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Idiopathic CSF dynamic disturbances causing headache: a narrative review.

Marina Romozzi1,2, Fabio Zeoli3, Federico Tosto4

  • 1Department of Neuroscience, Università Cattolica del Sacro Cuore, Rome, Italy. marinaromozzi@gmail.com.

Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
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Summary

Headache presentations in idiopathic cerebrospinal fluid (CSF) disorders like IIH, SIH, and iNPH are diverse and often missed. Recognizing atypical symptoms and headache in iNPH is crucial for diagnosis and management.

Keywords:
IIHIdiopathic intracranial hypertensionIntracranial hypotensionNPHNormal pressure hydrocephalusPapilledemaPseudotumor cerebri

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Area of Science:

  • Neurology
  • Neurosurgery
  • Headache Medicine

Background:

  • Idiopathic alterations in cerebrospinal fluid (CSF) dynamics frequently cause secondary headache disorders.
  • Idiopathic Intracranial Hypertension (IIH), Spontaneous Intracranial Hypotension (SIH), and Idiopathic Normal Pressure Hydrocephalus (iNPH) are common, but their headache presentations are not fully understood.
  • Headache in iNPH is often overlooked despite real-world evidence suggesting its frequency.

Purpose of the Study:

  • To review the clinical characteristics and pathophysiology of headache in IIH, SIH, and iNPH.
  • To synthesize evidence on the management and outcomes of headache in these CSF dynamic disorders.
  • To highlight atypical and overlapping headache presentations.

Main Methods:

  • Comprehensive literature search across multiple databases.
  • Review of representative clinical cases to illustrate imaging and headache features.
  • Synthesis of existing evidence on management and outcomes.

Main Results:

  • IIH headaches are typically bilateral, pressing/pulsatile (migraine-like/tension-type).
  • SIH classically presents with orthostatic headache, but atypical forms (non-orthostatic, thunderclap, chronic daily) exist.
  • Headache in iNPH (dull, holocranic) is reported in ~30% of patients; overlapping phenotypes and medication-overuse headache are common.

Conclusions:

  • Headache in idiopathic CSF dynamic disorders is diverse and often underrecognized, particularly atypical or overlapping cases.
  • Increased awareness of atypical headache features and the presence of headache in iNPH is essential for accurate diagnosis.
  • Further research into the pathophysiology and management of headache in these conditions is warranted.