BRAIN SPECT PERFUSION OF FRONTOTEMPORAL DEMENTIA ASSOCIATED WITH MOTOR NEURON DISEASE

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D.H. Benninger, MD J. Gandjour, MD D. Georgiadis, MD E. Sto¨ckli, MD M. Arnold, MD R.W. Baumgartner, MD

BENIGN LONG-TERM OUTCOME OF CONSERVATIVELY TREATED CERVICAL ANEURYSMS DUE TO CAROTID DISSECTION

Few data exist on long-term outcome of patients with cervical aneurysm due to spontaneous dissection of the internal carotid artery (sICAD).1,2 In this prospective observational study, we assessed the long-term risk of stroke, rupture, and development of local symptoms or signs on the side of dissection (headache, neck pain, Horner syndrome, cranial nerve palsy) in cervical aneurysms caused by sICAD. Methods. We included 279 consecutive patients with sICAD (diagnosed as previously reported)3 who underwent cerebral MR angiography (MRA; n ⫽ 195), digital subtraction angiography (DSA, n ⫽ 72), or both (n ⫽ 12) at two academic centers from January 1987 until March 2005. Five of these patients died from stroke within 2 weeks after symptom onset; an ICA aneurysm was diagnosed in one of them. A second angiogram (MRA, n ⫽ 218; DSA, n ⫽ 17; CT angiography, n ⫽ 6) was done in 236 (86%) of 274 survivors. Two neurologists blinded to the patients’ identity reviewed the angiographies and characterized the aneurysms (localization: postbifurcation, midcervical, or subpetrous third of the ICA; saccular or fusiform; size). Clinical follow-ups were done 3 and 6 months after symptom onset and annually thereafter. Baseline investigations3 were repeated in patients with suspicion of stroke, TIA, retinal ischemia, aneurysm rupture, recurrent dissection, or new local symptoms or signs. The study was approved by the local ethics committee. Results. Thirty-eight (14%; 25 men; mean age 47 ⫾ 11 years, range 24 to 68 years) of 279 included patients had 42 cervical ICA and 4 vertebral artery aneurysms and 2 sICAD without aneurysm. Thirty-five carotid aneurysms involved symptomatic and seven asymptomatic sICAD. Twenty-three carotid aneurysms were diagnosed on first angiography, performed 11 days (median; range, 0 to 59) after symptom onset. The

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Table

Presenting characteristics and treatment in 38 patients with 42 cervical carotid aneurysms* n (%)

Men

25 (66)

History of minor trauma

13 (34)

Smokers

13 (34)

Hypertension

16 (42)

Hypercholesterolemia

14 (37)

Carotid territory ischemia

20 (53)

Ischemic stroke

14 (37)

TIA

4 (11)

Retinal ischemia

2 (5)

Local symptoms and signs

26 (68)

Headache

24 (63)

Neck pain

11 (29)

Pulsatile tinnitus

9 (24)

Horner syndrome

9 (24)

Cranial nerve palsy

5 (13)

Treatment Aspirin alone†

17 (45)

Anticoagulation followed by aspirin

17 (45)

Warfarin alone‡

3 (8)

Balloon occlusion of carotid supplying aneurysm and extracranial–intracranial bypass surgery

1 (3)

* Mean age was 47 ⫾ 11 years (range, 24 to 68) years. †One patient also underwent endovascular occlusion of the aneurysm. ‡Two patients ages 58 to 68 years had atrial fibrillation and hypertension and one patient a mechanical heart valve.

remaining 19 carotid aneurysms were detected on second angiography, performed 9 months (median; range, 3 to 73) after symptom onset. Twelve aneurysms had an angiographic follow-up after 301 ⫾ 281 days (mean ⫾ SD; range, 77 to 987). Presenting patient characteristics and treatment are shown in the table. Aneurysms were mainly located in the subpetrous ICA (n ⫽ 33, 79%; midcervical, n ⫽ 8, 19%; postbifurcation, n ⫽ 1, 2%). Twenty-four aneurysms were fusiform (median extension: 11 mm, range 2 to 37 mm) and 18 aneurysms saccular (median length: 11 mm, range 2 to 19 mm; median

