Long-Term Follow-up of Pseudotumor Cerebri Syndrome in Prepubertal Children, Adolescents, and Adults

• Published in: Pediatric neurology Vol. 101; pp. 57 - 63
• Main Authors: Hilely, Assaf, Hecht, Idan, Goldenberg-Cohen, Nitza, Leiba, Hana
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01-12-2019
• Subjects: Acetazolamide; Body mass index; Cerebrospinal fluid; Idiopathic intracranial hypertension; Intracranial hypertension; Optic neuropathy; Pseudotumor cerebri syndrome; Body mass index; Optic neuropathy; Idiopathic intracranial hypertension; Intracranial hypertension; Acetazolamide; Cerebrospinal fluid; Pseudotumor cerebri syndrome
• ISSN: 0887-8994; 1873-5150
• DOI: 10.1016/j.pediatrneurol.2019.04.018

Pseudotumor cerebri syndrome can have a recurrent course. We compared the long-term disease course, recurrences, and final visual outcomes in prepubertal children, adolescents, and adults.
In this retrospective observational study, patients were divided into prepubertal children (group A) adolescents (group B), and adults (group C).
Sixty-five patients (56 females, nine males) were included, 26.2% in group A, 24.6% in group B, and 49.2% in group C. Age at diagnosis was 8.6 ± 2.0 years, 14.3 ± 1.5 years, and 31.9 ± 9.7 years for the prepubertal children, adolescents, and adults, respectively. Medical treatment duration was similar (2.4 to 3.3 years, P > 0.05). Recurrences were observed in 23.5% of prepubertal children, 50% of adolescents, and 28.1% of adults. Recurrences occurred within 1.3 ± 0.6 years from treatment cessation in the prepubertal group compared with 3.8 ± 5.1 years in adolescents and 2.7 ± 2.0 years in adults (P = 0.267). Optic neuropathy was evident in 41% of group A, 31% of group B, and 87.5% of group C (P < 0.001). Obesity and cerebrospinal fluid opening pressures were unassociated with either relapsing rates or final visual outcomes in all groups.
Pseudotumor cerebri syndrome exhibits a relapsing course in a third of cases. Recurrences tend to occur within one year after treatment cessation in prepubertal children, and within three years in older patients, revealing the importance of longer follow-up, especially in adults. Optic neuropathy was more common in adults along with a tendency for visual decline. Longer treatment times were associated with fewer recurrences.