Primary antiphospholipid antibody syndrome presenting with cortical venous sinus thrombosis

Full text

NJM
An Official Publication of
Nigerian Association of Resident Doctors (NARD)
Print ISSN : 1115-2613
Online ISSN : 2667-0526
Vo lume 30 • Issue 3 • May-J u ne 202 1
NIGERIAN
JOURNAL OF MEDICINE
ww w.njmonline.org
Spine 7.5 mm
Nigerian Journal of Medicine • Volume 30 • Issue 3 • May-June 2021 • Pages ***-***

Letter to Editor
To the Editor,
We report the case of a 22‑year‑old unmarried female patient
with severe diuse headache, bilateral vision loss for two days,
and multiple episodes of generalized tonic–clonic seizures
with alteration of sensorium on the day of presentation to
the casualty. There is no preceding history of fever, arthritis,
or skin rash. There was no past medical history of any
chronic comorbid illness. On physical examination, she
was unconscious with normal pupillary size and reaction;
bilateral fundi showed papilloedema. The neck was supple;
Kernig’s sign was negative. Flexor plantar response was
present bilaterally with no lateralizing sign on neurological
examination. Blood investigations including blood counts,
hepatic, renal, thyroid, lipid, and glycemic prole were within
normal limits; inammatory markers including erythrocyte
sedimentation rate and C-reactive protein were raised. HIV
test by Enzyme-Linked Immunosorbent Assay (ELISA),
antinuclear antibody and antineutrophil cytoplasmic antibody
test by indirect immune fluorescence assay, and urine
pregnancy test were negative. Urine and blood culture yielded
no organism. Magnetic resonance (MR) imaging of the brain
showed parasagittal T2/uid‑attenuated inversion recovery
hyperintensity with diusion restriction and hemorrhagic
transformation suggesting cerebral venous infarcts [Figure 1].
MR venogram conrmed superior sagittal sinus thrombosis.
Antiphospholipid antibody (immunoglobulin G) level by
ELISA was very high (186.6 U/L; N < 12 U/L), done in view of
venous thrombosis in a young patient. The patient was treated
with subcutaneous low-molecular-weight heparin at a dose
of 1 mg/kg twice daily and oral warfarin titrated according
to international normalized ratio target value of 2.0–3.0. The
patient gradually improved over the next few weeks and was
discharged on warfarin and oral antiepileptic drugs.
Antiphospholipid antibody syndrome (APS) is a thrombophilic
disorder. It usually occurs in young females. It is diagnosed
based on the presence of antiphospholipid antibodies.[1] It causes
venous or arterial thrombosis and obstetric complications.[1]
Venous thrombosis is more common compared to arterial
thrombosis in APS. Venous thrombosis commonly presents
as deep vein thrombosis in the legs or livedo reticularis in the
skin.[1,2] Primary APS presenting with cerebral venous sinus
thrombosis (CVT) is a rare complication in primary APS.
Central nervous system (CNS) complications usually occur in
secondary APS with systemic lupus erythematosus (SLE).[2,3]
We excluded other common causes of CVT such as drugs,
pregnancy or postpartum state, SLE, and immune-mediated
vasculitis by history, examination, and relevant investigations.
The treatment of APS mainly relies on anticoagulation and
managing CNS complications including seizures and raised
intracranial tension, if any. We suggest that APS should be kept
in mind while managing a case of CVT, especially in young
women of childbearing age to prevent fatal complications.
Declaration of patient consent
In the form the patient has given her consent for her images
and other clinical information to be reported in the journal.
The patients understand that her name and initials will not be
published and due eorts will be made to conceal her identity,
but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conicts of interest.
Abhishek Juneja1, Kuljeet Singh Anand1, Rakesh Kumar Mahajan2
Departments of 1Neurology and 2Microbiology, Dr. RML Hospital,
New Delhi, India
Address for correspondence: Dr. Abhishek Juneja,
Department of Neurology, Dr. RML Hospital, New Delhi, India.
E‑mail: drabhishekjuneja@gmail.com
Primary Antiphospholipid Antibody Syndrome Presenting with
Cortical Venous Sinus Thrombosis
© 2021 Nigerian Journal of Medicine | Published by Wolters Kluwer - Medknow 349
Figure 1: Magnetic resonance imaging brain T2 sequence showing
bilateral parasagittal hyperintensity suggestive of cortical venous infarcts

Letter to Editor
references
1. Dhir V, Pinto B. Antiphospholipid syndrome: A review. J Mahatma
Gandhi Inst Med Sci 2014;19:19-27.
2. Sarkar R, Paul R, Thakur I, Sau TJ, Roy D, Pandey R, et al. Multiple
cerebral venous sinus thrombosis as st manifestation of primary
anti-phospholipid antibody syndrome. J Assoc Physicians India
2018;66:11-2.
3. Sanna G, Bertolaccini ML, Cuadrado MJ, Khamashta MA, Hughes GR.
Central nervous system involvement in the antiphospholipid (Hughes)
syndrome. Rheumatology (Oxford) 2003;42:200-13.
This is an open access journal, and arcles are distributed under the terms of the Creave
Commons Aribuon‑NonCommercial‑ShareAlike 4.0 License, which allows others to
remix, tweak, and build upon the work non‑commercially, as long as appropriate credit
is given and the new creaons are licensed under the idencal terms.
How to cite this article: Juneja A, Anand KS, Mahajan RK. Primary
antiphospholipid antibody syndrome presenting with cortical venous sinus
thrombosis. Niger J Med 2021;30:349-50.
Submitted: 17-Oct-2020 Revised: 18-Nov-2020
Accepted: 23-Apr-2021 Published: 19-Jun-2021
© 2021 Nigerian Journal of Medicine | Published by Wolters Kluwer - Medknow
Access this article online
Quick Response Code:
Website:
www.njmonline.org
DOI:
10.4103/NJM.NJM_191_20
Nigerian Journal of Medicine ¦ Volume 30 ¦ Issue 3 ¦ May-June 2021
350