NOTES, CASES, INSTRUMENTS of this condition has been stressed, as has the importance of a history of bilateral dimming of vision and pain in the temporal areas occurring in a patient in the age group of 54-84 years. A n increased sedimentation rate is an important sign in the absence of temperature rise. T h e history of a
70-year-old man who presented little more than acute visual loss in one eye and dimming of vision 24 hours later in the other eye is detailed. T h e diagnosis was confirmed by biopsy of the right temporal artery. 57 Midwood
1. Hutchinson, J. : Diseases of arteries. Arch. Surg. (Lond.). 1:323, 1890. 2. Horton, B. T., Magath, T. B., and Brown, G. E. : An undescribed form of arteritis of the temporal vessel. Proc. Mayo Clin, 7 :700, 1932. 3. Simmons, R. J., and Cogan, D. G. : Occult temporal arteritis. Arch. Ophth., 68:8, 1962. 4. Parsons-Smith, G. : Ophthalmic manifestations of temporal arteritis. Brit. J. Ophth., 36 :615, 1952. 5. Wagener, H. P., and Hollenhorst, R. W. : Ocular lesions of temporal arteritis. Am. J. Ophth., 45 : 617, 1958. 6. Palin, E. : The ocular crisis of the temporal arteritis syndrome (Horton). Acta ophth. (Kbh.), 36:208, 1958. VISUAL ANOSOGNOSIA IN CORTICAL BLINDNESS* A N T O N ' S SYMPTOM H . S A U L SUGAR,
AND A B E S. GOLDSTEIN,
Detroit, Michigan Cases of denial of blindness have been well described in the neurologic literature but have had little attention from ophthalmologists. Experience with such a case has stimulated this report. The term anosognosia, coined by Babinski, 1 although originally used to designate failure to recognize the existence of paralysis, etymologically refers to failure to recognize the presence of any physical disability as due to disease and is so used today. Visual anosognosia, where the patient is "blind to his blindness," is usually associated with confabulation or hallucinations in which the patient acts as though he can see, reports visual experiences in detail as if he actually saw them, and denies that he is blind when asked if he can see. T h e visual anosognosia usually occcurs in * From the Sinai Hospital of Detroit and the Wayne State University College of Medicine.
the presence of bilateral homonymous hemianopia. However, if only one side is involved, it is believed by some that the major lesion probably involves the thalamoparietal bundle of the nondominant right side which prevents messages essential for the integration of bodily sensation from reaching the supramarginal cortex (Schlaegel 3 ). T h e first case of symptom denial was described nearly 2,000 years ago by Seneca (cited by M. Critchley 2 2 ). I n 1816, Anton verbally reported a case with bilateral softenings of the visual radiations which was followed by complete blindness of which the patient himself was not aware. In 1898 and 1899, he added three cases. 4 The first was that of a 56-year-old woman who was totally blind but unaware of her visual loss. H e r spatial orientation was impaired and she could not localize voices or distances by sound. At autopsy, she was found to have bilaterally symmetric lesions of the angular gyrus and the first and second occipital gyri, involving the medullary substance, and a small lesion in the splenium of the corpus callosum. Anton's second case was that of a 64-year-old man with complete deafness who thought he could hear well. T h e third case was that of a 69-year-old woman who
NOTES, CASES, INSTRUMENTS
was unaware of her inability to understand spoken words. Autopsy revealed bilateral destruction of the first and second temporal gyri, extending toward the occipital lobes. Anton stressed the importance of destruction of the association tracts between the occipital, temporal and lower parietal lobes. Only the first case was later referred to as manifesting Anton's syndrome which is now considered to include cortical amaurosis due to a bilateral homonymous hemianopia associated with denial of blindness, amnestic aphasia, loss of recent memory and retention, confabulation and, at times, varying degrees of intellectual deterioration ( S m i t h 5 ) . Some authors refer to the denial of blindness alone as Anton's symptom. Previous cases of blindness from cerebral disease with denial of blindness were gathered by Redlich and Dorsey. 