Abstract
Posterior reversible encephalopathy syndrome (PRES) is a reversible neuroradiological syndrome characterized by reversible vasogenic edema. The pathophysiological mechanism is still unclear, but PRES may be triggered by various etiologies. To date, only a few PRES cases linked to cerebrospinal fluid (CSF) hypovolemia were reported. The association between PRES and CSF hypovolemia needs to be explored. We presented a case of PRES with CSF hypovolemia as a result of an inadvertent dural puncture and reviewed the literature to identify the clinical characterization and pathophysiological mechanism of PRES following CSF hypovolemia. A total of 31 cases of PRES-CSF hypovolemia was included for analysis. The median age was 33 years, with a notable female predominance (87.1%). Fifteen patients (48.4%) didn't have either a history of hypertension nor an episode of hypertension. The most common cause of CSF hypovolemia was epidural or lumbar puncture (n = 21), followed by CSF shunt (n = 6). The median interval between the procedure leading to CSF hypovolemia and PRES was 4 days. Seizure, altered mental state, and headache were the most frequent presenting symptom. The parietooccipital pattern was most frequent (71.0%). Conservative management remains the mainstay of treatment with excellent outcomes. Three patients had a second episode of PRES. CSF hypovolemia is a plausible cause of PRES via a unique pathophysiologic mechanism including arterial hyperperfusion and venous dysfunction. Patients with CSF hypovolemia is more susceptible to PRES, which is potentially life-threatening. Given that CSF hypovolemia is a common complication of anesthetic, neurological, and neurosurgical procedures, PRES should be early considered for prompt diagnosis and appropriate management.
Highlights
Posterior reversible encephalopathy syndrome (PRES), initially described by Hinchey et al in 1996 [1], refers to a reversible clinical and neuroradiological syndrome characterized by acute headache, seizures, visual disturbances, impaired consciousness, focal neurological deficits, or combinations of them [2]
The additional 30 cases (29 articles) in the PubMed and Web of Science database from inception to July 2019 using a combination with “PRES” and various terms related to Cerebrospinal fluid (CSF) hypovolemia or high risks of CSF hypovolemia including “cerebrospinal fluid hypovolemia,” “intracranial hypotension,” “CSF leakage,” “epidural puncture,” “epidural anesthesia,” “spinal puncture,” “spinal anesthesia,” “lumbar puncture,” “cerebrospinal fluid shunt,” “spinal surgery,” and “cranial surgery.”
The present case and reviewed literature highlight the pathophysiological link between PRES and CSF hypovolemia
Summary
Posterior reversible encephalopathy syndrome (PRES), initially described by Hinchey et al in 1996 [1], refers to a reversible clinical and neuroradiological syndrome characterized by acute headache, seizures, visual disturbances, impaired consciousness, focal neurological deficits, or combinations of them [2]. Cerebrospinal fluid (CSF) hypovolemia, which is used to be referred to as intracranial hypotension (IH) synonymously, is increasingly recognized as a critical but often a misdiagnosed cause of new-onset cephalalgia [6, 7]. It included IH, but it was not an unequivocal definition of IH as a normal or even an increased CSF pressure was not rare in reported cases [8]. Atypical clinical presentations including non-orthostatic headaches, visual defects, neurocognitive decline, epilepsy, and focal neurological deficits, which are similar to PRES, have already been reported. The association between PRES and CSF hypovolemia has not been fully elucidated
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