Man presents with homonymous

hemianopia, rapidly progressive

dementia
http://www.healio.com/ophthalmology/neurosciences/news/print/ocularsurgery-news/{c5fe23a0-06e9-4a41-b8cb-c1225c677d96}/man-presents-withhomonymous-hemianopia-rapidly-progressive-dementia?page=0&Filter=

Ocular Surgery News U.S. Edition, September 10, 2013

Jennifer Renz, MD; Thomas R. Hedges, MD

A 66-year-old patient first noticed trouble with his right peripheral

vision 2 weeks prior to presentation while he was playing golf. He
also complained of more trouble with light to dark adaptation and
reading.
The patient had no ocular history. He had had a cardiac ablation
for atrial fibrillation in 2011, a right bundle branch block and
cardiomyopathy. His family history was notable for Alzheimers
disease, prostate cancer and myocardial infarction in his father.
The patient was a retired engineer who drank alcohol socially and
quit smoking at age 26. He took 81 mg of aspirin, a multivitamin,
vitamin C and a magnesium supplement daily.

History
Upon outside ophthalmology exam, the patients best corrected
visual acuity was 20/30 in his right eye and 20/20 in his left. He
had full color vision on Ishihara plates, and his pupils were equal,
round and reactive to light with no afferent pupillary defect. His
motility exam was reportedly normal, and the slit lamp exam was
unremarkable. On dilated fundus exam his cup-to-disc ratio was
0.4, and he had trace extrafoveal drusen in both eyes. Automated
perimetry revealed a right homonymous hemianopia (Figure 1).
The patient underwent a stroke workup, including diffusionweighted MRI, which showed hyperintensities in the left occipital
and inferior parietal cortexes that were consistent with a possible
early left occipital stroke. Magnetic resonance angiography,
transesophageal echocardiography and 24-hour Holter readings
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were all normal. He was evaluated by a neurologist and

cardiologist, and started on Xarelto (rivaroxaban, Janssen).
In the next month, the patient had progressive problems with word
finding and memory. His wife prompted him to seek emergency
care when he could not compute a simple arithmetic problem. At
this point, he was able to perform activities of daily living but was
not driving or shaving, which he claimed was due to his visual
difficulties.
The patient had another MRI, which demonstrated a single small
focus of prolonged T2 hyperintensity in the white matter of the
frontal lobe. The previously noted left occipital hyperintensity was
no longer noted, and there was no evidence of a previous stroke.
Electrocardiogram and routine labs were normal.
The next day, another visual field test that showed possible
progression of the right hemianopic field defect into the left visual
field but was significantly more unreliable. Two days later, the
patient was admitted to our hospital. Neurologic exam documented
increased muscle tone, cognitive decline, ataxia, perseveration of
speech and myoclonus. He was then admitted for further workup.

Figure 1. Right homonymous hemianopia on automated perimetry at presentation.

Images: Renz J, Hedges TR

Figure 2. Diffusion-weighted MRI showing hyperintensity of the caudate nucleus of

the basal ganglia, left greater than right (black arrow), and ribbon-like signal in the
frontal and occipital lobes (white arrows).Examination

Copper, Lyme, erythrocyte sedimentation rate and C-reactive

protein, antinuclear antibodies, rapid plasma reagin, and a
paraneoplastic panel were all normal. A lumbar puncture was
unremarkable, and an electroencephalogram (EEG) showed
periodic sharp and slow wave complexes. Yet another MRI noted
faint, relatively slow cortical diffusion that was worse in the left
frontal temporal and occipital cortex, as well as the persistent
hyperintensity in the caudate nucleus of the left basal ganglia
(Figure 2).
Upon neuro-ophthalmologic exam, the patient appeared to have
visual field loss to confrontation on the right as well as the left.
Although visual acuity could not be tested by Snellen chart and he
repeatedly stated, I cant see, the patient was able to recognize
objects and family members. He was unable to initiate appropriate
saccades, made inaccurate saccades, and had perseverated
fixation consistent with Balints syndrome. His ability to pursue
targets was poor. These abnormalities rapidly worsened over the
course of several days, and he became minimally verbal.
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What is your diagnosis?

Homonymous hemianopia
The first concern in any patient with apparently acute
homonymous hemianopia is stroke. Brain tumor with hemorrhage
would be the next possibility. However, the differential diagnosis of
homonymous hemianopia and rapidly progressive dementia is
narrow.
Degenerative dementias, such as Alzheimers, Picks disease or
Lewy body dementia, can rarely be associated with hemianopias
when degenerative neurofibrillary tangles are focused primarily in
the occipital cortex; however, the clinical course is typically more
gradual in these disorders. Occipital seizures typically have
autonomic findings, such as tachycardia, and occipital spikes on
EEG. Functional vision loss would be difficult to rule out, but is
unlikely given the severity of the patients cognitive decline and the
lack of previous psychosocial abnormalities.
The patients homonymous hemianopia, rapidly progressive
dementia, later neurologic findings of ataxia, increased muscle
tone, perseveration of speech and movement, his MRI showing
hyperintensity of his basal ganglia, a ribbon-like signal in the
frontal and occipital cortices, and his EEG all lead to the diagnosis
of the Heidenhain variant of Creutzfeldt-Jakob disease. He was
discharged to hospice and died 12 days later. His cerebrospinal
fluid was later found to be positive for 14-3-3 protein.

Discussion
Creutzfeldt-Jakob disease is rare and fatal, with an incidence of
one in 2 million people, 75% of whom die within 6 months. There is
no sex predilection, and the mean age of onset is in the seventh
decade. Symptoms primarily include rapidly progressive dementia,
myoclonus, ataxia and personality changes. Pathologically, prion
protein accumulates in the brain, resulting in spongiform
degeneration, loss of granular cells, astrocytic gliosis and amyloid
plaque. Prion protein is stable in high temperature and acid and is
thus not destroyed by routine sterilization.
Eighty-five percent of cases are sporadic. Less commonly it is
familial, although some small case series noted a strong genetic
basis in the Heidenhain variant.
There have been three cases of spongiform encephalopathy after
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cornea transplant, but no conclusive evidence of transmission from

intraocular surgery or ophthalmic procedures. Diagnosis is made
clinically but can be confirmed by 14-3-3 protein in cerebrospinal
fluid, hyperintense basal ganglia on MRI, EEG with periodic sharp
wave complexes, and definitively by brain biopsy.
The Heidenhain variant affects predominantly the occipital lobe.
Initial symptoms are primarily visual, with varied complaints
including decreased acuity; an altered perception of color, such as
a description of green vision; visual hallucinations; visual field
defects and, in late stages, cortical blindness. MRI findings are
ribbon-shaped hyperintensities in the occipital cortex on diffusionweighted MRI, increased signal in the basal ganglia, and later,
occipital atrophy.
A significant number of these cases present first to an eye care
provider with vision loss.
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Kher M, et al. Ann Indian Acad Neurol. 2009;doi:10.4103/0972-2327.48856.
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