6 Von Monakow described two cases. 7 Dejerine and Vialet 8 and Rossolimo 9 each reported one. Since Anton's reports, other cases have been reported by Mayer, 1 0 Lunz, 1 1 Bonhoeffer, 12 Lejeune, Raymond and Galezewski, 13 Probst, 1 4 Redlich and Bonvicini, 15 Albrecht, 16 Poetzl, 17 Tunero, 1 8 Weber, 1 9 Raney and Nielsen 20 and Brockman and von Hagen. 2 1 Since denial of blindness is concerned with cases of cortical blindness, let us consider the signs of this disease. T h e outstanding feature is a loss of vision which may be sudden in onset (as with basilar artery involvement), or gradual, or may develop in two separate stages (vascular thrombosis causing unilateral hemianopia, followed later by vascular occlusion in the opposite hemisphere). 2 2 T h e pupillary reactions to light and convergence are retained, although the latter is often difficult to elicit. There is loss of reflex lid closure to bright illumination and to threatening gestures. T h e ocular motility and fundi remain normal. No optokinetic nystagmus is demonstrable. There may be less constant unformed visual hallucinations due to cortical stimulation and such associated mental signs as anosognosia,
loss of apperception, agnosia and apathy. Denial of blindness usually occurs in elderly arteriosclerotics with bilateral hemianopia. Most have lesions of the occipital lobes or temporoparietal lesions but cases have occurred with generalized disease of the brain with blindness. All patients with denial of blindness show at least a moderate amount of intellectual deterioration, disturbed orientation, defects of memory and retention and amnestic aphasia. They are not able to learn from experiences. W h e n they bump into furniture or the wall, they do not change their behavior. W h e n asked why they do not recognize an object held before them, they may reply that they forgot their glasses or that the room is dark or they may ask why the lights were not turned on. A patient of Probst 1 4 thought he was in a dark cellar most of the time. Another patient said he had tears in his eyes. 8 Others explained that the blinds were drawn or that eyeglasses were inadequate. 15 I n our case the patient explained the blindness by saying that her eyelids were closed. A considerable controversy exists as to whether the denial is primarily due to emotional causes rather than to brain injury alone. 6 - 15> 22 Perhaps both explanations are valid. C A S E REPORT
I. S., a 72-year-old Jewish woman, who had been previously treated for arteriosclerotic heart disease with coronary insufficiency, suffered a sudden left hemiparesis in July, 1961. She suddenly fell to the floor, had difficulty getting up, finally collapsed and was subsequently hospitalized. A left hemiparesis and left facial paresis were present. Left hemianopia was also present. The spinal fluid was normal. She was discharged from the hospital after four weeks and was maintained on anticoagulants. She had improved considerably but continued to complain of unsteadiness in walking, of bumping into things with her left hand, and of iight-headedness, difficulty in swallowing, blurred vision and depression. Her blood pressure varied from 140/80 to 170/90 mm Hg. On several occasions it was found that the systolic blood pressure fell 20 mm Hg and the diastolic fell 10 mm Hg upon sitting up quickly. She was treated with Coumadin, Butisol Sodium and Doriden. In 1953, the patient had been hospitalized for an
NOTES, CASES, INSTRUMENTS acute myocardial infarction. This was followed by recurrent episodes of myocardial insufficiency, relieved by nitroglycerine. In 1956, she had pain in her left lower extremity. No posterior tibial or dorsalis pedis pulse could be felt. Neurologic examination given by Dr. J. S. Meyer in January, 1962, revealed a hémiplégie gait on the left with small steppage, a rather shuffling gait. The faciès were immobile, rather parkinsonian. A definite bradykinesis was present. No murmurs were heard over the heart or neck vessels. Fine movements were poorly performed bilaterally. The left hand grip and left biceps were weak. Dorsiflexion of the left wrist was weak. The