Infantile Idiopathic Intracranial Hypertension: A Case Study and Review of the Literature

Abstract

Idiopathic intracranial hypertension, or pseudotumor cerebri, is an increase in cerebrospinal fluid pressure of unknown etiology. It is mostly seen in adults, less frequently in adolescents, rarely in younger children. Only 5 infants meeting idiopathic intracranial hypertension criteria have been mentioned in the literature. We report a case of a previously healthy 9-month-old boy who presented with irritability, decreased appetite, and a bulging fontanelle. Computed tomography (CT) head imaging and cerebrospinal fluid studies revealed normal results. The patient’s symptoms transiently resolved after the initial lumbar puncture, but 11 days later, his fontanelle bulged again. A second lumbar puncture revealed an elevated opening pressure of 35 cmH2O and led to a diagnosis of idiopathic intracranial hypertension in accordance with the modified Dandy Criteria. Treatment with acetazolamide at a dose of 25 mg/kg/d was initiated and the patient remained symptom-free for 6 weeks, followed by another relapse. His acetazolamide dose was increased to 37 mg/kg/d, with no further relapses to date. A diagnosis of idiopathic intracranial hypertension is challenging in infants, because the patients cannot yet verbalize typical idiopathic intracranial hypertension–related symptoms such as positional headaches, diplopia, or pulsatile tinnitus. Furthermore, it is more difficult to assess papilledema in that age group. If undetected and untreated, idiopathic intracranial hypertension may result in permanent visual deficits. Little is known about idiopathic intracranial hypertension in infants, and age-specific treatment guidelines are lacking. We discuss this rare case of infantile idiopathic intracranial hypertension and provide a review of the literature, including an overview of disease characteristics and outcomes of idiopathic intracranial hypertension in this very young age group.

Infantile idiopathic intracranial hypertension: case report and review of the literature

“…In children, the incidence is 0.6–0.9/100,000/year [ 10 ], and it is very rare in infancy. To our knowledge, only 26 infants have been described so far; among them only 6 met all the diagnostic criteria [ 1 – 5 ]. In order to find literature on already described cases of infantile PTCS, we searched Pubmed from inception to February 2021; age limit was 0–23 months and we used the following terms combined with boolean operators AND and OR: idiopathic intracranial hypertension , infantile idiopathic intracranial hypertension , pseudotumor cerebri , infants .…”

“…Idiopathic intracranial hypertension (IIH) is a clinical syndrome caused by elevated cerebrospinal fluid (CSF) pressures with no evidence of mass lesion on neuroimaging. It is infrequent in childhood, and extremely rare in infancy [ 1 – 5 ]. We describe the case of an 8-month-old male affected by IIH, followed by a brief review of the literature.…”

Pseudotumor Cerebri Syndrome: From Childhood to Adulthood Risk Factors and Clinical Presentation

“…Patients were defined as overweight if body mass index was above 25 or body weight was above the 90th percentile, and obese if body mass index was 30.0 or higher, or over the 97th percentile for age. Subsequently, we merged and combined their clinical data with the information retrieved from 15 previous clinical studies published since 2001 3,14 -32 and performed a pooled analysis. In addition to patient’s weight and gender, data that were analyzed for that purpose included the finding of papilledema and the symptoms of headache, vomiting, visual impairment, dizziness, tinnitus, and associated drug therapies regarded as precipitating factors.…”

“…12,13 The fact that pseudotumor cerebri syndrome is associated with male gender and normal body mass in children raises the possibility that the underlying mechanisms are different at a younger age. Most previous studies on pediatric pseudotumor cerebri syndrome 14 -32 included only children, which made the comparison between pediatric and adult patients challenging.…”

Idiopathic intracranial hypertension with multiple cranial nerve palsies in 10 years old thin Sudanese boy: case report

“…The incidence is estimated by about 0.9 cases per 100,000 in the general population [ 2 ]. Patients are usually present with signs and symptoms of raise intracranial pressure with normal cerebrospinal fluid (CSF) composition and normal brain neuroimaging [ 3 ]. The diagnosis is based on the presence of all five items of diagnostic criteria for IIH proposed by Friedman [ 3 ] which include (1) normal neurological examination except for cranial nerve abnormalities, (2) presence of papilledema, (3) normal brain neuroimaging, (4) normal CSF composition, and (5) raised lumbar puncture (LP) opening pressure (≥ 250 cm H2O in adults and ≥ 280 cm H2O in children) in a properly performed LP (Table 1 ).…”