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Epileptic facial metamorphopsia
Epileptic Facial Metamorphopsia Ruth Nass, MD, S Sinha, MD and Gail Solomon, MD
A 14-year-old patient with a right parietal arteriovenous malformation presented with seizures characterized by metamorphopsia of faces. Unlike adults with right hemisphere pathology she performed like an age matched control on a task requiring recognition of unfamiliar faces. This likely reflects maturational changes in hemispheric dominance for face recognition. Nass R, Sinha S, Solomon G. Epileptic facial metamorphopsia. Brain Dev 1985; 7:50-2
Patients with complex partial seizures may have psychosensory symptoms for some time before the appearance of automatisms. If isolated psychosensory symptoms persist, a psychiatric explanation is often erroneously accepted. Particularly in a patient with lack of self reference in the illusions or hallucinations and with no previous emotional disturbance, a high index of suspicion can lead to diagnostic evaluation and in some patients treatment of a structural lesion.
Case Report A l4-year-old white right-handed female experienced several episodes of visual distortion. Frequently she reported that people looked different than their known appearance, e.g., "their faces looked older." At times the white walls of her kitchen appeared yellow or the shapes of objects appeared distorted. Consequently, the patient was referred to a psychiatrist. On one occasion several months later
From the Departments of Neurology and Pediatrics (RN, GS) and Child Psychiatry (SS), The New York Hospital-Cornell Medical College, New York. Received for publication: October 9, 1984. Accepted for publication: January 8, 1985.
Key words: Face recognition, epilepsy, metamorphopsia, right hemisphere dominance. Correspondence address: Ruth Nass, MD, The New York Hospital-Cornell Medical Center, 525 E 68th St, New York, NY 10021 , USA.
she continued to fill her glass with milk even though it was full . This incident lead her therapist to obtain a neurologic consult and an EEG because of suspicion of partial complex epilepsy. Neurologic examination was normal including funduscopic and visual fields. Electroencephalogram showed posterior temporal slow and sharp wave activity synchronously bilaterally , as well as independently from the right hemisphere. Visual evoked responses were normal. Computerized transaxial tomography (CT) scan revealed a calcified right parietal temporal occipital lesion which showed slight enhancement with contrast (Fig 1). Cerebral angiography was normal. The patient was treated with carbamazepine for seizures. At surgery an arteriovenous malformation with areas of thrombosis and calcification was partially excised. The patient was continued on carbamazepine and had rare episodes of visual distortions. Two and one half years later she developed severe headaches. The follow-up CT scan showed hemorrhage into the arteriovenous malformation. Surgery was performed and the arteriovenous malformation was again resected . Follow-up CT scan six months later showed a small residual calcified lesion. No further seiZures have occurred and the patient is still receiving carbamazepine. The intracerebral arteriovenous malformation was detected only by CT scan and not demonstrated by angiography in this patient. Although it is a vascular lesion, thrombosis and infarction within the lesion produced no changes on arteriography.
prosopagnosia - failed recognition of familiar faces-or difficulty on an experimental recognition matching task of unfamiliar faces, a review of these topics places her particular ictal deficit in perspective. Prosopagnosia occurs with bilateral lesions in the mesial occipitotemporal region [1] . Damasio et al suggest that failure of familiar face recognition could be caused by destruction of the face template, inaccessibility of the template, or blocking of the activation produced by the template. Although our patient had only a right occipitotemporal lesion, epileptiform activity on EEG was bilateral over this region, and could conceivably have produced such an ictal deficit. Our patient may have misactivated the template, with resultant distortion rather than failed recognition. Metamorphopsia for three dimensional faces with retained recognition has occasionally been reported [2-4]. Recognition of unfamiliar faces in the experimental situation is impaired primarily in Fig 1 CT scan shows a calcified contrast enhancing patients with right posterior lesions [5, 6] . lesion in the right parietal region. Some patients with· prosopagnosia perform satisfactorily on such tasks [7-10]. Using Methods and Results tachistoscopic assessment of visual hemifield Because of the ictal phenomenon of facial performance, a left visual field (LVF) advandistortion, the process of face recognition was tage, reflecting right hemisphere dominance, studied in this patient with a congenital right has been demonstrated for matching of unfamiliar faces in normal adults [11] . hemisphere lesion. Both the ability to recognize unfamiliar Facial Recognition faces and right hemisphere dominance evolve In the unfamiliar face recognition task teenage during childhood. Not only is the young child male and female faces were presented 6° to less competent at this task, compared even to the right or left of a central fixation stimulus the right brain damaged adult, but differences for 150 milliseconds. Immediately following in face encoding strategies are present: 1) stimulus presentation, the subject was required young children do not have the same difficulty to choose the matching face from an array of recognizing inverted faces as adults [12, 13]; 10 faces presented in full view. Seventy trials 2) younger children rely on more superficial, were given. Both this patient and an age, sex, piecemeal cues than the older child or adult handedness matched control made correct [12]; 3) children under 10 do not show the matches on 65% of the left visual field (LVF) usual LVF advantage for discriminating unand 78% of the right visual field (RVF) trials. familiar faces [14]. While these data are not The performance difference between the two conclusive, the strategy differences suggest that hemifields was not significant by T test. prior to puberty, facial encoding is mediated by the left hemisphere. Around puberty, a drop in total accuracy occurs, followed by a spurt Discussion to adult norms [12]. Physiological events at A frequent ictal phenomenon for this patient puberty may directly interfere with the funcwas the illusion that familiar faces looked old, tioning of the right posterior cortex, or may although they could be accurately identified. change the balance between left and right Thus, while our patient had neither classic hemisphere involvement in the face encoding.
