Seizure 21 (2012) 655–657
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Case report
Complex epileptic palilalia: A case report Doriana Landi a,*, Antonella Benvenga a, Carlo C. Quattrocchi b, Luca Vollero c, Giovanni Assenza a, Giovanni Pellegrino a, Chiara Campana a, Mario Tombini a a
Unita` di Neurologia, Campus Bio-Medico University of Rome, Italy Unita` di Radiologia, Campus Bio-Medico University of Rome, Italy c Laboratorio di Sistemi di Elaborazione e Bioinformatica, Campus Bio-Medico University of Rome, Italy b
A R T I C L E I N F O
A B S T R A C T
Article history: Received 5 April 2012 Received in revised form 11 June 2012 Accepted 13 June 2012
Palilalia is a kind of motor perseveration involving speech, consisting in the continuous repetition of words or phrases, or both. Rarely it occurs in seizure disorders as ictal or post-ictal manifestation. We report a case of epileptic palilalia in a 71-year-old patient with a previous history of intracerebral haemorrhage in the left frontal region, characterized by the continuous repetition of the same speech sequence for few minutes. EEG recordings during NREM sleep and wakefulness showed recurrent and prolonged focal subclinical epileptiform paroxysms in the left mesial frontal region. Our case confirms the role of the left frontal lobe, reliably of the supplementary motor area (SMA), as neuroanatomic origin of ictal palilalia. ß 2012 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.
Keywords: Supplementary motor area Language Automatisms
1. Introduction Palilalia is a kind of motor perseveration involving speech, consisting of compulsive repetition of normally articulated words, phrases or syllables. Palilalia has been described in several neurological disorders such as cerebrovascular and degenerative diseases, encephalitis or tic syndromes. Very rarely it has been associated to seizures disorders as ictal or post-ictal phenomena. In these cases of epileptic palilalia repetition of single words or simple syllables has been reported.1–3 Here we present the first case of symptomatic ‘complex’ epileptic palilalia in a 71-year-old patient with a previous history of intracerebral haemorrhage in the left fronto-temporal region.
2. Methods A 71-year old, right-handed man, was admitted to our hospital because he had developed confusion, aphasia and clonic jerks in the right upper limb during the preceding night. His past medical history was unremarkable except for a car accident which had occurred two years earlier in which he had sustained head trauma and an intracerebral haemorrhage in the left fronto-temporal area. Permanent clinical sequelae of brain damage included mild anomic
* Corresponding author at: Unita` di Neurologia, Policlinico Universitario Campus Bio-Medico di Roma, Via Alvaro del Portillo 200, Roma 00128, Italy. Tel.: +39 06225411313; fax: +39 06225411936. E-mail address:
[email protected] (D. Landi).
aphasia and impairment of attention. On admission, while he was telling the interviewer of his car accident, he started compulsively to reiterate the narrative of the accident (‘I felt someone running into my car, the man in the car realized that I was not going out of the car and moved out his car to check what was going on. . .) with stereotypic prosody. During each repetition he used exactly the same sentences and adopted repetitive facial expressions closely linked to emotions roused by the account. The entire sequence was repeated for about 7 times. Although he was able to interact with his environment non-verbally (i.e. turning his head to localize doctor’s voice), he could not suppress the phrase repetition in order to answer doctor’s questions. After about 4 min the palilalic activity stopped and he spontaneously moved forward with his tale. He was not aware of the event. On the basis of this observation and of the description of the nocturnal symptoms, a clinical diagnosis of seizure disorder was made and Levetiracetam therapy was started. One day later 24-h EEG monitoring recorded recurrent and prolonged (10–14 s) focal epileptiform paroxysms in the left mesial frontal region during NREM sleep. On the standard EEG (Fig. 1) performed two days later we registered an electrical seizure in the left frontal region during hyperventilation without overt symptoms. No palilalic reiteration was observed during the ictal discharge. At this time the patient was taking Levetiracetam 500 mg twice daily. In this patient we obtained morphological and functional MRI images using a Siemens 1.5 Tesla MR scanner (Avanto, Erlangen, Germany). The imaging protocol included axial FLAIR, coronal TSE T2 fat suppressed weighted, diffusion ecoplanar images with a B0 and a B1000 weighting and 3d MPRAGE T1
1059-1311/$ – see front matter ß 2012 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.seizure.2012.06.009
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D. Landi et al. / Seizure 21 (2012) 655–657
Fig. 1. Standard EEG showing electrical seizure in the left frontal region during hyperventilation.
Fig. 2. Brain morphologial and functional MR imaging. Axial FLAIR images (A and B) show post-traumatic sequelae with a left frontal lesion, peripheral gliosis and enlargement of the frontal horn of the left ventricle. Coronal TSE T2 weighted image (C) shows extension of the lesion with enlarged left frontal subarachnoid spaces; a word generation task shows BOLD signal activation in the site of the expected Broca’s area (D).
