Infantile spasms: Etiology, lead time and treatment response in a resource limited setting

Surana, Priyanka; Symonds, Joseph D.; Srivastava, Prabhar; Geetha, Thenral S; Jain, Romit; Vedant, Ramprasad; Murugan, Sakthivel; Mahalingam, Subathra; Bhargava, Vivek; Goyal, Pradeep; Zuberi, Sameer M.; Jain, Vivek

doi:10.1016/j.ebr.2020.100397

Cited by 24 publications

(36 citation statements)

References 32 publications

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“…Epidemiologically, genetic and particularly metabolic 21 subcategories contribute to a small proportion of symptomatic ISS. In a recent study from India, genetic etiology was identified in 10% of children with ISS (39% of children with ISS and normal neuroimaging) even after detailed investigations, 22 while none had an identified metabolic etiology. Similarly, the proportions of ISS with immunogenic etiology is likely to be miniscule; there were none identified in the National Infantile Spasm Consortium.…”

Section: Discussionmentioning

confidence: 97%

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Classifying etiology of infantile spasms syndrome in resource‐limited settings: A study from the South Asian region

et al. 2021

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Section: Discussionmentioning

confidence: 97%

“…Neonatal hypoglycemia is an important etiological factor in India. 22 , 30 Therefore, using detailed history and MRI imaging, a significant proportion of children in our setting can be identified to have an etiology. The proportion due to genetic and metabolic subgroups which are difficult to diagnose may be small.…”

Section: Discussionmentioning

confidence: 99%

Classifying etiology of infantile spasms syndrome in resource‐limited settings: A study from the South Asian region

et al. 2021

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“…Infantile spasms (IS) are age‐specific seizures characterized by clusters of axial epileptic flexion and extension spasms in infancy 1–7 . In ~24% there is a genetic or genetic‐structural etiology, whereas in ~33% the cause is unknown 7–11 . In ~60% of affected infants, IS are associated with pre‐existing structural or metabolic brain pathologies, and have worse prognosis than IS of unknown etiology, including persistence of intellectual and other neurological deficits as well as epilepsies that are often drug‐resistant 4,5,7,12 .…”

Section: Introductionmentioning

confidence: 99%

Antiepileptogenic effects of rapamycin in a model of infantile spasms due to structural lesions

Akman¹,

Briggs²,

Mowrey³

et al. 2021

Epilepsia

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Objective Infantile spasms may evolve into persistent epilepsies including Lennox‐Gastaut syndrome. We compared adult epilepsy outcomes in models of infantile spasms due to structural etiology (multiple‐hit model) or focal cortical inflammation and determined the anti‐epileptogenic effects of pulse‐rapamycin, previously shown to stop spasms in multiple‐hit rats. Methods Spasms were induced in 3‐day‐old male rats via right intracerebral doxorubicin/lipopolysaccharide (multiple‐hit model) infusions. Controls and sham rats were used. Separate multiple‐hit rats received pulse‐rapamycin or vehicle intraperitoneally between postnatal days 4 and 6. In adult mice, video‐EEG (electroencephalography) scoring for seizures and sleep and histology were done blinded to treatment. Results Motor‐type seizures developed in 66.7% of multiple‐hit rats, usually from sleep, but were reduced in the pulse‐rapamycin–treated group (20%, p = .043 vs multiple‐hit) and rare in other groups (0–9.1%, p < .05 vs multiple‐hit). Spike‐and‐wave bursts had a slower frequency in multiple‐hit rats (5.4–5.8Hz) than in the other groups (7.6–8.3Hz) (p < .05); pulse rapamycin had no effect on the hourly spike‐and‐wave burst rates in adulthood. Rapamycin, however, reduced the time spent in slow‐wave‐sleep (17.2%), which was increased in multiple‐hit rats (71.6%, p = .003). Sham rats spent more time in wakefulness (43.7%) compared to controls (30.6%, p = .043). Multiple‐hit rats, with or without rapamycin treatment, had right more than left corticohippocampal, basal ganglia lesions. There was no macroscopic pathology in the other groups. Significance Structural corticohippocampal/basal ganglia lesions increase the risk for post‐infantile spasms epilepsy, Lennox‐Gastaut syndrome features, and sleep dysregulation. Pulse rapamycin treatment for infantile spasms has anti‐epileptogenic effects, despite the structural lesions, and decreases the time spent in slow wave sleep.

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“…26 Delayed diagnosis of infantile spasms has been described in resource limited settings. 27 Concerns regarding inpatient hospitalizations during the pandemic to diagnose and treat infantile spasms were acknowledged in an online Child Neurology Society statement 28 and later formalized into published recommendations. 29 Recommendations include utilizing outpatient telemedicine, home videos of patient events, outpatient EEGs and MRIs, and preference for oral prednisolone over ACTH for ease and safety of initiation in the outpatient setting.…”

Section: Discussionmentioning

confidence: 99%

Multicenter Study of the Impact of COVID-19 Shelter-In-Place on Tertiary Hospital-based Care for Pediatric Neurologic Disease

Hutchinson

Nash

Abend

et al. 2022

The Neurohospitalist

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Objective To describe changes in hospital-based care for children with neurologic diagnoses during the initial 6 weeks following regional Coronavirus 2019 Shelter-in-Place orders. Methods This retrospective cross-sectional study of 7 US and Canadian pediatric tertiary care institutions included emergency and inpatient encounters with a neurologic primary discharge diagnosis code in the initial 6 weeks of Shelter-in-Place (COVID-SiP), compared to the same period during the prior 3 years (Pre-COVID). Patient demographics, encounter length, and neuroimaging and electroencephalography use were extracted from the medical record. Results 27,900 encounters over 4 years were included. Compared to Pre-COVID, there was a 54% reduction in encounters during Shelter-in-Place. COVID-SiP patients were younger (median 5 years vs 7 years). The incidence of encounters for migraine fell by 72%, and encounters for acute diagnoses of status epilepticus, infantile spasms, and traumatic brain injury dropped by 53%, 55%, and 56%, respectively. There was an increase in hospital length of stay, relative utilization of intensive care, and diagnostic testing (long-term electroencephalography, brain MRI, and head CT (all P<.01)). Conclusion During the initial 6 weeks of SiP, there was a significant decrease in neurologic hospital-based encounters. Those admitted required a high level of care. Hospital-based neurologic services are needed to care for acutely ill patients. Precise factors causing these shifts are unknown and raise concern for changes in care seeking of patients with serious neurologic conditions. Impacts of potentially delayed diagnosis or treatment require further investigation.

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Infantile spasms: Etiology, lead time and treatment response in a resource limited setting

Cited by 24 publications

References 32 publications

Classifying etiology of infantile spasms syndrome in resource‐limited settings: A study from the South Asian region

Classifying etiology of infantile spasms syndrome in resource‐limited settings: A study from the South Asian region

Antiepileptogenic effects of rapamycin in a model of infantile spasms due to structural lesions

Multicenter Study of the Impact of COVID-19 Shelter-In-Place on Tertiary Hospital-based Care for Pediatric Neurologic Disease

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