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Show Journal of (/"" eal Neuro- ol'hthalm,,/ ogy 9( 4J: N~-~ 46, 1989. Palinopsia as a Presenting Manifestation of Creutzfeldt- Jakob Disease Valerie Purvin, M. D., Jose Bonnin, M. D., and Julius Goodman, M. D. © 1989 Raven Press, Ltd., New York A 70- year- old man developed a syndrome of progressive nondominant parietal and occipital dysfunction including palinopsia and a visual field defect. Despite the marked focality of his clinical findings, radiologic studies were normal. Myoclonus and ataxia began 6 weeks after onset of his illness at which time brain biopsy confirmed Creutzfeldt- Jakob disease ( CJD). This is the first reported case of palinopsia due to CJD. Key Words: Palinopsia- Creutzfeldt- Jakob disease ( CJD)-- Dementia. From the Midwest Eye Institute ( V. P.), Neuropathology Section O. B.) and Indianapolis Neurosurgical Group ( J. G.), Methodist Hospital, Indianapolis, Indiana and Indiana University Medical Center Departments of Neurology ( V. P.) Neurosurgery ( J. G.), and Ophthalmology ( V. P.). Address correspondence and reprint requests to Dr. V. PurYin at Midwest Eye Institute, 1800 North Capitol Avenue, P. O. fic' · 13<; 7, Irdi: mapoIis, IN 46206- 1367, U. S. A 242 Palinopsia is a rare disorder of visual perception characterized by persistence of an image after removal of the exciting stimulus. Such images usually persist for several minutes, although occasional cases may last for hours or days. Palinopsia often becomes evident early in the course of a progressive hemianopic visual field defect and may resolve as the defect becomes complete ( 1). Other abnormalities of visual perception such as illusory motion, distortion of size and shape, and transposition of objects in space are frequently present ( 2). Most reported cases are due to a structural lesion in the nondominant parietooccipital area. We report a patient in whom vivid palinopsia was the initial manifestation of Creutzfeldt- Jakob disease ( CJD). CASE REPORT A 70- year- old right- handed man was in good health until June 1987, when he noticed problems with reading. Specifically, he described abnormal persistence of visual images. One example of this occurred when he looked at a mustard jar and then at a piece of bread. He believed the bread had become moldy until he realized he was still seeing the green label from the mustard. Palinoptic images lasted from minutes up to an hour, and each image did not recur after its resolution. Images were not confined to a particular area of the visual field. Perception of these images was not associated with any alteration of consciousness or focal neurologic deficits. He also noted problems reading, which he attributed to a " missing portion" in the center of each word. Over the next 4 weeks, he experienced a variety of other visual distortions, illusions, and hallucinations. On one occasion, a moving car appeared to him to be 6 inches off the ground. On another occasion, every building he PALINOPSIA IN CREUTZFELDT-/ AKOB DISEASE 243 looked at appeared to have a " bite" taken out of the roof. He denied language difficulty, numbness, weakness, headache, fatigue, weight loss, or any other constitutional symptoms. Past medical history included essential hypertension and a balloon angioplasty in 1985. Ophthalmologic examination revealed a visual acuity of 20/ 50 in each eye with normal pupillary responses. On testing with Ishihara pseudoisochromatic plates, he was able to identify only the screening plate in each eye. Goldmann perimetry ( Fig. 1) revealed an absolute left homonymous hemianopic scotoma and a smaller homonymous central defect to the right of fixation. Ocular motility was normal as was a dilated fundus exam. On neurologic testing, he was alert and attentive and was oriented to person, place, and time. He recalled two of three items at 5 min. Verbal output was fluent and grammatically correct with intact repetition. He followed two- step commands easily but had mild difficulty with more complex instruction. There was no anomia, he was able to name colors correctly, and there was no right- left confusion. He had difficulty with serial subtraction. There was marked constructional apraxia with failure to close each of his figures. He extinguished the left hand on simultaneous stimulation. Tone, strength, and coordination were intact. There was mild flattening of the left nasolabial fold; cranial nerves were otherwise intact. A complete blood count, chemistry profile, erythrocyte sedimentation rate, VORL, fluorescent treponemal antibody ( FTA), serum protein electrophoresis, urinalysis, chest x- ray, and EKG were normal or negative. Computed tomographic ( CT) scan of the head before and after intravenous