width: 6 mm, range, 4 to 10 mm). No change in diameter or morphology was observed in 12 aneurysms with angiographic follow-up. Clinical follow-up was obtained in all 38 patients 6.5 ⫾ 5.5 years (mean ⫾ SD) after symptom onset (1 year, n ⫽ 33; 5 years, n ⫽ 23; 10 years, n ⫽ 9; total duration, 430 patient-years). Thirtyseven had antithrombotic treatment including oral aspirin 100 mg/day (n ⫽ 34) and anticoagulation (n ⫽ 3). Ischemic events included two fatal strokes (sICAD related, after 14 days; cause unknown, after 5,477 days) and a capsular stroke (small artery disease) after 1,653 days. No clinical features of aneurysm rupture or new local symptoms or signs were observed. Discussion. This study confirms that the longterm outcome of cervical aneurysm due to sICAD—regardless of location, size, and morphology—is benign under conservative treatment including mainly aspirin, as no aneurysm ruptured or caused ischemia or new local symptoms or signs. Although this finding has been described in two previous studies reporting 44 sICAD patients with 49 cervical aneurysms,1,2 these were based on mean follow-ups of 3 to 3.5 years as opposed to the 6.5 years in the current study. Prevalence of cervical carotid aneurysm was 13% as compared with 9 to 49% in previous studies.1,2,4 Aneurysms were mainly located in the subpetrous ICA, and prevalence of fusiform and saccular forms was similar; both findings are in accordance with previous results.1 Size and morphology of 12 aneurysms with angiographic follow-up remained unchanged. Previous studies reported constant sizes in 59 to 65%,1,2,5 decreased sizes in 18 to 30%, and resolution in 5 to 25%.1,2,5 Prevalence of carotid territory ischemia was 50% in this and 53% in a previous study1 and thus markedly lower compared with previous reports of 79 to 83% described in sICAD patients, 9 to 12% of whom had carotid aneurysms.3,4 It appears that cervical sICAD aneurysms are not associated with a higher prevalence of cerebral or retinal ischemia.

We observed a predominance of male patients and a high prevalence of local symptoms and signs. Similar results were reported by the only other study that separately assessed patients (n ⫽ 16) with carotid aneurysms.1 Obviously, the low number of enrolled patients precludes definitive statements. This study is limited by its retrospective analysis of prospectively collected data. Another drawback is the long observation period with different diagnostic methods. However, sICAD aneurysms are rare, and patients had to be recruited for a long time period. In conclusion, our data suggest that conservative management of cervical aneurysms caused by sICAD is associated with a benign long-term outcome. From the Departments of Neurology, University Hospitals of Zu¨rich (D.H.B., J.G., D.G., E.S., R.W.B.) and Bern (M.A.), Switzerland. Disclosure: The authors report no conflicts of interest. Received September 8, 2006. Accepted in final form February 27, 2007. Address correspondence and reprint requests to Dr. R.W. Baumgartner, Department of Neurology, University Hospital, Frauenklinikstrasse 26, CH-8091 Zu¨rich, Switzerland; [email protected] REFERENCES 1.

2.

3.

4.

5.

Guillon B, Brunerau L, Biousse V, Djouhri H, Le´vy C, Bousser MG. Long-term follow-up of aneurysms developed during extracranial internal carotid artery dissection. Neurology 1999;53:117–122. Touze´ E, Randoux B, Me´ary E, Arquizan C, Meder J-F, Mas J-L. Aneurysmal forms of cervical artery dissection. Associated factors and outcome. Stroke 2001; 32:418–423. Baumgartner RW, Arnold M, Baumgartner I, et al. Carotid dissection with and without ischemic events: local symptoms and cerebral artery findings. Neurology 2001;57:827–832. Biousse V, D’Anglejan-Chatillon J, Massiou H, Bousser MG. Time course of symptoms in extracranial carotid artery dissections. A series of 80 patients. Stroke 1995;26:235–239. Djouhri H, Guillon B, Brunereau L, Levy C, Bousson V, Biousse V, et al. MR angiography for the long-term follow-up of dissecting aneurysms of the extracranial internal carotid artery. AJR Am J Roentgenol 2000; 174:1137–1140.

