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Epilepsy & Behavior 6 (2005) 463–466

Case Report

Writing epilepsy: A neurophysiological,neuropsychological and neuroimaging study

Pedro Abreu a,*, Mendes Ribeiro b, Alessandra Forni b, Isabel Pires b, Georgina Sousa b

a Department of Neurology, Hospital S. Joao, Porto, Portugalb Neurophysiology Unity, Hospital S. Joao, Porto, Portugal

Received 1 December 2004; revised 6 February 2005; accepted 8 February 2005

Abstract

Writing epilepsy is a rare reflex syndrome in which seizures are triggered by writing. We describe a 33-year-old, right-handedman, with a history of juvenile absence epilepsy in remission and a family history of epilepsy, in whom myoclonic jerks precipitatedexclusively by writing started at the age of 30. Intensive video/EEG monitoring during neuropsychological tests revealed, at about1 minute after starting to write, a dystonic posture, followed by myoclonic jerks involving the right hand that shortly after becamegeneralized. Concomitantly, the ictal EEG documented generalized hypersynchronous polyspike–wave discharges, maximal over theright parietocentral area. SPECT revealed an ictal hyperperfusion and interictal hypoperfusion over right parietofrontal regions,and fMRI showed extensive and intense left frontal, supplementary motor area activation, induced by writing. This case study pro-vides some evidence supporting the hypothesis that the mechanism underlying writing-triggered seizures may be a generalized sei-zure process, with a focal cortical trigger zone, presumed to be the left frontal lobe as suggested by clinical and fMRI data. Arelevant role played by the right hemisphere (right parietofrontal region) is postulated in the full-blown expression of reflex epilep-togenesis, as supported by EEG and SPECT findings.� 2005 Elsevier Inc. All rights reserved.

Keywords: Writing-induced epilepsy; Graphogenic epilepsy; Language epilepsy; Reflex epilepsy

1. Introduction

In language-induced epilepsy, a well-known syn-drome proposed by Geschwind and Sherwin [1], differ-ent aspects of language function, namely, reading,writing, and speaking, may precipitate seizures. In themajority of the reported cases [2–4] reading is the majortriggering factor and writing is only an additional pre-cipitating stimulus. Furthermore, seizures inducedexclusively by writing are very rarely described in the lit-

1525-5050/$ - see front matter � 2005 Elsevier Inc. All rights reserved.

doi:10.1016/j.yebeh.2005.02.004

* Corresponding author. Present address: Servico de Neurologia,Hospital S. Joao, Alameda Prof. Hernani Monteiro, 4200-319, Porto,Portugal.

E-mail addresses: pmabreu@portugalmail.pt, pmabreu@netcabo.pt (P. Abreu).

erature [5], and in the reported cases of graphogenic epi-lepsy, the reflex seizures were related to juvenilemyoclonic epilepsy [4–8]. To our knowledge, a puregraphogenic epilepsy syndrome associated with a pasthistory of juvenile absence epilepsy has not yet beendescribed.

This case is a good experimental paradigm for study-ing writing-induced epilepsy with neuropsychologicaltests under video/EEG monitoring and functional neu-roimaging (SPECT and fMRI).

2. Case report

The 33-year-old, right-handed man has been attend-ing our epilepsy clinic in S. Joao Hospital in northern

464 Case Report / Epilepsy & Behavior 6 (2005) 463–466

Portugal since he was 12 years old. At that age, he waselectroclinically diagnosed with juvenile absence epi-lepsy and, shortly after onset, became seizure-free.

At the age of 30, he began to complain about jerksinvolving initially the right hand and immediatelyspreading to the whole body. This ictal behavior wasreproducibly precipitated by handwriting, with a regular

Table 1Neuropsychological tests

Reading silently and aloudRegular textNoncomprehensible text

WritingSpontaneous writing, dictation, copyingSpontaneous writing and dictation blindfolded

SpeakingSpontaneous speaking and repeating

Other verbal activitiesVisualizing numbers and lettersVisual labyrinthConstructing sentences in the mind

CalculationWritten and mental calculation

Spatial constructionCopying figures (Rey figure)Spontaneous drawingBlock Design test of Wais-RMental construction of block design

Fig. 1. Spontaneous asymmetrical discharges (maximal over right parietocen

latency of 1 minute. His family history included an auntwith epilepsy. His neurological exam was normal(WAIS: 34). Interictal EEGs and cerebral MRI scanswere normal.

We obtained video/EEG recordings during neuropsy-chological tests (NPT) (Table 1) using the same method-ology as described by Matsuoka et al. [6] and Pegnaet al. [9]. Ictal and interictal HMPAO-SPECT andfunctional MRI using semantic decisions, reading, fingertapping, and writing paradigms were also performed.

For the ictal SPECT study, 740 mBq of 99mTC-HMPAO was injected intravenously at the onset of aclinical seizure (right-hand myoclonus), which occurred1 minute after the patient started writing.