D. Landi et al. / Seizure 21 (2012) 655–657
weighted sequences. For BOLD functional MR imaging, twenty-five contiguous axial 4 mm thick functional images were acquired parallel to the AC-PC line using a T2* EPI sequence (matrix = 128 128, voxel size 1.72 mm 1.72 mm 4 mm, TR = 3000 ms). The fMRI task was conducted by using a block design fMRI paradigm consisting of 5 time blocks of 30 s in which the patient performed the task interleaved with 30 s time blocks of rest. The patient had to silently generate as many words as possible for every semantic category required. Processing of images and statistical analyses were carried out using the software SPM8 (http://www.fil.ion.ucl.ac.uk/spm/). Structural cerebral MR scan detected an area of encephalomalacia with peripheral gliosis in the left frontal lobe likely to have resulted from the reported previous head trauma. fMRI acquired during language production task did not show abnormalities in activation pattern, confirming the integrity of primary language areas in this patient (Fig. 2). At three month follow-up the patient was seizure free. 3. Discussion We report a case of epileptic palilalia with repetition of an entire part of a narrative. It is likely that the seizures was related to an area of cerebral scarring caused by an intracerebral haemorrhage in the left frontal region occurred two years before the presentation with paroxysmal palilalia. We talk of ‘complex’ palilalia to distinguish our case from previous description of palilalia only involving the repetition of single words, syllables or phonemes. Even if we were not able to record an ictal EEG during overt symptoms, the available clinical and electroencephalographic data suggests that the left mesial frontal region, namely the supplementary motor area (SMA), may have been the neuroanatomic substrate of the observed ictal palilalia. The SMA is known to be a key structure for motor control, as it is directly involved in the selection, planning and production of voluntary movements. The SMA is also involved in speech processes. In standard models of speech production the formulation and articulation of a linguistic plan relies on different anatomical areas.4 Specifically, the SMA plays a role in different aspects of speech articulation: initiation, tuning and maintenance of speech fluency and volume.5 Lesions of SMA, tumors and infarctions, as well as intracerebral electrical stimulation of this area, are associated with speech impairment.6 Interestingly, language disorders observed in these cases occur more frequently after dominant SMA damage, as in our patient.7 They typically consist of a reduction and slowing of speech, complete mutism or the production of repetitive vocalization patterns (palilalia).8 Palilalia was first recognised as a speech disorder different from aphasia by Souques in 1908.9 Later it was observed in postencephalitic Parkinsonism and in cerebrovascular disease.10 Epileptic palilalia and/or vocalization due to a lesion located in the mesial side of the left frontal lobe, anterior to paracentral region corresponding to SMA, was first observed by Alajouanine et al.1 Bancaud and Talairach described four patients with SMA epilepsy characterized by speech arrest, vocalization or palilalia associated to motor signs such as abduction and elevation of the contralateral upper limb, adversive movement of the head and eyes to the side of the limb elevation.11 Linetsky et al. reported a case of non-convulsive status epilepticus characterized by
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echolalia–palilalia sustained by generalized polyspike and slow wave EEG activity with the occasional predominance in the left frontal leads; they supposed that epileptic discharge caused functional isolation and disinhibition of the repetition network.2 Cho et al. described a case of ictal palilalia, echolalia, and echopraxia–palipraxia in a patient showing encephalomalacia on the left superior frontal gyrus. Ictal single photon emission computed tomography detected hyperperfusion above the lesion, corresponding to the left SMA and subcortical nuclei.3 Moreover, palilalia and echolalia were reported as a post-ictal manifestation combined with transcortical aphasia due to a left frontal parasagittal meningioma resection.12 Consistent with literature, we believe that the epileptic focus is more reliably localized in the left SMA, rather than in the language area in our patient. An fMRI task of words generation, in fact, showed a normal activation pattern of primary language areas, supporting their integrity. Thus in keeping with previous interpretations,3 we speculate that ictal activity arising from the left SMA causes a disconnection between this region and the motor speech area, altering normal reciprocal interactions between the two regions during speech processes and inducing palilalia. More specifically, we hypothesize that the story telling sequence started correctly in the language area, which is substantially intact, but disconnection from the SMA prevented the completion of the speech, triggering a continuous loop, like a ‘jammed disc’. On the other hand the lacking of the ictal EEG does not allow us to rule out the potential involvement of surrounding associative areas. Finally the abrupt onset and the stereotypy of the palilalia observed in our patient make it less likely to be a postictal mechanism.
Conflict of interest statement None of the authors has any conflict of interest to disclose. The work described is consistent with the Journal’s guidelines for ethical publication. References 1. Alajouanine T, Castaigne P, Sabouraud O, Contamin F. Palilalie paroxystique et vocalisations iteratives au cours de crises epileptiques par lesion interessant l’aire motrice supplementaire. Revue Neurologique (Paris) 1959;101:685–99. 2. Linetsky E, Planer D, Ben-Hur T. Echolalia–palilalia as the sole manifestation of nonconvulsive status epilepticus. Neurology 2000;55:733–4. 3. Cho YJ, Han SD, Song SK, Lee BI, Heo K. Palilalia, echolalia, and echopraxia– palipraxia as ictal manifestations in a patient with left frontal lobe epilepsy. Epilepsia 2009;50(6):1616–9. 4. Levelt WJM. Speaking: from intention to articulation. Cambridge, MA: MIT Press; 1989. 5. Krainik A, Lehericy S, Duffau H, Capelle L, Chainay H, Cornu P, et al. Postoperative speech disorder after medial frontal surgery—role of the supplementary motor area. Neurology 2003;60:587–94. 6. Benke T. Palilalia and repetitive speech: two case studies. Brain and Language 2001;78:62–81. 7. Zentner J, Hufnagel A, Pechstein U, Wolf HK, Schramm J. Functional results after resective procedures involving the supplementary motor area. Journal of Neurosurgery 1996;85(4):542–9. 8. Alario FX, Chainay H, Lehericy S, Cohen L. The role of the supplementary motor area (SMA) in word production. Brain Research 2006;1076(1):129–43. 9. Souques A. Palilalia. Revue Neurologique (Paris) 1908;16:340–3. 10. Critchley M. On palilalia. Journal of Neurology and Psychopathology 1927;18: 23–31. 11. Bancaud J, Talairach J. Clinical semiology of frontal lobe seizures. Advances in Neurology 1992;57:3–58. 12. Yankovsky AE, Treves TA. Postictal mixed transcortical aphasia. Seizure 2002;11:278–9.
