Spinal cord reversible cerebral vasoconstriction syndrome

Abstract

We present the case of a 19-year-old woman who developed reversible cerebral vasoconstriction syndrome (RCVS) with primarily posterior circulation infarcts, including a high cervical spinal cord infarct. RCVS is thought to be a transient vasospastic condition of the brain and, to our knowledge, has not been previously described to affect the spinal cord.

KEY POINTS

• Acute atraumatic quadriparesis in a young woman has classically been most concerning for demyelinating disease of the central nervous system, but workup should include an evaluation for strokes and acute neuropathies as well.

• The history of stimulant use may help guide the workup to include autonomic sensitive systems, including cerebral vasculature disease.

CME

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CASE SUMMARY

A 19-year-old woman with a history of migraines and epilepsy presented with severe thunderclap headache (TCH) after exercising at the gym; over the next 2 days, the headache progressed to acute quadriparesis. She admitted to taking dextroamphetamine a week before, alongside a reported habit of consuming five to six cups of coffee daily and at least one or two Red Bulls daily for approximately 1 week leading up to the incident. Additionally, she was concurrently taking topiramate and lamotrigine.

The neurological exam conducted at the time of presentation was remarkable for 0/5 proximal strength in all extremities and minimal bilateral grip strength. Her cranial nerves and extraocular muscles were intact without any visual abnormalities. Her progressive weakness and worsening respiratory distress necessitated intubation, eventually resulting in tracheostomy and percutaneous enterogastric tube placement for presumed permanent quadriparesis due to spinal cord infarct. She received a one-time dose of methylprednisolone, which was discontinued as there was no immediate neurologic improvement, and the pattern of cord involvement proved to be ischemic. Her blood pressures were low, such that she could not tolerate calcium channel blockers and briefly needed to be on pressors.

Magnetic resonance imaging (MRI) of the brain and cervical spine demonstrated scattered acute diffusion restricting strokes, mostly in the posterior circulation (Figure 1), and a T2 hyperintense lesion with enhancement in the C2–C3 cord (Figure 2). Her presenting symptoms were attributed to these findings. A computed tomography (CT) angiogram of the head and neck and a formal cerebral angiogram (four-vessel digital subtraction angiography) showed no significant stenosis, aneurysm, vascular malformation, dissections, or branch arterial occlusion in the intracranial and extracranial arterial vasculature. There was diminished flow in the region of the anterior spinal artery (Figure 3) on the formal angiogram.

Figure 1.

Diffusion-weighted imaging showing predominantly posterior circulation acute infarcts with two middle cerebral artery territory infarcts as well.

Figure 2.

Sagittal T2 fast spin echo and axial T2 showing a cord infarct at the C2–C3 disk levels.

Figure 3.

(a) CT angiogram and (b) formal cerebral angiogram revealing diminished flow in anterior spinal arteries (yellow arrow) of our patient. (c) A normal anterior spinal artery (black arrow) from our institution as a comparison.

Her treatment regimen included a daily multivitamin, thiamin, aspirin, and continuation of her home seizure medications of lamotrigine and topiramate. She underwent inpatient rehabilitation and eventually regained full neurological function, allowing for decannulation of tracheostomy and removal of the percutaneous endoscopic gastrostomy tube. Repeat contrasted MRI of the brain and cervical spine 6 weeks after symptom onset demonstrated a marked decrease in the T2 fluid-attenuated inversion recovery (FLAIR), with near complete resolution of the enhancement in the cord at C2–C3.

Extensive workup for the etiology of her strokes was performed. Cerebrospinal fluid analysis was unremarkable for infection, inflammation, or subarachnoid hemorrhage (red blood cell count 1/uL, white blood cell count 1/uL, protein 25 mg/dL, glucose 54 mg/dL). Cerebrospinal fluid fungal and bacterial cultures and a meningitis polymerase chain reaction panel were all negative. Her methylmalonic acid level, anti-nuclear antibodies, and neuromyelitis optica antibodies were normal or negative. Hypercoagulable laboratory results, including antithrombin III, factor 5, lupus-like anticoagulant, JAK2, prothrombin gene mutation, and antiphospholipid antibody panels were also negative. Other “unremarkable” imaging studies included CT of the chest, abdomen, and pelvis, transthoracic echocardiogram with bubble study, and transesophageal echocardiograms. Additionally, an implantable loop recorder was placed, which more than 2 years after the event, remained without evidence of any significant arrhythmia.

CLINICAL QUESTIONS

1. Which of the following statements about RCVS is false?

   1. RCVS is more common in women.

   2. RCVS usually resolves with minimal disability.

   3. RCVS has a high frequency with prior migraine, depression, and anxiety.

   4. RCVS can be diagnosed solely by cerebral arteriogram.

2. A 35-year-old woman who recently delivered a healthy baby, from a pregnancy complicated by preeclampsia, had been drinking caffeine due to being sleep deprived with the new baby. On the first day of trying a new exercise routine, she developed a diffuse headache that rapidly progressed to “the worst headache of my life.” Which of the following statements about the evaluation of a patient presenting with a TCH is false?

   1. Negative noncontrast brain CT findings within 6 hours from ictus essentially excludes subarachnoid hemorrhage

   2. CT angiography is a reasonable alternative to lumbar puncture if clinical suspicion of an intracranial source of subarachnoid hemorrhage is high

   3. TCH is not considered a neurological emergency

   4. What differentiates TCH from other headache types is how rapidly they reach their peak intensity, not the headache intensity itself

Answers are provided at the end of the article.

