disorder.’ Sociery j o r Neuroscience Abstracts, 15(1), 302. blesulam, hl.-M. (1985) ‘Patterns in behavioral neuroanatomy: association areas, the limbic system and hemispheric specialisat ion. In Principles of Behavioral Neurology. Philadelphia: F. A. Davis. Mork. S. J.. Lindcgaard, K . , Halvorsen, T. B., Lehmann, E. H., Solgaard, T., Hatlevoll, R., Harvci, S., Ganz, J . (1985) ‘Oligodendroglioma: incidence and biological bchavior in ,a defined
. population.’ Journal of Neurosurgery, 63,
Visual Hallucinations after Acute Visual Loss in a Young Child
glioma) an 18-month course of chemotherapy was instituted. At the age of 2 % years his.optic discs were noted to be pale and his recognition visual acuity was 6/30 in both eyes. There were no other neurological abnormalities and he was otherwise well. CT scanning showed the tumour to have not increased in size. At the age of 3’1: years he was found to have a left temporal ficld loss, with no change in visual acuity. Over the next six months his vision gradually deteriorated until he had only light perception in his right eye and 6/60 in his left eye with severe peripheral field loss. A repeat C T scan revealed an increase in rhe size of the tumour and it was decided to recommence chemotherapy and debulk thc tumour. After surgery the child was torally blind; the postopcrative course otherwise was uneventful. One week after the operation he suddenly began to experience visual hallucinations: he saw people, including his brother and Santa Claus, animals and other familiar objects. The hallucinations moved around in both visual fields. They lasted for three days, abating only in sleep. No provoking factors were identified. There were no auditory, olfactory or tactile accompaniments. Initially he was frightened by the hallucinations, and became angry when told that they were imaginary. There was no previous history of visual hallucinations. The child’s mental status appeared normal apart from the visual hallucinations, and neurological and fundoscopic examinations were unchanged apart from the sudden visual loss. He was receiving no. medication and was afebrile. There was no evidence of ‘electrolyte imbalance o r other pituitary dysfunction. An electroencephalogram was normal and a repeat C T scan showed a significant reduction in the size of the tumour, but no new abnormalities.
C. P. White J. E. Jan
’
Visual hallucinations are common manifestations of toxic confusional states and psychoses. They may also arise secondary to lesions of the visual pathway anywhere from the eye to the cortex in otherwise healthy people. They are most commonly reported in older individuals with gradually deteriorating vision from a variety of causes, and are thought of as release phenomena (Cogan 1973). Rarely, they may be a manifestation of seizure activity in the occipital or temporal regions (Russell and Whitty 1955, Lance 1976). Hallucinations with visual loss have not been reported previously in young children.
Case report At the age of I 1 months the patient presented with a four-week history of nysragmus. Pregnancy and delivery had been normal, as had been his development until that time. Initial cxamination revealed the presence of an involuntary, bilateral, conjugate nystagmus of similar amplitude in both eyes. The horizontal nature of the nystagmus was maintained in upgaze. Neurological examination was unremarkable and there was no evidence of neurofibromatosis. A C T scan revealed the presence of a large suprasellar tumour extending posteriorly along the optic radiations. Following stereotactic biopsy (confirming the presence o f a low-grade optic
881-889. Ornitz, E. M. (1985) ‘Neurophysiology of infantile autism.’ Journal o/ rhe Amerjcan Acadenty of Child Psychiatry, 24, 25 1-262. Reiss. A. L., Fcinstein, C., Rosenbaum, K . N. (1986) ‘Autism and genetic disorders.’ Schizophrenia Bulletin, 12, 724-738. Tomira, T.. McLone, D. G . (1985) ‘Brain tumors during the first twenty-four months of life.’ Neurosurgery, 17, 913-919.
