Roth Spot and a Homonymous Hemianopsia as the Presenting Manifestation of Polyarteritis Nodosa

Clinical Correspondence Section Editors: Robert Avery, DO Karl C. Golnik, MD Caroline Froment, MD, PhD An-Guor Wang, MD Roth Spot and a Homonymous Hemianopsia as the Presenting Manifestation of Polyarteritis Nodosa Arjun V. Jogimahanti, BS, Subahari Raviskanthan, MBBS, Peter W. Mortensen, MD, Sarah A. Kazzaz, MD, Andrew G. Lee, MD T he etiology of stroke in young patients differs substantially from older patients whose strokes are typically carotid stenosis or atrial fibrillation related. Young patients with stroke require assessment for hypercoagulability, alternate cardioembolic sources including patent foramen ovale, and vasculitis. The white-centered hemorrhage (Roth spot) is an interesting but nonsensitive ophthalmoscopic finding. We present a case of a patient who presented with a Roth spot and left homonymous hemianopsia secondary to polyarteritis nodosa (PAN). To the best of our knowledge, this is the first such case to be reported in the English language ophthalmic literature. A 41-year-old man presented with acute right arm and leg weakness. His medical history was significant for a vasectomy .10 years before. He took no medications. He previously smoked, drank alcohol, and used nonintravenous recreational drugs, which he stopped 18 years before. During this first outside hospital admission, his blood pressure peaked at 246/145 mm Hg. His other vital signs were normal. Electrocardiogram showed sinus rhythm. Random blood glucose was .300 mg/dL. MRI of the brain revealed acute left basal ganglia, left occipital lobe, and right centrum semiovale infarcts. Workup for underlying etiology of the stroke was unremarkable. His symptoms resolved, and he was dis- University of Texas Medical School at Houston (AJ), Houston, Texas; Department of Ophthalmology (SR, PWM, AGL), Blanton Eye Institute, Houston Methodist Hospital, Houston, Texas; Department of Rheumatology (SAK), Houston Methodist Hospital, Houston, Texas; Departments of Ophthalmology, Neurology, and Neurosurgery (AGL), Weill Cornell Medicine, New York, New York; University of Texas MD Anderson Cancer Center (AGL), Houston, Texas; Department of Ophthalmology (AGL), University of Texas Medical Branch, Galveston, Texas; Texas A and M College of Medicine (AGL), Bryan, Texas; and Department of Ophthalmology (AGL), The University of Iowa Hospitals and Clinics, Iowa City, Iowa. The authors report no conflicts of interest. All named authors meet the International Committee of Medical Journal Editors (ICMJE) criteria for authorship for this article, take responsibility for the integrity of the work as a whole, and have given their approval for this version to be published. Address correspondence to Andrew G. Lee, MD, Department of Ophthalmology, Blanton Eye Institute Houston Methodist Hospital, 6560 Fannin Street, Suite 450, Houston, TX 77030; E-mail: aglee@ houstonmethodist.org e482 charged home on aspirin, clopidogrel, and atorvastatin, with cardiovascular risk factor management education. The patient re-presented 2 weeks after exhibiting acute left-sided weakness, homonymous hemianopia, and facial droop. Repeat MRI of the brain revealed multiple new acute infarcts in the right thalamus, lateral geniculate body, pons, and cerebellar vermis (Fig. 1). An external optometrist noted a Roth spot in the right eye on further evaluation, and the patient was referred to the Blanton Eye Institute, Houston Methodist Hospital. Initial neuro-ophthalmic examination 2 months after his first presentation revealed visual acuity of 20/20 in the right eye and 20/25 in the left eye. His pupils were isocoric with no relative afferent pupillary defect. He had a dense left homonymous hemianopsia. Dilated fundus examination revealed the Roth spot in the right eye and multifocal cotton wool patches in both eyes (Fig. 2). A splinter hemorrhage was noted on the middle fingernail of the right hand. Given the Roth spot and homonymous hemianopsia, the patient was readmitted for workup of possible subacute bacterial endocarditis (SBE) to the Houston Methodist Hospital. Erythrocyte sedimentation rate (ESR) was normal at 17 mm/hour, and laboratory screening results for infection, vasculitis, and hypercoagulability were negative. Repeat stroke workup was otherwise unremarkable. Given the multifocal recurrent strokes, and