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Case Reports
. 2021 Jan 27;14(1):e239307.
doi: 10.1136/bcr-2020-239307.

Moyamoya angiopathy unmasking systemic lupus erythematosus

Shambaditya Das  1 , Souvik Dubey  2 , Alak Pandit  1 , Biman Kanti Ray  1
Affiliations
Case Reports

Moyamoya angiopathy unmasking systemic lupus erythematosus

Shambaditya Das et al. BMJ Case Rep. .

Abstract

A 47-year-old woman with history of seizure disorder (semiology of seizure unknown), not well controlled with antiepileptic drugs since last 30 years presented with 1-year history of intermittent throbbing headache. On the day prior to admission, she experienced worst headache, followed by loss of consciousness. On regaining consciousness, she had neck pain without any focal neurological deficit, but examination was marked by positive meningeal signs. She had history of oral ulceration, photosensitivity and small joints pain for which no medical consultancy was sought until. Following relevant investigations, this case came out to be moyamoya angiopathy secondary to underlying systemic lupus erythematosus. She was put on immunosuppressive and immunomodulator as per recommendations. Among neurological symptoms, headache improved dramatically without any further seizure recurrence till the 6 months of follow-up.

Keywords: epilepsy and seizures; headache (including migraines); neuroimaging; stroke; systemic lupus erythematosus.

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Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
NCCT brain showing hyperdensities in right temporal lobe (A), along with hyperdensities in the ventricles (B). Intracerebral haemorrhage with intraventricular extension. NCCT, non-contrast CT.
Figure 2
Figure 2
MR angiography showing cut-off at the terminal portions of bilateral internal carotid artery with non-visualisation of bilateral middle cerebral artery and anterior cerebral artery with evidence of collaterals and intracerebral haemorrhage.
Figure 3
Figure 3
DSA showing evidence of stenosis of terminal ICA along with formation of collaterals in different views, right ICA anteroposterior (A), Left ICA anteroposterior (B), right ICA lateral (C), left ICA lateral (D). DSA, digital substraction angiography; ICA, internal carotid artery.
See this image and copyright information in PMC

References

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    1. Das S, Dubey S, Acharya M, et al. . The disease presentation of moyamoya angiopathy in eastern India. J Stroke Cerebrovasc Dis 2020;29:104957. 10.1016/j.jstrokecerebrovasdis.2020.104957 - DOI - PubMed
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