Spontaneous intracranial hypotension: key features for a frequently misdiagnosed disorder

Spontaneous intracranial hypotension (SIH) is a rare neurological condition caused by low cerebrospinal fluid (CSF) volume, most commonly due to a CSF leak. The most common presenting symptom is an orthostatic headache, but some patients may present with atypical neurological manifestations such as cranial nerve palsies, an altered mental status, and movement disorders, which complicate the clinical diagnosis. Therefore, the diagnosis is based on the combination of clinical signs and symptoms, neuroimaging, and/or a low cerebrospinal fluid pressure. In this review, we describe the wide variety of neurological manifestations and complications seen in patients with SIH as well as the most common features described on imaging studies, including both subjective and objective measurements, in order to lead the clinician to a correct diagnosis. The prompt and correct management of patients with SIH will help prevent the development of life-threatening complications, such as subdural hematomas, cerebral venous thrombosis, and coma, and avoid unnecessary invasive procedures.