Idiopathic Intracranial Hypertension in Children and Adolescents: An Update.

PREMISE: Idiopathic intracranial hypertension (IIH), previously known as pseudotumor cerebri syndrome (PTC) is a serious neurological disorder that can lead to irreversible visual loss. Predominantly a disorder affecting women in reproductive years, the pediatric population is not spared. PROBLEM: In the past few years, the condition has been redefined, due to new accepted values for opening pressure in children and advances in neuroimaging. Emerging techniques in ophthalmology are being increasingly used to monitor disease in these patients. And, although the treatment tools have not changed in several years, important evidence for efficacy for acetazolamide finally came to light in recent years in the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT). POTENTIAL SOLUTION: This review article provides an overview on recent advances in diagnosis, evaluation and treatment of IIH.

Lyme disease-related intracranial hypertension in children: clinical and imaging findings.

Lyme disease (LD) is a tick-borne infection that is endemic to multiple areas of the United States. Patients with LD may present with sign and symptoms of intracranial hypertension (IH). The objective of this study is to evaluate the history, clinical findings, CSF analysis, and brain imaging results in pediatric patients with increased intracranial pressure secondary to LD. A retrospective database search was performed using the International Classification of Diseases (ICD) 9/10 codes to identify patients diagnosed with LD and IH between 2004 and 2014 at a tertiary referral pediatric hospital. Clinical, laboratory and neuroimaging data for each patient were reviewed. Seven patients met inclusion criteria; mean age was 9.6 years (standard deviation 4.0 years); 4/7 patients were male. Average body mass index was 18.8 kg/m(2) (standard deviation 3.0 kg/m(2)). Fever was present in four patients. Four had a history of LD related erythema migrans. All had elevated CSF opening pressure with leukocytosis and lymphocytic predominance. MRI obtained in six patients showed contrast enhancement of various cranial nerves. Tentorial enhancement was noted in all patients. In addition, patients had widening of the optic nerve sheath (ONS), optic nerve protrusion, and flattening of the posterior globe consistent with increased intracranial pressure. All patients had resolution of their symptoms after initiation of antibiotic therapy. In endemic areas, LD should be included in the differential of IH. MRI can help distinguish IH due to LD from its idiopathic form due to the presence of tentorial and cranial nerve enhancement in the former in addition to abnormal CSF showing leukocytosis with lymphocyte predominance.

Magnetic resonance angiography evidence of vasospasm in children with suspected acute hemiplegic migraine.

Hemiplegic migraine is a rare subtype of migraine that is differentiated by motor weakness in the aura phase. The purpose of this case series was to examine the magnetic resonance angiogram findings of patients suffering from suspected acute hemiplegic migraine. This was a retrospective institutional board review protocol study of 8 patients. All patients received full brain magnetic resonance imaging under a 1.5-T magnet. The scans were subsequently evaluated by a neuroradiologist and 2 neurologists who were blinded to the study. The magnetic resonance angiogram findings of this study showed the presence of vasospasm within the intracranial vasculature during suspected acute hemiplegic migraine. This case series suggests that routine use of magnetic resonance angiography might be beneficial in both managing patients with acute hemiplegic migraine and helping to further understand the pathophysiology of this complicated disease process.