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Chronic intracranial hypertension with unexplained cerebrospinal fluid pleocytosis
Yaniv Barkana1, Neta Levin, Yochanan Goldhammer
1Department of Neurology, Sheba Medical Center, Tel Hashomer, Tel Aviv, Israel. idityaniv@yahoo.com
Insights
Idiopathic intracranial hypertension (IIH) can present with cerebrospinal fluid pleocytosis, mimicking chronic meningitis. This condition, characterized by elevated intracranial pressure, often responds to standard IIH treatments.
Area of Science:
- Neurology
- Neuroscience
- Ophthalmology
Background:
- Idiopathic intracranial hypertension (IIH) is a neurological disorder characterized by elevated intracranial pressure without a clear cause.
- Typical IIH presentation excludes cerebrospinal fluid (CSF) abnormalities like pleocytosis.
- Chronic meningitis can present with elevated intracranial pressure and CSF pleocytosis.
Purpose of the Study:
- To identify and characterize patients with IIH exhibiting cerebrospinal fluid (CSF) pleocytosis.
- To investigate the clinical course, diagnostic challenges, and treatment outcomes in these atypical IIH cases.
- To determine if IIH treatment protocols are effective for patients presenting with both elevated intracranial pressure and CSF pleocytosis.
Main Methods:
- Retrospective review of patients diagnosed with idiopathic intracranial hypertension (IIH) over a nine-year period across two academic neurology departments.
- Inclusion criteria focused on patients with IIH-like symptoms but also cerebrospinal fluid (CSF) pleocytosis.
- Comprehensive laboratory and neuroimaging evaluations were performed to exclude other causes.
Main Results:
- Six patients (five female, one male; mean age 25.7 years) presented with IIH symptoms and CSF pleocytosis, predominantly lymphocytes.
- All patients were obese; no other risk factors for IIH were identified.
- Medical management for elevated intracranial pressure was effective in five patients; one required shunting. Ophthalmic symptoms resolved or stabilized.
Conclusions:
- A subset of patients may present with idiopathic chronic meningitis and elevated intracranial pressure.
- This condition appears to respond favorably to treatments typically used for idiopathic intracranial hypertension (IIH).
- Recognition of this IIH variant is crucial for appropriate diagnosis and management, preventing unnecessary investigations for chronic meningitis.
Abstract:
In a retrospective review of all cases with a diagnosis of idiopathic intracranial hypertension in two academic departments of neurology over a nine-year period, the authors identified six patients with a clinical course typical of idiopathic intracranial hypertension (IIH) except for the finding of cerebrospinal fluid pleocytosis. There were five women and one man with a mean age at presentation of 25.7 years (range, 25-32 yr). All were obese but had no other associated medical conditions or identifiable risk factors for IIH. In five patients, all or most cerebrospinal fluid cells were lymphocytes. Cerebrospinal fluid pleocytosis persisted for several months in all patients. Patients underwent a thorough laboratory and neuroimaging evaluation that did not reveal a primary cause. Medical treatment directed solely at lowering intracranial pressure was effective in five patients; one patient required lumboperitoneal shunting. Ophthalmic manifestations of increased intracranial pressure stabilized or remitted after treatment was withdrawn with a mean follow-up period of 33 months (range, 14-55 mo). Some patients may present with idiopathic chronic meningitis and elevated intracranial pressure that responds to treatment used for IIH.
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