Reversible Cerebral Vasoconstriction Syndrome Presenting with Thunderclap Headache in a Child

Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is a clinical and radiological diagnosis characterized by the acute onset of headache, multiple constrictions of several cerebral blood vessels, and remission within 3 months [1]. Thunderclap headache, which usually lasts for 1 to 3 hours, is a typical symptom accompanying RCVS, and focal neurologic deficits may also occur due to hemorrhages, infarcts, and even posterior reversible encephalopathy syndrome (PRES) [2]. RCVS predominantly occurs in middle-aged women aged 30 to 50 years [1]. Only small case series and individual cases of RCVS have been reported in children [2]. We describe a healthy child with a thunderclap headache associated with RCVS, which is the first pediatric case of this condition in Korea. An 8-year-old boy presenting with sudden-onset, severe, and diffuse headache was referred to our pediatric neurology clinic. He described thunderclap headaches with a verbal numeric pain scale of 9 to 10 intensity within 2 hours of falling asleep. He woke up with a severe headache, and taking acetaminophen did not help. The patient was born full-term without any perinatal problems, and his medical history was unremarkable. He and his family had no history of headaches, migraines, or neurological diseases. He was not taking any medications. On admission, his neurological examination results were normal. His blood pressure was 110/70 mm Hg. A complete blood count, serum electrolyte concentrations, serum glucose level, hepatic and renal function tests, thyroid function test, and routine urinalysis all showed normal results. Brain magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) showed multiple segmental narrowing of the cerebral arteries without brain parenchymal lesions (Fig. 1A); however, electroencephalography showed normal findings without focal slow waves. Several laboratory tests were performed under the suspicion of arteriopathy or an infectious or rheumatologic disorder. The erythrocyte sedimentation rate and C-reactive protein level were normal. Cerebrospinal fluid (CSF) analysis revealed a normal cell count (0/μL), protein level (21 mg/dL), and glucose level (72 mg/dL), with a normal opening pressure (17 cmH2O). The CSF bacterial culture was negative, and polymerase chain reaction tests did not detect herpes simplex virus, human herpes virus 6, varicella zoster virus, or enterovirus. The plasma von Willebrand factor antigen test, performed to detect primary angiitis of the central nervous system, was normal. He had normal procoagulant screening results, including protein C, protein S, antithrombin III, homocysteine levels, negative lupus anticoagulant, and cardiolipin antibodies. His autoimmune workup, including rheumatoid factor,