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\begin{document}

\title{Paroxysmal pain as the only presentation of focal epilepsy}



\author[1]{Daniela Garcez}%
\author[1]{Mariana Fernandes}%
\author[1]{Joana Marques}%
\author[1]{John Foreid}%
\affil[1]{Instituto Portugues de Oncologia de Lisboa Francisco Gentil EPE}%


\vspace{-1em}



  
  \date{June 18, 2024}


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\begin{abstract}
A 55-year-old man with left temporo-insulo-parietal glioblastoma was
admitted because of abrupt excruciating pain in right arm and
ipsilateral face. EEG showed a left central frontal rhythmic activity
and complains were controlled with anti-epileptic drugs. Paroxysmal pain
is an unusual manifestation of focal seizures and can be the only
symptom.%
\end{abstract}%



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\textbf{Key Clinical Message:}

- Painful seizures can be the only feature of focal epilepsy.

- Its localization on a limb favors the epileptic etiology while
abdominal or cephalic paroxysmal pain is more unspecific.

- Painful somatosensory seizures arise from operculo-insular cortex.

\textbf{Background:}

Ictal pain is a rare manifestation of focal seizures, with a frequency
ranging from 0,2 to 2,8\% in patients with epilepsy\textsuperscript{1}.
Classically, it is categorized according to its localization as
cephalic, abdominal (visceral or pneumogastric) or unilateral
somatosensory pain\textsuperscript{2}. The last is usually striking and
can be described as a burning, stabbing, prickling, throbbing, or
tearing sensation. In most cases they are accompanied by other motor,
sensory or behavioral features which can denote their ictal
origin\textsuperscript{3}. When it occurs in isolation, seizures are
often misdiagnosed and patients can go through unnecessary diagnostic
procedures and inadequate treatment. While most patients who present
with paroxysmal headache or abdominal pain do not have an epileptic
cause in their origin, the localization of paroxysmal pain on a limb is
more suggestive of an underlying epileptic cause\textsuperscript{1}.

\textbf{Case Presentation:}

A 55-year-old man was diagnosed with a left temporo-insulo-parietal left
tumor after developing focal cognitive seizures with aphasia and complex
visual hallucinations (fig. 1). He underwent partial tumor resection
resulting in persistent dysphasia. The histopathology diagnosis was
compatible with a glioblastoma. He was medicated with levetiracetam and
sodium valproate due to increasing of seizure frequency, characterized
by transitory worsening of previous dysphasia. He started treatment with
chemotherapy and radiotherapy (according to STUPP protocol), with an
initial good radiological response. While undergoing oral chemotherapy
cycles, he was admitted in the Emergency Room, desperately screaming due
to acute and excruciating pain on the right arm and ipsilateral face,
described as shock-like and stabbing. Each episode lasted a few seconds
but they had been repeating in cluster for over an hour. General blood
analyses were unremarkable and the electroencephalography showed a left
central frontal rhythmic activity during the pain episodes (fig. 2). The
add-on of intravenous lacosamide resolved the painful complaint in a few
minutes. The brain MRI performed a few days later showed local tumor
recurrence (fig. 2).

\textbf{Discussion:}

Although ictal pain is often associated with other seizure symptoms, it
can be the only manifestation of focal epilepsy and differentiating them
from others can be challenging. Among patients with ictal pain, those
who report it to their limbs are more likely to have an epileptic
cause\textsuperscript{1}.

Under some pathophysiological conditions, operculo-insular lesions may
produce location-specific painful epileptic seizures. Functional imaging
studies have identified several cortical areas activated by painful
stimuli, referred as ``pain matrix'', including, among others, the
primary somatosensory area, the supplementary motor area, the insula,
the anterior frontal or the posterior parietal
cortices\textsuperscript{4}. Some cases of ictal pain with foci
originating in the frontal, parietal and temporal regions have been
described with surface EEG\textsuperscript{3,5}. However, data from
functional cortical mapping by using direct cortical stimulation
suggests that the origin of painful somatosensory seizures arises solely
from the medial part of parietal operculum or the posterior and upper
part of the insular cortex\textsuperscript{6,7}.

Taking into account the cortical mapping studies and the localization of
our patient's glioblastoma, we suggest that the painful seizure has a
left operculo-insular origin and that the rhythmic activity captured on
the surface EEG represents propagation to the primary parietal cortex.

Paroxysmal contralateral pain can be the only manifestation of epileptic
seizures with operculo-insular cortex involvement. This knowledge is
useful to avoid misdiagnosis and to prompt appropriate management with
antiepileptic drugs.

\textbf{Author Contribution Statement:}

DG: wrote the manuscript; MF and JM: evaluated the patient and revised
the manuscript; JF: carried out the EEG assessment. All authors approved
the final version to be published.

\textbf{References:}

1 - Asadi-Pooya, Ali A. et al. Ictal pain: occurrence, clinical
features, and underlying etiologies, \emph{Epilepsy \& Behavior} ,
Volume 61 (2016), 59 - 62. DOI: 10.1016/j.yebeh.2016.05.006

2 - G. Bryan Young, Warren T. Blume. Painful epileptic
seizures,\emph{Brain} , Volume 106, Issue 3, September (1983), Pages
537--554. https://doi.org/10.1093/brain/106.3.537

3 - Hwang, S.T., Goodman, T. \& Stevens, S.J. Painful Seizures: a Review
of Epileptic Ictal Pain.~\emph{Curr Pain Headache Rep~} 23\textbf{,~} 83
(2019). https://doi.org/10.1007/s11916-019-0825-6

4 - R. Peyron, B. Laurent, L. Garc\selectlanguage{ngerman}ía-Larrea. Functional imaging of brain
responses to pain. A review and meta-analysis
(2000),\emph{Neurophysiologie Clinique/Clinical Neurophysiology} ,
Volume 30, Issue 5, (2000), Pages 263-288.
https://doi.org/10.1016/S0987-7053(00)00227-6

5 - Dileep R. Nair, Imad Najm, Juan Bulacio, Hans Lüders. Painful auras
in focal epilepsy, \emph{Neurology,} Aug (2001), 57 (4) 700-702. DOI:
10.1212/WNL.57.4.700

6 - Laure Mazzola, Jean Isnard, Roland Peyron, François Mauguière.
Stimulation of the human cortex and the experience of pain: Wilder
Penfield's observations revisited,~\emph{Brain} , Volume 135, Issue 2,
February (2012), Pages 631--640.~https://doi.org/10.1093/brain/awr265

7- Alexandra Montavont, Francois Mauguière et al. On the origin of
painful somatosensory seizures\emph{, Neurology} Feb (2015), 84 (6)
594-601. DOI: 10.1212/WNL.0000000000001235\selectlanguage{english}
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