Spontaneous low cerebrospinal pressure/volume headaches.

Area of Science:

• Neurology
• Radiology
• Neurosurgery

Background:

• Spontaneous intracranial hypotension (SIH) is primarily caused by spontaneous cerebrospinal fluid (CSF) leaks.
• SIH exhibits a wide spectrum of clinical and imaging features, with significant variability.
• Headache, typically orthostatic, is the most common symptom, potentially becoming chronic.

Purpose of the Study:

• To delineate the clinical and imaging spectrum of SIH.
• To emphasize the central role of decreased CSF volume in SIH pathogenesis.
• To discuss diagnostic findings and therapeutic challenges.

Main Methods:

• Review of clinical presentations, including headache, neck pain, and neurological deficits.
• Analysis of characteristic imaging findings such as diffuse pachymeningeal enhancement and brain sagging.
• Evaluation of cerebrospinal fluid (CSF) analysis, including opening pressure and composition.

Main Results:

• Common imaging findings include diffuse pachymeningeal enhancement, brain sagging, and subdural collections.
• CSF opening pressure is typically low; CSF analysis may show elevated protein and lymphocytic pleocytosis.
• Decreased CSF volume is identified as the core pathogenetic factor influencing all clinical and imaging variables.

Conclusions:

• SIH is a complex disorder with diverse manifestations, distinct from post-spinal puncture headaches.
• Effective management requires recognizing the variability and understanding the underlying CSF volume loss.
• While many patients respond to treatment, a subset presents persistent therapeutic challenges.