Asymptomatic Idiopathic Intracranial Hypertension: Is It a Precursor to Symptomatic Disease in Children?

Less pronounced clinical and radiologic features in asymptomatic idiopathic intracranial hypertension (IIH) may serve as early indicators for symptomatic IIH in children, according to results published in Child Neurology.

The goal of this retrospective case-control study was to assess the clinical features of asymptomatic IIH compared with those of symptomatic IIH in children. Researchers focused on patients aged ≤18 years who were diagnosed with IIH between 2005 and 2016. Patients were classified into 2 groups: symptomatic IIH (at least one symptom related to increased intracranial pressure), or asymptomatic IIH (no symptoms related to increased intracranial pressure). All patients underwent lumbar puncture, fundus examination, and radiologic neuroimaging on initial diagnosis.

In total, 53 children with a diagnosis of IIH were identified. Of these, 41 patients (77.4%) were found to have symptomatic IIH and 12 patients (22.6%) were asymptomatic. The most common symptoms reported were headache (69.8%), vision loss (22.6%), diplopia (20.8%), and pulsatile tinnitus (11.3%).

Compared to patients with symptomatic IIH, those with the asymptomatic disorder were younger at time of diagnosis (mean age 12.54±3.14 years vs. 10.0±5.27 years, P <.05), had lower initial opening pressure on lumbar puncture (34.25 vs. 46.24 cmH2O, P =0.0133), lower optic nerve edema grades bilaterally (P <.05), lower likelihood of globe flattening on magnetic resonance imaging (33.3% vs. 46.1%, P =.0296), and smaller required dose of acetazolamide (361.11 vs. 986.88 mg daily, P =.0019) for resolution of papilledema.

The major limitation of the study, according to the researchers, is the retrospective design, small sample size, and inconsistent time intervals between visits.

The investigators concluded that this study establishes, “the importance of asymptomatic IIH as a disease state among pediatric neurology and ophthalmology patients.” They believe that asymptomatic IIH may exist as a separate, less severe form of the disease that precludes development of the symptomatic disease state. “Proper identification and diagnosis in children can guide clinical management and warrants close follow-up through the disease course,” the authors stated in a final remark.

Reference

Gondi KT, Chen KS, Gratton SM. Asymptomatic versus symptomatic idiopathic intracranial hypertension in children [published online July 1, 2019]. J Child Neurol. doi:10.1177/0883073819858455