LETTERS TO THE EDITOR
|Year : 2020 | Volume
| Issue : 4 | Page : 563-566
Young male with paraparesis and vision loss- A rare presentation of Eales' Disease
Tanushree Chawla, Anu Gupta, Kamakshi Dhamija, Debashish Chowdhury
Department of Neurology, Govind Ballabh Pant Institute of Post Graduate Medical Education and Research (GIPMER), New Delhi, India
|Date of Submission||09-Nov-2019|
|Date of Decision||19-Nov-2019|
|Date of Acceptance||15-Dec-2019|
|Date of Web Publication||29-Jun-2020|
Department of Neurology, Govind Ballabh Pant Institute of Post Graduate Medical Education and Research (GIPMER), New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Chawla T, Gupta A, Dhamija K, Chowdhury D. Young male with paraparesis and vision loss- A rare presentation of Eales' Disease. Ann Indian Acad Neurol 2020;23:563-6
|How to cite this URL:|
Chawla T, Gupta A, Dhamija K, Chowdhury D. Young male with paraparesis and vision loss- A rare presentation of Eales' Disease. Ann Indian Acad Neurol [serial online] 2020 [cited 2020 Sep 21];23:563-6. Available from: http://www.annalsofian.org/text.asp?2020/23/4/563/288778
Eales' disease, first described by British ophthalmologist, Sir Henry Eales, in 1882 is an idiopathic obliterative retinal peri-phlebitis, occurring in young adult males with a mean age of onset of 20-30 years (age range 11-40 years). It is more commonly seen in the Indian subcontinent (frequency of 1 in 135-200 ophthalmic patients reported from a referral eye hospital in India).
It starts as an active perivenular inflammation associated with retinal hemorrhages, and hemorrhagic infarctions of the retina, in multiple quadrants, starting at or anterior to the equator and progressing posteriorly. It may also be associated with arteritis, papillitis or uveitis. Recurrent vitreous hemorrhage, tractional retinal detachment, rubeosis iridis, and neovascular glaucoma are its sequelae. Bilateral eye involvement, simultaneous or sequential, may be seen in 70-80% of the cases. The pathophysiology is hitherto unknown, but hypersensitivity to tuberculin protein has been postulated based on epiretinal membrane polymerase chain reaction (PCR) studies. The role of various proteins, retinal autoimmunity, HLA class I and II, oxidative stress, VEGF overexpression has also been studied.
Various neurological manifestations have been described in Eales' disease including subacute myelopathy, strokes, seizures and nonspecific white matter changes.,,,, They can present within few days to several years after the onset of the disease and may precede the diagnosis in a few.
We report a 23-year-old male who presented with insidious onset, progressive, asymmetric spastic paraparesis, gait imbalance and bladder dysfunction, in the form of frequency, urgency and urge incontinence of 3 months duration.
A month later, he developed floaters in the right eye which gradually progressed to complete visual blurring in the right eye. It was not associated with painful eye movements or local signs of irritation. He did not report any neck or back pain, fever, cough, breathlessness, loss of weight or appetite, features of connective tissue disease, joint pain, joint swelling, skin rash, oral and genital ulcers, or a high risk behavior. His past and family history was unremarkable.
Examination revealed reduced visual acuity in right eye, (finger counting at 3 meters), hazy media, and a normal pupillary light reflex. He had asymmetric spastic paraparesis (Medical research council grade 4- to 4 in left lower limb, grade 4+ in right lower limb), with brisk deep tendon jerks and extensor plantar response. Joint position and vibration sense were impaired in both lower limbs with a positive Romberg's sign.
On indirect ophthalmoscopy (IO) perivascular hemorrhages [Figure 1]a and perivascular sheathing [Figure 1]b involving both arteries and veins, hard exudates [Figure 1]b and decreased perfusion of the superior and inferotemporal quadrants [Figure 1]a and [Figure 1]b, were seen which was suggestive of retinal vasculitis. Fundus fluorescein angiography (FFA) demonstrated abrupt cut off of vessels involving both arteries and veins, areas of ischemia and cuffing of vessels, suggesting perivascular inflammation [Figure 1]c. Contrast enhanced magnetic resonance imaging (CEMRI) of dorsolumbar spine revealed two non-enhancing hyperintensities (HI) with cord edema extending from D9 to D12 and involving the conus with more than 2/3rd intramedullary involvement [Figure 1]d and [Figure 1]e.