Nass et al: Facial metamorphopsia 51
2. Bodamer J. Die Prosop-Agnosie. Arch Psychiatr Levine [14] found that a LVF advantage was Zeitschrift Neural 1947; 179:654. present at ages 10 and 12, but was absent at 3. Hecaen H, Albert M. Human neuropsychology. 8 years and transiently disappeared at 14. In New York: Wiley & Sons, 1978. contrast to 8-year-olds where absence of LVF 4. Whiteley AM, Warrington EK. Prosopagnosia: advantage was due to poor left field recognia clinical, psychological and anatomical study of three patients. J Neurol Neurosurg Psychiatry tion, absence at 14 was due to improved 1977;40:395-403. performance in the right visual field. This 5. Benton AL, Van Allen MW. Impairment in facial suggests that the balancing of right/left hemirecognition in patients with cerebral disease. sphere input may be the crucial maturational Cortex 1968;4:344-58. change. 6. DeRenzi E, Spinnler H. Facial recognition in brain damaged patients. Neurology 1966 ; 16 : We tested our subjects with the same facial 145-52. recognition paradigm that has shown a marked 7. Benton AL, Van Allen MW. Prosopagnosia and left field/right hemisphere advantage in corpus facial discrimination. J Neuroscience 1972;15 : callostomy patients [15]. Accuracy was simi167-72. 8. Assai G. Regression des troubles de la reconlar, but our subjects showed no field advantage. naissance des physiognomies de Ie memoire topoThis probably reflects the age at testing-both graphique chez un malade opere d'un hematome are 14. While not statistically significant the intracerebral parieto-temporal droit. Rev Neurol trend toward a right field/left hemisphere 1969; 121: 184-5. 9. Tzavaras A, Hecaen H, LeBras H. Le probleme advantage suggests that the left hemisphere in de la specificite du deficit de la reconnaissance du this age group is still crucial to the facial revisae humain. Neuropsychologia 1970;8:403-16. cognition process. The maturation pattern of 10. De Renzi E, Faglioni P, Spinnler H. The performface recognition suggests that deficits due to ance of patients with unilateral brain damage right posterior pathology may be masked in on facial recognition tasks. Cortex 1968;4 : 17-34. prepubertal children or in those tested during transition periods. The fact that facial dis- 11. Bertelson P, Vanhaelen H, Morais J . Left hemifield superiority and the extraction of phytortion occurred ictally suggests that bihemisiognomic information. In: Steel Russell I, Van spheric epileptic activity is necessary for such Hof MW, Berlucchi G, eds. Structure and function of cerebral commissures. London: McMillan, a deficit in this age group. 1979.
Acknowledgments This work was aided in part by an NIH grant F532HD06151 (National Research Service Award), and by a grant from United Cerebral Palsy to Dr. Ruth Nass, M.D. References 1. Damasio A, Damasio H, Van Hoesen GW. Prosopagnosia: anatomical basis and behavioral mechanisms. Neurology 1982;32:331-41.
52 Brain & Development, Vol 7, No 1, 1985
12. Carey S, Diamond R. Maturational determina-
tion of the development course of face encoding. In: Caplan D, ed. Biological studies of mental processes. Cambridge: MIT Press, 1980. 13. Yin R. Looking at upside.{)own faces. J Exp Bioi 1969 ; 18: 141-7 . 14. Levine S. Developmental changes in right hemisphere involvement in face recognition. Technical Report, University of Chicago, 1981. 15. Gazzaniga M, Smylie C. Facial recognition and brain asymmetries. Clues to underlying mechanisms. Ann Neural 1983;13:536-40.
