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10 marksDrNB 2025NeurologyCSF Disorders

Discuss the various presentations, diagnosis and management of spontaneous intracranial hypotension. [3+3+4]

Spontaneous intracranial hypotension (SIH) is a syndrome caused by cerebrospinal fluid (CSF) volume depletion due to spontaneous CSF leak, leading to low CSF pressure and orthostatic headache (Harrison's 21e, Ch. 265).

SIH results from spontaneous dural tears or CSF leaks along the spinal axis, often at the cervicothoracic junction. Loss of CSF volume decreases buoyancy and causes brain sagging, leading to characteristic symptoms.

MechanismDescription
CSF LeakSpontaneous dural tear or meningeal diverticula rupture
Typical SitesCervicothoracic spine, thoracic spine
Risk FactorsConnective tissue disorders (e.g., Marfan, Ehlers-Danlos), trivial trauma, spinal degenerative changes
Pathophysiology↓CSF volume → ↓CSF pressure → compensatory venous dilation and brain sagging

SIH classification by leak type:

TypeDescription
Type 1Ventral dural tear
Type 2Ruptured meningeal diverticula
Type 3CSF-venous fistula

(Harrison's 21e, Ch. 265; Bradley's Neurology in Clinical Practice, 8e)

References

Harrison's 21e, Ch. 265Harrison's 21e, Ch. 265; Bradley's Neurology in Clinical PracticeHarrison's 21e, Ch. 265; Bradley's Neurology in Clinical Practice, 8e