Optic Neuropathy Secondary to Polyarteritis Nodosa, Case Report, and Diagnostic Challenges

Abstract

PurposeTo describe a case of optic neuropathy as a primary manifestation of polyarteritis nodosa (PAN) and discuss diagnostic challenges.MethodsCase report.ResultsA 41-year-old Hispanic man presented with a 2-day history of reduced visual acuity in his left eye. Physical examination revealed a complete visual field loss in the affected eye. Best-corrected visual acuity (BCVA) in the left eye was hand motion, and fundus examination revealed a hyperemic optic disk with blurred margins, swelling, retinal folds, dilated veins, and normal size arteries. BCVA in the right eye was 20/20; no anomalies were seen during examination of the fundus. The patient was started on oral corticosteroids and once the diagnosis of PAN was made, cyclophosphamide was added to the treatment regimen. Six months later, the patient recovered his BCVA to 20/20 in his left eye.ConclusionRarely does optic neuropathy present as a primary manifestation of PAN; nevertheless, it represents an ophthalmologic emergency that requires expeditious anti-inflammatory and immunosuppressive treatment to decrease the probability of permanent visual damage. Unfortunately, diagnosing PAN is challenging as it necessitates a high index of suspicion. In young male patients who present for the first time with diminished visual acuity, ophthalmologists become cornerstones in the suspicion of this diagnosis and should be responsible for continuing the study until a diagnosis is reached to ensure rapid commencement of immunosuppressive treatment.

Neurological Involvement in Primary Systemic Vasculitis

“…In addition, patients with PAN may also develop pachymeningitis (43) and present with headache or cranial nerve palsy. Sudden bilateral hearing loss (44) and visual alterations caused by optic neuropathy (45) have also been reported.…”

Oculomotor nerve palsy, an unusual onset of polyarteritis nodosa

“…Cranial nerve involvement in PAN is a rare manifestation, being less frequent than the central nervous sys-tem involvement and peripheral neuropathy. According to our literature review, the most frequent cranial nerves involved in PAN are the optic (II), oculomotor (III) and abducent (IV) [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21]. We did not find any case report with involvement of olfactory, glossopharyngeal, vagus, accessory or hypoglossal nerves in patients with PAN.…”

“…1). Each case is described in Table I [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21]. In 10 of the 16 patients, cranial neu-ropathy was the initial manifestation, as in our case.…”

“…Treatment of cranial involvement in PAN without hepatitis B infection included glucocorticosteroids, as first-line treatment in monotherapy to rapidly control inflammation. Iimmunosuppressive agents, such as cyclophosphamide or AZT with combination with GCs may be a second line treatment [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21]. The described patient responded very well to intravenous methylprednisolone pulse therapy, followed by oral prednisolone and AZT, remaining in sustained remission with AZT in monotherapy.…”

Polyarteritis nodosa presenting with posterior reversible leukoencephalopathy syndrome

“…Finally, there are a few case reports in the literature proposing other CNS manifestations of PAN, but these associations are not confirmed and there is diagnostic doubt around PAN in all. These include a case of bilateral optic neuropathy,22 a patient presenting with pachymeningitis23 and a woman in her 30s with a 20-year history in keeping with clinical and radiological appearances of relapsing-remitting multiple sclerosis 24…”