Discussion In 1769 Charles Bonnet published an account of the visual hallucinations experienced by his grandfather, an 89year-old man with severely decreased visual ‘acuity as a result of bilateral cataracts, who otherwise was in excellent physical and mental health. Since then there have been a number of other reports of such phenomena occurring in individuals, especially the elderly, in the absence of psychiatric disorder (DamasMora el al. 1982, Olbrich et al. 1987), and
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the phenomenon is often referred to as Charles Bonnet syndrome. It has been recognised that visual hallucinations can occur after visual loss due to disease of other areas of the visual pathway (Cogan 1973) and with field defects in the presence of otherwise normal visual acuity (Lance 1976). In 1973 Cogan suggested that visual hallucinations occurring in the absence of psychiatric disorder be divided into two categories: those occurring in the presence of an irritative cerebral lesion and those occurring with deteriorating vision. Hallucinations occurring secondary to an irritative lesion are thought to be an ictal phenomenon resulting from epileptic discharges from lesions usually located in the occipital or temporal cortex (Penfield and Perot 1963, Lance 1976). When sensory deprivation occurs as a result of deteriorating vision, visual hallucinations are ‘released’ by the removal of normal visual input. Visual hallucinations may be simple or elementary phenomena consisting of photopsias-basic geometric or unformed shapes. More elaborate visions, such as people, faces and everyday objects, are classified as complex phenomena. Ictal visual hallucinations may be simple or complex, but are characteristically brief, intermittent and stereotyped. They most often are associated with sensory or motor symptomatology, suggesting that , the process is epileptic in nature (Lance 1976). The hallucinations ‘released’ by deteriorating vision may also be simple or complex: they are usually continuous, colourful and often moving, and while the theme may be fixed the ’mages are not repetitive or stereotyped (Cogan 1973). Release phenomena are more common than previously thought, because people are reluctant to report such experiences for fear of being thought disturbed. In a recent study of 104 consecutive cases with retinal or neural visual loss, Lepore (1990) found that 57 per cent of individuals had experienced elementary or complex visual phenomena. Although more commonly reported in the context of gradual visual loss, they have also been noted following sudden visual loss (Cogan 1973, Olbrich et al. 1987). In adults, gradual visual deterioration is far more common than
sudden visual loss. Both these situations are rare in childhood, where the most common causes of severe visual impairment are congenital and non-progressive (Robinson el al. 1987). Release visual hallucinations may arise in individuals with both ocular and cerebral pathology, involving the visual system anywhere from the eye to the occipital cortex, and do not help to locate the lesion (Weinberger and Grant 1940, Cogan 1973, Lepore 1990). The greater the decrease in visual acuity, and thus visual input, the more likely the emergence of hallucinations (Lepore 1990); improvement in vision may result in the cessation of the hallucinations (Olbrich et al. 1987). Similarly, when normal individuals are visually deprived they begin to experience visual hallucinations-initially simple, becoming complex later (Heron er al. 1956). These observations support the hypothesis that these are release phenomena. In this patient the hallucinations were complex, continuous and occurred in the context of sudden total visual lossfactors suggesting that they were release phenomena. There were no associated disorders of thought or personality and no other clinical explanation, such as fever, to account for the phenomena. Complex release visual hallucinations may be more common in children than previously supposed, since children may not be able clearly to articulate or describe their hallucinations, so their caregivers may misinterpret their complaints as being due to emotional or other causes. The knowledge that release visual hallucinations can occur in children with gradual and abrupt severe bilateral visual loss, and that they are benign in nature, is important to avoid misdiagnosis and inappropriate intervention. These children require only understanding and reassurance. Accepted for publicaiion 3rd Ociober 1991. Auiliors’ Appornrnienrs ‘C. P. White, M.B., B.S., M.R.C.P. (UK), Fellow; J. E. Jan, M.D., F.R.C.P. (C), Professor; Division of Child Neurology, University of British Columbia, British Columbia’s Children’s Hospital, 4480 Oak Street, Vancouver. ‘Correspondence 10 firsi auihor at Department of Neurology, Royal Liverpool Children’s Hospital, Eaton Road, Liverpool LIZ 2AP.
SUMMARY A four-year-old boy developed complex visual hallucinations consisting of moving figures, animals and other familiar objects one week after becoming totally blind. This followed a six-month period of gradually deteriorating vision and was precipitated by the debulking of a large optic nerve glioma. Although visual hallucinations are well recognised in adults with visual loss, secondary to both ophthalmological and neurological causes, they have not been reported in young children. RESUME Hallucina,ions visuelles apres line perte aigue de la vision chez un jeirne enfani L’article decrit un garcon d e quatre ans qui presenta des hallucinations visuelles complexes, consistant en figures mobiles, animaux et autres objets familiers, une semaine apres Ctre devenu totalement aveugle. Cela survint apres une periode de perte partielle d e la vision de six mois et une aggravation apres ablation d’un gliome d e nerf optique. Les hallucinations visuelles apres perte visucllc, sccondaire a des causes ophtalmologiques ou nrurologiques, sont bien connues chez I’adulte mais n’avaient pas encore ete decrites chez le jeune enfant.