finger to nose test was poorly performed on the left. The tendon jerks were increased on the left, as compared to the right. There was dimnished pain sensation in the left leg to the thigh. A diagnosis of generalized atherosclerosis with vertebral-basilar artery insufficiency was made. An electrocencephalogram, done in June, 1962, revealed a diffusely abnormal record, nonspecific in clinical correlation but compatible with a mild generalized alteration of cortical function. In September, 1962, the patient had an anginal attack which lasted 15 minutes. Electrocardiography indicated subendocardial ischemia in the left ventricle, an increase since the previous electrocardiogram. On at least two occasions between September, 1961, and October, 1963, the patient appeared to have a left homonymous hemianopia on double simultaneous stimulation. This was not present when each visual field was stimulated independently. This was considered to be a parietal lobe sign. In September, 1963, anticoagulants were dis-
continued because of melena and hematuria. On October 31, 1963, the patient awoke with a severe headache, nausea, vomiting, incontinence and total blindness which she denied. She explained her inability to see by saying her lids were closed. When the lids were hold open by the observer the patient did not change her explanation for the blindness. Examination revealed normal pupillary reactions to light. Spontaneous comitant extraocular muscle movements were present. The fundi were normal except for moderate arteriolosclerosis. No ocular pathology was found. A lower left hemiparesis was present. Weakness of the left leg, arm and hand were present. Peripheral sensation was normal. There was displacement of left-sided tactile stimuli to the right side of the body. Lumbar puncture was negative. The cortical blindness was considered to be due to occlusion of the terminal portion of the basilar artery. The patient's vision remained unchanged. She remained moderately disoriented. In April, 1964, thrombophlebitis of the lower left extremity developed. Several days later the patient died of what appeared to be a massive pulmonary embolus. Permission for autopsy was not obtained. SUMMARY
Denial of blindness in a case of hemianopia is presented to acquaint mologists with this symptom which known to neurologists but is rarely opthalmologists. 18140 San
bilateral ophthalis wellseen by
1. Babinski, M. : Contribution a l'étude des troubles mentaux dans l'hémiplégie organique cérébrale (anosognosie). Rev. Neurol., 27:845, 1914. 2. Lhermitte, J., and De Ajuriaguerra, J. : Psychopathologie de la Vision. Paris, 1942. 3. Schlaegel, T., Jr. : Psychosomatic Ophthalmology. Baltimore, Williams & Wilkins, 1957. 4. Anton, G. : Ueber Herderkrankungen des Gehirns welche vom Patienten selbst nich wahrgenommen werden. Wien. klin. Wschr., 11:227, 1898. : Ueber die Selbstwahrnehmung der Herderkvankungen des Gehirns durch den Kranken bei Rindenblindheit und Rindentaubheit. Arch. Psychiat. Nervenkr., 32:86, 1899. 5. Smith, J. : Optokinetic Nystagmus. Thomas, Springfield, 111., 1963. 6. Redlich, F., and Dorsey, J. : Denial of blindness by patients with cerebral disease. Arch. Neurol. & Psychiat., 53:407, 1945. 7. von Monakow, A. : Experimentelle und pathologisch-anatomische Untersuchungen über die Beziehungen der Sogennanten Sehspare zu den infracorticalen Opticuscentren und zum Nervus opticus. Arch. Psychiat. Nervenkr., 16:151, 1885. 8. Dejerine and Vialet: Sur un cas de cécité corticale. C. R. Soc. Biol. (Paris), 11:983, 1893. 9. Rossolimo, G. : Ueber Hemianopsie und einseitige Ophthalmoplegie vascularen Ursprungs. Neurol. Cbl., 15 :626, 1896. 10. Mayer, C. : Eine doppelseitige homonyme Hemianopie mit Orientierungstörungen. Mschr. Psychiat. Neurol., 8:440, 1900. 11. Lunz, C. : Zwei Fälle von Korticaler Seelenblindheit. Dtsch. med. Wschr., 1:381, 1897. 12. Bonhoefïer, K. : Casuistische Beitrage zur Aphasielehre. Arch. Psychiat. Nervenkr., 37 :564, 1903. 13. Lejeune, Raymond, and Galezowski: Cécité corticale par double hemianopie. Rev. Neurol., 19:680, 1906. 14. Probst: Ueber einen Fall vollständiger Rindenblindheit und vollständiger Amniesie. Mschr. Psychiat. Neurol., 9:5, 1901.
