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Eales' disease presenting as stroke in the young adult.

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BRIEF COMMUNICATIONS
Eales’ Disease
Presenting As Stroke
in the Young Adult
Mark Forrest Gordon, MD,” P. K. Coyle, MD,*
and Harry Golub, M I 3
Eales’ disease is a n uncommon idiopathic disorder characterized by retinal perivasculitis and recurrent vitreous
hemorrhages in young males. Associated neurological
involvement is rare. We report a 38-year-old man w h o
presented with stroke attributed to Eales’ disease.
Gordon MF, Coylc PK, Golub €3. Eales’ disease
presenting as stroke in the young adult.
Ann Neurol 1988;24:264-266
Eales’ disease was first described in 1882 as “retinal
hemorrhage associated with epistaxis and constipation”
seen in young m e n in southern England El]. This unc o m m o n idiopathic ophthalmological disorder affects
young males preferentially and is characterized by retinal perivasculiris with recurrent episodes of retinal and
vitreous hemorrhage. Neurological involvement is
rarely seen “1. We report a 38-year-old man who
p r e s e n t d with stroke most likely d u e to Eales’ disease.
Case Report
A 38-year-old right-handed man was transferred from an
outlying hospital to University Hospital at Stony Brook after
the sudden onset of right body hemiparesis and dysarthria.
He had no associated visual or sensory changes, headache,
dysphasia, o r seizure activity. Past medical history was notable for an acute decrease in the vision in his left eye 6
months earlier, hypertension for 5 years under good control
with chlorthalitlone and metoprolol tartrate, and alcohol and
cigarette use for 20 years. He denied illicit drug use, transfusions, or previous surgery.
General physical examination showed absence of carotid
bruits and cardiac. murmurs or rhythm irregulariries. There
was mild dysarthria but no dysphasia. Visual acuity was 20140
OD and 201400 0s with a left afferent pupillary defect.
Funduscopic examination showed a vitreous hemorrhage
that precluded visualization of the left fundus. I n the right
eye, the vitreous was clear; the disc and macula were normal.
Multiple peripheral retinal venous occlusions were present,
some of which were bridged by telangiectatic collateral vessels. Retinal neovascularization was present in the mid-
From the Departmcnts of ‘Neurology and ?Ophthalmology, State
University of New York ar Stony Brook, Stony Brook, N Y
Received O c r 1i. 1087, and
for public,ttion Feb i, 1988
111
revised form Feb 2, 1988 Accepted
Address correspondence to Dr Gordon, Department of Neurology,
Health Sciences Center, T-12, State University of New York, Stony
Brook, NY I 17‘)1
periphery, distal to the venous occlusions. There was mild
left central facial weakness. Motor examination showed mild
increase in tone with 5 - out of 5 (British Medical Research
Council scale) weakness on the right side. A metabolic
tremor was present in both hands. Primary and secondary
sensory modalities were intact, except for mild symmetrical
decrease to vibration in the legs. Reflexes were 3 + throughout with positive Hoffmann’s sign but no ankle clonus, and
bilateral flexor plantar responses. Cerebellar testing, stance,
and gait were normal except for decreased right arm swing.
Laboratory evaluation showed elevated platelets at
46 1,000. Otherwise studies were normal, including serum
electrolytes, hemarocrit, leukocyte count, erythrocyte sedimentation rate, prothrombin and partial thromboplastin
times, bleeding time, angiotensin converting enzyme,
VDRL, FTA-antibody, hemoglobin electrophoresis, Lyme titer, cryoglobulins, antinuclear antibody, rheumatoid Factor,
Vitamin BIZ,folate, immune complexes, hepatitis B screen,
Monospot test, Brucella antibody, and serum and urine protein electrophoreses. C3 complement level was slightly low
but C4 was normal. Chest radiograph, electrocardiogram,
echocardiogram, and Holter monitor were normal. Lumbar
puncture yielded entirely normal cerebrospinal fluid (CSF).
Computed tomographic scan of the head with and without
contrast showed focal hypodensities (consistent with old irifarctions) in the left anterior thalamic and right basal ganglta
regions (Fig 1). Purified protein derivative (PPD) and anergy
panel showed a normal response to mumps antigen with no
reaction to PPD. Electroencephalogram, brainstem auditory
evoked response, and visual evoked response were normal
except for a conduction delay in the left eye due to the
vitreous hemorrhage.