A 14-year-old patient with a right parietal arteriovenous malformation presented with seizures characterized by metamorphopsia of faces. Unlike adults with right hemisphere pathology she performed like an age matched control on a task requiring recognition of unfamiliar faces. This likely reflects maturational changes in hemispheric dominance for face recognition. Nass R, Sinha S, Solomon G. Epileptic facial metamorphopsia. Brain Dev 1985; 7:50-2
Patients with complex partial seizures may have psychosensory symptoms for some time before the appearance of automatisms. If isolated psychosensory symptoms persist, a psychiatric explanation is often erroneously accepted. Particularly in a patient with lack of self reference in the illusions or hallucinations and with no previous emotional disturbance, a high index of suspicion can lead to diagnostic evaluation and in some patients treatment of a structural lesion.
Case Report A l4-year-old white right-handed female experienced several episodes of visual distortion. Frequently she reported that people looked different than their known appearance, e.g., "their faces looked older." At times the white walls of her kitchen appeared yellow or the shapes of objects appeared distorted. Consequently, the patient was referred to a psychiatrist. On one occasion several months later
From the Departments of Neurology and Pediatrics (RN, GS) and Child Psychiatry (SS), The New York Hospital-Cornell Medical College, New York. Received for publication: October 9, 1984. Accepted for publication: January 8, 1985.
Key words: Face recognition, epilepsy, metamorphopsia, right hemisphere dominance. Correspondence address: Ruth Nass, MD, The New York Hospital-Cornell Medical Center, 525 E 68th St, New York, NY 10021 , USA.
she continued to fill her glass with milk even though it was full . This incident lead her therapist to obtain a neurologic consult and an EEG because of suspicion of partial complex epilepsy. Neurologic examination was normal including funduscopic and visual fields. Electroencephalogram showed posterior temporal slow and sharp wave activity synchronously bilaterally , as well as independently from the right hemisphere. Visual evoked responses were normal. Computerized transaxial tomography (CT) scan revealed a calcified right parietal temporal occipital lesion which showed slight enhancement with contrast (Fig 1). Cerebral angiography was normal. The patient was treated with carbamazepine for seizures. At surgery an arteriovenous malformation with areas of thrombosis and calcification was partially excised. The patient was continued on carbamazepine and had rare episodes of visual distortions. Two and one half years later she developed severe headaches. The follow-up CT scan showed hemorrhage into the arteriovenous malformation. Surgery was performed and the arteriovenous malformation was again resected . Follow-up CT scan six months later showed a small residual calcified lesion. No further seiZures have occurred and the patient is still receiving carbamazepine. The intracerebral arteriovenous malformation was detected only by CT scan and not demonstrated by angiography in this patient. Although it is a vascular lesion, thrombosis and infarction within the lesion produced no changes on arteriography.
prosopagnosia - failed recognition of familiar faces-or difficulty on an experimental recognition matching task of unfamiliar faces, a review of these topics places her particular ictal deficit in perspective. Prosopagnosia occurs with bilateral lesions in the mesial occipitotemporal region [1] . Damasio et al suggest that failure of familiar face recognition could be caused by destruction of the face template, inaccessibility of the template, or blocking of the activation produced by the template. Although our patient had only a right occipitotemporal lesion, epileptiform activity on EEG was bilateral over this region, and could conceivably have produced such an ictal deficit. Our patient may have misactivated the template, with resultant distortion rather than failed recognition. Metamorphopsia for three dimensional faces with retained recognition has occasionally been reported [2-4]. Recognition of unfamiliar faces in the experimental situation is impaired primarily in Fig 1 CT scan shows a calcified contrast enhancing patients with right posterior lesions [5, 6] . lesion in the right parietal region. Some patients with· prosopagnosia perform satisfactorily on such tasks [7-10]. Using Methods and Results tachistoscopic assessment of visual hemifield Because of the ictal phenomenon of facial performance, a left visual field (LVF) advandistortion, the process of face recognition was tage, reflecting right hemisphere dominance, studied in this patient with a congenital right has been demonstrated for matching of unfamiliar faces in normal adults [11] . hemisphere lesion. Both the ability to recognize unfamiliar Facial Recognition faces and right hemisphere dominance evolve In the unfamiliar face recognition task teenage during childhood. Not only is the young child male and female faces were presented 6° to less competent at this task, compared even to the right or left of a central fixation stimulus the right brain damaged adult, but differences for 150 milliseconds. Immediately following in face encoding strategies are present: 1) stimulus presentation, the subject was required young children do not have the same difficulty to choose the matching face from an array of recognizing inverted faces as adults [12, 13]; 10 faces presented in full view. Seventy trials 2) younger children rely on more superficial, were given. Both this patient and an age, sex, piecemeal cues than the older child or adult handedness matched control made correct [12]; 3) children under 10 do not show the matches on 65% of the left visual field (LVF) usual LVF advantage for discriminating unand 78% of the right visual field (RVF) trials. familiar faces [14]. While these data are not The performance difference between the two conclusive, the strategy differences suggest that hemifields was not significant by T test. prior to puberty, facial encoding is mediated by the left hemisphere. Around puberty, a drop in total accuracy occurs, followed by a spurt Discussion to adult norms [12]. Physiological events at A frequent ictal phenomenon for this patient puberty may directly interfere with the funcwas the illusion that familiar faces looked old, tioning of the right posterior cortex, or may although they could be accurately identified. change the balance between left and right Thus, while our patient had neither classic hemisphere involvement in the face encoding.
