Recognition of Infantile Spasms Is Often Delayed: The ASSIST Study

Hussain, Shaun A.; Lay, Johnson; Cheng, Emily H.; Weng, Julius; Sankar, Raman; Baca, Christine B.

doi:10.1016/j.jpeds.2017.08.009

Cited by 37 publications

(37 citation statements)

References 22 publications

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“…Also, there are several other crucial facets that need to be addressed in the management of WS. These include frequent misdiagnosis, diagnostic and treatment lag, availability of and familiarity with treatment modalities, quantification of response to treatment, and neurodevelopmental sequelae 10,11 . These fronts are handled differently in not only different clinical setups but also in different nations 12–14 …”

Section: Introductionmentioning

confidence: 99%

Management practices for West syndrome in South Asia: A survey study and meta‐analysis

Madaan

Chand

Linn³

et al. 2020

Epilepsia Open

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Objectives Considering the dearth of literature on West syndrome (WS) from South Asian countries, this study aimed to evaluate the management practices in South Asia by an online survey and meta‐analysis. Methods An online questionnaire was sent to 223 pediatric neurologists/pediatricians in India, Pakistan, Myanmar, Sri Lanka, Bhutan, Nepal, and Bangladesh. Their responses were evaluated and supplemented by a meta‐analysis. Results Of 125 responses received (response rate: 56%), around 60% of responders observed male preponderance and an approximate lead‐time‐to‐treatment (LTTT) of 4‐12 weeks. The commonest etiology observed was a static structural insult (88.6% of responders). Most commonly used first‐line drug (country‐wise) was as follows: India—adrenocorticotropin hormone (ACTH, 50%); Pakistan—oral steroids (45.5%); Myanmar, Sri Lanka, and Nepal—oral steroids (94.4%); Bangladesh—ACTH (2/2); Bhutan—vigabatrin (3/5). ACTH and vigabatrin are not available in Myanmar and Nepal. The most commonly used regime for ACTH was maximal‐dose‐at‐initiation‐regime in India, Sri Lanka, and Bangladesh and gradually escalating‐regime in Pakistan. Maximum dose of prednisolone was variable—most common response from India: 3‐4 mg/kg/d; Pakistan, Bhutan, and Bangladesh: 2 mg/kg/d; Sri Lanka, Nepal, and Myanmar: 5‐8 mg/kg/d or 60 mg/d. The total duration of hormonal therapy (including tapering) ranged from 4 to 12 weeks (67/91). Most responders considered cessation of spasms for four weeks as complete response (54/111) and advised electroencephalography (EEG; 104/123) to check for hypsarrhythmia resolution. Difficult access to pediatric EEG in Bhutan and Nepal is concerning. More than 95% of responders felt a need for more awareness. The meta‐analysis supported the preponderance of male gender (68%; confidence interval [CI]: 64%‐73%), structural etiology(80%; CI 73%‐86%), longer LTTT (2.4 months; CI 2.1‐2.6 months), and low response rate to hormonal therapy(18% and 28% for ACTH and oral steroids respectively) in WS in South Asia. Significance This study highlights the practices and challenges in the management of WS in South Asia. These include a preponderance of male gender and structural etiology, a longer LTTT, difficult access to pediatric EEG, nonavailability of ACTH and vigabatrin in some countries, and low effectiveness of hormonal therapy in this region.

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Section: Introductionmentioning

confidence: 99%

Management practices for West syndrome in South Asia: A survey study and meta‐analysis

Madaan

Chand

Linn³

et al. 2020

Epilepsia Open

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“…In this review, IS is used in the broad syndromic sense to identify infants with ES, but not exclusively those who present with hypsarrhythmia and developmental regression. Although IS is the most common epilepsy syndrome in the first year of life, diagnosis and treatment are often significantly delayed with potentially catastrophic consequences . Too often, IS masquerades as gastroesophageal reflux (Sandifer syndrome), benign sleep myoclonus, and a variety of “normal” infant behaviors.…”

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confidence: 99%

“…Although IS is the most common epilepsy syndrome in the first year of life, 4 diagnosis and treatment are often significantly delayed with potentially catastrophic consequences. 5 Too often, IS masquerades as gastroesophageal reflux (Sandifer syndrome), benign sleep myoclonus, and a variety of "normal" infant behaviors. The diagnosis is established with video-electroencephalography (EEG) to confirm the presence of ES and ascertain the presence or absence of hypsarrhythmia.…”

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confidence: 99%

Treatment of infantile spasms

Hussain

2018

Epilepsia Open

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SummaryThe treatment of infantile spasms is challenging, especially in the context of the following: (1) a severe phenotype with high morbidity and mortality; (2) the urgency of diagnosis and successful early response to therapy; and (3) the paucity of effective, safe, and well‐tolerated therapies. Even after initially successful treatment, relapse risk is substantial and the most effective therapies pose considerable risk with long‐term administration. In evaluating any treatment for infantile spasms, the key short‐term outcome measure is freedom from both epileptic spasms and hypsarrhythmia. In contrast, the most important long‐term outcomes are enduring seizure‐freedom and measures of intellectual performance in later childhood and adulthood. First‐line treatment options—namely hormonal therapy and vigabatrin—display moderate to high efficacy but also exhibit substantial side‐effect burdens. Data on efficacy and safety of each class of therapy, as well as the combination of these therapies, are reviewed in detail. Specific hormonal therapies (adrenocorticotropic hormone and various corticosteroids) are contrasted. Those etiologies that prompt specific therapies are reviewed briefly, as are an array of second‐line therapies supported by less‐compelling data. The ketogenic diet is discussed in greater detail, with a focus on the limitations of numerous available studies that generally suggest that it is efficacious. Special discussion is allocated to cannabidiol—the investigational therapy that has received the most attention, and which is already in use in the form of various artisanal cannabis extracts. Finally, a treatment algorithm reflecting the concepts and controversies discussed in this review is presented.

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“…2 Lactate, as well as ketones, emerges as an important energy substrate during early development. 3 In addition to higher ketones levels, the physiological situation of breastfed neonates during the early neonatal period is that they have higher plasma concentrations of lactate, 4 as well as lower levels of serum bicarbonate 5 or net acid excretion 6 than formula-fed babies.…”

Section: Replymentioning

confidence: 99%