|Figure 1: Panel (a), (b) Indirect ophthalmoscopy showing perivascular hemorrhages (black arrow head), perivascular sheathing (dashed black arrow), Hard exudates in the macular area (white arrow) and decreased perfusion in the superior and inferotemporal quadrant (black arrow); Panel c- Flourescein angiography demonstrating abrupt cut off of vessels (curved white arrow), area of retinal ischemia (white arrow head) and dye leakage (dashed white arrow); Panel (d), (e)- MRI dorsolumbar spine (T2 Weighted image) showing two long segment cord hyperintensity extending from D9-D12 and involving conus (dashed white arrows), cross section showing more than 2/3rd cord involvement; Panel (f, g) – MRI Brain (T2 Fluid attenuated inversion recovery image) depicting periventricular hyperintensity (f) and periependymal hyperintensity around the fourth ventricle (g) (black arrow)|
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Patient was investigated further keeping in mind differentials of Longitudinally extensive transverse myelitis (LETM) [Table 1] with retinal vasculitis [Table 2].
|Table 1: Differential Diagnosis of Longitudinally extensive transverse myelitis|
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|Table 2: Differential diagnosis of fundus findings of retinal vasculitis|
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Serum investigations are summarized in [Table 3]. Cerebrospinal fluid (CSF) was acellular, with 62.4 mg/dl protein and normal CSF: Plasma glucose ratio (0.53). Mantoux was positive (11 mm). Contrast enhanced computed tomography of chest and abdomen was normal. Pathergy was negative. He had positive proliferating cell nuclear antigen (PCNA) and PM-Scl antibodies. *PCNA is detected in low frequency in SLE  and PM-Scl is found in overlap syndromes. Clinically he did not have an overlap syndrome. Although Lyme's antibody titre, serology for rickettsial diseases and PCR for pathogens in ocular specimens, were not done, in the absence of rash and retinal infiltrates or necrosis, clinically these possibilities were less likely.
Brain CEMRI revealed white matter hyperintensities along fourth ventricle and the posterior horn of the lateral ventricle [Figure 1]f and g]. Serum and CSF was negative for anti-aquaporin 4 antibodies and our patient did not fulfill the international consensus diagnostic criteria for Neuromyelitis optica spectrum disorders. The pattern of cord and brain involvement with presence of hemorrhages in the fundus and absence of optic disc pallor did not favor a diagnosis of Multiple Sclerosis. Conus involvement in young males can occur in MOG-related diseases, but retinal vasculitis is not a feature of MOG. Susac syndrome characterized by encephalopathy, hearing loss and vision loss is a close differential of Eales disease but it shows branched retinal artery occlusion whereas Eales shows predilection for retinal veins, which was seen in our case.
With a negative work up for various etiologies of LETM and retinal vasculitis, Eales' disease was considered as the probable diagnosis, and the patient given a 5-day course of 1 gram intravenous methylprednisolone, followed by slow oral taper of prednisone over 6 months. He also underwent laser photocoagulation in the right eye twice. At one year follow-up, his visual aquity was 6/12 in the affected eye and his power in his both lower limbs was MRC grade 5/5 and he was off corticosteroids.
Neurological involvement in Eales' disease was first reported by Silverskoid in 1947 in three patients who presented with fairly rapidly progressive myelopathy along with pleocytosis in acute phase of Eales' disease. White  and Singhal and Dastur  have also reported neurological involvement in Eales' disease in their respective case series.
The development of myelopathy was found to be acute to subacute and dorsal spine was the most common site of involvement. Not much is known about the pathological findings in Eales'. Singhal and Dastur  performed autopsy of one of their cases and postulated that Eales' is a syndrome of “vasculopathy with episodic demyelinating retino-encephalo-myelopathy”, which develops as a result of hypersensitivity of retinal tissues to an unknown infective agent.
To conclude, Eales' is an uncommon, idiopathic ophthalmological disease, and an important treatable cause of myelopathy with retinitis. Simultaneously, bearing in mind that it is a diagnosis of exclusion, an exhaustive workup is prerequisite before clinching on to its diagnosis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Table 1], [Table 2], [Table 3]