ADHD in West syndrome

Sir,

West syndrome comprises a triad of infantile spasms seen in clusters of 2–3/day, hypsarrhythmia on EEG, and may or may not be associated with developmental delay usually noticed in the first year of life. Spasms are seen as symmetrical, brief, and sudden axial contractions lasting anywhere between less than 1 second to 3 seconds and repeat in clusters every 5–15 seconds for up to minutes. Spasms are a type of seizure and are often very subtle. EEG pattern shows a pattern of high-amplitude slow waves with discharges of spikes and waves varying in duration and amplitude. The spasms typically occur in quick succession, often right before falling asleep or upon waking. Following the spasms, affected children may exhibit irritability or drowsiness. Most children are seizure-free by the time they are 5 years old. Occasionally, other forms of seizures (atonic spasms, myoclonic seizures, and focal and absence seizures) or persisting cognitive or motor delay may appear.[1] It can be caused by several etiologies, usually regarded as a response of an immature brain to any pre-, peri-, or post-natal brain insult.[2] West syndrome is also associated with cognitive decline and persisting behavioral abnormalities in the child, such as autism and attention deficit hyperactivity disorder. Intellectual disability is seen in approximately 71%–80% of children with West syndrome, 13% suffer from autism, and hyperkinetic disorder is seen in 15%.[3]

A 22-year-old unmarried male presented to the outpatient department with his parents and elder brother, with complaints of restlessness, excessive fidgeting, demanding behavior, an inability to wait for rewards, and constant interruption of others’ conversations. Irritability and reduced sleep worsened over the last 4 months after the patient stopped taking his regular medications. Birth history was normal. The patient’s parents noticed a delay in developmental milestones as compared to his elder brother and observed that he was not able to hold his neck by himself or stand without support till he was the age of 16 months. His parents noticed spasms, which they described as contractions along the length of his body associated with stiffness of his arms and legs almost looking like a startle that would last for a couple of seconds occurring in clusters. His parents consulted a pediatrician, and he was diagnosed with West syndrome when an EEG was performed, showing hypsarrhythmia. The patient was administered ACTH injections (0.02 mg/kg/day) every week for around 2 months with complete resolution of the spasms. The patient was seizure-free since around 4 years of age. At 5 years of age, parents noted hyperactive behavior in the form of easy distractibility, refusal to sit in one place, and inability to focus on given tasks. The patient’s class teachers reported that the patient had difficulty communicating his needs and could not socialize as well as other children his age. They consulted a doctor who treated him with methylphenidate, but the family reported a worsening of hyperactive behavior with the medications. After multiple trials of medicines, the patient showed partial improvement on risperidone 2 mg, trihexyphenidyl 2 mg, and lorazepam 2 mg. Compliance with medications was poor owing to over-sedation and inability to attend school. They completely stopped his medications 4 months before he presented to us during which his symptoms worsened. No similar symptoms were noted in the family or any of the patient’s siblings. Physical examination revealed no abnormalities. A mental status examination revealed a hyperactive, young male extremely restless and refusing to sit in his chair. The speech was spontaneous with a reduced reaction time. No abnormalities of thought or perception. Insight was 1/5, and social judgment was impaired. A diagnosis of attention deficit hyperactive disorder (ADHD), predominantly hyperactive type was made. The patient was started on T. risperidone 4 mg, T. trihexyphenidyl 3 mg, and t. divalproate 750 mg and was advised to follow up regularly. The family displayed a good knowledge of the patient’s condition, and social support was satisfactory.

ADHD is seen in 15% of children with West syndrome; thus, this case report aims to highlight the importance of diagnosis and prompt treatment of the associated residual sequelae of such diseases. Controlling hyperactive symptoms in such patients is difficult.[3] Therefore, early detection of such cases is important. The neural mechanism of ADHD in such patients could be different from the children without comorbidities apart from ADHD. Cognitive, behavioral, and emotional problems are commonly seen in children with epilepsy. Apart from the burden of epilepsy in itself, the most common problems are impaired attention and hyperactivity,[4] and these symptoms are often misdiagnosed. Delay in treatment is associated with worse cognitive outcomes. Therefore, further evaluation and study of hyperactive symptoms in West syndrome and other epileptic syndromes need to be undertaken to ensure prompt diagnosis and treatment as well as allay the concerns of family members.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Authors contribution

Concept, design, definition of intellectual content, literature search, data acquisition: AP, SC, AJ, SRM Data acquisition: AP, SRM Manuscript preparation: AP, SC, Manuscript editing and manuscript review. AJ, SRM Guarantor: SC.

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.