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E. Guedj, MD I. Le Ber, MD L. Lacomblez, MD B. Dubois, MD P. Verpillat, MD M. Didic, MD F. Salachas, MD P. Vera, MD D. Hannequin, MD J.-A. Lotterie, MD M. Puel, MD M. Decousus, MD C. Thomas-Ante´rion, MD C. Magne, MD M. Vercelletto, MD A.-M. Bernard, MD V. Golfier, MD J. Pasquier, MD B.-F. Michel, MD I. Namer, MD F. Sellal, MD J. Bochet, MD M. Volteau A. Brice, MD V. Meininger, MD, PhD French Research Network on FTD/ FTD-MND* M.-O. Habert, MD

BRAIN SPECT PERFUSION OF FRONTOTEMPORAL DEMENTIA ASSOCIATED WITH MOTOR NEURON DISEASE

A continuum may exist between frontotemporal dementia (FTD) and motor neuron disease (MND).1 However, conflicting SPECT and PET studies on small groups of patients raise the question of whether the functional changes in patients with FTD and FTD/MND are similar.2-4 We therefore conducted a national multicentric SPECT study to characterize brain perfusion SPECT patterns in patients with FTD/MND in comparison with a group of patients with FTD and a control group of healthy subjects. Voxeland volume-of-interest-based analyses were performed to evaluate both significant regional hypoperfusion and significant interhemispheric perfusion asymmetry. Methods. Twenty-four patients with FTD/MND were compared with 28 age-matched (65.6 ⫾ 10.1 years) healthy control subjects and 54 FTD patients. All patients had frontal variant of FTD. FTD/MND and FTD groups had similar ages at onset, gender, FTD duration, and neuropsychological performances (table E-1 on the Neurology Web site at www.neurology.org). Fourteen patients with FTD/MND initially developed FTD, with a mean interval between FTD and MND of 3.2 ⫾ 2.6 years, one had MND 2 years before the FTD, and nine simultaneously developed FTD and MND. All FTD patients were followed at least 5 years to ascertain that none secondarily developed MND. Brain SPECT was performed in seven centers, after IV injection of 99mTc-ethylcysteinate dimer. A voxel-by-voxel intergroup study was performed with SPM2. The center effect was minimized by filtering and masking.5 We compared the perfusion brain images of the FTD/MND patients, FTD patients, and healthy controls. SPM {T} maps were initially obtained at a height threshold of p ⫽ 0.05, corrected for multiple comparisons for the cluster. The anatomic localization of significantly hypoperfused regions between patients and the control group were obtained from the segmentation of AAL software (http://www.cyceron.fr/freeware/), using the MARSBAR routine (http://marsbar.sourceforge. net/). An index of left/right asymmetry was then calculated for these regions to determine asymmetry of hypoperfusion in each patient. Two stan-

dard deviations above or below the mean index of the control group was considered significant. This study was approved by the Ethics Committee of Pitie´-Salpeˆtrie`re Hospital. Results. Patients with FTD/MND and FTD, compared with healthy subjects, had the same pattern of predominant asymmetric and anterior hypoperfusion, involving association frontal, temporal, cingular, and insular cortices. Frontal hypoperfusion included the premotor cortex. There was also bilateral hypoperfusion of the thalamus and striatum (figure; table E-2). For a corrected statistical threshold, perfusion was similar in FTD and FTD/MND patients and in patients for whom FTD only or FTD/MND was the initial symptom. However, for a noncorrected statistical threshold (figure), patients with FTD/ MND had greater left premotor and precentral hypoperfusion than FTD patients (p voxel level ⬍ 0.05). This was confirmed using small volume correction with a left precentral region of interest (ROI) (p ⫽ 0.011, with Family-Wise Error correction). No hypoperfusion was found with a right precentral ROI. In addition, interhemispheric perfusion was similar in both groups of patients. Discussion. Similar patterns of predominant anterior and asymmetric hypoperfusions, including the premotor cortex, were found in patients with FTD and with FTD/MND, matched for age, gender, FTD duration, and severity of dementia. The only difference was greater hypoperfusion in the left precentral cortex in FTD/MND than in FTD patients that could clinically be related to bulbar signs in MND. A left predominance of lesions is found in progressive aphasia, a variant of FTD. However, all our FTD/MND patients had a frontal variant of FTD. An asymmetric involvement of motor cortex in MND was previously described with PET.6 This asymmetry could be due to a distribution at random or to statistical bias. It has also been suggested that lateralization in the cerebral cortex could be dependent on the intensity of callosal inhibition and that part of the asymmetry observed in MND patients could reflect an unbalance between the excitatory and inhibitory callosal projections.6 On previous functional neuroimaging studies comparing FTD/MND and FTD patients, similar patterns of hypoperfusion in the two groups have been reported,2 but also more se-

