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Pseudotumour cerebri in children: etiological, clinical features and treatment modalities
Nilufer Hacifazlioglu Eldes1, Yuksel Yilmaz
1Department of Pediatrics, Division of Child Neurology, Marmara University School of Medicine, Istanbul, Turkey. ninieldes@yahoo.com
Insights
Pseudotumour cerebri (PTC) in children often requires identifying the underlying cause. While treatments like acetazolamide and lumbar punctures can help, addressing the root issue is crucial for managing this condition.
Area of Science:
- Pediatric Neurology
- Ophthalmology
- Neurosurgery
Background:
- Pseudotumour cerebri (PTC), also known as idiopathic intracranial hypertension, is a neurological disorder.
- It primarily affects children and is characterized by increased intracranial pressure without a clear cause.
- Understanding the etiological and clinical features is vital for effective management.
Purpose of the Study:
- To investigate the etiological and clinical features of pseudotumour cerebri (PTC) in pediatric patients.
- To evaluate the effectiveness of different treatment modalities for PTC in children.
- To highlight the importance of identifying underlying causes for successful PTC management.
Main Methods:
- A retrospective review of 12 pediatric patients diagnosed with PTC.
- Documentation of etiological factors, clinical presentations, and treatment responses.
- Analysis of outcomes following medical management (acetazolamide, prednisone) and interventions like lumbar punctures and shunting.
Main Results:
- Etiology was identified in 6 patients; the remaining 6 were classified as idiopathic.
- Acetazolamide was effective in 4 patients without an underlying cause.
- Repeated lumbar punctures were ineffective in 5 out of 6 patients.
- Prednisone showed limited efficacy, effective in only one patient.
- Cerebrospinal fluid (CSF) pressure normalized in 3 patients after drug withdrawal and in 2 with cerebral venous thrombosis after ventricular-peritoneal shunting.
Conclusions:
- Medical treatments and lumbar punctures are not always effective in pediatric PTC.
- Identifying and addressing the underlying cause of PTC is critical for successful treatment outcomes.
- Ventricular-peritoneal shunting may be necessary for refractory cases, especially those with cerebral venous thrombosis.
Abstract:
To investigate the etiological and clinical features of pseudotumour cerebri (PTC) in children, features of 12 children with PTC were documented. The etiology could be clarified in 6 patients. The other 6 patients were accepted as idiopathic intracranial hypertension (primary PTC). Acetazolamide was used as the first drug and was effective in only 4 patients who had no underlying cause. Repeated lumbar punctures (LP) were performed in 6 patients and 5 of them were unresponsive. Prednisone was used in 3 patients and was effective in only one patient. The CSF pressure continued to be high in five patients and could be normalized only by withdrawing of the precipitating drug in three patients and by ventricular-peritoneal shunting in two patients with cerebral venous thrombosis. Although, medical treatment and repeated LP are usually effective in children with PTC, clarifying and solving the underlying cause can be crucial.

