Reversible Cerebral Vasoconstriction Syndrome Presenting as Micturition Headaches in a Pediatric Patient
Brianna Brun1, Jennifer McKinney2, Melissa Chung3
1Strong Memorial Hospital, 2Nationwide Childrens Hospital, 3Nationwide Children'S Hospital
Objective:
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Background:

Reversible cerebral vasoconstriction syndrome (RCVS) presents with recurrent thunderclap headaches and is characterized by cerebral artery vasoconstriction that resolves within 3 months. Although well described in adult patients, pediatric cases are exceedingly rare. Here we present a child who presented with recurrent abrupt onset micturition headaches found to have RCVS. 

Design/Methods:
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Results:

This patient is a 10-year-old male with no prior headache history who developed new onset daily headaches. He was initially diagnosed with migraine, but he developed progressive visual impairments and encephalopathy. Careful review of history revealed that the patient recently used phenylephrine containing cough medication and headaches were sudden onset, severe, occipital-predominant in nature. Headaches occurred immediately after micturition with significant improvement within 30 minutes. Emergent MRI demonstrated restricted diffusion in watershed regions bilaterally with associated FLAIR signal abnormalities, perfusion deficits larger than the areas of restricted diffusion, and diffuse multifocal irregularity of intracranial arteries. Diagnosis of RCVS was suspected based upon imaging and history, so he was started on verapamil and aspirin. Diagnosis of RCVS was confirmed via diagnostic cerebral angiogram showing improved vasoconstriction after intraarterial verapamil. During hospitalization, he developed stereotyped episode of abrupt awakening overnight with intense fear, hallucinations, and hypermotor activity with EEG findings suggestive of electroclinical seizures arising from the left hemisphere. These episodes resolved with levetiracetam. Repeat imaging four months later showed normal vasculature and areas of cortical encephalomalacia in the bilateral frontal, parietal, and occipital lobes.  Five months after symptom onset, he had persistent left inferior quadrantanopia, but resolution of seizures and headache. 

Conclusions:

This case highlights the importance of careful clinical history and neuroimaging in pediatric patients presenting with thunderclap headaches. 

10.1212/WNL.0000000000205866