despite the normal ESR, the patient underwent digital subtraction cerebral angiography, which was consistent with cerebral vasculitis. Further rheumatology evaluation revealed multiple features consistent with the diagnosis of PAN, including headaches, epididymo-orchitis, skin lesions suggestive of leukocytoclastic vasculitis, 70-pound unintentional weight loss, weakness not consistent with his stroke distribution, and axonal polyneuropathy on nerve conduction studies. The patient met 6 of the 10 American College of Rheumatology (ACR) 1990 diagnostic criteria for PAN and was treated with 3 days of intravenous methylprednisolone, followed by oral prednisone and concurrent intravenous cyclophosphamide and had marked improvement in his symptoms after treatment commencement (1). Polyarteritis nodosa is a systemic vasculitis that most commonly presents in men in their fifties (2). It affects Jogimahanti et al: J Neuro-Ophthalmol 2022; 42: e482-e484 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence FIG. 1. MRI diffusion-weighted imaging sequences highlighting areas of diffusion restriction (arrows) across multiple bilateral deep vascular territories. medium-sized blood vessels (2). Polyarteritis nodosa is rare, with a prevalence of 31 cases per 1,000,000 adults (2). Unlike other ANCA-related vasculitides, the diagnosis of PAN is not typically made on serology but requires at least 3 of the 10 criteria proposed by the ACR. These include musculoskeletal (myalgias and tenderness), renal (renal failure typically without hematuria), neurological (mono/ polyneuropathy), angiographic vasculitis, gastrointestinal, FIG. 2. Fundus photographs showing (A), Roth spot in the right eye (blue circle) with multiple cotton wool patches and (B), multiple cotton wool patches in the left eye. Jogimahanti et al: J Neuro-Ophthalmol 2022; 42: e482-e484 e483 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence and dermatological (livedo reticularis) (1). P-ANCA may also be positive but is not in the diagnostic criteria (2). The criteria’s sensitivity and specificity for PAN is 82 and 87%, respectively (2). The prognosis of untreated PAN is poor with an average 5-year survival rate of 13% but increases 6-fold with treatment (2). Hepatitis B or having recurrent flares portend a poorer prognosis (2). The finding of a Roth spot, low-grade fevers, leukocytosis, and a drug use history led to multiple cardioembolic stroke workups for this patient. Roth spots, which are white fibrin-centered retinal hemorrhages, are ocular manifestations of many systemic conditions (3). It is presumed that endothelial dysfunction causes rupture of retinal capillaries, leading to intraretinal hemorrhage and this characteristic appearance (3). Histologically they contain platelet and fibrin thrombus (3). The differential diagnosis for Roth spot is broad, but it can occur in any condition causing endothelial dysfunction. Classical teaching associates Roth spots with SBE but other causes include vasculitis from any etiology, sepsis, hypertension, infections including HIV, blood dyscrasias (anemia, leukemia, and thrombocytopenia), and trauma (3). Roth spots are not sensitive for SBE and are found in less than 5% of SBE cases (5). To the best of our knowledge, this is the first case to be reported in the English language ophthalmic literature of PAN presenting with a Roth spot and homonymous hemianopsia. Clinicians should note that although the e484 Roth spot can be suggestive of SBE, it is not pathognomonic and alternative etiologies including vasculitis (i.e., PAN) should be considered in the differential diagnosis. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: A. Jogimahanti, S. Raviskanthan, P. Mortensen, S. A. Kazzaz, and A. G. Lee; b. Acquisition of data: A. Jogimahanti, S. Raviskanthan, P. Mortensen, S. A. Kazzaz, and A. G. Lee; c. Analysis and interpretation of data: A. Jogimahanti, S. Raviskanthan, P. Mortensen, S. A. Kazzaz, and A. G. Lee. Category 2: a. Drafting the manuscript: A. Jogimahanti, S. Raviskanthan, P. Mortensen, S. A. Kazzaz, and A. G. Lee; b. Revising it for intellectual content: A. Jogimahanti, S. Raviskanthan, P. Mortensen, S. A. Kazzaz, and A. G. Lee. Category 3: a. Final approval of the completed manuscript: A. Jogimahanti, S. Raviskanthan, P. Mortensen, S. A. Kazzaz, and A. G. Lee. REFERENCES 1. 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