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 » Case Illustration
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Table of Contents    
CASE REPORT
Year : 2021  |  Volume : 69  |  Issue : 4  |  Page : 1040-1042

Ictal Smile and Fear In A Child With Refractory Epilepsy - An Interesting Electro Clinical Correlation


1 Department of Neurology, Krishna Institute of Medical Sciences, Secunderabad, Telangana, India
2 Department of Radiology, Krishna Institute of Medical Sciences, Secunderabad, Telangana, India
3 Department of Neurosurgery, Krishna Institute of Medical Sciences, Secunderabad, Telangana, India

Date of Submission05-Sep-2019
Date of Decision18-May-2020
Date of Acceptance14-Jul-2020
Date of Web Publication2-Sep-2021

Correspondence Address:
Sita Jayalakshmi
Senior Consultant Neurologist, Krishna Institute of Medical Sciences, Minister Road, 1-8-31/1, Secunderabad - 500 003, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.325357

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 » Abstract 


Ictal fear is a common manifestation with seizures originating from temporal lobe, orbitofrontal region and cingulate cortex. Ictal smile has been described in association with seizures from hypothalamus, cingulate cortex, lateral and prefrontal cortex. Isolated fear or ictal smile has been reported in various published reports in the past in patients with cingulate epilepsy. We report an unusual semiology with ictal smiling along with fear and preserved sensorium during the event, in a four-year-old child; Our hypothesis was that the ictal origin, most likely from the right mesial frontal or cingulate cortex. The child underwent resective surgery after multimodality evaluation and has been seizure free for 6 years post-surgery.


Keywords: Epilepsy, frontal lobe, ictal fear, ictal smile, invasive EEG
Key Message: Occurrence of ictal smiling along with intense fear could be a semiological occurrence in patients with cingulate epilepsy.


How to cite this article:
Jayalakshmi S, Nanda SK, Vooturi S, Vadapalli R, Panigrahi M. Ictal Smile and Fear In A Child With Refractory Epilepsy - An Interesting Electro Clinical Correlation. Neurol India 2021;69:1040-2

How to cite this URL:
Jayalakshmi S, Nanda SK, Vooturi S, Vadapalli R, Panigrahi M. Ictal Smile and Fear In A Child With Refractory Epilepsy - An Interesting Electro Clinical Correlation. Neurol India [serial online] 2021 [cited 2021 Sep 18];69:1040-2. Available from: https://www.neurologyindia.com/text.asp?2021/69/4/1040/325357




Ictal fear is a common manifestation with seizures originating from temporal lobe, orbitofrontal region and cingulate cortex.[1],[2],[3] The network for fear involves orbitoprefrontal, anterior cingulate, and temporal limbic cortices. Ictal smile is an uncommon manifestation of partial seizures and has been described in association with seizures from hypothalamus, cingulate cortex, lateral and prefrontal cortex and even parietal lobe.[3],[4] We report an unusual semiology with ictal smiling along with fear in a four-year-old child.


 » Case Illustration Top


The case report is from a tertiary referral care centre and necessary consents were obtained.

A 4-year-old child has presented with refractory partial epilepsy. The child was born of full term normal delivery. Two days after birth, he developed respiratory distress that was managed in neonatal intensive care unit. He had normal motor, social and language development and started attending play school. The child had his first unprovoked seizure at the age of one year eight months, three days after an episode of fever and was given sodium valproate for one month. There was no family history of epilepsy or febrile convulsions. He had the second unprovoked seizure after four months and continued to have infrequent seizures, once in six months for next two years.

The seizure description included sudden brief behavioral arrest followed by smiling for few seconds where mother could identify the onset. This was followed by intense fear and crying and purposeless movements of the right hand. The child was aware of the fear and could respond to questions during the event. Over time, the epilepsy became refractory to multiple anti-epileptic drugs (AEDs). He had 2 episodes of status epilepticus requiring hospitalization. During these events, the child had behavioral arrest followed by fear with abduction and extension of left upper limb and with loss of awareness. Despite multiple AEDs, the frequency of seizures increased to 3-4 per week in clusters, 90% during sleep and later became multiple daily seizures of 8-10 per hour for one month prior to his visit to our centre.

Non-invasive investigations

During the Video EEG, multiple events were recorded at a frequency of 4-8 per hour. The seizures started with a sudden behavioral arrest, then smiling for a few seconds, followed by intense fear and crying, right hand automatisms with immobile left upper limb, and preserved awareness throughout the event. The inter-ictal electroencephalogram (EEG) showed rhythmic right frontal sharp and slow wave discharges [Figure 1]a and intermittent generalized sharp and slow wave activity. The ictal EEG showed a rhythmic 2-3/second sharp and slow wave discharges, confined to the right frontal and parasagittal region throughout the event [Figure 1]b.
Figure 1: (a) Inter-ictal EEG showing rhythmic right frontal and parasagittal sharp and slow wave discharges (F4-C4, FZ-CZ) with a surface maximum negativity at F4 and (b) The ictal EEG showing rhythmic 2.5-3/second sharp and slow wave activity, confined to the right frontal leads FP2 and F4 and FZ (maximum negative at F4)