Supplemental data at www.neurology.org *See appendix for a complete list of members of the French Research Network on FTD/FTD-MND. 488

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Figure

Anatomic localization of peaks of hypoperfusion when comparing FTD/MND and FTD patients and healthy subjects

(A) Patients with FTD/MND and (C) patients with FTD exhibited similar bilateral predominant anterior hypoperfusion in comparison with healthy subjects (SPM2 surface rendering; p cluster level ⬍ 0.05, corrected for multiple comparisons). (B) Patients with FTD/MND exhibited in comparison with FTD patients left premotor and precentral hypoperfusions (sections of a normal MRI set spatially normalized into the standard SPM2 template; p voxel-level ⬍ 0.05 uncorrected, not significant for the cluster, p cluster level ⫽ 0.337, uncorrected; k ⫽ 990, T score ⫽ 3.80; peak Talairach coordinates: ⫺42, 0, 41). This result was confirmed using small volume correction with left precentral region of interest (p ⫽ 0.011, with FWE correction). No hypoperfusion was found with a right precentral region of interest.

vere hypometabolism in medial temporal regions in FTD/MND patients.3 A third study found that FTD/MND had only frontal alterations that were more symmetric than in FTD patients.4 Smaller groups, differences in age, type of frontotemporal lobar degeneration, and duration and severity of the disease might explain these discrepancies. Our results are concordant with studies showing similar clinical presentations in FTD/MND and FTD.7 Moreover, several SPECT and PET studies confirmed that functional changes in MND included the frontal association cortex in addition to the primary motor cortex.6 These results provide further evidence that there is a continuum from FTD to FTD/MND and to MND. From the Service Central de Biophysique et Me´decine Nucle´aire (E.G.), Hoˆpital de la Timone, APHM, and Service de Neurologie et Neuropsychologie (M.D.), Hoˆpital de la Timone, and Laboratoire de Neurophysiologie et de Neuropsychologie, INSERM U-751, De´partement de Me´decine Nucle´aire (J.P.), Institut Paoli-Calmettes, and Service de Neuroge´riatrie (B.-F.M.), Hoˆpital de Sainte-Marguerite, Marseille; Centre de Neuropsychologie (I.L.B., B.D.), APHP, INSERM UMRS679 (I.L.B., P.V., A.B.), Federation of Neurology (I.L.B., L.L., B.D., F.S., A.B., V.M.), Department of Genetics and Cytogenetics (A.B.), APHP, and

INSERM U610 (B.D., M.V.), Pitie´-Salpeˆtrie`re Hospital, Department of Nuclear Medicine (M.-O.H.), CHU Pitie´Salpeˆtrie`re, AP-HP, INSERM U678 (M.-O.H.), and Universite´ Pierre et Marie Curie–Paris 6 (I.L.B., L.L., B.D., A.B., M.-O.H.), Paris; De´partement et GIE de Me´decine Nucle´aire (P.V.), CRLCC Henri Becquerel–CHU de Rouen and LITIS Quant. IF, Universite´ de Rouen, France, and De´partement de Neurologie and INSERM U-614 (D.H.), CHU Rouen; Service de Me´decine Nucle´aire (J.-A.L.), Hoˆpital Rangueil, and Service de Neurologie (M.P.), Hoˆpital Purpan, Toulouse; Service de Me´decine Nucle´aire (M.D.) and Service de Neurologie (C.T.-A.), Hoˆpital Bellevue, Saint-E´tienne; Service de Me´decine Nucle´aire (C.M.) and Service de Neurologie (M.V.), Hoˆpital Guillaume et Rene´ Lae¨nnec, Nantes; Service de Me´decine Nucle´aire (A.-M.B.), Centre Euge`ne Marquis, Rennes; Service de Neurologie (V.G.), Hoˆpital de SaintBrieuc; Service de Me´decine Nucle´aire (I.N.), Hoˆpital de Hautepierre, and Service de Neurologie (F.S.), Hoˆpitaux Universitaires and INSERM U-692, Strasbourg; and Service de Me´decine Nucle´aire (J.B.), Polyclinique Saint-Claude, Saint-Quentin, France. Received October 20, 2006. Accepted in final form February 27, 2007. Address correspondence and reprint requests to Dr. E. Guedj, Service Central de Biophysique et de Me´decine Nucle´aire. Assistance Publique des Hoˆpitaux de Marseille, Centre Hospitalo-Universitaire de la Timone, 264, rue Saint Pierre, 13385 Marseille Cedex 5, France; [email protected] ap-hm.fr Neurology 69

