Importance of Recognizing Frontal Lobe Seizures in Down Syndrome: A Case Report

Dear Editor,

Around 8%–26.1% of patients with Down syndrome have seizures. ¹ Frontal lobe seizures have a varied presentation with complex semiology, which can be challenging to interpret. It can also present as episodes of crying along with features of anxiety, bizarre behavior, shouting, and other aggressive behavior. In individuals with Down’s syndrome with intellectual disability and language difficulty, there is a higher chance of these features getting misinterpreted as an affective disorder or as behavioral problems. Here, we present a case of a 17-year-old adolescent diagnosed with Down’s syndrome, mild mental retardation, and mixed receptive- expressive language disorder who was initially misdiagnosed with depression with complete improvement on sodium valproate after being assessed for seizure disorder.

Case Report

This case involves a 17-year-old adolescent male, diagnosed with Down’s syndrome, mild mental retardation, and mixed receptive-expressive language disorder, diagnosed as per ICD-10 criteria. ² He presented with an eight month history of episodes of sudden inconsolable crying for a few minutes along with complaints of headache, poor sleep, and poor attendance while at special boarding school with no clear stressors or precipitating factors. Subsequently, he was treated at a local hospital where he received independent trials of escitalopram 20 mg/day, amisulpride 100 mg/day, risperidone 0.5 mg/day, and aripiprazole 5 mg/day, each lasting for approximately two months with no improvement of symptoms and side effects of excessive drowsiness.

Due to this, he started attending a special school near his house as a day scholar. However, he continued to have these sudden crying episodes multiple times daily, along with headaches lasting for a few minutes in the frontal region with head banging, even at home and at his new school. He was also observed to have brief episodes of staring (a few seconds) and a headache for a few minutes at home. EEG was done twice; one had no abnormal findings, and the second had bi-frontal discharges. All the blood tests were within normal limits.

Due to these paroxysmal episodes of staring or crying, headaches, and mood- related symptoms, he was started on sodium valproate 1,000 mg/day as it could help with all of these symptoms. On initiation of sodium valproate 1,000 mg/day, the patient showed complete improvement with no further episodes of crying, headache or head-banging in the subsequent monthly follow-ups for 6 months. He was also noted to be regular to school and is currently being maintained on 750 mg/day of sodium valproate.

Discussion

Frontal lobe seizures are still considered one of the most difficult diagnosed conditions by clinicians, especially in populations with intellectual disabilities or language disorders. ³ The challenges lie in the complexity and heterogeneity of their clinical expression or semiology, and the typical rapid spread of electrical discharge that often makes surface EEG difficult to interpret.

Prefrontal seizures can present as ictal emotional, behavioral changes, with or without reported subjective emotional feeling, reflecting the key role of the frontal cortex in emotion perception and expression. Emotional expressions could be in the form of crying or laughing, or intense anxiety with or without hyperkinetic behavior. ⁴ Due to this, it can be commonly misdiagnosed as an affective disorder, especially when it is difficult to assess the emotional state of children and adolescents. ⁵ Diagnosis is more difficult when the patient has speech difficulties and is unable to express their difficulties in words, in disorders like mixed receptive-expressive language disorder. As antiepileptic agents like sodium valproate or carbamazepine can adequately control these episodes, early detection and management are essential for alleviating symptoms and improving quality of life. ⁶ However, in this case, the limitations include the lack of a detailed treatment history, the unavailability of neuroimaging, and a follow-up EEG after the remission of symptoms. A thorough evaluation is ideally needed in the management of this disorder.

Conclusion

Paroxysmal episodes with an affective component in patients with language disorder need a thorough evaluation of seizures with adequate antiepileptic treatment.

Consent for Publication

Informed assent from the adolescent and parental consent were obtained from the adolescent’s parent to publish the case report. Our institution does not require ethical approval for reporting individual cases.

Supplemental Material

Supplemental material for this article available online: The CARE checklist. ⁷