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.

Related Concept Videos

Increased Intracranial Pressure l: Introduction01:14

Increased Intracranial Pressure l: Introduction

Intracranial hypertension is a sustained elevation of intracranial pressure (ICP) above 22 mm Hg. In supine adults, normal ICP is ~7–15 mm Hg.The rigid, nonexpandable cranium contains three components—brain tissue, blood, and cerebrospinal fluid (CSF)—that total ~1,700 mL in a typical adult: 1,400 mL brain (~80%), 150 mL blood (~10%), and 150 mL CSF (~10%). According to the Monro–Kellie doctrine, total intracranial volume is effectively fixed. When one component expands, CSF and venous blood...
Increased Intracranial Pressure ll: Pathophysiology01:29

Increased Intracranial Pressure ll: Pathophysiology

Increased intracranial pressure (ICP) refers to a potentially life-threatening rise in pressure inside the skull. This usually happens when there is a major change in the volume of brain tissue, blood, or cerebrospinal fluid (CSF) — the three components inside the skull. According to the Monro-Kellie doctrine, if the volume of one component increases, the volumes of the other components must decrease to maintain normal pressure. If this does not happen, ICP rises.The process often begins with...
Cerebral Edema ll: Pathophysiology01:22

Cerebral Edema ll: Pathophysiology

Vasogenic edema is a major form of cerebral edema characterized by abnormal accumulation of fluid in the brain’s extracellular space due to disruption of the blood–brain barrier (BBB). The BBB is a specialized structure composed of endothelial cells connected by tight junctions, supported by astrocytic endfeet and a basement membrane. Under normal conditions, it tightly regulates the movement of ions, proteins, and solutes between the bloodstream and brain parenchyma. When this barrier loses...
Brain Abscess l: Introduction01:26

Brain Abscess l: Introduction

A brain abscess is a focal, intracerebral infection characterized by a localized collection of pus within the brain parenchyma, resulting from microbial invasion and the body’s inflammatory response. It progresses through stages: early and late cerebritis, followed by early and late capsule formation, reflecting tissue destruction, immune response, and eventual encapsulation.Etiology and PathogenesisCausative organisms vary with source and host factors, often involving polymicrobial infections,...
Cerebral Edema l: Introduction01:19

Cerebral Edema l: Introduction

Cerebral edema is a pathological increase in brain water content that disrupts intracranial pressure regulation and impairs neurological function. Because the cranial vault is rigid, even modest increases in tissue volume can compromise cerebral perfusion, distort neural structures, and initiate secondary injury. Cerebral edema develops through four principal mechanisms: vasogenic, cytotoxic, interstitial, and ionic.Vasogenic EdemaVasogenic edema arises from disruption of the blood–brain...
Cryptococcal Meningitis01:27

Cryptococcal Meningitis

Cryptococcal meningitis is a life-threatening opportunistic infection predominantly associated with HIV/AIDS, accounting for over 100,000 deaths annually worldwide. However, it also affects individuals with other forms of immunosuppression, including those undergoing immunosuppressive therapy, organ transplant recipients, patients with innate immunodeficiencies, and individuals with hematological disorders. The infection is caused mainly by Cryptococcus neoformans and Cryptococcus gattii,...