Features of headache due to spontaneous intracranial hypotension

What are the features of headache due to spontaneous intracranial hypotension (SIH) or low CSF volume syndromes

SIH almost always results from spontaneous CSF leaks, typically at the spinal level (most commonly thoracic) and rarely at the skull base.

The annual incidence may be 5/100,000 cases, with a peak age of onset of 40 years and a female-to-male ratio of 1.5:1. It can occur in patients of all ages. 

Orthostatic headache (a headache while upright, relieved while lying down) is the most common clinical manifestation.

The headache may either appear or be relieved after a change in posture.

The headache may be dull, throbbing, or pressure-like, mild to severe in intensity, and is usually but not always bilateral. The headache can be frontal, fronto-occipital, generalized, or occipital.

It can gradually evolve into a nonorthostatic chronic daily headache or be a nonorthostatic chronic daily headache from onset.

Other, less common types include exertional headaches, cough headaches, acute thunderclap onset, second-half-of-the-day headaches, paradoxical orthostatic headaches (present in recumbency, relieved when upright), intermittent headaches, and the acephalgic form (no headaches).

Sources

  • Dilli E: Thunderclap headache. Curr Neurol Neurosci Rep 14(4):437, 2014. 
  • Armon C, Evans RW, Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology: Addendum to assessment: prevention of post-lumbar puncture headaches: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology 65:510-512, 2005. 
  • Mokri B. Spontaneous Intracranial Hypotension. Continuum 21:1086-1108, 2015. 
  • Schievink WI, Deline CR: Headache secondary to intracranial hypotension. Curr Pain Headache Rep 18(11):457, 2014.
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