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[A prognostic study of cryptogenic infantile spasms]
J Boulloche1, P Dautreme, B Le Luyer
1Département de Pédiatrie, Centre Hospitalier, Le Havre.
Insights
Cryptogenic infantile spasms, without brain lesions, often have a good prognosis with treatment. However, long-term outcomes show varied cognitive and psychiatric challenges in affected children.
Area of Science:
- Neurology
- Pediatrics
- Developmental Neuroscience
Context:
- Infantile spasms (IS) prognosis is generally poor with detectable brain lesions.
- Cryptogenic infantile spasms (CIS), lacking identifiable lesions, typically show a better treatment response.
- Limited research exists on the long-term outcomes specifically for children with CIS.
Purpose:
- To investigate the long-term prognosis and developmental outcomes in children diagnosed with cryptogenic infantile spasms.
- To identify early clinical and electrophysiological factors associated with favorable or unfavorable prognoses in CIS.
Summary:
- This study followed 111 pediatric patients with IS, identifying 23 (21%) with CIS.
- Outcomes included IQ levels, school attendance with learning disabilities, psychiatric disorders, developmental delay, and severe epilepsy.
- Favorable prognostic indicators were mild psychomotor regression and prompt treatment response; poor indicators included severe regression and late-onset spasms.
Impact:
- Highlights the significant long-term neurodevelopmental and psychiatric challenges in children with CIS, despite initial favorable response to treatment.
- Identifies key early predictors for prognosis, aiding in clinical management and parental counseling for infantile spasms.
- Underscores the need for continued monitoring and intervention for children with CIS to mitigate long-term sequelae.
Abstract:
The prognosis of infantile spasms is grim when a detectable brain lesion is present. In contrast, cryptogenic infantile spasms, in which there is no identifiable brain lesion, usually run a favorable course under treatment. Few studies have focused on the outcome in children with cryptogenic infantile spasms. Among 111 pediatric patients with a history of infantile spasms hospitalized over 18 consecutive years, 23 (21%) were given a diagnosis of cryptogenic infantile spasms. Follow-up ranged from 4 to 21 years. Outcome was as follows: the IQ was above 80 in 39% of cases and above 100 in 13% of cases; 42% of patients of school age were attending school but half of these had learning disabilities; 30% of patients had severe psychiatric disorders, and 22% had developmental delay and severe epilepsia. Early factors apparently associated with a good prognosis included the mild nature of psychomotor regression, persistence of spindles on EEGs recorded during NREM sleep, and prompt improvement of clinical status and EEG recordings under treatment. Conversely, severe regression, focalized EEG anomalies, failure of development to resume promptly after initiation of therapy, recurrence of spasms and hypsarrhythmia at discontinuation of treatment, and onset before 5 months or after 11 years of age were associated with a poor prognosis.