• Users Online: 49
  • Print this page
  • Email this page

Table of Contents
Year : 2020  |  Volume : 11  |  Issue : 1  |  Page : 56-57

Cerebral vasculopathy in progressive systemic sclerosis: A rare presentation

1 Department of Neurology, Dr. RML Hospital, Delhi, India
2 Department of Medicine, UCMS and GTB Hospital, Delhi, India

Date of Submission24-Dec-2019
Date of Acceptance26-Dec-2019
Date of Web Publication07-Feb-2020

Correspondence Address:
Dr. Abhishek Juneja
A-15, Old Quarter, Back Side, Ramesh Nagar, Delhi - 110 015
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/INJMS.INJMS_155_19

Rights and Permissions

How to cite this article:
Juneja A, Jhamb R. Cerebral vasculopathy in progressive systemic sclerosis: A rare presentation. Indian J Med Spec 2020;11:56-7

How to cite this URL:
Juneja A, Jhamb R. Cerebral vasculopathy in progressive systemic sclerosis: A rare presentation. Indian J Med Spec [serial online] 2020 [cited 2022 Nov 30];11:56-7. Available from: http://www.ijms.in/text.asp?2020/11/1/56/277932

Dear Editor,

We report the case of a 30-year-old female patient with diffuse systemic sclerosis presenting with sudden-onset left hemiparesis. The patient was diagnosed as a case of diffuse systemic sclerosis 2 years back when she developed skin tightening and difficulty in deglutition [Figure 1] and [Figure 2]. She developed sudden-onset right-sided body weakness with inability to speak when cooking in the kitchen. Her blood pressure was 116/64 mmHg and a regular pulse rate of 98/min. All peripheral pulses were palpable. She had spontaneous eye opening with normal extraocular movements. She could not speak anything despite apparently normal comprehension. She could not move her right upper limb and could slightly move the right lower limb in bed. The power in the left upper and lower limb was normal. Noncontrast computed tomography (CT) of the brain showed right-sided middle cerebral artery (MCA) infarct [Figure 3]. CT angiogram of the head and neck vessels showed decreased opacification of the right MCA in comparison to the left with right MCA infarct. Furthermore, right posterior communicating artery was more prominent in comparison to the left suggestive of stenotic right MCA with collateral circulation from the right posterior communicating artery [Figure 4]. Her erythrocyte sedimentation rate (68 mm/h) and C-reactive protein levels were high. Serum anti-Scl 70 antibody was positive whereas antiphospholipid antibodies were negative. Serum homocysteine levels were normal. A surface echocardiogram was normal with no cardioembolic source. She was started on high-dose intravenous methylprednisolone. The power improved over next 2–3 weeks to 2/5 (Medical Research Council grading) in the right upper limb and some antigravity effort in the lower limb. She was discharged on aspirin and tapering dose of oral prednisolone. She gradually recovered and started walking with support after 2 months of starting steroid therapy.
Figure 1: Sclerodermic facies with left-sided facial nerve paly

Click here to view
Figure 2: Sclerodactyly

Click here to view
Figure 3: Noncontrast computed tomography head showing hypodensity in the right middle cerebral artery territory

Click here to view
Figure 4: Computed tomography angiogram head and neck vessels showing stenosis of the right middle cerebral artery (arrow)

Click here to view

Progressive systemic sclerosis (PSS) is a multisystem disease affecting multiple organs, commonly skin, lungs, myocardium, kidneys, and gastrointestinal tract.[1] It is characterized by tissue fibrosis due to increased collagen deposition and vasculopathy. Raynaud's phenomenon commonly occurring in scleroderma is a manifestation of peripheral vascular involvement.[2] However, cerebral vasculopathies resulting in neurological complications are extremely rare in PSS.[2]

Primary central nervous system (CNS) involvement in scleroderma is extremely rare.[3] Four-vessel cerebral angiography should be performed in a patient with PSS presenting with neurological complications to look for abnormal CNS vasculature. Leptomeningeal biopsy remains the gold standard to confirm cerebral vasculitis suggestive of angiographic evidence. In our case, steroids were given empirically without histopathological evidence of cerebral vasculitis. However, in view of background illness and clinicoradiological presentation, possibility of cerebral vasculitis is more likely. Early initiation of immunosuppressive therapy can be lifesaving in such cases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Lucivero V, Mezzapesa DM, Petruzzellis M, Carella A, Lamberti P, Federico F. Ischaemic stroke in progressive systemic sclerosis. Neurol Sci 2004;25:230-3.  Back to cited text no. 1
Cutolo M, Sulli A, Smith V. Assessing microvascular changes in systemic sclerosis diagnosis and management. Nat Rev Rheumatol 2010;6:578-87.  Back to cited text no. 2
Pathak R, Gabor AJ. Scleroderma and central nervous system vasculitis. Stroke 1991;22:410-3.  Back to cited text no. 3


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

  In this article
Article Figures

 Article Access Statistics
    PDF Downloaded176    
    Comments [Add]    

Recommend this journal