DISCUSSION

This patient experienced a sudden TCH that progressed to acute quadriparesis, occurring concurrently with stimulant use and exercise. The cerebral vasculature receives heavy innervation from sensory fibers of both the trigeminal nerve and the dorsal root of C2.¹ Although the pathophysiology of RCVS is not clearly understood, the dysregulation of cerebrovascular tone shows significant impairment of vasodilatory reserve, at least during the acute phase. The changes in cerebrovascular tone may be driven by heightened sympathetic activity, endothelial dysfunction, and oxidative stress.² Typically, TCH, which could be attributed to the dilatation of distal arterioles and meningeal arteries, resolves within 2 to 3 weeks. The vasoconstriction with consequent edema and stroke-related weakness do not completely resolve for 3 months on average.³

RCVS may be primary (idiopathic) or secondary (60% to 80%), influenced by many precipitating factors, of which the most common are vasoactive substance use and postpartum state.⁴ The vascular smooth muscle can be altered by various stimulants resulting in vasoconstriction, which leads to an increase in systemic blood pressure and a decrease in localized blood flow. Certain stimulants like caffeine, cocaine, and amphetamine derivatives are closely related to vasospasm and brain hypoperfusion.⁴ RCVS carries multiple risk factors including an age of 20 to 50 years, female gender, and stimulant use,⁵ apart from use of central nervous system stimulants and physical exertion. Our patient was on lamotrigine and topiramate at the time of presentation. It remains unclear whether these medications predisposed her to vasoreactivity or if her seizure and migraine disorders were contributory factors. Both migraines⁶ and epilepsy^7,8 are associated with abnormal cerebrovascular tone. The patient did not have any of her typical migraines or seizures around the time of her presentation.

A combination of an appropriate clinical presentation and diagnostic imaging may lead to the diagnosis of RCVS. To diagnose RCVS, T2-weighted FLAIR and T1-weighted imaging are used to analyze white matter lesions and evaluate for the possibility of subarachnoid hemorrhage, intracerebral hemorrhage, and pituitary apoplexy. Vascular imaging, including MRI and CT angiography and formal cerebral angiogram (in increasing order of accuracy and invasiveness), are used to exclude cerebral aneurysms, arterial dissections, vasoconstriction, and venous sinus thrombosis.⁹ An abnormal cerebral angiogram showing smooth-tapered narrowing followed by abnormal dilated segments in the cerebral arteries is suggestive of RCVS. Due to the imaging similarities with central nervous system vasculitis, the diagnosis cannot be made strictly based on angiographic findings,¹⁰ and the clinical presentation is important. Diagnostic imaging is reliable within the first 30 days of the condition’s development.

The posterior circulation of the brain, which is based around the vertebral arteries and basilar artery, also supplies the cervical spinal cord. A single anterior spinal artery (ASA), arising by fusing the medial branch of two vertebral arteries, supplies the anterior two-thirds of the cord, whereas the two posterior spinal arteries arising directly from each vertebral artery supply the posterior third of the cervical cord. The posterior circulation is densely innervated by the sympathetic nervous system¹ and hence is likely more susceptible to RCVS as is seen in posterior reversible encephalopathy syndrome.⁹

We reported here a patient who presented with diffuse strokes, mostly in the posterior circulation, with clinically characteristic circumstances of RCVS. The anterior spinal artery, which is supplied by the vertebral arteries, might also become involved in severe vasoconstriction and was the only location where vascular imaging was abnormal in our patient. In older patients, the more common stroke demographic, the imaging appearance of the ASA is one of diminished flow, which makes it difficult to distinguish any abnormality. However, in our young patient, the flow to her ASA should have been robust.

ANSWERS TO CLINICAL QUESTIONS

Question 1, d. Among RCVS patients, 81% are female.⁵ Overall, 90% to 95% of admitted patients achieve complete or major resolution of symptoms and clinical deficits within days to weeks. Recurrence is exceptional, although 5% of patients can later develop isolated TCH with or without mild cerebral vasoconstriction.¹¹ Multifocal stenosis (84.0%) and beading shape (82.4%) were the leading abnormal findings on angiography, while cerebral ischemic lesions (47.6%) and cerebral hemorrhage (mainly subarachnoid hemorrhage) (35.1%) were the main findings on brain CT/MRI.⁵ RCVS has a high frequency of occurrence with serotoninergic conditions such as prior migraine, depression, and anxiety¹² and sympathetic vasoconstrictive triggers, including exertion. The patient’s first angiogram will be normal in up to 30% of cases if done early in the disease process (within 1 week).¹³ The RCVS2 score relies heavily on clinical presentation, including the presence of THC, a vasoconstrictive trigger, female gender, and the presence of subarachnoid hemorrhage. Intracranial carotid involvement makes RCVS less likely.¹⁰

Question 2, c. TCH might be the first sign of subarachnoid hemorrhage, intracranial aneurysm, cerebral venous sinus thrombosis, cervical artery dissection, acute hypertensive crisis, spontaneous intracranial hypotension, ischemic stroke, retroclival hematoma, pituitary apoplexy, third ventricle colloid cyst, intracranial infection, and RCVS.¹⁴ Day and Raskin are credited with coining the term THC. The term was used in a case report published in 1986 of a 42-year-old woman who presented with an intense, sudden-onset headache described as: “like a hammer hitting my head.” Commonly referred to as “the worst headache ever” or “worst headache of my life,” TCH is considered a medical emergency until proven otherwise because many of the possible causes are potentially life threatening. What differentiates TCH from other headache types is how rapidly they reach their peak intensity, not the headache intensity itself.¹⁵

Disclosure statement/Funding

The planners and faculty for this activity have no relevant financial relationships to disclose. The authors report no funding. The patient consented to publication of this case report.