Pseudotumor Cerebri Syndrome Headache Is Often Episodic With Neck, Shoulder Pain

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A medical chart review of children with pseudotumor cerebri syndrome found that approximately half of headaches were episodic in nature.
A medical chart review of children with pseudotumor cerebri syndrome found that approximately half of headaches were episodic in nature.

In children with pseudotumor cerebri syndrome (PTCS), the incidence of headache is typically episodic or constant, and its presence generally involves the neck and shoulders, according to findings from a medical chart review published in Headache.

Investigators retrospectively reviewed medical charts of patients with suspected or confirmed PTCS who were treated at a single center in Philadelphia (median age, 13.6 years). The investigators further identified patients with suspected or confirmed PTCS and headache. According to updated diagnostic criteria, the researchers classified patients as having definite PTCS (n=61), probable PTCS (n=10), elevated opening pressure (OP) without papilledema (n=31), or normal OP without papilledema (n=25). A review of the patients' medical charts was performed to identify the characteristics of headache in pediatric PTCS.

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Approximately half of the headaches were episodic in nature (49%; 95% CI, 34%-64%) among patients with definite PTCS, whereas 18% (95% CI, 6%-37%) of individuals with elevated OP without papilledema (P =.008 compared with definite PTCS) and 16% (95% CI, 5%-36%) of patients with normal OP but without papilledema (P =.006 compared with definite PTCS) had episodic headache. The primary regions involved with headache in patients with definite PTCS vs patients with elevated OP without papilledema (odds ratio, 7.2; 95% CI, 1.9-27.6) and normal OP (odds ratio, 4.5; 95% CI, 1.3-15.6) included the head, as well as the neck or shoulders.

The study's retrospective design and the reliance on patient data from one center were the main limitations of the analysis.

The investigators of this study also found that headache was absent in PTCS more frequently in the setting of normal vs elevated opening pressure, which suggested that "intracranial hypertension plays at least a partial role in the pathogenesis of headache in PTCS."

Reference

Hamedani AG, Witonsky KFR, Cosico M, et al. Headache characteristics in children with pseudotumor cerebri syndrome, elevated opening pressure without papilledema, and normal opening pressure: a retrospective cohort study. Headache. 2018;58:1339-1346.

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