Idiopathic CSF dynamic disturbances causing headache: a narrative review.

BACKGROUND

Idiopathic alterations in CSF dynamics can lead to secondary headache disorders, with Idiopathic Intracranial Hypertension (IIH), Spontaneous Intracranial Hypotension (SIH), and Idiopathic Normal Pressure Hydrocephalus (iNPH) being the most frequent. While common headache features in IIH and SIH are relatively well-characterized, less is known about their atypical presentations. Moreover, although headache is not typically considered a hallmark of iNPH, real-world evidence suggests that headache is a frequent symptom; however, its clinical features and pathophysiology remain poorly defined. This review summarizes headache clinical characteristics andpathophysiology, and synthesizes the available evidence on the management and outcomes of headache in these conditions..

METHODS

A comprehensive literature search was conducted through multiple databases. Additionally, representative clinical cases from our Institution were reviewed to highlight typical imaging findings and headache characteristics of the three conditions.

RESULTS

IIH typically manifests with bilateral, pressing, or pulsatile headache resembling migraine or tension-type headache; SIH is characteristically associated with orthostatic headache. However, diverse clinical presentations associated with SIH have been reported, including non-orthostatic, thunderclap-onset, and chronic daily headache. Additionally, both disorders frequently coexist with medication-overuse, further blurring diagnostic boundaries. Headache associated with iNPH has historically received limited attention, yet evidence suggests that 30% of patients may present with dull, holocranic headache. Overlapping headache phenotypes can occur across these entities, including orthostatic headache following ventriculoperitoneal shunt overdrainage in iNPH and rebound intracranial hypertension following epidural blood patch in SIH.

CONCLUSION

Headache manifestations in idiopathic CSF dynamic disorders are diverse and frequently underrecognized, especially in atypical/overlapping presentations. Awareness of atypical features and the potential for headache in iNPH is essential.