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Supported by CRIC/AP-HP 01107 (to A.B.) and GIS-Institut des Maladies Rares A03081DS/APS03002DSA (to A.B.). 2. APPENDIX The French Research Network on FTD/FTD-MND includes Alexis Brice (Hoˆpital de la Salpeˆtrie`re, Paris), Franc¸oise Clerget-Darpoux (Hoˆpital Paul Brousse, Villejuif), Gilles Defer (CHU Cote de Nacre, Caen), Mira Didic (CHU La Timone, Marseille), Claude Desnuelle (CHU Nice), Bruno Dubois (Hoˆpital de la Salpeˆtrie`re, Paris), Charles Duyckaerts (Hoˆpital de la Salpeˆtrie`re, Paris), Ve´ronique Golfier (CH Saint-Brieuc), Didier Hannequin (CHU de Rouen), Lucette Lacomblez (Hoˆpital de la Salpeˆtrie`re, Paris), Isabelle Le Ber (Hoˆpital de la Salpeˆtrie`re, Paris), Bernard-Franc¸ois Michel (CH Sainte-Marguerite, Marseille), Florence Pasquier (CHU Roger Salengro, Lille), Catherine Thomas-Anterion (CHU Bellevue, Saint-Etienne), Miche`le Puel (CHU Purpan, Toulouse), Franc¸ois Salachas (Hoˆpital de la Salpeˆtrie`re, Paris), Franc¸ois Sellal (Hoˆpitaux Universitaires, Strasbourg), Martine Vercelletto (CHU Laennec, Nantes), Patrice Verpillat (Hoˆpital de la Salpeˆtrie`re, Paris), and William Camu (CHU Gui de Chauliac, Montpellier).

3.

4.

5.

6.

REFERENCES 7. 1.

F. Bartolomei, MD, PhD A. McGonigal, MD M. Guye, MD, PhD E. Guedj, MD P. Chauvel, MD

Supplemental data at www.neurology.org 490

Lomen-Hoerth C, Murphy J, Langmore S, Kramer JH, Olney RK, Miller B. Are amyotrophic lateral sclerosis

CLINICAL AND ANATOMIC CHARACTERISTICS OF HUMMING AND SINGING IN PARTIAL SEIZURES

Few reports have described the occurrence of complex musical automatisms during seizures, including singing1,2 or humming.3,4 The anatomic origin of these manifestations and the types of seizures in which they occur are not well known. In a recent report, the authors estimated that singing during seizures was difficult to correlate with anatomy.2 Humming has been described in seizures beginning in the anterior part of the temporal lobe. 4 It is thus possible that ictal humming and ictal singing could reflect different anatomic origins. In addition, differences in the functional anatomy of ictal humming or singing may be supported by different brain networks involved in the normal processing of humming and singing.5 We assessed the frequency and features of “musical automatisms” (MAs) during partial seizures in an adult population of epileptic patients undergoing presurgical evaluation. All patients with partial seizures admitted to the Adult Epilepsy Unit of Timone Hospital (Marseille, France) between 2000 and 2005 for video-EEG recording were reviewed. Using a patient database, we retrospectively searched for the occurrence of MAs (singing or humNeurology 69

