Spontaneous intracranial hypotension associated with headache: a literature review

Monika Šerelytė1, Džonardas Šleiteris1

1 Lithuanian university of health sciences, Medicine Academy, Faculty of Medicine


Spontaneous intracranial hypotension (SIH) is a rare neurological condition typically characterized by orthostatic headache, below-average intracranial pressure and pathological cerebrospinal fluid leaks. Cerebrospinal fluid leaks are one of the main causes of SIH. The most common symptom of SIH is positional headache, usually associated with dilatation of pain-sensitive intracranial veins or sag of brain due to traction of associated brain structures. SIH related headaches remain undiagnosed and unspecified in 94% cases. SIH is a fairly rare disease. It is estimated that there are only 2 to 5 cases per 100,000 persons per year. One of the main etiopathogenetic hypothesis claims that the weakness or thinning of spinal dural tissue are associated with spontaneous cerebrospinal leakage. The most important in SIH orthostatic headaches treatment is control of intracranial pressure. Although, SIH is not novel pathology, but often it is confused with other diseases. Also, SIH affects the quality of patients lives, limiting ability to work and it remains a relevant problem in today’s society. This article reviews the major causes, symptoms, diagnostic possibilities, and treatment for SIH induced headache.

Keywords: headache, orthostatic headache, spontaneous intracranial hypotension, cerebrospinal fluid leak.