Secondary Logo

Journal Logo

Triptan-induced Reversible Cerebral Vasoconstriction Syndrome Presenting With Thunderclap Headache and Paraparesis

Ramineni, Kiran K. MD, DM; Jakkani, Ravi K. MD, FRCR; Girgani, Suresh MD, DM; Balani, Ankit DMRD, DNB; Satyanarayan, Sandeep MD

doi: 10.1097/NRL.0000000000000193
Case Report/Case Series
Free

Reversible cerebral vasoconstriction syndrome is a rare clinical syndrome characterized by sudden thunderclap headache often an under diagnosed neurological emergency. It is often provoked by postpartum state or exposure to provocative drugs. We report a rare case of Rizatriptan-induced reversible cerebral vasoconstriction syndrome presenting with thunderclap headache and paraparesis with complete recovery of neurological and imaging findings.

Yashoda Hospital, Malakpet, Hyderabad, Telangana, India

The authors declare no conflict of interest.

Reprints: Ravi K. Jakkani, MD, FRCR, Yashoda Hospital, Malakpet, Nalgonda Cross Roads, Hyderabad 5000036, Telangana, India. E-mail: ravikanthjakkani@gmail.com.

Back to Top | Article Outline

CASE REPORT

A 32-year-old female presented to the neurology department of our hospital with acute onset thunderclap headache associated with lower limb weakness. There was no history of seizures. Neurological examination showed normal sensorium, higher intellectual functions and cranial nerve examination. She had paraparesis with grade 2 power in right and grade 3 power in left lower limb with associated pyramidal signs. There was no sensory deficit. No history of bowel or bladder incontinence. No evidence of neck stiffness. Patient had history of chronic common migraine. She was prescribed oral Rizatriptan elsewhere and was advised to take it only during episodes of severe headache not relieved by conventional analgesics. However she self administered oral Rizatriptan 5 mg daily for >10 consecutive days till she was brought to our hospital. She also used paracetamol tablets twice a day over the past 10 days for headache. In the past she never used Triptans, other vasoactive drugs, and illicit drugs like marijuana, etc. No history of usage of antidepressants like selective serotonin reuptake inhibitors. She had 2 live children and last child birth was 5 years back following which she had undergone family planning surgery. No past history of thunderclap headaches. She was not a known hypertensive or diabetic. No family history of stroke in young. At the time of presentation she was afebrile, her blood pressure was 120/70 mm Hg, normal peripheral pulses, unremarkable general and cardiac examination. Chest radiograph, electrocardiogram, 2D echocardiogram, transesophageal echocardiogram, and ultrasound abdomen were normal. Laboratory investigations including complete blood picture, erythrocyte sedimentation rate, random blood sugar, serum creatine, liver function tests were normal. HIV1 and 2, VDRL were negative. Normal homocysteine levels and prothrombotic profile. Nerve conduction tests were normal. Magnetic resonance imaging (MRI) of brain and spine was done for further evaluation. MRI of spine was unremarkable with no intrinsic cord lesion or cord compression. MRI of brain showed subtle areas of restricted diffusion in the white matter of bilateral centrum semiovale (Fig. 1A). Magnetic resonance angiogram showed multifocal areas of narrowing and dilatation of circle of Willis branches, more prominent in bilateral anterior cerebral arteries (ACA’s) both in their proximal and distal portions giving beaded appearance (Figs. 1B, C). Computed tomography (CT) angiography of neck vessels and intracranial circulation better delineated the findings of multifocal areas of narrowing and dilatation of bilateral ACA’s (Fig. 2A). Follow-up CT brain after 3 days showed hypodensities in white matter of bilateral centrum semiovale suggesting ischemic lesions (Fig. 2B). On the basis of imaging findings and clinical history we considered drug-induced cerebral vasoconstriction syndrome as provisional diagnosis and central nervous system (CNS) vasculitis as differential diagnosis. Work up for vasculitic markers including antinuclear antibodies, antineutrophil cytoplasmic antibody and rheumatoid factor assay was negative. Cerebrospinal fluid analysis could not be done due to negative patient consent; however, she was counselled regarding its importance in this clinical context. Illicit drug screening of urine was done to exclude other vasoactive drugs like marijuana and cocaine and was negative. Rizatriptan was stopped. Patient was treated with course of oral nimodipine which was tapered gradually over 4 weeks. Deep vein thrombosis prophylaxis and physiotherapy was given. As our first diagnosis was drug-induced cerebral vasoconstriction syndrome, and we monitored her closely in the hospital we consciously avoided steroids in view of the possible worsening described with steroid usage in this condition. Patient had good clinical improvement and with relief in headache by fifth day and was able to walk without support after 2 weeks and complete recovery by 4 weeks. Follow-up CT angiogram done after 6 weeks showed complete resolution of vascular narrowing with good caliber of vessels (Figs. 3A, B). Clinical, imaging features were consistent with drug-induced reversible cerebral vasoconstriction syndrome (RCVS). At the time of discharge she was counselled not to use Triptans and other drugs known to cause RCVS including illicit drugs, marijuana, etc. Possible recurrence of RCVS was explained. She was prescribed divalproex sodium 500 mg once daily, naxdom 250 mg sos for severe headache typical of migraine and explained to come to us at the earliest if she develops thunderclap headache or focal deficits. Possible adverse drug reactions also explained. She and her family members were counselled regarding beneficial life style measures including good sleep hygiene, routine meal schedules, exercise, coping with headache triggers, etc. She had undergone family planning surgery 5 years back and not willing to become pregnant again, we have explained the postpartum risk.

