CSF nerve growth factor (β-NGF) is increased but CSF insulin-like growth factor-(IGF-1) is normal in children with tuberous sclerosis and infantile spasms. (January 2019)

• Record Type: Journal Article
• Authors: Riikonen, Raili; Kokki, Hannu
• Is Part Of: European journal of paediatric neurology. Volume 23:Number 1(2019:Jan.)
• Page Start: 191
• Page End: 196
• Publication Date: 2019-01
• Subjects: Tuberous sclerosis -- Nerve growth factor -- Insulin-like growth factor -- Infantile spasms; Pediatric neurology -- Periodicals; Nervous System Diseases -- Periodicals; Child -- Periodicals; Infant -- Periodicals; Neurologie pédiatrique -- Périodiques; Pediatric neurology; Electronic journals; Periodicals; Electronic journals; 618.928
• DOI: 10.1016/j.ejpn.2018.11.001
• Languages: English
• ISSNs: 1090-3798

Abstract

Abstract: Tuberous sclerosis is associated with epilepsy that is often refractory. We examined cerebrospinal fluid (CSF) concentrations for neurotrophins, nerve growth factor (β-NGF) and insulin-like growth factor (IGF-1) in children with infantile spasms between 1997 and 2010. We classified the patients as follows: tuberous sclerosis (n = 5), cryptogenic spasms (n = 6), postinfectious spasms (n = 5) and other symptomatic spasms (n = 22). We had 22 age- and sex-matched controls for CSF-NGF and 14 for CSF-IGF-1. The median of CSF-NGF was higher in those with tuberous sclerosis, 56 (minimum–maximum, 8.0–131) ng/L, in relative to age- and sex-matched controls, 6.7 (0.0–22) ng/L, and symptomatic infantile spasms, 0.0 (0.0–4.5) ng/L or cryptogenic cases of infantile spasms, 6.2 (3.9–8.8) ng/L, respectively. CSF-NGF were highest in children with postinfectious aetiology, 408 (89–778) ng/L. CSF-IGF-1 of tuberous sclerosis, 0.65 (0.35–0.98) μg/L, did not differ from the cryptogenic spasms, 0.68 (0.32–0.87) μg/L, or from age- and sex-matched controls 0.52 (0.22–0.77) μg/L. Patients with tuberous sclerosis and cryptogenic spasms had normal development prior the ACTH therapy. We suggest that increased CSF-NGF might indicate a persistent activation of inflammatory pathways in cortical tubers in tuberous sclerosis and this would reflect in CSF concentrations. Highlights: Inflammatory processes may be involved in epileptic mechanisms of tuberous sclerosis complex. CSF-NGF was increased in children with tuberous sclerosis compared to those with symptomatic infantile spasms and controls. CSF-NGF concentrations might be a feasible biomarker of immune functioning within the cortical tubers in tuberous sclerosis.