Nass et al: Facial metamorphopsia 51
2. Bodamer J. Die Prosop-Agnosie. Arch Psychiatr Levine [14] found that a LVF advantage was Zeitschrift Neural 1947; 179:654. present at ages 10 and 12, but was absent at 3. Hecaen H, Albert M. Human neuropsychology. 8 years and transiently disappeared at 14. In New York: Wiley & Sons, 1978. contrast to 8-year-olds where absence of LVF 4. Whiteley AM, Warrington EK. Prosopagnosia: advantage was due to poor left field recognia clinical, psychological and anatomical study of three patients. J Neurol Neurosurg Psychiatry tion, absence at 14 was due to improved 1977;40:395-403. performance in the right visual field. This 5. Benton AL, Van Allen MW. Impairment in facial suggests that the balancing of right/left hemirecognition in patients with cerebral disease. sphere input may be the crucial maturational Cortex 1968;4:344-58. change. 6. DeRenzi E, Spinnler H. Facial recognition in brain damaged patients. Neurology 1966 ; 16 : We tested our subjects with the same facial 145-52. recognition paradigm that has shown a marked 7. Benton AL, Van Allen MW. Prosopagnosia and left field/right hemisphere advantage in corpus facial discrimination. J Neuroscience 1972;15 : callostomy patients [15]. Accuracy was simi167-72. 8. Assai G. Regression des troubles de la reconlar, but our subjects showed no field advantage. naissance des physiognomies de Ie memoire topoThis probably reflects the age at testing-both graphique chez un malade opere d'un hematome are 14. While not statistically significant the intracerebral parieto-temporal droit. Rev Neurol trend toward a right field/left hemisphere 1969; 121: 184-5. 9. Tzavaras A, Hecaen H, LeBras H. Le probleme advantage suggests that the left hemisphere in de la specificite du deficit de la reconnaissance du this age group is still crucial to the facial revisae humain. Neuropsychologia 1970;8:403-16. cognition process. The maturation pattern of 10. De Renzi E, Faglioni P, Spinnler H. The performface recognition suggests that deficits due to ance of patients with unilateral brain damage right posterior pathology may be masked in on facial recognition tasks. Cortex 1968;4 : 17-34. prepubertal children or in those tested during transition periods. The fact that facial dis- 11. Bertelson P, Vanhaelen H, Morais J . Left hemifield superiority and the extraction of phytortion occurred ictally suggests that bihemisiognomic information. In: Steel Russell I, Van spheric epileptic activity is necessary for such Hof MW, Berlucchi G, eds. Structure and function of cerebral commissures. London: McMillan, a deficit in this age group. 1979.
Acknowledgments This work was aided in part by an NIH grant F532HD06151 (National Research Service Award), and by a grant from United Cerebral Palsy to Dr. Ruth Nass, M.D. References 1. Damasio A, Damasio H, Van Hoesen GW. Prosopagnosia: anatomical basis and behavioral mechanisms. Neurology 1982;32:331-41.
52 Brain & Development, Vol 7, No 1, 1985
12. Carey S, Diamond R. Maturational determina-
tion of the development course of face encoding. In: Caplan D, ed. Biological studies of mental processes. Cambridge: MIT Press, 1980. 13. Yin R. Looking at upside.{)own faces. J Exp Bioi 1969 ; 18: 141-7 . 14. Levine S. Developmental changes in right hemisphere involvement in face recognition. Technical Report, University of Chicago, 1981. 15. Gazzaniga M, Smylie C. Facial recognition and brain asymmetries. Clues to underlying mechanisms. Ann Neural 1983;13:536-40.