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Epilepsy & Behavior 10 (2007) 626–628

Case Report

Nonconvulsive status epilepticus manifesting as pure alexia(alexia without agraphia)

Ekrem Kutluay a,*, Burak Pakoz a, Alper Yuksel b, Ahmad Beydoun c

a Department of Neurology, Kent Hospital, Izmir, Turkeyb Department of Radiology, Kent Hospital, Izmir, Turkey

c Division of Neurology, American University of Beirut, Beirut, Lebanon

Received 9 February 2007; accepted 7 March 2007Available online 5 April 2007

Abstract

Pure alexia is a rare disorder usually caused by an ischemic or hemorrhagic stroke. We describe a case of pure alexia due to noncon-vulsive status epilepticus (NCSE). This 57-year-old man presented in a hyperosmolar, nonketotic state with fluctuations in mental statusand an inability to read. His EEG was diagnostic of NCSE originating from the left temporo-occipital region. MRI of the brain revealedincreased FLAIR signal over the left occipitotemporal region. Following initiation of antiepileptic treatment, his neurological examina-tion normalized concomitantly with resolution of the NCSE. A follow-up MRI scan of the brain obtained 1 month later was normal.NCSE can have unusual clinical manifestations, and a high index of suspicion is necessary to correctly diagnose these patients.� 2007 Elsevier Inc. All rights reserved.

Keywords: Nonconvulsive status epilepticus; Alexia; Hyperosmolar state; Brain MRI

1. Introduction

Alexia is an acquired reading disorder, which usuallyoccurs as part of a more generalized language disorder[1]. When it occurs as an isolated disorder, the cause is usu-ally a stroke in the area of the left posterior cerebral artery[1]. However, alexia has also been reported to occur follow-ing intracerebral hemorrhage, brain tumor, central nervoussystem infection, multiple sclerosis, eclampsia, and rightposterior head region lesions in left-handed patients [2–8].Nonconvulsive partial status epilepticus has not been citedas one of the etiologic factors.

2. Case report

A 57-year-old man presented to the emergency depart-ment with a 2-day history of fluctuations in mental status

1525-5050/$ - see front matter � 2007 Elsevier Inc. All rights reserved.

doi:10.1016/j.yebeh.2007.03.001

* Corresponding author. Address: Epilepsy and Sleep Disorders Center,Department of Neurology, Kent Hospital, 8229 sok. No: 30, Cigli, Izmir,Turkey. Fax: +90 232 386 7071.

E-mail address: ekutluay@yahoo.com (E. Kutluay).

and inability to read. Although not known to be diabetic,he was found to be in a hyperosmolar state with a bloodsugar level of 678 mg/dL. Blood gas analysis did not revealacidosis.

Following stabilization of his blood sugar with insulintherapy, an inpatient neurology consult was requested toevaluate the patient’s persistent inability to read. Thepatient was fully awake and not in acute distress. Hisspeech and verbal output were normal. He was able tocomprehend and to follow verbal commands. Visual acuityand funduscopic examinations were normal. Visual testingby confrontation revealed a right homonymous hemiano-pia. The patient was unable to read any written wordsdespite his ability to recognize individual letters and towrite normally. The rest of his neurological examinationwas normal.

On the second day of admission, a brain MRI scan anda 1-hour EEG were obtained. MRI revealed evidence ofcortical swelling and increased signal in FLAIR sequencesover the left temporo-occipital junction involving themiddle occipital and middle temporal gyri (Fig. 1). The

Fig. 1. MRI scan of the brain revealing cortical swelling and increasedsignal in FLAIR sequences over the left temporo-occipital junction.

Case Report / Epilepsy & Behavior 10 (2007) 626–628 627

interictal EEG revealed intermittent focal slowing over theleft posterior head region. In addition, four electrographicseizures originating from the left temporo-occipital regionand lasting 150 to 220 seconds were recorded. The ictal pat-tern started with periodic sharp waves originating from theleft temporo-occipital region, followed by rhythmic 8- to 9-Hz discharges from the same area with maximal field at theO1 electrode site and a field involving the T3 and T5 elec-trode sites (Fig. 2). This pattern subsequently evolved intohigh-amplitude, 1- to 2-Hz rhythmic delta activity involv-ing the entire left hemisphere. During the seizures, nystag-moid movements of the eyes to the right were observed,and the patient was unable to answer questions. He wasamnestic to the events.

Fig. 2. Bipolar EEG recording revealing ictal activity over the left temporo-o

A diagnosis of nonconvulsive status epilepticus (NCSE)was made, and the patient received 10 mg of diazepamintravenously followed by an intravenous loading dose ofphenytoin. No clinical seizures were observed following ini-tiation of antiepileptic drug treatment. Six hours later, thepatient started to read and was able to comprehend most ofthe written words. His alexia completely resolved 24 hourslater. A follow-up EEG did not show any epileptiformactivity or electrographic seizures. A follow-up brainMRI scan obtained 1 month later revealed complete nor-malization of the previously described abnormality. Inaddition, his visual fields evaluated by Goldman perimetrywere normal.

3. Discussion

Alexia is a reading disorder characterized by an inabilityto comprehend written language [1]. Isolated alexia occursas a consequence of brain damage within the distributionof the left cerebral artery, with an ischemic infarct as themost common etiology, followed by intracranial hemor-rhage and brain tumor [1]. The two common forms of iso-lated alexia are hemianopic alexia and pure alexia [9].Patients with hemianopic alexia are able to read individualwords, but generate inefficient saccades to be able to read aline of text [9]. Patients with pure alexia often have hemia-nopia but are more disabled, making frequent errors onindividual words [9], due to damage to a brain region orits connections that support efficient word identification.This region, known as the ‘‘visual word form area,’’ wasfound to be located within the left occipito-temporal sul-cus, between the left fusiform and inferior temporal gyri[10,11]. Although patients with pure alexia cannot read

ccipital region with a maximal field at the O1, T3, and T5 electrode sites.

628 Case Report / Epilepsy & Behavior 10 (2007) 626–628

words or sentences, they are able to identify individual let-ters and are usually able to write normally [9].

Our patient’s presentation was a good example of purealexia. He was unable to read or understand individual wordsand sentences; he could, however, correctly identify individ-ual letters and was able to write normally. There can be littledoubt that his alexia was a consequence of frequent focal sei-zures originating from the left posterior hemisphere. Indeed,the temporal relationship between the alexia and the occur-rence of focal seizures originating from the left temporo-occipital region, the gradual then full recovery of the alexiafollowing initiation of antiepileptic drug treatment and cessa-tion of seizures, as well as the reversibility of the MRI abnor-mality all strongly argue that the pure alexia was related tothe occurrence of focal NCSE in this patient.

The occurrence of focal seizures in patients with nonke-totic hyperglycemia is not unusual. It is estimated that 40%of patients with nonketotic hyperglycemia have seizures,most of which are focal [12]. The transient MRI abnormal-ities observed in our patient have been previously reportedas a consequence of partial NCSE [13–15]. The absence ofa structural abnormality over the left occipital lobe sug-gests that the patient’s hemianopia represented a Todd’sphenomenon related to the frequent seizures originatingor spreading to the left occipital cortex [16].

Our case demonstrates that pure alexia may occur as aclinical manifestation of NCSE. To our knowledge, thisis the first such patient reported in the literature.

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