East Asian Arch Psychiatry 2023;33:95-9 | https://doi.org/10.12809/eaap2306

CASE REPORT

Atypical Presentations of Childhood Simple Partial Seizures: a Case Series 

Rahul Saha, Abhilasha Yadav, Pankaj Verma, MK Srivastava

Rahul Saha, Department of Psychiatry, VMMC & Safdarjung Hospital, New Delhi, India
Abhilasha Yadav, Department of Psychiatry, VMMC & Safdarjung Hospital, New Delhi, India
Pankaj Verma, Department of Psychiatry, VMMC & Safdarjung Hospital, New Delhi, India
MK Srivastava, Centre of Excellence in Mental Health, ABVIMS & Dr RML Hospital, Delhi, India

Address  for correspondence: Dr Rahul Saha, Department of Psychiatry, VMMC & Safdarjung Hospital, New Delhi, India. Email: drrahul.saha19@gmail.com 

Submitted: 15 February 2023; Accepted: 8 September 2023

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Introduction

Epilepsy is the fourth-largest cause of global disease burden. A total of 65 million people are estimated to be affected by epilepsy worldwide, with an incidence of 15 to 113 per 100 000 people per year.^1-3 The incidence of epilepsy is 0.5% to 2% in the general population, but in children it is more than twice that of the adult population.^4,5 Childhood- onset epilepsy describes a heterogeneous group of disorders with varying presentations and causes. Despite advances in the diagnosis and treatment of epilepsy, misdiagnosis is common.

In this case series, we report on three children with simple partial seizures who presented to the outpatient department with atypical signs and symptoms. Each patient had the atypical presentation of migraine, tingling sensations, and/or crying spells, together with mood-, sleep-, and anxiety-related symptoms. More atypical presentation can include rage, episodic nervousness with sensory, motor and autonomic symptoms. These cases underscore the importance of taking a detailed history and sequence of ictal signs and symptoms to enable accurate diagnosis and determination of the underlying pathophysiology. If the core features (ie, abrupt onset, short duration, stereotyped sequence of ictal signs and symptoms) of an epileptic seizure are present, the general rule for atypical presentation of epilepsy should be applied, ie, the more unusual experiential or behavioural phenomena are, the more likely that they are of epileptic origin.⁶

Case presentations

Patient 1

In June 2019, an 11-year-old Hindu boy presented to the VMMC & Safdarjung Hospital with a 1-year history of episodic headache. His father explained that the patient had initially started complaining of episodic headache one to two times per month but that this had increased to >10 times per month during the 6 months prior to presentation. The severity of the headaches had also increased over this period, with aggravation following strenuous activities and stress. The headaches were sudden in onset with a gradual increase in severity, pulsating, localised to the parietotemporal region, and lasting for approximately 15 to 20 minutes. The headaches were precipitated by going out in the sun and were relieved by analgesics. Prodromal aura in the form of elementary visual hallucinations (such as flickering lights or spots) and tingling sensations in the arms and legs were reported. The episodes caused numbness in the whole body and were exhausting, causing the patient to fall asleep afterward. The patient had stopped attending school due to the increase in both the frequency and severity of the headaches.

The patient had no history suggestive of generalised tonic-clonic seizure. Mental status examination revealed no significant psychopathology. Past history was non- contributory to the presentation. His elder sister had a history of absence seizures and was receiving treatment. Child psychiatry assessment, using the Kiddie Schedule for Affective Disorders and Schizophrenia (K-SADS), and neurological examination did not reveal any significant abnormality. Routine blood and urine test results were unremarkable. Magnetic resonance imaging of the brain did not reveal any significant abnormality. The patient had been diagnosed with conversion disorder by two clinicians and prescribed fluoxetine 20 mg/day for 2 months, but he had not responded to this treatment. He had also been diagnosed with migraine and prescribed propranolol 40 mg for 1 month and naproxen 250 mg as required, but he had no relief in symptoms.

The patient was suspected of having simple partial seizures because (1) a childhood presentation of migraine is rare; (2) there was no response to treatment; (3) simple partial seizures commonly present with headache; and (4) the episodes of headache were followed by exhaustion of the body.

