Case
Presentation
A 33-year-old woman with a history of recurrent hospitalization due to
headaches with blurred vision was admitted to our neurology department.
In January 2018, she had cerebral vein thrombosis (CVT) in the left
transverse sinus with left mastoiditis, treated with anticoagulants and
antibiotics. Extensive work-ups for finding the etiology produced
negative results, including acquired and genetic thrombophilia tests. In
December 2020, she was diagnosed with a new CVT in the sigmoid sinus.
Again she was discharged with a 5mg daily dose of Warfarin. In March
2021, she presented horizontal binocular diplopia, blurry vision, and
hemicranial headache in the last hospitalization. The patient also
complained of right eye proptosis, which developed in a period of 3
months. She did not report any fever, seizures, neck stiffness, or
weight loss. Except for the history of recurrent CVTs, the patient did
not have any specific diseases. She had stopped using her drugs
individualistically four months ago due to the improvement of her
symptoms.
The patient had a smoke-free life and never used illicit drugs. Also,
there was no history of drugs that induced a hyper coagulated state
(e.g., Oral Contraceptive Pill or OCPs). On physical examination, the
vital sign was stable (Blood Pressure:133/78 mmHg, Pulse Rate: 92
pr/min, Respiratory Rate: 18 pr/min, Temperature: 37.6 C), and the
mental status was not impaired (Glasgow Coma Scale:15/15). An
ophthalmometer established a 5mm protrusion from the temporal orbital
rim in the right eye. On ophthalmoscopy, bilateral papilledema was
detected. Except for a right sixth nerve palsy, other cranial nerve
examinations were not involved. Sensory and motor examinations were
unremarkable, the muscle tones were normal, and the forces were 5/5.
Deep Tendon Reflexes were not impaired, and the plantar reflexes were
downward. Also, the patient had a normal gait.
Brain Magnetic Resonance Imaging (MRI), Magnetic Resonance angiography
(MRA), Magnetic Resonance Venography (MRV), and a Computed Tomography
(CT) scan was performed. The T1 weighted MRI with
Gadolinium contrast demonstrated diffuse pachymeningeal thickening
(Figure 1A, B) and an enhanced
intra-orbital mass measuring 9*15*24mm, which seems to be originated
from right lateral and superior rectus muscles (Figure
2A, B) . Compared to the previous neuroimaging of the
patient admissions in 2018 and 2020, this mass was defined as a new
finding. MRV revealed CVT within the left transverse sinuses and
superior sagittal sinus (Figure 3) . In addition to
confirming the intra-orbital mass presence and size, Orbit MRI disclosed
the tortuous appearance of both optic nerves with dilation in nerve
sheets and superior ophthalmic veins were dilated. Laboratory test
findings were unremarkable, except for hemoglobulin (Hgb:11.9 g/dl),
high serum C-reactive protein (CRP:52 mg/L), Erythrocyte Sedimentation
Rate (ESR:76mm/h), serum IgG (34,6 g/L;normal range:7-16) and serum IgG4
(141 mg/dl) was above the upper limit of the normal range (4–90 mg/dl).
Other specific serum rheumatological or vasculitis markers include
P-ANCA, C-ANCA, ANA, Anti-dsDNA, anti-Smith, Anti-RNP, anticardiolipin
antibodies, β2-glycoprotein, C3, C4, RF, Anti-CCP, Anti-SSA/ Ro,
Anti-SSB/ La were normal in the results. In addition, Acquired
and genetic thrombophilia tests findings (e.g., protein C deficiency,
protein S deficiency, Antithrombin III deficiency, Factor V Leiden
mutation, Homocysteine level, and Prothrombin 20210 mutation) were
negative. The tumor marker assessment demonstrated negative results for
CA5.3, CA19.9, CA125, α-fetoprotein (α-FP), and carcinoembryonic antigen
(CEA)). Lumbar puncture (LP) indicated a low elevation in opening
pressure (30 cm H2O), absence of WBC and RBC, a normal
protein level (33 mg/dl), and an average glucose level (63 mg/dl). CSF
isoelectric focusing showed an oligoclonal IgG banding pattern. There
was no evidence of bacterial growth in CSF gram staining and culture. A
surgical biopsy from the intra-orbital mass was accomplished. In the
pathology report, microscopic examination of the specimen revealed
fragments of fibroconnective tissue containing inflammatory cells
infiltration, composed mostly lymphoplasmacytic admixed with histiocytes
and polymorphonuclears (PMNs). Foci of necrosis fibrosis and lymphoid
follicles formation were also seen. Special stainings for acid-fast
bacilli and fungi were negative. Immunohistochemistry showed
approximately 60 IgG4-positive plasma cells per high-power field (HPF),
of which almost more than 40 of the IgG+ plasma cells were IgG4+. Also,
the IgG4+/IgG+ plasma cell ratio was more than 40%(Figure
4) . For other organ work-ups, CT-scan from chest and MRI from abdomen
and pelvic were conducted, but they didn’t have any specific findings.
The patient received Prednisolone (1 mg/kg/day) for three months and a
5mg daily dose of Warfarin. She felt a rapid improvement after
initiating the treatment. Azathioprine (2 mg/kg/day) was also prescribed
without any interval. After eight months, all neurological symptoms had
resolved.