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Impaired cerebrospinal fluid pressure.

Jan Hoffmann1

  • 1Department of Systems Neuroscience, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

Handbook of Clinical Neurology
|November 8, 2017
PubMed
Summary

Abnormal cerebrospinal fluid (CSF) pressure, including idiopathic intracranial hypertension (IIH) and spontaneous intracranial hypotension, causes various symptoms. Acetazolamide is effective for IIH, while hypotension often resolves spontaneously.

Keywords:
headacheidiopathic intracranial hypertensionpainspontaneous intracranial hypotension

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Area of Science:

  • Neurology
  • Neurosurgery
  • Ophthalmology

Background:

  • Cerebrospinal fluid (CSF) pressure abnormalities are common, presenting with diverse symptoms, most notably headache.
  • Altered CSF pressure can mimic primary headache syndromes, necessitating accurate diagnosis and management.
  • Pathologic increases in CSF pressure can be primary or secondary, while decreases typically result from CSF leakage.

Purpose of the Study:

  • To review the current understanding of the pathophysiology and treatment of conditions involving abnormal CSF pressure.
  • To highlight the diagnostic challenges and therapeutic strategies for idiopathic intracranial hypertension (IIH) and spontaneous intracranial hypotension.
  • To discuss recent advancements in the treatment of IIH, including pharmacologic interventions.

Main Methods:

  • Review of existing literature on CSF pressure abnormalities, focusing on idiopathic intracranial hypertension (IIH) and spontaneous intracranial hypotension.
  • Analysis of clinical presentations, pathophysiologic mechanisms, and treatment outcomes.
  • Evaluation of evidence from randomized controlled trials (RCTs) and clinical studies.

Main Results:

  • The pathophysiology of IIH is not fully understood, but impaired CSF outflow and absorption are implicated.
  • A randomized, double-blind, placebo-controlled trial confirmed acetazolamide's efficacy in managing IIH symptoms like headache and visual disturbances.
  • Spontaneous intracranial hypotension often resolves without intervention, but treatments like epidural blood patches may be necessary.

Conclusions:

  • Effective management of IIH involves lifestyle modifications (weight loss) and pharmacotherapy, with acetazolamide demonstrating proven efficacy.
  • While topiramate and furosemide show potential for IIH treatment, further RCTs are needed.
  • Spontaneous intracranial hypotension management ranges from conservative measures to invasive procedures if spontaneous remission does not occur.