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patients cognitively normal? Neurology 2003;60:1094– 1097. Talbot PR, Goulding PJ, Lloyd JJ, Snowden JS, Neary D, Testa HJ. Inter-relation between “classic” motor neuron disease and frontotemporal dementia: neuropsychological and single photon emission computed tomography study. J Neurol Neurosurg Psychiatry 1995;58:541–547. Garraux G, Salmon E, Degueldre C, Lemaire C, Franck G. Medial temporal lobe metabolic impairment in dementia associated with motor neuron disease. J Neurol Sci 1999;168:145–150. Jeong Y, Park KC, Cho SS, et al. Pattern of glucose hypometabolism in frontotemporal dementia with motor neuron disease. Neurology 2005;64:734–736. Le Ber I, Guedj E, Gabelle A, et al. Demographic, neurological, behavioural characteristics and brain perfusion SPECT in frontal variant of frontotemporal dementia. Brain 2006;129:3051–3065. Habert MO, Lacomblez L, Maksud P, El Fakhri G, Pradat PF, Meininger V. Brain perfusion imaging in amyotrophic lateral sclerosis: extent of cortical changes according to the severity and topography of motor impairment. Amyotrophic Lateral Sclerosis 2007;8:9 –15. Neary D, Snowden JS, Mann DM. Cognitive change in motor neurone disease/amyotrophic lateral sclerosis (MND/ALS). J Neurol Sci 2000;180:15–20.

ming) during video-EEG recorded seizures. Among patients undergoing presurgical evaluation, some had depth electrodes recording using stereo-EEG (SEEG) according to the Talairach stereotactic method.4 Among 416 patients, 7 (1.4%) met inclusion criteria for study of MA ( table). In five patients, MA consisted of humming, defined here as a nonarticulated, wordless, and relatively discreet automatic musical production (see, e.g., video 1 on the Neurology Web site at www.neurology.org). Four of these five patients had typical mesial temporal lobe seizures. Humming occurred relatively late (mean 23 seconds) in the seizure course. This could be a relatively isolated clinical sign but was always associated with loss of consciousness. Humming was associated with a right (Patient 2), left (Patients 3, 5, 6), or a bilateral (Patient 1) hemispheric discharge. In Patient 5, seizures occurred in clusters, and the humming that occurred at seizure onset was associated with ictal aphasia and loss of contact. A left temporal rapid discharge was seen on the scalp EEG. Conversely, two patients presented with MAs more suggestive of singing (see, e.g., video 2). In these cases MA consisted of an articulated vocal automatism with musical intonation. Singing occurred abruptly, very early in the seizure course, and was associ-

Table

Main patient clinical characteristics and electrophysiologic correlations Patient 1

Patient 2

Patient 3

Patient 4

Patient 5

Patient 6

Patient 7

Age, y/gender

48/F

36/F

14/F

47/F

50/F

39/F

11/M

Epilepsy duration

35

26

12

37

33

14

10

MRI

N

HS

FCD left T

HS

N

Orbitofrontal contusion

N

Etiology

Crypt

HS

FCD

HS

Crypt

Posttraumatic

FCD*

Epilepsy type

TLE

TLE

TLE

TLE

TLE

FLE

FLE

Side

Bilat

Right

Left

Left

Left

Right

Right

Type of MA

Humming

Humming

Humming

Humming

Humming

Singing

Singing

Delay

55 s

14 s

20 s

8s

5s

⬍3 s

5s

Associated signs

Left head version, groaning, loss of contact

Staring, loss of contact, chewing

Aphasia, gestural automatism

Groaning, early loss of contact

Jargonophasia, gesticulation

Dancing, euphoric face, complex automatisms

Rubefaction, euphoric behaviour, gestural automatisms

Ictal scalp EEG discharge

Bilateral, theta, temporofrontal

Right theta, temporal

Left temporal, theta

Left temporal, theta

Left temporal, fast discharge

Right frontal

Right frontal

Depth correlation (SEEG)











⫹ prefrontal lateral and mesial

⫹ prefrontal right

Crypt ⫽ cryptogenic; HS ⫽ hippocampal sclerosis; FCD ⫽ focal cortical dysplasia; left T ⫽ left temporal lobe; TLE ⫽ temporal lobe epilepsy; FLE ⫽ frontal lobe epilepsy; FTE ⫽ frontotemporal epilepsy; MA ⫽ musical automatism; SEEG ⫽ stereo-EEG.

