Epilepsy & Behavior 49 (2015) 198–202

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Brief Communication

De novo status epilepticus with isolated aphasia Dominique Flügel a,⁎,1, Olaf Chan-Hi Kim b,1, Ansgar Felbecker a,1, Barbara Tettenborn a,1 a b

Department of Neurology, Kantonsspital St. Gallen, Rorschacherstrasse 95, 9000 St. Gallen, Switzerland Department of Neuroradiology and Nuclear Medicine, Kantonsspital St. Gallen, Rorschacherstrasse 95, 9000 St. Gallen, Switzerland

a r t i c l e

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Article history: Revised 4 May 2015 Accepted 5 May 2015 Available online 1 June 2015 Keywords: Status epilepticus Aphasia

a b s t r a c t Background: Sudden onset of aphasia is usually due to stroke. Rapid diagnostic workup is necessary if reperfusion therapy is considered. Ictal aphasia is a rare condition but has to be excluded. Perfusion imaging may differentiate acute ischemia from other causes. In dubious cases, EEG is required but is time-consuming and laborious. We report a case where we considered de novo status epilepticus as a cause of aphasia without any lesion even at follow-up. A 62-year-old right-handed woman presented to the emergency department after nurses found her aphasic. She had undergone operative treatment of varicosis 3 days earlier. Apart from hypertension and obesity, no cardiovascular risk factors and no intake of medication other than paracetamol were reported. Neurological examination revealed global aphasia and right pronation in the upper extremity position test. Computed tomography with angiography and perfusion showed no abnormalities. Electroencephalogram performed after the CT scan showed left-sided slowing with high-voltage rhythmic 2/s delta waves but no clear ictal pattern. Intravenous lorazepam did improve EEG slightly, while aphasia did not change. Lumbar puncture was performed which likely excluded encephalitis. Magnetic resonance imaging showed cortical pathological diffusion imaging (restriction) and cortical hyperperfusion in the left parietal region. Intravenous anticonvulsant therapy under continuous EEG resolved neurological symptoms. The patient was kept on anticonvulsant therapy. Magnetic resonance imaging after 6 months showed no abnormalities along with no clinical abnormalities. Conclusions: Magnetic resonance imaging findings were only subtle, and EEG was without clear ictal pattern, so the diagnosis of aphasic status remains with some uncertainty. However, status epilepticus can mimic stroke symptoms and has to be considered in patients with aphasia even when no previous stroke or structural lesions are detectable and EEG shows no epileptic discharges. Epileptic origin is favored when CT or MR imaging reveal no hypoperfusion. In this case, MRI was superior to CT in detecting hyperperfusion. This article is part of a Special Issue entitled “Status Epilepticus”. © 2015 Elsevier Inc. All rights reserved.

1. Introduction Sudden onset of aphasia can underlie various etiologies such as ischemia, trauma, infection (especially herpes encephalitis), or intoxication (lithium), but most often it is due to stroke. Aphasia may also present as an ictal or postictal state of temporal or frontal lobe seizures. In 16–17% of focal seizures, aphasia has been found as a part of ictal aura often occurring at the end of an aura and together with other symptoms such as familiarity and thought disorder [1]. Ictal aphasia as a manifestation of status epilepticus is rare and, most often, underlying lesions such as acute or remote cerebrovascular accident (hemorrhage/stroke)

⁎ Corresponding author. E-mail addresses: dominique.fl[email protected] (D. Flügel), [email protected] (O.C.-H. Kim), [email protected] (A. Felbecker), [email protected] (B. Tettenborn). 1 Tel.: +41 71 4941111; fax: +41 71 4942895.

http://dx.doi.org/10.1016/j.yebeh.2015.05.009 1525-5050/© 2015 Elsevier Inc. All rights reserved.

and tumor, among others, can be found [2,3]. A case of de novo ictal status without any lesion even at follow-up is reported. 2. Case report A 62-year-old right-handed woman was transferred to the neurological department from surgery where she had undergone operative treatment of varicosis 3 days earlier to the neurological department. Nurses found her “changed” in the morning at 5:30 a.m., and she could not remember her name nor follow instructions. Apart from hypertension and obesity, no cardiovascular risk factors, no history of migraine, and no intake of medication other than paracetamol were reported. The first computed tomography (CT) scan – initiated by the surgeons at 7:25 a.m. – was normal. Blood tests were normal. Neurological examination revealed global (expressive and receptive) aphasia and pronation in the right upper extremity on position testing. Repeated CT with angiography and perfusion at 11:25 a.m. showed no abnormalities, especially no signs of stroke. Electroencephalogram (EEG) performed

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Fig. 1. (Longitudinal bipolar montage): high-voltage semirhythmic 2-Hz delta activity on the left side, most often generalized, waxing and waning, and partly suppressed by eye opening. Intermittent 8- to 9-Hz normal background activity can be seen. Lorazepam did improve EEG slightly, while aphasia did not change.

following normal CT scan was pathological with left-sided slowing, with high-voltage 2/s delta waves which were mostly generalized, but no epileptiform discharges (Fig. 1). Intravenous lorazepam did improve EEG only slightly, while aphasia did not change. Lumbar puncture results were normal, which likely excluded encephalitis. Magnetic resonance imaging (MRI, 1.5 T) at 1 p.m. (with the patient still aphasic) showed no edema in the fluid-attenuated inversion recovery (FLAIR) sequences (Fig. 2), while diffusion-weighted images (DWIs) showed cortical diffusion restriction in the left parietal lobe (Fig. 3) with no signal loss in this region in the apparent diffusion coefficient (ADC) map (Fig. 4).

