Spontaneous intracranial hypotension (SIH) occurs secondary to cerebrospinal fluid (CSF) hypovolemia in the setting of noniatrogenic spinal CSF leak. Although orthostatic headache is characteristic, atypical presentations can occur. Cranial imaging can disclose characteristic imaging features of SIH but spinal imaging is needed for leak localization. Although advanced diagnostic workup and treatment depend on the type of CSF leak, differentiation of SIH from other headache pathologic conditions, such as Chiari I malformation, is crucial to prevent misdiagnosis and ineffective treatment.
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