FIGURE 1

FIGURE 1

FIGURE 2

FIGURE 2

FIGURE 3

FIGURE 3

Back to Top | Article Outline

DISCUSSION

RCVS is a diverse clinical syndrome characterized by cerebral vasoconstriction associated with severe headache and may be associated with seizures or neurological deficits. It commonly affects second to fifth decade age groups with female preponderance.1 Common clinical presentation includes sudden onset thunderclap headache usually associated with nausea, vomiting, photophobia, and blurring of vision.2 The etiology is diverse and it usually manifests as multifocal vasoconstriction and dilatation of intracranial arteries.3

Exact pathogenesis of RCVS is not fully established; however, it is possibly due to transient dysregulation of cerebral vascular autonomic regulation mechanism resulting in multifocal vasoconstriction. In about half of the cases likely cause is postpartum state or exposure to vasoactive substance.1 Triptans are a rare cause of RCVS with only a handful of cases reported previously in literature.4 The major complications are localized convexity nonaneurysmal subarachnoid hemorrhage (22%) and ischemic stroke or intracerebral hemorrhage (7%), which may leave permanent residual neurological deficits.1 The incidence of cerebral infarction in patients of RCVS is around 6—39% in previous studies.5 In our patient who had chronic history of migraine on treatment, development of paraparesis warranted further evaluation with MRI spine and brain with angiogram which clinched the diagnosis. Acute headache of RCVS can often be mistaken for severe migraine attack in a patient with history of migraine. However, the thunderclap characteristic may help to differentiate it from acute migraine attack.5

As the common presentation of RCVS is thunderclap headache, CT is the initial investigation done. CT is normal in majority of the cases, but may occasionally show focal sub arachnoid hemorrhage (22%) or intracranial bleed (7%).1 Magnetic resonance angiogram or CT angiogram usually shows multisegmental vasoconstriction with associated dilatation involving large and medium sized intracranial arteries giving beaded appearance, seen in up to 90% cases.6 Initial angiogram study may be negative; however, follow-up study after 1 to 2 weeks is required for confirmation. MRI can also show small acute ischemic foci. Noninvasive vascular imaging has replaced catheter angiogram, which, however, still remains gold standard in inconclusive cases. There is reversal of angiographic findings with normal caliber of vessels in 1 to 3 month interval which is a prerequisite for diagnosis.3

RCVS has to be differentiated from CNS vasculitis which poses a great diagnostic dilemma. Symptoms in RCVS usually evolve rapidly as compared with insidious onset in CNS vasculitis. Headaches are frequent in vasculitis and are followed by a stepwise deterioration with transient deficits, several infarcts, or cognitive decline.5 Presence of secondary causes like postpartum state or exposure to vasoactive drugs and absence of vasculitic markers favors RCVS as in our case. Complete reversibility of angiogram findings also favors RCVS.7

Treatment of RCVS is usually supportive with a general agreement on discontinuation of the offending vasoactive drugs.8 Calcium channel blockers like nimodipine, verapamil, and nifedipine are most commonly used with initial intravenous regimen followed by oral course for a period of 4 to 8 weeks.9Steroids should be avoided in RCVS in view of possible worsening as described previously by Singhal and Topcuoglu.10 Patients should be counselled regarding the avoidance of potential triggers and possibility of reccurence of RCVS.11

Back to Top | Article Outline

CONCLUSIONS

RCVS is a rare cerebrovascular disorder which still remains to be under diagnosed and misdiagnosed. RCVS with predominant ischemic presentation is less commonly described. Present case illustrates one of the rare cerebrovascular causes of paraparesis as a manifestation of Triptan-induced ischemic RCVS.

Back to Top | Article Outline

REFERENCES

1. Ducros A. Reversible cerebral vasoconstriction syndrome. Presse Med. 2010;39:312–322.
2. Singhal AB. Cerebral vasoconstriction syndromes. Top Stroke Rehabil. 2004;11:1–6.
3. Hantson P, Forget P. Reversible cerebral vasospasm, multilobular intracerebral hemorrhages, and nonaneurysmal subarachnoid hemorrhage: review of possible interrelationships. Curr Pain Headache Rep. 2010;14:228–232.
4. Kato Y, Hayashi T, Mizuno S, et al. Triptan-induced reversible cerebral vasoconstriction syndrome: two case reports with a literature review. Intern Med. 2016;55:3525–3528.
5. Ducros A. Reversible cerebral vasoconstriction syndrome. Lancet Neurol. 2012;11:906–917.
6. Ducros A, Boukobza M, Porcher R, et al. The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. Brain. 2007;130:3091–3101.
7. Calabrese LH, Dodick DW, Schwedt TJ, et al. Narrative review: reversible cerebral vasoconstriction syndromes. Ann Intern Med. 2007;146:34–44.
8. Singhal AB, Caviness VS, Begleiter AF, et al. Cerebral vasoconstriction and stroke after use of serotonergic drugs. Neurology. 2002;58:130–133.
9. Elstner M, Linn J, Muller-Schunk S, et al. Reversible cerebral vasoconstriction syndrome: a complicated clinical course treated with intra-arterial application of nimodipine. Cephalalgia. 2009;29:677–682.
10. Singhal AB, Topcuoglu MA. Glucocorticoid-associated worsening in reversible cerebral vasoconstriction syndrome. Neurology. 2017;88:228–236.
11. Chen SP, Fuh JL, Lirng JF, et al. Recurrence of reversible cerebral vasoconstriction syndrome: a long-term follow-up study. Neurology. 2015;84:1552–1558.
Keywords:

RCVS; Triptan; vasculitis; paraparesis

Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.