Electroencephalography (EEG) showed diffuse epileptiform discharges, which confirmed the diagnosis of simple partial seizure (epileptic headache), as there was no loss of consciousness during the episodes of headache. The patient was started on carbamazepine and the dosage was gradually increased to 400 mg/day over 4 weeks. There were symptom improvement and considerable reduction in the frequency of headache episodes. The patient returned to school after 1 month of treatment and was maintained on carbamazepine 400 mg/day.

Patient 2

In September 2022, an 8-year-old Hindu girl presented to the VMMC & Safdarjung Hospital with a 2-year history of episodic tingling sensations all over the body. The sensation was reported as being like “ants crawling all over her body.” The episodes occurred initially two or three times per month, but the frequency increased to two to three times per day during the 6 months prior to the presentation. Episodes were sudden in onset, lasted for approximately 15 minutes, and were associated with crying and clinginess as she sought comfort from family members. Her father reported that the patient could not localise the tingling sensation to any particular part of her body. The sensations sometimes occurred in a single dermatome but could also occur across multiple dermatomes in a single episode. There was no precipitating or aggravating factor that brought on the episodes. The tingling sensation was not relieved by any medication including non-steroidal anti-inflammatory drugs and antidepressants. During the episodes, the patient became very anxious. Psychological and emotional support from family members did not alleviate this. She gradually became withdrawn and anxious with the increase in frequency of episodes. She had also stopped attending school in the 2 months prior to presentation due to the increased frequency of episodes.

There was no history suggestive of any neurological illness, generalised tonic-clonic seizure, or other Axis I psychiatric illness. Past history and family history were non-contributory to the presentation. Blood and urine tests did not reveal any abnormalities. Child psychiatry assessment using the K-SADS revealed only mild anxiety and sadness due to the repeated episodes. The patient had been diagnosed with conversion disorder by at least four different clinicians and prescribed fluoxetine 20 mg/day for 3 months. The patient had also been prescribed escitalopram 10 mg/day for 6 weeks and had received counselling sessions from a psychologist, but she had not responded to these treatments. Mental status examination revealed mild anxiety and sadness. Her thought content revealed worries related to the tingling sensation.

The patient was referred to the department of psychiatry and was suspected to have dissociative disorder and partial seizures. The rationales were that she did not respond to treatment, had stereotyped episodes of tingling sensations, absence of stressors, a very early presentation of dissociative symptoms (which is rare), and non-specific dermatomal presentation.

EEG showed diffuse epileptiform discharges, and the diagnosis was revised to simple partial seizure (somatosensory seizure), with anxiety not otherwise specified, as there was no loss of consciousness during the episodes. The patient was started on sodium valproate, with the dose gradually increased to 400 mg/day over 4 weeks. She was maintained on valproate 400 mg/day. The tingling sensation stopped and anxiety and depressive symptoms improved. There was no need to add antidepressants, as her anxiety and depressive symptoms were secondary to the seizures.

Patient 3

In January 2023, a 9-year-old Hindu girl presented to the VMMC & Safdarjung Hospital with a 1-year history of episodic crying spells. Her mother reported that the patient would suddenly start crying without apparent reason one to two times per month; the frequency of these episodes had gradually increased to one to two times per week at the time of the first consultation. These episodes lasted for up to 5 minutes and were self-remitting. The patient would sometimes wake from deep sleep and would start crying for 4 to 5 minutes at night. She did not remember the episodes afterwards and there was no precipitating factor.

The patient’s mother believed that the crying spells were due to depressive symptoms, but the patient did not seek any comfort or respond to affection from family members during the episodes. A video recording of one of the episodes showed abnormal movement of the fingers of her right hand and abnormal repeated clenching and opening movements of her right fist. These movements were present in all episodes. The patient was asymptomatic between episodes.

There was no history suggestive of any neurological illness, generalised tonic-clonic seizure, or any Axis I psychiatric illness. Past history and premorbid temperament were non-contributory to the presentation. Blood and urine tests did not reveal any abnormalities. Psychiatric assessment using the K-SADS and neurological examination did not reveal any abnormality. There was a family history of depression in a maternal uncle. The patient had been assessed by two paediatricians and prescribed clonazepam 0.25 mg as required, but there was no improvement in her symptoms.