contrast infusion was normal except for mild ventricular enlargement. A magnetic resonance imaging ( MRI) scan contained some motion artifact but revealed no abnormalities. An EEG was generally slow especially posteriorly with no paroxysmal discharges. Two weeks after his initial presentation, he reported increased visual difficulty, dressing apraxia, and spatial disorientation. His family also described occasional brief jerking movements of the extremities more frequent on the left side. Examination revealed marked progreSSion of his left homonymous hemianopsia with some worsening of the right hemi- field defect ( Fig. 2), abnormal posturing and dysmetria of the left upper extremity, mild gait instability, and left astereognosis and agraphesthesia. There was again no evidence of aphasia. CSF exam and three- vessel angiography were normal. To confirm the clinical suspicion of CJO, a brain biopsy was performed. A I- cm3 sample taken from the right parietal cortex was examined by light and electron microscopy. The neuropil had marked spongiform change with many small, primarily " " ," '.' , ~ O. t--- ,...- . IIOotL _ 1.. 2. _ '.~ • ~. 9 .. .4l . lD -" . , ~ ,., . I, ll_ -"' 0 i I I I . J4~ "]) 0 " JI) 10 <~. n,. I~••",. •••"", ,.- do. " 0", ,.... ~... 110 I , .. t. o.,,,,. nd Io" llI t"'''' FIG. 1. Goldmann visual fields at initial presentation showing. a congruous left homonymous hemianopic scotoma. A small homonymous defect IS also present In each eye just to the right of fixation. / Clill Neuro- ophthalmol, Vol, 9, No. 4, 1989 244 V. PURVIN ET AL. I!~ I~) " 1\ 50 ll~ l~) .. 1\ -~ -,-~-. ,'- 0---,"-:::" r IH,, , \ / \ ." " ~.., \ .. '. , \,' J ! '.~ " \ "', 1 \ /" ~ " '.. I!... //: . , \ ")~____ •• o.! L- U-- ~~ ----- t+ L- ~ " .~ o " .\... 19)....- · , ] 34) 210' · 330 '---~- ---#-~---- '-,. ~~-~ ",' '] 1) _. -?( j- ,;, , ~ l10 28~ ,,; '" 2~-) 210 .... I......'.~. 4.~. i. l". O..,"~•',•.....,....... ..':':':'::'; 9;..,'::;,-::_ FIG. 2. Goldmann visual fields performed 2 weeks later. There is now complete loss of the left hemifield with some early loss of central field on the right. rounded or oval vacuoles in the deeper cortical layers. Ultrastructurally, the vacuoles could be localized in the cell bodies and processes of neurons and astrocytes. There was also a moderate loss of neurons, and some of the remaining ones had large vacuoles that distended the cell bodies and compressed the nuclei. Reactive proliferation of the astrocytes was also a prominent feature. The triad of spongiform change, neuronal loss, and astrocytosis is characteristic of C] D. The diagnosis was further supported by some negative observations such as the absence of an inflammatory reaction, infiltration with macrophages, necrosis, or the morphological hallmarks of any dementing process. The patient continued his rapid neurologic decline. Seven weeks after initial onset of his illness, he was lethargic, unable to stand, and displayed frequent diffuse myoclonic jerks. EEG at this stage showed marked slowing with periodic spike and sharp wave discharges over the right posterior region. He was transferred to a nursing home where he died 1 week later. An autopsy was not performed. DISCUSSION The pathophysiology of palinopsia is not yet fully defined. There is probably more than one form of the disorder. In some cases, palinopic images occur episodically, recur at some time after the initial event, and respond to anticonvulsants. Such cases probably represent a form of sensory seizure ( 3,4). In other cases, there is evidence of prolongation of the normal afterimage ( 5,6). Such prolongation may be selective for specific stimulus parameters such as shape, color, and duration ( 6). In the majority of cases, there is no evidence of altered physiologic afterimage. The mechanism in these instances may be loss of normal inhibition of visual- memory pathways, thus creating an " uninhibited reverberating circuit" ( 2). Most cases of palinopsia have been associated with a focal lesion in the nondominant parietooccipital area ( 1), although rare cases have involved the posterior left hemisphere ( 1,5,7) or more anteriorly placed lesions ( 1,8). Specific causes have included tumor ( 1), ischemia ( 9), trauma ( 5), arteriovenous malformation ( 3), migraine ( 10), and abscess ( 11, 12). In rare instances, no structural lesion is found, including cases of carbon monoxide poisoning, drug intoxication ( 13), and one case in which cerebral vasculitis was suspected ( 6). In our case, palinopsia was the presenting manifestation of C] D. A variety of different visual abnormalities have been described in patients with C] D. Nonspecific complaints of blurred or dim vision are common ( 14). Higher cortical visual disturbances including