During NPTs and only when writing, EEGs showedgeneralized hypersynchronous polyspike–wave dis-charges, maximal over the right parietocentral area(Fig. 1), concomitant with a dystonic posture of thewriting hand, followed by a brief myoclonic jerk (fewseconds) that affected the arm and the axial musclesand, finally, the entire body. The patient was fully alertduring the seizure and remembered all the actions re-quested. He had clinical and subclinical seizures precip-itated by all forms of writing (spontaneous writing,dictation, and copying), even when he was writing blind-folded. In comparison to dictation and copying, sponta-neous writing induced the shortest latency to discharges,longest duration of discharges, and largest number ofseizures. No clinical seizure or EEG discharges were in-duced by other NPTs such as written and mental calcu-

tral area) with clinical seizures, observed when our patient was writing.

Case Report / Epilepsy & Behavior 6 (2005) 463–466 465

lation, copying of figures, spontaneous drawing, andfine finger movements. Furthermore, other languagestimuli (speaking or reading) were not able to triggerany electroclinical manifestations.

HMPAO-SPECT revealed an ictal hyperperfusionand interictal hypoperfusion in the right parietofrontalarea (Fig. 2).

Functional MRI (1.5 T) showed intense and extensiveleft frontal, supplementary motor area activation (Fig.3), when using a writing paradigm, which was suddenlyterminated by the occurrence of myoclonic seizures.

Fig. 2. HMPAO-SPECT reveals ictal hyperperfusion (arrows) andinterictal hypoperfusion (thin arrows) in right parietofrontal area.

Fig. 3. fMRI reveals left frontal supplementary

A slight improvement in seizure status was notedafter increasing sodium valproate 500 mg three timesdaily (25 mg/kg/day).

3. Discussion

This case has two interesting features. To our knowl-edge, this is the first published report of a pure reflexwriting epilepsy associated with a documented past his-tory of juvenile absence epilepsy. Second, the electroclin-ical and functional neuroimaging aspects of thisgraphogenic epilepsy, as studied by neuropsychologicaltesting under intensive video/EEG monitoring, SPECT,and fMRI, are described.

Seizures induced exclusively by writing have been de-scribed mainly in relationship to juvenile myoclonic epi-lepsy and are considered rare in other generalizedidiopathic epilepsies [4,6,8] and even rarer in focal epi-lepsies [10,11].

This case exhibited writing-induced seizures, shownby clinical semiology and fMRI to be activated in theleft frontal lobe, arguing in favor of the thesis that lan-guage-induced epilepsy may have a focal cortical acti-vating mechanism [5,12–16]. This is especiallyinteresting in view of the fact that this case had a pasthistory of generalized idiopathic (absence) epilepsy.

Furthermore, and consistent with a generalized, neu-ronal dysfunction (cortical hyperexcitability), with a fo-cal cortical activation, the fMRI findings demonstratedintense left frontal, supplementary motor area activation(right-handedness), as compared with controls, suggest-ing a role of the left hemisphere in the very beginning ofthe neuronal process. This is a logical finding, consideringthat the patient does not have spontaneous seizures and,therefore, writing had to be a very powerful trigger to ini-tiate the brain cascade, leading to the pure reflex seizures.

motor area activation (writing paradigm).

466 Case Report / Epilepsy & Behavior 6 (2005) 463–466

Otherwise, the right hemisphere seemed to play a rel-evant role in the ongoing pathophysiological processunderlining the writing seizures, as we may deduce fromthe ictal EEG and SPECT findings, which revealed max-imal electrical and cerebral blood perfusion activationover the right parietocentral region. This is in keepingwith Price and colleagues� studies suggesting that pro-cessing of written words is not restricted to the left hemi-sphere, even in normal right-handed subjects [17].

Another relevant issue that merits consideration per-tains to the place of this case of epilepsy in the Interna-tional Classification of Syndromes and Epilepsies [18]and, additionally, in the recent Proposed DiagnosticScheme for People with Epileptic Seizures and with Epi-lepsy [19]. Rudolf et al. [20] classified reflex epilepsy intotwo main groups: non-verbal-induced epilepsy (praxis-induced seizures: thinking epilepsy, reflex decisionepilepsy, eating epilepsy) and verbal-induced epilepsy(language-induced epilepsy: reading, writing, andspeaking epilepsies). In the light of this classification,writing or graphogenic epilepsy is listed in both groups.We think that we have a strong argument in favor ofpraxis-triggered seizures: the hand motor activity impli-cated in the writing process seemed to be a major ele-ment in the seizure-precipitating mechanism.

In conclusion, this case is an example of pure graph-ogenic epilepsy, shown by clinical semiology and fMRIstudies to be generalized reflex epilepsy, with a focal/re-gional cortical activation, but including, in the timecourse of the reflex epileptogenesis, a relevant righthemisphere participation, as suggested by ictal EEGand SPECT findings. This interpretation parallels pho-tosensitive epilepsy studies, in which visual stimuli leadto occipital cortical participation in the activation of ageneralized epileptic process.

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