The patient was suspected of having depression and partial seizure because she had stereotyped episodes of crying spells, abnormal movements of the fingers and clenching and opening of the fist, no memory of the episodes, absence of other depressive symptoms, and did not seek comfort during the crying spells.

EEG showed diffuse epileptiform discharges, and a diagnosis of simple partial seizure was made. The patient was started on sodium valproate and the dose was gradually increased to 400 mg/day over 4 weeks. The crying spells stopped and she was maintained on valproate 400 mg/day.

Discussion

Differentiating between simple partial seizure and psychiatric symptoms is a challenge in psychiatric practice.^1-3 Clinicians are often not aware of the psychiatric symptoms and tend to make a diagnosis of hysteria, conversion disorder or dissociative disorder if the presenting symptoms are uncommon. Even psychiatrists tend to focus on the anxiety or mood symptoms and less on symptoms characteristic of simple partial seizure. It is challenging to distinguish simple partial seizure from other neuropsychological disorders such as migraine, tingling sensations, and crying spells. In our three patients, the diagnosis of simple partial seizure was made after detailed investigation of personal and family history, presenting symptoms, and drug response.

Presenting symptoms

Patients with simple partial seizure may present with a variety of psychiatric symptoms (depression, anxiety, psychosis, hypomania, neurovegetation, fatigue, and cognitive issues) and unusual symptoms (headache, pain sensations, crying, singing, episodic dyspnoea, and vomiting).^1,7 Such patients are often misdiagnosed with psychiatric illnesses. Patient 1 presented with migraine at 12 years of age, which itself is rare. There are reports of episodes of headache, sometimes with features of migraine. These headaches are not followed or accompanied by other manifestations of a clear epileptic nature. They often present with diffuse epileptiform discharges on the EEG that begin and end with the headache if not followed by other epileptic manifestations. This is a true simple partial seizure manifesting as headache, known as an epileptic headache.⁸

Patient 2 presented with tingling sensations. These types of seizures are called somatosensory seizures. Somatosensory auras are simple partial seizures that typically manifest as a localised cutaneous tingling sensation.^9,10 Patient 3 presented with crying spells in the absence of any other depressive symptoms. These unusual findings gave the initial clue to the eventual diagnosis of simple partial seizure.

Associated specific and stereotyped symptoms

Stereotyped presentation of symptoms is the most specific for the diagnosis of simple partial seizures. Patient 1 presented with preictal aura followed by migraine headache with severe exhaustion of the body. Patient 2 presented with non-specific tingling sensations not localised to a dermatome and clinginess. Patient 3 presented with crying spells in the absence of depressive symptoms and amnesia regarding the episodes. The sudden onset of symptoms was also one of the most typical indicators of simple partial seizure.

Lack of response to previous treatment

In our patients, there was no response to the initial treatments. Patient 1 did not respond to anti-migraine medication and antidepressants. Patient 2 did not respond to antidepressants and psychological counselling. Patient 3 did not respond to treatment with clonazepam. In medical practice, when there is no response to the standard treatment for any illness, the diagnosis should be reviewed.

Family history

Only patient 1 had a family history of absence seizures in his elder sister. Patient 2 had no significant family history. Patient 3 had a family history of depressive disorder in a maternal uncle, which prompted her mother to seek consultation from our hospital.

Response to antiseizure treatment

In our patients, symptoms improved significantly after receiving antiseizure treatment, which is the strongest indicator for the diagnosis of simple partial seizure. All three patients had adjusted well to their daily routine following 4 to 8 weeks of antiseizure treatment. Patient 2 had considerable improvement in depressive and anxiety symptoms, even with no antidepressant treatment.

Investigation tools

EEG is useful for localising seizure foci and determining severity. Simple partial seizures are seldom revealed by routine scalp EEG studies, as they usually present with non- specific or unremarkable EEG features even during attacks, because the electric discharges during simple partial seizures may only involve subcortical regions.¹¹ Therefore, EEG findings are only supplementary; the diagnosis should be based on clinical presentation. In our patients, EEG findings confirmed our clinical findings and supported the diagnoses.