visual distortions, dyschromatopsia, visual agnosia, and micropsia have also been reported ( 15). Homonymous hemianopic visual field defects are sometimes suspected, but formal perimetry is usually not feasible due to declining intellect and attention. In some cases, a hemi- , Clin Neuro- ophlhalmol, Vol. 9, No. 3, 1989 PALINOPSIA IN CREUTZFELDT-/ AKOB DISEASE 245 anopic defect is impossible to distinguish from hemiinattention due to parietal lobe dysfunction. In more advanced stages, cortical blindness ( loss of vision with normal pupils and optic discs) is frequent. Ocular motility disturbances are less common and include supranuclear ( 16) and infranuclear palsies ( 15), ptosis ( 15), and apraxia of eyelid closure ( 17). Variability in the clinical and pathologic manifestations of CJD has led to attempts to divide the disorder into subgroups including one form with prominent visual manifestations. This entity was first described by Heidenhain in 1929 ( 18). He described three patients with a dementing disease, the course and histology of which were compatible with CJD. Two of these patients had cortical blindness as a prominent finding, and in these cases autopsy revealed the most severe changes in the occipital cortex. A similar case was reported in 1954 by Meyer et a1. ( 19) who proposed the term " Heidenhain syndrome." The subject of this report had " failing vision," normal appearing optic nerves, and a possible right homonymous hemianopic defect. Two similar cases were described by Jones and Nevin ( 20), who believed this condition was vasculopathic in nature and proposed the term " subacute vascular encephalopathy." In 1967, Nevin ( 21) reviewed 102 cases gathered from the literature or studied personally. Based on clinical and pathologic features, he differentiated between cases of CJD and an entity he called " subacute spongiform encephalopathy" ( SSE), the latter category containing cases of the " Heidenhain variant" of CJD. According to his schema, SSE is distinguished clinically from CJD by its later age of onset, more rapid progression and paucity of upper and lower motor signs in SSE. Pathologically cases of CJD show focal involvement of frontal and temporal regions, whereas SSE cases exhibit more diffuse changes with particular occipito- parietal predominance. Siedler and Malamud ( 22) reviewed a total of 72 cases and came up with a similar classification but felt that these subgroups did not constitute two different diseases. The basis for diverse expressions of an apparently identical pathogen remains obscure. Factors that might influence disease expression include age at which infection occurs, route of entry, and differing strains of infectious agents ( 23). Although some degree of asymmetry is common early in the course of CJD, marked or prolonged predominance of signs and symptoms referable to one area of the brain is rare. Two recent reports describe patients with C] D affecting the left hemisphere preferentially. One patient, a 61- year- old man, had progressive, isolated aphasia for - 1 year before the onset of a fatal dementing illness with typical features of CJD ( 24). Another, a 73- year- old woman, presented with right hemiparesis, deteriorated rapidly to somnolence, and died 8 weeks after onset ( 25). Autopsy showed typical changes of CJD affecting the left hemisphere with only minimal abnormality of the right hemisphere. Our patient's initial complaint was palinopsia, suggesting a lesion of the right occipitoparietal area. Neurologic examination revealed a congruous left hemianopic defect compatible with a right occipital lesion. He also exhibited constructional apraxia and left- sided extinction indicating right parietal lobe involvement. He subsequently experienced progression of the hemianopic visual field defect and more severe nondominant parietal dysfunction before developing evidence of widespread involvement including myoclonic jerks, rigidity, and dementia. Our patient represents an example of the Heidenhain variant of CJD. Typical features include the patient's advanced age, rapid progression, and absence of motor signs. In addition to the characteristic predominance of occipital and parietal dysfunction, our patient also exhibited preferential involvement of the right hemisphere with striking preservation of dominant hemisphere function. This occurrence is consistent with the concept of CJD as a multifocal rather than diffuse degenerative disease ( 26). A notable feature was the presence of focal clinical findings and the absence of focal change on CT, MRI, and angiography. Focal findings with normal radiologic studies should suggest the possibility of spongiform encephalopathy. REFERENCES 1. 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