medicina

Case Report
Cognitive Status Epilepticus: Two Case Reports
Eleni Karantali 1, *,† , Symela Chatzikonstantinou 1 , Ioannis Mavroudis 2 , Constantin Trus 3, * and
Dimitrios Kazis 1

1 Third Neurological Department, Faculty of Medicine, Aristotle University of Thessaloniki,

57010 Thessaloniki, Greece; [email protected] (S.C.); [email protected] (D.K.)
2 Department of Neurosciences, Leeds Teaching Hospitals NHS Trust, Leeds LS97TF, UK; [email protected]
3 Department of Morphological and Functional Sciences, Faculty of Medicine, Dunarea de Jos University,
800008 Galati, Romania
* Correspondence: [email protected] (E.K.); [email protected] (C.T.); Tel.: +30-2313307301 (E.K.)
† Third Neurological Department, General Hospital of Thessaloniki, «G. Papanikolaou», Aristotle University of
Thessaloniki, Leoforos Papanikolaou, 57010 Thessaloniki, Greece.

Abstract: Cognitive status epilepticus is an uncommon form of focal status epilepticus presenting
with a dysfunction of language, thinking or associated higher cortical functions. The absence of ictal
manifestations can be misleading and delay a prompt diagnosis. Here we present two patients; one
with amnesic and one with aphasic status epilepticus. Through these cases, we aim to highlight the
value of EEG performance early in the diagnostic work-up and early antiepileptic drug initiation in
cases where an epileptic disorder cannot be excluded.

Keywords: status epilepticus; amnesia; aphasia; EEG







Citation: Karantali, E.; 1. Introduction

Chatzikonstantinou, S.; Mavroudis, I.; Focal status epilepticus (FSE) with or without an impairment of consciousness can
Trus, C.; Kazis, D. Cognitive Status be presented with motor or non-motor symptoms; the latter was formerly known as aura
Epilepticus: Two Case Reports. continua [1,2]. Typically, the duration is over an hour but on several occasions it can last
Medicina 2021, 57, 799. https://
for years. EEG findings represent locally restricted epileptic activity. FSE, when presented
doi.org/10.3390/medicina57080799
with non-motor symptoms, may be very challenging to diagnose.
In the present study, we describe the cases of two patients with cognitive status
Academic Editor: Marco Carotenuto
epilepticus; the first suffering from amnestic and the second from aphasic status epilepticus.
These cases address the difficulty of diagnosing such rare clinical entities.
Received: 12 July 2021
Accepted: 2 August 2021
2. Case Presentation
Published: 3 August 2021
2.1. Patient One; Amnestic SE
Publisher’s Note: MDPI stays neutral
A 49-year-old right-handed female patient presented with an amnestic disorder that
with regard to jurisdictional claims in started upon waking. Three months prior, a similar incident was reported, which lasted for
published maps and institutional affil- a day and was spontaneously resolved. A neurological examination revealed anterograde
iations. and autobiographical amnesia. The patient was afebrile and otherwise cooperative and
relatively calm. The emergency brain non-contrast computed tomography (NCCT) was
normal. Considering the patient’s personal history, we conducted an emergent scalp EEG,
which revealed continuous spike-and-wave complexes 3–3.5 Hz/s over the left hemisphere,
Copyright: © 2021 by the authors.
which consequently confirmed the diagnosis of amnestic status epilepticus (Figure 1A). No
Licensee MDPI, Basel, Switzerland.
other ictal features were seen. The patient was treated with intravenous benzodiazepines
This article is an open access article and valproic acid. Two days later, we performed a second EEG because of a residual
distributed under the terms and amnestic disorder, which revealed very frequent spike-and-wave activity (Figure 1B).
conditions of the Creative Commons The patient commenced brivaracetam as an add-on therapy and her symptoms and EEG
Attribution (CC BY) license (https:// findings resolved completely. When completely improved, the patient recollected all the
creativecommons.org/licenses/by/ incidence of her amnestic phase (Figure 1C). A brain MRI scan was performed within 24 h
4.0/).

Medicina 2021, 57, 799. https://doi.org/10.3390/medicina57080799 https://www.mdpi.com/journal/medicina

Medicina 2021, 57, 799 2 of 5

from the symptom onset and it was reported as being unremarkable. Two months later, on
follow-up, the patient remained seizure-free.

Figure 1. EEGs: Patient One. (A) Continuous spike-wave activity over the left hemisphere (maximum over frontotemporal
areas); (B) very frequent spike-wave activity (double banana montage, 0.3–70 Hz); (C) Intermittent theta wave activity over
the left frontotemporal region (average monopolar montage, 0.3–70 Hz).
Medicina 2021, 57, 799 3 of 5

2.2. Patient Two; Aphasic SE

A 48-year-old right-handed female patient presented with acute onset speech diffi-
culties upon waking. During the previous two weeks, similar episodes with a shorter
duration (few minutes) and a spontaneous remission were reported. Her past medical his-
tory included a chronic ischemic stroke with mild residual aphasia and right hemiparesis,
essential hypertension, hyperhomocysteinemia and hypercholesterolemia. A neurological
examination revealed receptive aphasia, right spastic hemiparesis and mild psychomotor
agitation. The patient was afebrile. A brain NCCT revealed a hypoattenuation of left
temporal lobe in keeping with a chronic stroke. The following day, a scalp EEG was
performed to exclude symptomatic epilepsy. No other ictal features were noticed. Con-
tinuous periodic epileptiform discharges (PEDs) on the left temporal region were noted,
confirming the diagnosis of focal aphasic status epilepticus (Figure 2A). After the initiation
of levetiracetam followed by the addition of valproic acid, the patient gradually improved
(Figure 2B). A brain MRI was performed within 24 h from symptom onset and revealed a
chronic ischemic stroke of the left temporal lobe. The rest of the diagnostic work-up was
unremarkable. On follow-up, two months later, the patient remained seizure-free.