Only <5% of patients with epilepsy present with headache as the only manifestation of their seizures. According to the International Classification of Headache Disorders, ictal epileptic headache is defined as a condition when headache is the only clinical feature of epilepsy.^8,12-14

There were only 12 such cases in which the epilepsy was well documented using ictal EEG. Nonetheless, no conclusion can be made about the frequency of ictal epileptic headache.¹⁴ Ictal pain can sometimes be the only presenting symptom of a seizure and it can present as lateralised peripheral, abdominal or cephalic pain.^15,16 The most common brain area involved in the cephalic ictal pain seems to involve the parietal lobes, although epileptic activity can occur in a different location.^11,17 In addition, both migraine and epilepsy are characterised by paroxysmal bursts of transient cerebral dysfunction. These disorders might have similar pathophysiological mechanisms and risk factors. Ictal headache may present as a sensation of electricity, with varying intensity, or as a dull bilateral headache with a sensation of pressure across the frontal region and piercing retro-orbital pain.¹⁸ Rarely, it may be the sole symptom of a simple partial seizure or focal status epilepticus.^19,20 The clinical presentation of patient 1 seems to be one these rare cases.

Somatosensory auras are simple partial seizures that typically manifest as a localised cutaneous tingling sensation,^9,10 as seen in patient 2. The prevalence of somatosensory auras in patients with parietal lobe epilepsy was reported to be 63% and 44%.^21,22 Somatosensory auras are common in patients with simple partial seizure originating from the insular cortex and second sensory area,^23,24 but are less common in patients with frontal lobe epilepsy (with the prevalence being 16%, 17.5%, and 22%^22,25,26) and are rare in patients with temporal lobe seizures (with the prevalence being 1.7% to 4.5%^22,27,28). Although the affected brain area could not be localised in patient 2, the diagnosis of simple partial seizure was confirmed by symptom improvement after receiving antiepileptic medication.

Reports of crying spells as a presenting feature of epilepsy were few. Of seven cases in which the ictal or post-ictal semiology, as determined by ictal EEG, involved crying spells, five had a medial temporal onset of seizures and the remaining two had a midline frontal onset.²⁹ The epidemiology of crying spells is not known, as the only available data are from incidental reports.²⁹ In patient 3, the child presented with crying spells only, without any affect or emotional problems. This raised our suspicion of simple partial seizure and led to an EEG investigation.

In patients with a presumed first seizure, EEG shows epileptiform abnormalities in approximately 7% to 34% of episodes. When EEG is performed in the first 24 hours after an attack, the yield increases to 51%.³⁰ Repeated EEGs, sleep-deprived EEGs, and sleep EEGs all increase the likelihood of capturing epileptiform discharges.³¹

Nevertheless, some patients with epilepsy persistently have normal EEGs and some persons with epileptiform spike discharges may have an increased risk but never experience a seizure.³² EEG almost always show some paroxysmal change in seizures with altered consciousness, usually an evolving rhythmic ictal discharge is apparent if the seizures are epileptic.³³ Ideally, an ictal EEG should be performed to detect the underlying pathogenic mechanism when there are any atypical episodic presentations. Such categorical determination of pathogenesis is not always achievable by taking a history and following guidelines only.²⁷ A personalised and multiaxial diagnostic approach may be more helpful in making the diagnosis, even though ictal EEG is seldom practised in daily clinical settings.

Conclusion

The consequences of misdiagnosis and missed treatment of simple partial seizures are profound. Under- or over- diagnosis of simple partial seizure remains a challenge. Careful consideration of the differential diagnoses is of utmost importance when there are atypical presentations and no response to previous treatments. Prolonged video and ambulatory EEG monitoring provide additional help to determine the diagnosis.

Contributors

All authors designed the study, acquired the data, analysed the data, drafted the manuscript, and critically revised the manuscript for important intellectual content. All authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity.

Conflicts of Interest

All authors have disclosed no conflicts of interest.

Funding / Support

This study received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Data Availability

All data generated or analysed during the present study are available from the corresponding author on reasonable request.

Ethics Approval

The patients were treated in accordance with the tenets of the Declaration of Helsinki. The patients or their parents provided written informed consent for all treatments and procedures and for publication.

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