NOTES, CASES, INSTRUMENTS
15. Redlich, E., and Bonvicini, G. : Weitere klinische und anatomische Mitteilungen über das Fehlen der Wahrenhmung der eigenen Blindheit bei Hernkrankheiten. Neurol. CbL, 30:227, 301, 1911. 16. Albrecht, O. : Drei Fälle mit Antons Symptom. Arch. Psychiat. Nervenkr., 59 :883, 1918. 17. Poetzl, O. : Ueber Störungen der Selbstwahrnehmung bei linkseitiger Hémiplégie. Z. ges. Neurol. Psychiat., 93:117, 1924. 18. Tunero, J.: Ein Fall mit Antons Symptom. Psychiat. Neurol. jap., 41:679, 1931. 19. Weber, F. : Agnosia of hemiplegia and of blindness after cerebral embolism. Lancet, 1:44, 1942. 20. Raney, A., and Nielsen, J. : Denial of blindness (Anton's symptom). Bull. Los Angeles neurol. Soc, 7:150, 1942. 21. Brockman, N., and Von Hagen, K. : Denial of own blindness (Anton's syndrome). Bull. Los Angeles neurol. Soc, 11:178, 1946. 22. Critchley, M. : The Parietal Lobes. London, Harold, 1933, p. 258.
MARGINAL DEGENERATION OF CORNEA WITH KERATECTASIA A C L I N I C A L CASE P R E S E N T A T I O N T H O M A S E.
Lawton, Oklahoma History. The patient, a 74-year-old white man, was admitted to the Baltimore City Hospital with pneumonia, congestive heart failure and uremia secondary to chronic obstructive uropathy. Due to the striking appearance of the eyes an ophthalmologic consultation was requested. Because of marked senile dementia an adequate ocular history was unobtainable. He was not aware of anything being wrong with his eyes. Ocular examination. On external examination there was prominent marginal degeneration with perilimbal excavation, extending circumferentially around both corneas (fig. 1). The marked keratectasia formed a picture of exaggerated keratoglobus (fig. 2). A pseudo-Munson's sign was present (%■ 3 ) .
On slitlamp examination the central cornea was clear in both eyes without demonstrable defects in Descemet's membrane. A broad, deep gutterlike furrow with sloping peripheral edge and sharp perpendicular central wall extended around the periphery of both corneas. The furrow was quite broad temporally. Fine radial vessels extended into the floor of the furrow from the limbal arcade. The anterior chambers were deep and clear and the
Fig. 1 (Acers). Anterior view.
Fig. 2 (Acers). Lateral view. lenses were in place. There was prominent sectorial iris atrophy. Retinoscopy revealed a refractive error of approximately —15D sph with a prominent irregular astigmatism. Visual acuity was not ascertainable due to lack of communication and co-operation on the part of the patient. The fundi were essentially normal except for prominent arteriosclerotic retinal vascular changes. There were no significant degenerative changes in the posterior ocular segments. DISCUSSION
It is considered that the condition described represents an advanced keratectasia secondary to marked peripheral degeneration of the cornea. Even though this condition of peripheral marginal degeneration is well documented in the ophthalmic literature, this far-advanced stage must be rather unusual. Most of the cases have been reported in the German and French literature. According to Duke-Elder 1 this type of corneal degeneration was first described by