ated with complex behavioral changes including euphoric appearance, laughing, gestural automatisms, and in one case dancing-like behavior. In both of these cases, SEEG recordings demonstrated a seizure onset in the right prefrontal region, mainly originating from the dorsolateral prefrontal cortex. However, automatic activity, including singing, occurred when distinct prefrontal and premotor regions were the seat of the epileptic discharge, while the temporal lobe was spared (see figure E-1). Our results suggest that at least two different types of MA of different anatomic origin may be observed in patients with partial seizures. Humming is particularly observed in seizures primarily affecting the temporal lobe, whereas singing is more suggestive of seizures affecting the frontal lobe and in particular the right prefrontal cortex. From a previous series of patients, we reported three cases of humming observed in patients with right or left mesial temporal lobe seizures recorded by intracerebral electrodes (SEEG). 4 Humming was associated with synchronization (coherence analysis)of areas remote from the epileptogenic zone: the prefrontal cortex and the superior temporal gyrus.4 These structures are part of normal networks underlying musical processing in the brain.6 Singing has been reported in some previously published cases. In two cases, the origin was the frontal lobe,1,7 and in two other cases the anatomic origin of the seizures was not defined.2 In our two cases of

singing, right-sided prefrontal seizures were demonstrated after depth SEEG recordings. The mechanisms of singing in seizures are unknown. In a recent fMRI study in normal subjects, it was shown that brain regions involved with both perception and production for singing included the left planum temporale/superior temporal parietal region as well as left and right premotor cortex, anterior superior temporal gyrus, and planum polare.6 In another recent study, it has also been shown that singing more than humming showed additional right lateralized activation of the superior temporal gyrus, inferior central operculum, and particularly the inferior frontal gyrus.5 The emergence of ictal singing in our two patients may therefore be related to the involvement of the right frontal region. However, given the number of regions involved in normal musical processing, other sites of origin for ictal singing can not be excluded. From the Laboratoire de Neurophysiologie et Neuropsychologie (F.B., A.McG., M.G., P.C.), INSERM, U 751, Faculte´ de Me´decine (F.B., A.McG., M.G., E.G., P.C.), Universite´ de la Me´diterrane´e, and Service de Neurophysiologie Clinique (F.B., A.McG., M.G., P.C.) and Service de Me´decine Nucle´aire (E.G.), CHU Timone, Marseille, France. Disclosure: The authors report no conflicts of interest. Received October 22, 2006. Accepted in final form February 27, 2007. Address correspondence and reprint requests to Dr. F. Bartolomei, Service de Neurophysiologie Clinique, CHU Neurology 69

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Timone, 264 Rue st Pierre, 13005-Marseille, France; [email protected]

4.

REFERENCES

5.

1.

2.

3.

492

Vidailhet M, Serdaru M, Agid Y. Singing in the brain: a new form of complex partial seizure? J Neurol Neurosurg Psychiatry 1989;52:1306. Doherty MJ, Wilensky AJ, Holmes MD, Lewis DH, Rae J, Cohn GH. Singing seizures. Neurology 2002;59: 1435–1438. Meierkord H, Shorvon S. Variations on a theme— singing as an epileptic automatism. J Neurol Neurosurg Psychiatry 1991;54:1114–1116.

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7.

Bartolomei F, Wendling F, Vignal JP, Chauvel P, Liegeois-Chauvel C. Neural networks underlying epileptic humming. Epilepsia 2002;43:1001– 1012. Ozdemir E, Norton A, Schlaug G. Shared and distinct neural correlates of singing and speaking. Neuroimage 2006;33:628–635. Callan D, Tsytsarev V, Hanakawa T, et al. Song and speech: brain regions involved with perception and covert production. Neuroimage 2006;31:1327–1342. McChesney-Atkins S, Davies KG, Montouris GD, Silver JT, Menkes DL. Amusia after right frontal resection for epilepsy with singing seizures: case report and review of the literature. Epilepsy Behav 2003;4: 343–347.

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