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His two previous 1.5 Tesla MRIs of the brain were normal. A repeat 3 Tesla MRI of the brain showed a subtle right cingulate and mesial frontal cortical thickening, a possible focal cortical dysplasia [Figure 2]a and [Figure 2]b. The inter-ictal 18-Fluoro deoxyglucose positron emission tomography (FDG PET) and the PET MRI fusion was suggestive of a right mesial frontal and cingulate mild hypometabolism [Figure 2]c and [Figure 2]d. The ictal single photon emission computerized tomographic (SPECT) scan performed after injection of the dye at 8 minutes in a seizure of 46 seconds duration, showed bifrontal hyper perfusion and non-lateralizing [Figure 3]. On Binet Kamath test, his mental age was four years two months, with an intelligence quotient of 105, but he had stopped going to school due to very frequent seizures. On parental interview, he had no psychiatric or behavioural problems, but quality of life was poor.
Figure 2: The 3 TMRI brain (a) Axial T1 and (b) Axial T2 weighted images showing a subtle right mesial frontal focal cortical thickening suggestive of a possible focal cortical dysplaisa (black arrow). Brain FDGPET (c) Axial image and (d) PET MRI fused image showing mild right mesial frontal hypometabolism (red arrow)

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Figure 3: The ictal SPECT scan showing bifrontal hyperperfusion at the convexity, with no clear cut lateralization

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Invasive evaluation

The child required an invasive monitoring due to lack of definite localization after multi-modality evaluation. As the pre-surgical evaluation data was lateralizing to the right hemisphere with a possible cingulate or a mesial frontal localization, an invasive grid placement (guided by neuronaviagation) was done as an emergency procedure when the child recovered from refractory status epilepticus but was still having frequent seizures. A 64-contact grid was placed on right frontal lobe covering the motor cortex and the prefrontal cortex, four 8-contact surface electrodes were placed each on the right orbitofrontal, right temporal, right inter-hemispheric region covering the posterior cingulate and paracentral lobule and left frontal cortex and one 8 contact depth electrodes in right cingulate cortex. The intracranial ictal EEG showed ictal EEG onset from cingulate region [Figure 4].
Figure 4: The histopathology suggestive of Focal cortical dysplasia Type IIA

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Surgery and outcome

The child underwent right cingulate, mesial frontal and prefrontal resection guided by electrocorticography. Histopathology was suggestive of focal cortical dysplasia type IIA [Figure 5]. All the AEDs were continued at the same doses for twelve months followed by gradual reduction. At last follow up, 6 years post-surgery, the child was seizure-free on one AED, and going to regular school.
Figure 5: The ictal invasive EEG recording (a) and (b- close up view)) showing 5 Hz rhythmic slow wave activity in anterior cingulate contacts for 2 seconds (arrow) followed by rhythmic spiking in the posterior cingulate (arrow) and 3 Hz slow wave activity in anterior cingulate contacts suggestive of ictal onset from the cingulate region

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 » Discussion Top


Ictal fear is a common manifestation with seizures originating from temporal lobe,[1] orbitofrontal region[2] and cingulate cortex.[3] Ictal smile has been described in association with seizures from hypothalamus,[4] cingulate cortex,[3] lateral and prefrontal cortex[4] and even parietal lobe.[4] Isolated fear or ictal smile has been reported in various published reports in the past in patients with cingulate epilepsy. Ictal smiling along with fear in the seizure semiology in the present case was an unusual combination. We hypothesized and confirmed that the ictal origin was from right cingulate cortex and the child is seizure free for 6 years after resection of this region.

The cingulate gyrus is part of the limbic system with extensive connectivity to different anatomic and functional areas, including the supplementary motor area,[3] hence an important structure in seizure propagation. Cingulate gyrus epilepsies comprise complex partial seizures with complex motor gestural automatisms at onset, autonomic signs, and changes in mood and affect. The affective behaviour can be diverse in form of fear, smile or laugh, crying, spitting, clapping, hand shaking and various other manifestations.[5],[6],[7] This can explain the semiology of ictal smile and fear in the present case.


 » Conclusion Top


Cingulate epilepsy can present with varied affective features and we propose that the occurrence of ictal smile and followed by intense fear could be a semiological occurrence in these patients.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 » References Top

1.
Biraben A, Taussig D, Thomas P, Even C, Vignal J, Scarabin J, et al. Fear as the main feature of epileptic seizures. J Neurol Neurosurg Psychiatry 2001;70:186-91.  Back to cited text no. 1
    
2.
La Vega-Talbot M, Duchowny M, Jayakar P. Orbitofrontal seizures presenting with ictal visual hallucinations and interictal psychosis. Pediatr Neurol 2006;35:78-81.  Back to cited text no. 2
    
3.
Alkawadri R, Mickey BE, Madden CJ, Van Ness PC. Cingulate gyrus epilepsy: Clinical and behavioral aspects, with surgical outcomes. Arch Neurol 2011;68:381-5.  Back to cited text no. 3
    
4.
Molinuevo JL, Arroyo S. Ictal smile. Epilepsia 1998;39:1357-60.  Back to cited text no. 4
    
5.
Oehl B, Biethahn S, Schulze-Bonhage A. Mirthful gelastic seizures with ictal involvement of temporobasal regions. Epileptic Disord l2009;11:82-6.  Back to cited text no. 5
    
6.
Mazars G. Criteria for identifying cingulate epilepsies. Epilepsia l1970;11:41-7.  Back to cited text no. 6
    
7.
Clusmann H, Schramm J, Kral T, Helmstaedter C, Ostertun B, Fimmers R, et al. Prognostic factors and outcome after different types of resection for temporal lobe epilepsy. J Neurosurg 2002;97:1131-41.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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