Sex: F
Vaccine: FLU4, FLUX
Symptoms: Biopsy brain abnormal, Blindness, Blood immunoglobulin G increased, Borrelia test negative, Brucella test negative
Clinical narrative: This literature report was received from Physician on 04-Sep-2017. This case refers to a 23-year-old female patient. The patient had no recent history of febrile illness or tick exposure. On an unspecified date, the patient was administered with influenza vaccine (dose, batch number, manufacturer, trade name, route of administration, anatomical location: not reported). On an unspecified date, after 2 weeks of vaccination, the patient presented to the neuro-ophthalmology clinic with a 5-day history of right frontal headache and a 3-day history of blurred vision and pain on eye movement of the right eye. It was reported that, she had no other constitutional symptoms such as skin rash, fever and muscle aches. The patient's initial ophthalmologic examination of the right eye showed reduced visual acuity (20/40), dyschromatopsia (4/10 on Hardy-Rand-Rittler (HRR) test), a visual field defect (constriction of peripheral visual field) on Humphrey automated perimetry, a relative afferent pupillary defect, and optic disc swelling suggestive of optic neuritis. An MRI of the brain and orbits with and without gadolinium showed enhancement of the right retrobulbar optic nerve and right hemispheric leptomeninges. Her serologic studies showed increased erythrocyte sedimentation rate (37 mm/h) and C-reactive protein (1.7 mg/L), mildly elevated immunoglobulin G4 (IgG4) (112.7 mg/dL) and low vitamin D. However, antinuclear antibody, antineutrophil cytoplasmic antibody, anti-Ro/La, C3/4, aquaporin 4 (AQ4) antibody, angiotensin-converting enzyme (ACE), Lyme, syphilis, human  immunodeficiency virus (HIV), Bartonella, Brucella, and quantiferon gold were negative. Vitamins B1, B6, B12, and E levels were normal. Opening pressure on lumbar puncture was 23 cm H2O, and cerebrospinal  fluid (CSF) showed leucocytosis (45/uL; mononuclear dominant). The patient's protein and glucose levels were normal, no oligoclonal IgG, negative tests for cytomegalovirus, Epstein-Barr virus, herpes simplex virus, and varicella-zoster virus, and negative cultures for Mycobacterium and other fungi. Chest computed tomography was normal except for a small thyroid nodule and positron emission tomography (PET) scan showed no lesion outside the brain and orbit. On an unspecified date, about 3 weeks after the initial visit, the patient developed mild pain and blurred vision in the fellow (left) eye and repeat brain MRI with and without gadolinium showed new abnormal enhancement of the left optic nerve which atypical presentation of the inflammatory optic neuritis and the involvement of left eye. Meningeal and brain biopsies with craniotomy from the right frontal lobe, which showed reactive astrogliosis with lymphocytic infiltration without evidence of neoplasm, granulomas, or vasculitis. Her vision started improving at hospital day 4 and had improved to 20/20 in both eyes by the time of discharge. The patient deferred intravenous corticosteroid therapy and received oral corticosteroid for 14 days with tapering. It was reported that, a week after completing the course of oral corticosteroids, she experienced worsening of the vision in her right eye (counting fingers at 3 ft.). The patient's repeat brain and spinal MRI showed enhancement of the right optic nerve. The repeated rheumatologic work-up, infectious disease work-up, CSF analysis, and paraneoplastic antibody assay, including repeated AQ4 antibody, were unremarkable or negative. The patient received a 3-day course of intravenous corticosteroid, with minimal improvement of her vision, and then received 5-day plasmapheresis. The patient's right eye vision was recovered to 20/25 in 2 weeks after the completion of the treatment and in 3 weeks after recurrence of vision loss. The reporter stated that, the concept of an immune-mediated attack against optic nerves after vaccination in genetically susceptible individuals does exist, and the possible link should be considered. Here we present a case of optic neuritis that could be linked to alteration of immune system by influenza vaccination. The exact pathophysiologic mechanism of post vaccination optic neuritis is not clearly defined. It could be triggered by a common denominator such as adjuvant14 used in the vaccine to enhance the antigen-specific immune response, which perhaps induces the release of inflammatory cytokines and interacts with Toll-like receptors and NLRP (NOD like receptor protein) inflammasomes. The existence of vaccine-associated optic neuritis is essentially based on the temporal relationship between the administration of a vaccine and the development of optic neuritis in patients with no other known aetiologies for infectious or non-infectious inflammation that could account for the optic neuritis. Influenza vaccine (inactivated or live attenuated) is considered to be one of vaccines that could be related to optic neuritis.