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Pseudotumour cerebri in children: Aetiology, clinical features, and progression
A Mosquera Gorostidi1, M Iridoy Zulet2, G Azcona Ganuza3
1Unidad de Neuropediatría, Servicio de Pediatría, Complejo Hospitalario de Navarra, Pamplona, Navarra, España.
Insights
Idiopathic intracranial hypertension (pseudotumor cerebri) in children is often diagnosed via lumbar puncture. Acetazolamide effectively treats this condition, offering an alternative to repeated procedures.
Area of Science:
- Pediatric Neurology
- Ophthalmology
- Infectious Diseases
Background:
- Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri (PTC), diagnosis and treatment are evolving in pediatric populations.
- PTC is characterized by increased intracranial pressure without identifiable space-occupying lesions.
Purpose of the Study:
- To analyze the clinical and epidemiological features of pediatric PTC.
- To evaluate diagnostic and treatment approaches for PTC in children.
- To estimate the incidence of PTC in children.
Main Methods:
- Retrospective analysis of 12 children under 15 years old diagnosed with PTC over 12 years.
- Diagnosis confirmed by CSF opening pressure >25cmH2O and normal brain MRI.
- Clinical data, symptoms, diagnostic methods, treatments, and outcomes were reviewed.
Main Results:
- The study included 12 children (90% girls, mean age 10 years); none were overweight.
- Headache was the most common symptom (66%), and papilledema was present in all patients.
- Mycoplasma pneumoniae infection was identified as a potential trigger in 3 cases. All patients had favorable outcomes.
Conclusions:
- The estimated incidence of pediatric PTC is approximately 1 per 100,000 child-years.
- Overweight was not a risk factor; Mycoplasma pneumoniae infection may trigger PTC.
- Acetazolamide is an effective treatment, serving as a viable alternative to repeated lumbar punctures.
Introduction:
The definition, associated aetiologies, diagnosis, and treatment of idiopathic intracranial hypertension, or pseudotumour cerebri (PTC), are constantly being revised in the paediatric population.
Objectives And Methods:
Our study included children younger than 15 years old with PTC and attended at a reference hospital in the past 12 years. We analysed the clinical and epidemiological features of our sample and the diagnostic and treatment approaches. PTC was defined as presence of intracranial hypertension (CSF opening pressure>25cmH2O) and absence of space-occupying lesions in brain MR images.
Results:
A total of 12 children with PTC were included; mean age was 10 years and 90% were girls. Weight was normal in all patients. Eighty-two percent of the patients had symptoms: headache (66%), diplopia (8%), and visual loss (8%). All of them displayed papilloedema (17% unilaterally). Lumbar puncture (LP) provided the diagnosis in all cases and 91% showed no relevant MRI findings. A potential cause of PTC was identified in 5 cases: pharmacological treatment in 2 and infection (Mycoplasma pneumoniae [M. pneumoniae]) in 3. Ninety-one per cent of the patients received treatment: 75% underwent several LPs and 42% received acetazolamide and/or prednisone. Outcomes were favourable in all cases.
Conclusions:
The incidence of PTC was estimated at approximately 1 case per 100 000 children/years, in line with data reported by previous studies. Overweight was not found to be a risk factor for PTC in this population. M. pneumoniae infection may trigger PTC and cause recurrences at later stages. The absence of symptoms seems to be independent from the degree of intracranial hypertension. Acetazolamide treatment is effective in most cases, and it represents a viable alternative to repeated LP.
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