Angiography demonstrated occlusion of the left middle
cerebral artery; the sylvian triangle was filled by collaterals
from the left anterior and posterior cerebral arteries. Both
carotid bifurcations were normal. The caliber of the intracranial vessels appeared to be normal except for a few branches
arising from the left posterior cerebral artery (Fig 2).
Fluorescein angiography of the right eye demonstrated
areas of venous occlusion with telangiectatic collaterals, focal
areas of vascular staining consisrent with ischemia or inflammation, and fluorescein leakage consistent with retinal
neovascularization (Fig 3).
The patient’s vitreous hemorrhage resolved gradually with
a concurrent improvement in vision. No steroid medication
or photocoagulation therapy was given. He had no further
vitreous hemorrhage over the next several months.
Discussion
Eales’ disease (also known as retinal periphlebitis, retinal perivasculitis, and angiopathia retinae juvenilis) is a
disorder best known t o ophthalmologists. Y o u n g adult
males between the ages of 20 and 30 are t h e primary
target, with a 4 to 1 male t o female ratio. Patients
present with s u d d e n monocular visual loss due to vitreous hemorrhage. Although ocular involvement is typically unilateral at onset, ultimately both eyes are involved. The disease persists for some time, with
recurrent hemorrhages over months to years, but recovery of vision is generally good and ultimately the
264 Copyright 0 1988 by the American Neurological Association
Fig 1 . Postcontrast head computed tornograph demonstrated hypodensities in the right basal ganglia and left anterior thalamus
(arrowhead) consistent with old cerebral infarctions.
attacks subside 131. There is relative sparing of the
macular vasculature and thus central visual impairment
is minimal.
Pathologically, avascular areas in the retinal periphery occur secondary to peripheral capillary closure.
More posteriorly one sees microaneurysms, dilated
capillary channels, tortuosity of neighboring vessels,
and spontaneous choreoretinal scars. The most common ophthalmologic findings are retinal neovascularization (84%), vitreous and retinal hemorrhages (58%),
obliterated vessels (37%), and vascular sheathing
(34%) [4}. Occasionally choreoretinal scarring, retinal
detachment, or anterior segment neovascularization
with hemorrhagic glaucoma or cataract formation is
seen with profound loss of vision [5].
There are no characteristic laboratory abnormalities
in Eales’ disease and the diagnosis is a clinical one
based on a typical funduscopic picture. In at least one
patient 161, abnormal platelet clumping was noted on
routine blood test (platelet count was normal) and further studies revealed “a large proportion of misshapen
platelets with reduced aggregation to adenosine
diphosphate, no aggregation to adrenaline, and reduced glass adhesion and spreading.” No platelet antibodies or cryoglobulins were found. Fluorescein angiography is helpful in the diagnosis of Eales’ disease.
Fig 2. Cerebral angiogram demonstrated occlusion of
middle cerebral artety (arrowhead).
the /dt
Fig 3. Lute arteriovenousphase ofjuorescein angiogrum demonstrated leakagefrom retinal newascularizution proximal t o the
peripheral zone of ischemia.
Brief Communication: Gordon et al: Eales’ Disease Presenting As Stroke
265
It delineates regional vascular abnormalities, such as
capillary nonperfusion, dye leakage secondary to increased vascular permeability, capillary dilatation, arteriovenous shunts, segmental aneurysms, and retinal
neovascularization 171.Treatment of Eales’ disease has
included corticosteroids as well as argon laser or xenon
arc photocoagulation, but results are controversial.
The differential diagnosis of Eales’ disease includes a
wide range of disorders that can produce vitreous
hemorrhage or retinal perivasculitis or both. Many disorders can produce vitreous hemorrhage IS], but only
retinal telangiectasia, sickle cell disease, and pars
planitis are occasionally associated with a concomitant
retinal perivasculitis.
Eales’ disease is a diagnosis of exclusion and other
conditions that may produce a true retinal perivasculitis need to be ruled out, such as connective tissue
disorders, granulomatous diseases, syphilis, Behfet’s
disease, inflammatory bowel disease, cytomegalovirus,
Hodgkin’s disease, acute ocular panvasculitis syndrome, multiple sclerosis, and pars planitis. In the past,
certain patients with multiple sclerosis associated with
retinal periphlebitis and venous cuffing and sheathing
have been mistakenly diagnosed as having Eales’ disease. In contrast to the situation with Eales’ disease,
optic pallor is a frequently associated finding in patients with multiple sclerosis, whereas microaneurysms, retinitis proliferans, and vitreous hemorrhage
have not been reported f5, 91.