Figure 2. EEGs: Patient Two. (A) Continuous periodic epileptiform discharges over the left temporal region; (B) very
frequent polymorphic theta and delta slow wave activity over the left hemisphere (double banana montage, 0.3–70 Hz).
Medicina 2021, 57, 799 4 of 5

3. Discussion
Based on ILAE epilepsy classification, the term focal status epilepticus (FSE) with or
without an impairment of consciousness is used to describe the entity formerly known
as partial status epilepticus [1]. The typical presentation is characterized by prominent
motor or sensorimotor symptoms. Rarely, focal status epilepticus can present with a
wide variety of non-motor symptoms with autonomic, cognitive, sensory, emotional or
behavioral arrest [1]. The minimum duration is reported to be over an hour with subtle
motor symptoms that can occur at time intervals not longer than 10 s whereas the symptoms
of non-motor FSE may be waxing and waning, continue uninterrupted and persist during
sleep [2]. In most patients with motor FSE, the impairment of consciousness can be safely
assessed. In contrast, a reliable assessment of consciousness in non-motor FSE is particularly
challenging especially when patients exhibit language or memory dysfunction [3].
Cognitive FSE presents with a dysfunction of language, thinking or associated higher
cortical functions [1]. These symptoms outbalance the presence of other epileptic manifes-
tations. In non-convulsive status epilepticus (NCSE), as the clinical signs and symptoms
are usually non-specific or diagnostic, the diagnosis is based on the EEG performance and
the initiation of an antiseizure drug. EEG patterns can be divided into diagnostic (e.g., focal
or generalized spikes, sharp waves or sharp-and-slow complexes) or uncertain significance
(e.g., periodic or rhythmic discharges) [4]. When the clinical suspicion of NCSE is high but
the EEG findings are non-diagnostic, a trial of an IV antiseizure drug is suggested [4].
Patient One presented with acute onset memory deficits and underlying focal spike-
wave complexes. Amnestic status epilepticus is a rare form of cognitive FSE. The dif-
ferentiation from transient global amnesia (TGA) can be particularly challenging. TGA
presents with an acute onset global (retrograde and anterograde) memory deficit lasting
less than 24 h. Other cognitive functions are typically normal. A single, definite etiology
has yet to been determined. The underlying pathophysiologic processes that have been
proposed include vascular, migraine, epileptic and psychogenic mechanisms. Transient
epileptic amnesia (TEA) can be presented as TGA. Typically, TEA episodes are shorter,
recurrent and, occasionally, accompanied by ictal manifestations such as oral automatisms
or hallucinations [5]. From a clinical point of view, the recurrence and atypical symptoms
such as confusion are the key to discriminating between TEA and TGA [5]. The diagnostic
value of an EEG in TGA may be limited. After a TGA episode, the EEG is typically normal.
On the contrary, an EEG in TEA is usually diagnostic. In patients presenting with atypical
TGA, an EEG must be performed. In our patient, the recurrent amnestic syndrome led us
to perform an EEG and diagnose the amnestic status epilepticus. In general, EEG findings,
a previous history of similar episodes, an atypical presentation and the improvement of
symptoms after the initiation of AEDs may contribute to the diagnosis.
Patient Two presented with an acute onset aphasia, which typically can be seen in
cerebrovascular events. Rarely, an acute onset aphasia can be the manifestation of epileptic
activity. In the absence of typical paroxysmal seizure symptoms, the differential diagnosis
solely on a clinical basis can be improbable. Both ictal aphasia and aphasic status epilepticus
vary in phenomenology (paraphasia to mutism) and EEG findings [3]. The initiation of
AEDs may be indicated in cases where epileptic aphasia cannot be excluded.
In conclusion, our cases highlight the difficulties of diagnosing cognitive status epilep-
ticus on a clinical basis and stress the value of performing an EEG early in the diagnostic
work-up.

Author Contributions: Conceptualization, E.K. and S.C.; methodology, not applicable; software, not
applicable; validation, not applicable; formal analysis, not applicable.; investigation, D.K.; resources,
C.T.; data curation, not applicable; writing—original draft preparation, E.K. and S.C.; writing—review
and editing, E.K., I.M. and C.T.; visualization, I.M.; supervision, D.K. and I.M.; project administration,
D.K.; funding acquisition, C.T. All authors have read and agreed to the published version of the
manuscript.
Medicina 2021, 57, 799 5 of 5

Funding: This research did not receive any specific grant from funding agencies in the public,
commercial or not-for-profit sectors.
Institutional Review Board Statement: The study was conducted according to the guidelines of the
Declaration of Helsinki and approved by the Institutional Review Board.
Informed Consent Statement: Informed consent was obtained from all subjects involved in the
study.
Conflicts of Interest: The authors declare no conflict of interest.

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