Case report
We describe a case of a 25-year-old South Asian gentleman with no past
medical history who suffered a seizure during an air flight and brought
to the hospital. The event started with jerky movements on the left side
of his body and numbness followed by secondarily generalized
tonic-clonic seizure (GTCs)and loss of consciousness lasting for a few
minutes. According to the patient, these attacks started one year ago,
with the last episode being two months prior. He also had a mild
nonspecific headache with right frontal swelling for the last two months
without any fever, cough, weight loss, and night sweats. There was no
history of focal weakness, vision problems, or balance problems. He did
not give a history of any myalgias, joint pain, dry eyes or mouth, skin
rash, or oral ulcers. A review of the system was negative for any
respiratory, gastrointestinal, and urinary symptoms. He did not take any
medical treatment for these events. He was a nonsmoker, nonalcoholic,
and denied any use of illicit recreational drugs. He was unmarried, and
his family history was negative for epilepsy or any other neurological
disease. He works as a cobbler in a leather factory. There is no sick
contact history.
He was vitally stable. His neurological and systemic exam was
unremarkable. He was started on phenytoin as an anti-seizure agent.
Initial blood work including complete blood count, coagulation profile,
blood chemistry, and renal and liver function tests did not show any
significant abnormality. His chest x-ray showed hazy ill-defined shadows
present in the right upper zone and to a lesser extent at the left upper
zone and the right base. CT scan head non-contrast showed right side
extra-axial hyperdensity along frontoparietal convexity of 12 to 13 mm
in maximum thickness with effacement of the adjacent sulci and 5 mm
midline shift to the left (fig1-a, b ) and was reported as
Subdural hematoma. However, there was no history of any trauma or
bleeding disorder. An osteolytic lesion in the right frontal bone
(fig1-b, c ). There was no history of trauma and no clinical
signs or laboratory abnormality indicative of bleeding diathesis for
spontaneous subdural bleeding. MRI of the head with contrast was done
after admission and the results showed: a right side intracranial
extra-axial hemispheric crescentic shaped mass lesion showing low signal
in T2, FLAIR, and T1 sequences with intense postcontrast enhancement and
underlying dural involvement having a maximum thickness of 13 mm,
extending along the surface of the right cerebral hemisphere more at the
higher cuts, reaching the ventral part of the interhemispheric fissure
with mild left frontal dural enhancement as well. There was the
associated irregularity of the adjacent inner skull table with right
frontal focal full-thickness bony defect, underlying diploic and
extracranial enhancing swelling as well as right frontal bone marrow
signal abnormality bright in T2. Partial effacement of the right
cerebral frontoparietal cortical sulci with mild compression of the
right lateral ventricle and midline shift to the left side of about 5 mm
was seen (fig2-a-e ).
The working differential diagnosis at the stage based on the clinical
and imaging findings was an infective process (TB, fungal infections),
neoplastic lesion (carcinomatosis, lymphoma, leukemia), histiocytosis,
and inflammatory granulomatous processes like Sarcoidosis and Wegner
granulomatous. Further work-up was tailored accordingly based on the
differential. CT scan of chest, abdomen, and pelvis showed multiple
enhancing pulmonary nodules of both lungs, in the upper lung lobe with
no other abnormality in abdomen and pelvis (fig3-a, c ).
Erythrocyte sedimentation rate was high 67 (reference 5 to 28), C -
reactive protein (CRP)
129 (reference 1to 5), procalcitonin was normal whilst TB PCR was
negative. The serum and urine protein electrophoresis were unremarkable.
The flow cytometry indicated no definitive immunophenotypic evidence of
a monotypic B-cell population. ANA and ANCA were negative.
He underwent right side craniotomy and had a right frontal skull
osteolytic lesion with dural thickening. Resection of the thickened
dural area and repair of skull defect were done. A Frozen Section
Diagnosis of the skull lesion with intracranial subdural collection
showed aggregates of chronic inflammatory cells, plasma cells, and
lymphocytes, while that of thickened dura had dense fibrous tissue with
a focal aggregate of chronic inflammatory cells. Histopathology Sections
show sclerosed fibrous tissue in storiform pattern and patchy chronic
inflammatory cell infiltrate, including lymphocytes, plasma cells, and
few histiocytes and eosinophils. Few neutrophils were also noted. The
inflammatory cells predominantly aggregated around blood vessels with no
granuloma, necrosis, or atypia. Ancillary Immunohistochemistry studies
with appropriate controls show the following results: CD138: Positive
and highlighting an enormous number of plasma cells. IgG: Positive in
plasma cells. IgG4: Positive in plasma cells (more than 10 positive
cells/HPF). Kappa and Lambda: Polyclonal. Alk-1: Negative. S-100:
Negative. Based on these findings definite diagnosis was an Inflammatory
sclerosing pseudo tumoral lesion compatible with IgG4-related meningeal
disease. Histopathology from CT-guided right lung biopsy was also
consistent with Ig4 related disease.
Serum immunoglobulin measurement was positive for elevation in total IgE
with a level 263 k units/L (reference range between 0 and 114) and in
IgG4 (223mg/dL with a reference range of 3–201). C3 and C4 194 and 193
were also elevated. Final cultures were negative for mycobacterium,
fungal, and any other bacterial organism.
During his hospital stay, our patient had experienced episodes of sudden
left side jerky movement with facial twitches followed by loss of
consciousness. Levetiracetam was added. After the diagnosis of IG4
related disease, He was treated on oral steroids 60mg OD dose and IV 1g
rituximab dose was given to be followed by 2nd dose in
14 days in his home country. On discharge, azathioprine 100 mg OD was
also prescribed He did not have further GTCs but reported episodic mild
numbness of the left side with minor jerky movements. Inflammatory
markers show CRP decrease to 53. He was repatriated to his home country
as he was a transit passenger.