The only extraocular manifestations reported in
Eales’ disease have involved the nervous system. Although neurological involvement is rare, with no central nervous system (CNS) complications reported in
one series of 947 cases [2), it definitely occurs. In one
report of 9 patients with neurological involvement,
8 had acute or subacute myelopathy with onset weeks
to years after ocular involvement [9]. Five patients
showed a lymphocytic pleocytosis in the CSF, and 4
had raised CSF protein levels. Chronic encephalomyelitis and CNS vasculitis or vasculopathy have also
been reported in association with Eales’ disease. One
patient suffered from a progressive brainstem and
cerebellar syndrome over a 10-year period. [lo]. Terminally he showed rapid progression with clinical involvement of the cortex. At postmortem examination
there was extensive lymphocytic infiltration of CNS
veins and venules, and to a lesser extent arterioles and
meninges, along with gliosis and demyelination. A few
multiple sclerosis-like plaques were noted in the
spinal cord. In another report of 17 cases of Eales’
disease, 7 showed evidence of neurological involvement [I 11. Three patients had only CSF abnormalities
(elevated total protein in 2 cases and a “paretic” colloidal gold curve in one). Two patients had a
paraparesis, one patient had a history of episodic
266 Annals of Neurology
Vol 24
No 2
A u g u s t 1988
neurological disturbances and carried a diagnosis of
multiple sclerosis, and a fourth patient had suffered a
stroke in the puerperium 8 years prior to the appearance of ocular disease. Herson and Squier 161 described a 39-year-old man with Eales’ disease who had
chronic meningitis with transient focal signs. Mental
deterioration progressed over 4 years, ending in death.
Pathologically, there was a lymphocytic meningeal
infiltrate with chronic vasculitis throughout the neuraxis, involving the small veins and occasionally the arterioles. In another study of 25 patients with Eales’
disease, 6 had unexplained bilateral sensorineural hearing loss and 19 had objective abnormalities on vestibular function testing 1121.
Our 38-year-old patient presented with a mild
stroke syndrome and had evidence on computed tomographic scan of previous bilateral infarcts. He had a
history of a vitreous hemorrhage with funduscopic and
angiographic evidence of retinal perivasculitis. The
vitreous hemorrhage and perivasculitis were felt to
be idiopathic, consistent with Eales’ disease. Multiple
strokes in a young adult are unusual, even in the setting of hypertension and cigarette use. No cardiac embolic source was documented. We feel that this case
report involves multiple cerebrovascular accidents
most likely secondary to Eales’ disease. His elevated
platelet count may have reflected an associated platelet
abnormality, and his angiogram did not rule o u t an
isolated CNS vasculitis. Eales’ disease should be added
to the list of conditions that may be associated with
stroke in the young adult.
References
1. Eales H. Cases of retinal hemorrhage associated with epistaxis
and constipation. Birmingham Med Rev 1880;9:262-273
2. Duke-Elder S, Dobree JH. Diseases of the retina. Sr Louis:
Mosby, 1967:222
3. Eliot A]. Thirty year observation of patients with Eales’ disease.
Am J Ophthalmol 1975;80:404-408
4. Spirznas M, Meyer-Schwickerath GM, Stephan 8,Albrecht v.
Graefes Arch Klin Exp Ophthalmol 1975;194:73-85
5. Wise GN, Dollery CT, Henkind P. Diseases of capillary and
venous insufficiency. In: Wise G N , Dollery CT, Henkind P,
eds. The retinal circulation. New York: Harper ti: Row,
197 1:37 6-3 8 1
6. Herson RN, Squier M. Retinal perivasculitis with neurological
involvement. J Neurol Sci 19?8;36:11l-117
7. Theodassiadis G. Fluorescein angiography in Eales’ disease. ,4m
J Ophthalmol 1970;69:271-2’77
8. Tasman W. The vitreous. In: I h a n e TD, Jaeger EA, eds. Clinical ophthalmology. Philadelphia: Harper & Row, 1984-14-16
9. Singhal BS, Dastur DK. Eales’ disease with neurological involvement. J Neurol Sci 1976;27:312-121, 323-345
0. Silfverskiold BP. Retina periphlebitis and chronic disseminated
encephalomyelitis. Acta Psychiatr Neurol Scand 1951; ‘4(Si:55
1. White RH. The etiology and rieurological complications of r-etinal vasculitis. Brain 1961;84:262-273
2. Renie WA, Murphy RP, Anderson KC, er al. The evaluatioil of
patients with Eales’ disease. Retina 1983;3:243-248
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