Reversible cerebral vasoconstriction syndrome presenting with symptoms of intracranial hypotension
Singh J, and Ali A. Reversible cerebral vasoconstriction syndrome presenting with symptoms of intracranial hypotension. Headache 2020; 60:134.
Background: Headache associated with spontaneous intracranial hypotension typically presents as an orthostatic headache that is improved in the supine position. Conversely, RCVS, a transient intracerebral arteriopathy, is classically characterized by recurrent thunderclap headache.
Methods: We report a 60 year old woman who presented to the emergency department with a sudden onset bilateral parietooccipital headache that developed after straining on the toilet. The headache was a throbbing, pounding sensation associated with neck stiffness, without photophobia, phonophobia, or nausea. CT head and MRI brain were unremarkable, and she was treated symptomatically. The headache persisted, and she also developed cognitive difficulty, prompting 2 additional ER visits the following week without additional testing. 2 weeks after symptom onset, a repeat MRI brain and CT Angiography were unremarkable at an ER visit. The patient was evaluated in our headache clinic 2.5 weeks after symptom onset. She would wake up symptom-free without head pain, and as the day progressed, would develop progressively worsening headache, cognitive, and memory symptoms. Given multiple negative neuroimaging studies, her current symptoms of position-dependent occipital headache, and cognitive impairment, there was concern for spontaneous intracranial hypotension. She was scheduled 2 days later for a diagnostic and therapeutic lumbar epidural blood patch. Just prior to the procedure, she described new onset vertigo and was taken to the ER for urgent evaluation. CT head revealed a hypodensity in the left posterior parietal lobe and follow up MRI brain confirmed an acute infarct. The following day she developed mild right upper extremity weakness, and a repeat CTH was stable, but CTA of the head and neck revealed numerous, bilateral, stenotic vessels intracranially. Given concern for RCVS, she was started on IV fluids and verapamil. Cerebral angiogram demonstrated findings consistent with RCVS LP for CSF studies was unremarkable. No clear etiology of her RCVS was identified.
Results: The patient was discharged with verapamil 80 mg three times daily. At her 2 month follow up, the patient's headaches and neurologic deficits had resolved. Repeat CTA completed 3 months after presentation showed resolution of previously noted intracranial stenoses, and the patient's verapamil was tapered down to 20 mg three times daily.
Conclusion: Although uncommon, RCVS may present with symptoms concerning for low CSF pressure headache. Both conditions can present with thunderclap headache, can occur following a Valsalva maneuver, and can be missed on initial neuroimaging studies.