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Infantile spasms: infectious disorders
1Department of Child Neurology and Pediatrics, Children's Hospital, University of Helsinki, Finland.
Abstract:
Infections were considered to be etiological factors in 29 patients (10%) with infantile spasms; congenital CMV (n = 5), congenital or acquired CMV (n = 1), acquired CMV (n = 5), congenital rubella (n = 2), herpes simplex virus (n = 5), enterovirus (n = 1), adenovirus (n = 1), viral encephalitis of unknown agent (n = 3), meningococcus (n = 4), pneumococcus (n = 1) and pertussis (n = 1). The children with congenital infections had long-lasting tremor and convulsions from birth. Early EEG pattern was characteristic for children with herpes encephalitis but not for other patients. Infantile spasms appeared only some weeks after viral encephalitis. One patient with enterovirus and another with probable adenovirus infection had necrotic changes in their brain CT resembling those of herpes encephalitis. The response to ACTH was poor (38%) compared to the whole series (60%). The long-term outcome was also poor compared to the whole series; mental retardation in 90%, convulsions in 62%, abnormal EEG in 89%. Four children died during the follow-up of 7 years. Autopsy showed disseminated CMV infection in one patient and chronic CMV infection in another. The outcome of children with infectious etiology appears to be particularly poor. Thus, the prevention and specific diagnosis and treatment are important. Steroid therapy should be avoided in children with a history of herpes virus encephalitis (CMV, herpes simplex) in the past.
Insights
Infections, including cytomegalovirus (CMV) and herpes simplex virus, are significant causes of infantile spasms, leading to poor long-term outcomes. Early diagnosis and targeted treatments are crucial for affected infants.
Area of Science:
- Neurology
- Infectious Diseases
- Pediatrics
Background:
- Infections are identified as potential etiological factors in infantile spasms.
- Congenital infections can manifest with early-onset tremor and convulsions.
Purpose of the Study:
- To investigate the role of infections in infantile spasms.
- To analyze the clinical presentation, EEG patterns, treatment response, and long-term outcomes in infants with infectious etiologies of infantile spasms.
Main Methods:
- Retrospective analysis of patients with infantile spasms.
- Identification of infectious agents including CMV, herpes simplex virus, rubella, enterovirus, adenovirus, and bacterial pathogens.
- Evaluation of EEG patterns, brain CT findings, response to ACTH therapy, and long-term neurological outcomes.
Main Results:
- Infections were identified in 10% of patients with infantile spasms.
- Congenital infections led to early tremor and convulsions.
- Herpes simplex virus encephalitis showed a characteristic early EEG pattern.
- Poor response to ACTH therapy (38%) and adverse long-term outcomes (90% mental retardation, 62% convulsions, 89% abnormal EEG) were observed.
- Four deaths occurred within 7 years, with autopsy confirming CMV infection in two cases.
Conclusions:
- Infectious etiologies, particularly viral encephalitis, are associated with particularly poor outcomes in infantile spasms.
- Prevention, specific diagnosis, and timely treatment are critical.
- Steroid therapy should be avoided in cases with a history of herpes virus encephalitis (CMV, herpes simplex).