosomal dominant partial epilepsy with auditory features (ADPEAF) is an idiopathic focal epilepsy syndrome with auditory symptoms or receptive aphasia as major ictal manifestations, frequently associated with mutations in the leucine-rich, glioma inactivated 1 (LGI1) gene. Although affected subjects do not have structural abnormalities detected on routine MRI, a lateral temporal malformation was identified through high resolution MRI in one family. We attempted to replicate this finding and to assess auditory and language processing in ADPEAF using fMRI and magnetoencephalography (MEG).

Methods: We studied 17 subjects (10 affected mutation carriers, 3 unaffected carriers, 4 noncarriers) in 7 ADPEAF families, each of which had a different LGI1 mutation. Subjects underwent high-resolution structural MRI, fMRI with an auditory description decision task (ADDT) and a tone discrimination task, and MEG. A control group comprising 26 volunteers was also included.

Results: We found no evidence of structural abnormalities in any of the 17 subjects. On fMRI with ADDT, subjects with epilepsy had significantly less activation than controls. On MEG with auditory stimuli, peak 2 auditory evoked field latency was significantly delayed in affected individuals compared to controls.

Conclusions: These findings do not support the previous report of a lateral temporal malformation in autosomal dominant partial epilepsy with auditory features (ADPEAF). However, our fMRI and magnetoencephalography data suggest that individuals with ADPEAF have functional impairment in language processing.

Abbreviations: ADDT = auditory description decision task; ADPEAF = autosomal dominant partial epilepsy with auditory features; AED = antiepileptic drug; AEF = auditory evoked field; ECD = equivalent current dipole; FLAIR = fluid-attenuated inversion recovery; IGE = idiopathic generalized epilepsy; IIED = identified interictal epileptiform discharges; MEG = magnetoencephalography; MP-RAGE = magnetization prepared rapid gradient echo; SPGR = spoiled gradient echo recalled; SPL = sound-pressure level.

Supported by NIH grants R01NS036319 and R01NS043472 (to R.O.), and the NINDS Division of Intramural Research.

Disclosure: The authors report no disclosures.

Received May 28, 2008. Accepted in final form September 12, 2008.






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