The New England Journal of MedicineCase 18-2024: A 64-Year-Old Woman with the Worst Headache of Her Life

Reversible Cerebral Vasoconstriction Syndrome: Diagnosing and Managing Thunderclap Headaches with Associated Takotsubo Cardiomyopathy

This article presents a comprehensive case study of a 64-year-old woman who experienced a sudden, severe headache characterized as a thunderclap headache. The patient’s symptoms, imaging results, and subsequent management led to a diagnosis of Reversible Cerebral Vasoconstriction Syndrome (RCVS) with Takotsubo Cardiomyopathy. The article outlines the differential diagnosis, imaging studies, management strategies, and outcomes, emphasizing the importance of recognizing and treating RCVS in clinical practice.

Key Points:

• Patient Presentation
  • A 64-year-old woman presneuroented with a sudden, severe bilateral frontal headache rated at 10/10.
  • The headache onset occurred during public speaking, accompanied by nausea but without vomiting.
  • Medical history included hypertension and cervical disk disease with chronic neck pain.
  • Initial vital signs showed elevated blood pressure (157/77 mm Hg) but normal temperature, heart rate, and oxygen saturation.
• Initial Examination and Testing
  • The neurological examination was normal; no signs of focal neurological deficits or meningeal irritation.
  • Blood tests revealed elevated troponin T levels (0.36 ng/mL), while other parameters were within normal ranges.
  • Non-contrast head CT showed subarachnoid hemorrhage in the left parietal and occipital lobes.
• Differential Diagnosis
  • Thunderclap headache differential diagnosis included aneurysmal subarachnoid hemorrhage, RCVS, cerebral venous sinus thrombosis (CVST), arterial dissection, and posterior reversible encephalopathy syndrome (PRES).
• Key Diagnostic Findings
  • CT angiography revealed segmental narrowing of distal arterial branches without evidence of aneurysm.
  • MRI showed no acute infarcts but confirmed the presence of subarachnoid hemorrhage.
• Final Diagnosis
  • Diagnosis of RCVS with associated Takotsubo Cardiomyopathy was made based on imaging, clinical presentation, and elevated troponin levels.
  • RCVS was confirmed by the presence of thunderclap headache, female sex, anxiety trigger (public speaking), and convexal subarachnoid hemorrhage.
• Management and Follow-Up
  • Initial treatment included intravenous morphine and promethazine for pain and nausea management.
  • Avoidance of physical exertion and administration of oral nimodipine were recommended.
  • On follow-up, the patient experienced recurrent thunderclap headaches, requiring readmission.
  • Subsequent imaging showed progression of subarachnoid hemorrhage and new infarctions.
  • Intra-arterial verapamil was used to treat vasoconstriction, resulting in clinical improvement.
  • The patient’s condition stabilized with mild disability and no recurrence of thunderclap headaches after six months.
• Clinical Implications
  • RCVS should be considered in patients with thunderclap headaches and normal initial neurological exams.
  • Early imaging and follow-up are crucial for diagnosis and management.
  • Avoiding misdiagnosis as migraine and inappropriate treatments like triptans is essential.
  • Management involves controlling pain, monitoring blood pressure, and avoiding triggers.
  • Recognition of RCVS’s association with conditions like Takotsubo Cardiomyopathy can guide comprehensive care.

A trigger for RCVS is identified in approximately 25% to 60% of cases. However, RCVS can occur spontaneously, as well. Some identified triggers include vasoactive drugs, such as over the counter sympathomimetic drugs and migraine abortive medications. The most significant contributor, accounting for approximately 50% to 60% of cases in most published series, is the postpartum state. (StatPearls)

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