Moaz Hamid contact

Western Sussex Hospitals, Worthing, United Kingdom

Acknowledgements

Nil.

Conflicts of Interest:

The author declares no conflicts of interest

Funding:

Nil received

1. Description

A 64-year-old lady presented to the emergency department with a headache and bilateral visual loss. She had recently visited France for two months to spend time with family, and had returned to the United Kingdom one week previously. The headache had developed around 2 weeks ago and whilst still on holiday, coming on suddenly. She had sought medical attention at the time of onset by visiting a french hospital, although assessment, including a CT head scan, had returned normal and thus she had been discharged.

However, given the persistence of her symptoms and also new symptoms of bilateral blurry vision for the past day, she sought medical attention again and therefore came to the emergency department.  She was reviewed by an emergency department doctor for initial work up and then referred to the ophthalmology team for further assessment.

A more focussed history and examination was then performed by the on-call ophthalmologist. It was ascertained that she had a diffuse headache, slightly more severe over her occipital region; the headache was extremely severe, with the patient rating it 9/10 in severity. She denied any other symptoms including jaw claudication, scalp tenderness and muscle weakness. Apart from a retinal tear around 10 years ago, she had no other past medical history and was on no regular medications. On examination her systolic blood pressure was raised around 30mmHg more than normal, although this was attributed to the severe pain she was experiencing and her other observations were normal. Ophthalmological assessment revealed significantly reduced visual acuity, being to hand motion only in both eyes. Pupils and intraocular pressures were normal. Visual fields and color blindness testing were unable to be performed. Slit lamp examination was unremarkable. Blood tests revealed a slightly raised erythrocyte sedimentation rate of 30, however other bloods such as her full blood count, liver function, and urea and electrolytes were normal.

Given the thunderclap nature of her headache and the significant visual loss, a CT angiogram of the head and neck was organised. This showed areas of infarction of her occipital lobe as well as narrowing of her PCA and MCA arteries bilaterally.

The patient was admitted to the hospital for further work up and pain control. Further autoimmune blood tests returned normal, an MRI angiogram of her head was also performed and this showed no signs of vasculitis and although corroborated the vasoconstriction found on the earlier CT scans. Given her history and imaging findings a diagnosis of reversible cerebral vasoconstriction syndromes, the patient was managed with supportive care and was started on oral amlodipine 5mg once daily for her blood pressure. Her headache and visual loss gradually resolved over the next 4 days.

2. Discussion

This case highlights a patient with a very commonly misinterpreted condition: reversible cerebral vasoconstriction syndrome (RCVS). The reasons for it being missed are due to it’s shared features with diagnoses such as subarachnoid haemorrhage or various vasculitides [1]. It has also been argued the reversible angiographic phenomenon poses a challenging diagnostic puzzle the clinician has to contend with [2].

Thankfully RCVS is become increasingly recognised and diagnosed, and is now understood as the encompassing term for a collection of similar entities such as postpartum cerebral angiopathy, migrainous vasospasm and thunderclap headache associated vasospasm. The pathophysiology involves reversible vasoconstriction of cerebral vasculature with an unclear precipitating event.

Its epidemiology is still being ascertained and indeed the true incidence of RCVS is unknown despite anecdotal reports suggesting it is fairly common. In terms of demographics, RCVS tends to predominate in women with a mean age of 42-45 years old being reported in the literature [3].

Classically in RCVS there appears to be a sudden onset, severe headache which settles and then recurs over days-weeks. There are less than 10% of patients who experience RCVS without a headache, therefore headache is a key diagnostic factor [1]. Neurological symptoms can subsequently develop in RCVS, with the visual symptoms including scotomas, hemianopias or cortical blindness being the most frequent.

Diagnosis in RCVS is based primarily on clinical findings and imaging. Laboratory tests are usually normal with neurovascular imaging revealing a “sausage on string” appearance of the Circle of Willis. However it is important to note that up to 70% of patients have no abnormality on initial brain scans despite there being widespread cerebral vasoconstriction present [4], as was likely the case with this patient initial imaging performed in France.

Management of RCVS is primarily centred around supportive care. Vasoconstrictive agents should be stopped and blood pressure should be normalised if high, with caution not to make the patient hypotensive and exacerbate any cerebral ischaemia. Calcium channel blockers are often used symptomatic relief of the headache despite there being no effect on the prognosis. Most patients symptoms resolve in days to weeks although around 20% do unfortunately have residual deficits from an ischaemic stroke that can occur as a complication of RCVS, these residual deficits are usually mild in severity [5].

3. Learning points

• Reversible cerebral vasoconstriction syndrome (RCVS) is a commonly missed diagnosis in patients with thunderclap headache and focal neurological signs
• Spontaneously resolving, recurrent thunderclap headaches are the pathognomic feature of RCVS
• Imaging is often normal in the initial stages of RCVS, despite diffuse cerebral-vasoconstriction occuring

References

1. “Reversible cerebral vasoconstriction syndrome – UpToDate.” [Online]. Available: https://www.uptodate.com/contents/reversible-cerebral-vasoconstriction-syndrome. [Accessed: 11-Nov-2020].
2. B. Grundt, T. Bolling, and M. L. Ritch, “Reversible Cerebral Vasoconstriction Syndrome: A Common Occurrence but Rare Diagnosis,” Cureus, vol. 12, no. 6, Jun. 2020.
3. J. Jensen, J. Leonard, K. Salottolo, K. McCarthy, J. Wagner, and D. Bar-Or, “The Epidemiology of Reversible Cerebral Vasoconstriction Syndrome in Patients at a Colorado Comprehensive Stroke Center.,” J. Vasc. Interv. Neurol., vol. 10, no. 1, pp. 32–38, Jun. 2018.
4. C. Y. Chen et al., “Vascular wall imaging in reversible cerebral vasoconstriction syndrome – A 3-T contrast-enhanced MRI study,” J. Headache Pain, vol. 19, no. 1, p. 74, Aug. 2018.
5. Sattar, G. Manousakis, and M. B. Jensen, “Systematic review of reversible cerebral vasoconstriction syndrome,” Expert Review of Cardiovascular Therapy, vol. 8, no. 10. NIH Public Access, pp. 1417–1421, Oct-2010.