Evaluation of the T2 perfusion study with OLEA® software showed a signal drop in the left parietal lobe in comparison to the contralateral region as a sign of hyperperfusion (Fig. 5). T1 imaging after gadolinium could not detect any structural abnormalities (Fig. 6). Magnetic resonance imaging revealed hyperperfusion in the left parietal region consistent with the diagnosis of nonconvulsive status epilepticus (NCSE). Intravenous anticonvulsant therapy (valproate) under continuous EEG was initiated, and neurological symptoms resolved within 48 h. Follow-up MRI as well as EEG 6 months later showed no abnormalities along with no clinical abnormalities.

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Fig. 4. ADC: No significant signal drop in the left parietal region. Fig. 2. FLAIR image shows no edema in the cortex of the parietal left lobe.

3. Discussion Ictal aphasia may present as pure motor, sensory, or mixed aphasia and is clinically undistinguishable from other causes [4,5]. The differential diagnosis includes stroke or other structural/metabolic damage, inflammation, persistent migraine aura, or postictal state. There was no history of migraine; therefore, migraine aura is unlikely to have caused these symptoms. Imaging, even with contrast enhanced images could not reveal structural pathology. On the other hand, EEG was pathological with high-voltage slow rhythmic delta activity. Criteria for NCSE have been proposed and include rhythmic delta/ theta activity with either EEG and clinical improvement after intravenous administration of anticonvulsant drugs or subtle clinical ictal phenomena during the EEG patterns or typical spatiotemporal evolution [6]. Benzodiazepines did improve EEG slightly, however, without clinical improvement. Improve the EEG under benzodiazepine treatment

Fig. 3. DWI shows reversible cortical diffusion restriction in the left parietal lobe.

without normalization of the clinical picture may not be helpful in differentiating NCSE, since EEG improvement can also be seen in metabolic encephalopathy [7]. Pure persistent rhythmic delta activity has been reported in NCSE [8]; more often toxic-metabolic encephalopathies and viral or limbic encephalitis [9,10] present with pure rhythmic delta activity. These conditions could more or less be excluded by normal spinal tap, and resolving aphasia under anticonvulsant therapy may support ictal origin. De novo status epilepticus presenting with aphasia has been reported in multiple sclerosis [11,12], diabetes [13], nonketotic hyperglycemia [14,15], and AIDS–toxoplasmosis complex [16] or induced by the cephalosporine cefepime [17]; these conditions could also be excluded. Nevertheless, the detection of ictal activity in prolonged aphasia can be difficult as scalp EEG might not show abnormalities. Hirsch et al. [18] described apparent postictal aphasia in a patient without scalp EEG correlate as an ongoing seizure was proven by intracranial depth electrodes. Ericson et al. [19] found standard EEG (30 min) in aphasic status epilepticus to be sensitive in only 5/9 (56%) patients, while continuous EEG confirmed seizure activity. Dong et al. [20] showed aphasic NCSE in 5 patients with (18)F-fluorodeoxyglucose positron emission tomography (FDG-PET), while EEG failed to show clear ictal activity. Seizure duration of aphasia over long periods of time is not uncommon and has been reported to last from several days [3,21,22] up to weeks [23], causing difficulties in differentiating between postictal and ictal states [24]. Computed tomography and magnetic resonance imaging are often more readily available than EEG in an emergency setting and helpful in diagnosing ictal activity. Focal hyperintensities on DWIs with ADC reduction have been found after prolonged seizures or status resembling hemodynamic changes with ictal hyperperfusion, cell swelling, and cytotoxic edema [25–28]. Magnetic resonance imaging abnormalities may be unilateral or bilateral, and location has been found to be cortical and subcortical, involving the basal ganglia, hippocampus, corpus callosum, and cerebellum, with variable reversibility [29]. In aphasic status epilepticus, Toledo et al. [30] found cortical DWI hyperintensity in the parietotemporal region in 6/8 patients (4 patients also showed hyperintensity in the ipsilateral pulvinar regions), along with hyperperfusion in this region in 5/6 patients and ADC reduction in the areas of most pronounced signal change on DWIs. Our patient

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Fig. 5. Evaluation of T2 perfusion imaging with OLEA® software shows a signal drop in the left parietal lobe in comparison to the contralateral region as a sign of hyperperfusion.

did not show ADC reduction. This might be due to only subtle signal change on DWIs. Magnetic resonance imaging changes do not necessarily represent the epileptic focus and are observed distant from the primary language areas [30], resembling involvement of sensitive areas during seizure spread [28].

ischemic lesion would be very unlikely in the presence of longstanding aphasia of lengthy duration (hours). Nonconvulsive status epilepticus is often underdiagnosed, and aphasic status epilepticus may present with no further signs of a seizure or precipitating lesions. Magnetic resonance imaging can be helpful in the diagnosis, with hyperperfusion and localization reflecting seizure spread.

4. Conclusion Since our MRI findings were only subtle and EEG was without clear ictal pattern, the diagnosis of aphasic status remains with some uncertainty. Ischemia or other nonictal etiologies cannot be excluded completely; however, hyperperfusion in the acute

Conflict of interests All authors confirm that they have read the Journal's position on issues involved in ethical publication and affirm that this report is

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Fig. 6. T1 imaging after gadolinium shows no pathological enhancement.

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De novo status epilepticus with isolated aphasia.

Sudden onset of aphasia is usually due to stroke. Rapid diagnostic workup is necessary if reperfusion therapy is considered. Ictal aphasia is a rare c...
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