Pearls & Oy-sters: Spontaneous intracranial hypotension and posterior reversible encephalopathy syndrome
PEARLS• Although rare, cases of posterior reversible encephalopathy syndrome (PRES) secondary to CSF leak and intracranial hypotension have been documented in the literature.• The pathogenesis of PRES in these settings has been associated with both arterial and venous cerebrovascular dysfunction.
OY-STERS• PRES should be suspected in patients with CSF leak who continue to experience headache, visual changes, and altered mentation despite conservative management.• The mainstay of treatment involves resolution of the CSF leak, typically with an autologous epidural blood patch.CASE REPORT A 65-year-old woman with no significant medical history was brought to the emergency room after experiencing 2 weeks of band-like occipital headaches and 2 days of progressive mental status changes. Her neurologic examination was significant for decreased alertness, disorientation to time, confabulation, loss of recent and remote memory, diffuse hyperreflexia, and a left Hoffmann sign. MRI of the brain with contrast showed bilateral subdural hygromas, diffuse enhancement of the dura mater, with mild sagging of the brainstem suggestive of intracranial hypotension (figure 1, A and B). MRI of the spine showed a large collection of CSF in the lumbar epidural space ( figure 2A); however, the tear site was not identified. EEG also revealed generalized slowing with no evidence of seizure activity. Her mental status continued to deteriorate despite conservative measures such as aggressive hydration, caffeine intake, and bedrest in the Trendelenburg position. A head CT was ordered for worsening mental status and revealed symmetric hypoattenuation of the bilateral parasagittal occipital cortices consistent with PRES ( figure 2B), a finding that was not present on the initial MRI. She then received an emergent lumbar epidural blood patch, resulting in dramatic improvement in mental status several hours later. She was discharged without any focal neurologic deficits. An MRI performed 10 days later revealed no evidence of PRES in the occipital region. An MRI of the lumbar spine performed 5 weeks later showed resolution of the fluid collection (figure 3).DISCUSSION Spontaneous intracranial hypotension (SIH) is a known neurologic condition characterized by occipital headaches, neck stiffness, and altered mentation that results from leakage of CSF through a structural break in the meninges. Distinct abnormalities on MRI support the diagnosis, including diffuse meningeal enhancement, subdural fluid collections, engorgement of the dural sinuses, and downward displacement of the brain that can result in compression of vital structures (figure 1, A and B). Stupor and coma, for example, have been linked to compression of the diencephalon. 1 While our patient's altered mental status was initially attributed to brain sagging, her clinical deterioration was complicated by a transient occipital hypodensity concerning for PRES (figure 2B). Although rare...