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Published on: February 2, 2024
Infantile spasms
1Pediatric Epilepsy Center, Washington University School of Medicine and St. Louis Children's Hospital, St. Louis, MO 63110-1093, USA.
Insights
Infantile spasms, also known as West syndrome, are a severe epilepsy. Recent advances in diagnosis and treatment offer hope for improved outcomes in affected infants.
Area of Science:
- Pediatric Neurology
- Epileptology
- Developmental Neuroscience
Background:
- Infantile spasms (IS) are a distinct seizure type and age-specific epilepsy syndrome.
- Classification has evolved, distinguishing seizure type (epileptic spasms) from epilepsy syndrome (West syndrome).
- Advances in neuroimaging and understanding pathophysiology are improving diagnostic yield.
Purpose of the Study:
- To review current understanding and management of infantile spasms.
- To highlight recent advances in diagnosis, treatment, and prognosis.
- To discuss the evolving classification and therapeutic landscape.
Main Methods:
- Review of recent literature on infantile spasms and West syndrome.
- Analysis of clinical, electrographic, and neuroimaging findings.
- Evaluation of current and emerging therapeutic options.
Main Results:
- Epileptic spasms and hypsarrhythmia have more detailed clinical and electrographic descriptions.
- Neuroimaging advances aid in identifying etiology and understanding pathophysiology.
- Vigabatrin is supported as first-line therapy; other agents like topiramate, lamotrigine, and zonisamide show promise.
- Adrenocorticotrophic hormone remains a common treatment; surgical options exist for select cases.
- Visual-field constriction is a concern with vigabatrin.
Conclusions:
- Prognosis for infantile spasms remains generally poor, but research offers hope.
- Identifying predictors of favorable prognosis is crucial.
- Advances in understanding pathophysiology may lead to safer, more effective therapies.
- Improved long-term outcomes are anticipated with continued research and therapeutic innovation.
Abstract:
Infantile spasms constitute both a distinctive seizure type and an age-specific epilepsy syndrome that have been extensively described for over a century. Standardization of the classification of infantile spasms has evolved, culminating in recent recommendations for separately recognizing and distinguishing the seizure type (spasms or epileptic spasms) and the epilepsy syndrome of infantile spasms (West syndrome). More-detailed descriptions of the clinical and electrographic features of epileptic spasms and hypsarrhythmia have emerged. Advances in neuroimaging techniques have revealed clues about pathophysiology and increased the etiologic yield of the diagnostic evaluation of patients with infantile spasms. Adrenocorticotrophic hormone remains the treatment of choice for many neurologists. Recent controlled studies support vigabatrin as first-line therapy, and open-label studies suggest that topiramate, lamotrigine, and zonisamide may be useful in treating spasms. Recent reports of visual-field constriction with vigabatrin may limit its use. Surgical treatment has been used successfully in a select subgroup of patients with secondarily generalized spasms from a single epileptogenic zone. Although the prognosis for most patients with infantile spasms remains poor, further studies identifying predictors of favorable prognosis and recent advances in understanding the pathophysiology of infantile spasms offer hope of safer and more-effective therapies that improve long-term outcome.
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