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ZUSAMMENFASSUNG Visuelle Hallazinationen nach akiiiern Sehverlust bei einem Kleinkind Ein vierjahriger Junge cntwickelte eine Woche nach totaler Erblindung komplexe visuelle Halluzinationen, bestehend aus bewegten Figuren, Tieren und anderen vertrauten Objekten. Dies trat nach einer Zeitspanne von seche Monaten auf, in der sich das Sehvermogen nach und nach verschlechtert hatte, und war bedingr durch ein grones Gliom des N. opticus. Obwohl visuelle Halluzinationen bei Erwachsenen rnit Sehverlust sowohl aufgrund ophthalmoligscher als auch neurologischer Ursachen gut bekannt sind, sind sie bei Kindern noch nicht beschrieben worden. RESUMEN Alircinaciones visuales despues de la perdida de la vision en un niiio pequeito Un muchacho de cuatro aiios de edad, una semana despues de quedar totalmente ciego, sufrio unas alucinaciones visuales complcjas consistentes en figuras moviles, animales y otros objetos familiares. Esto occurio despues d e un period0 de deterioracion gradual d e la vision y se precipito por el crccimiento de un gran glioma del nervio optico. Si bien las alucinaciones visuales son bien conocidas en adultos con perdida de vision, secundariamente a causas oftalmologicas y neurologicas, no se habian comunicado en el caso de niiios pequeiios. References Bonnet, C. (1769) Essai Analytique sur les Facultes de I’Ame, 2nd Edn., Vol. 2. Copenhagen and Geneva: Philibert. Cogan, D. G. (1973) ‘Visual hallucinations as release phenomena.’ Craefe’s Archive f o r Clinical and Experimental Ophthalmology. 188, 139-1 50. Damas-Mora, J., Skelton-Robinson, M., Jenner, F. A . (1982) ‘The Charles Bonnet syndrome in perspective.’ Psychological Medicine, 12, 25 1-261. Heron, W., Doane, B. K . , Scott, T. H. (1956) ‘Visual disturbances after prolonged perceptual isolation.’ Canadian Journal of Psychology. 10, 13-18, Lance, J . W. (1976) ‘Simple formed hallucinations confined to the area of a specific visual field defect.’ Brain, 99, 719-734. Leporc, F. E. (1990) ‘Spontaneous visual phenomena with visual loss: 104 patients with
lesions of the retinal and neural afferent pathways.’ Neurology, 40, 441-447. Olbrich, H . M.,Engclmeier, bl. P., Pauleikhoff, D., Waubke, T. (1987) ‘Visual hallucinations in ophthalmology.’ Graefe’s Archive f o r Clinical and Experimental Ophthalmology, 225, 2 17-220. Penfield, W., Perot. P. (1963) ‘The brain’s record of auditory and visual experience.’ Brain, 86, 595-696. Robinson, G . C., Jan, J. E., Kinnis, C. (1987) ‘Conaenital ocular blindness in children. 1945 to 1984.’ American Journal of Diseases of Children, 141, 1321-1324. Russell, W. R . , Whitty, C. W. M. (1955) ‘Studies in traumatic eoilemv. . . . 111: Visual fits.’ Journal o f Neurology, Neurosurgery and Psychiatry, I a, 79-96. Weinberger, L. M., Grant, F. C. (1940) ‘Visual hallucinations and their neuro-optical correlates.’ Archives of Ophthaltnology, 23, 166-199.
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. population.’ Journal of Neurosurgery, 63,
Visual Hallucinations after Acute Visual Loss in a Young Child
glioma) an 18-month course of chemotherapy was instituted. At the age of 2 % years his.optic discs were noted to be pale and his recognition visual acuity was 6/30 in both eyes. There were no other neurological abnormalities and he was otherwise well. CT scanning showed the tumour to have not increased in size. At the age of 3’1: years he was found to have a left temporal ficld loss, with no change in visual acuity. Over the next six months his vision gradually deteriorated until he had only light perception in his right eye and 6/60 in his left eye with severe peripheral field loss. A repeat C T scan revealed an increase in rhe size of the tumour and it was decided to recommence chemotherapy and debulk thc tumour. After surgery the child was torally blind; the postopcrative course otherwise was uneventful. One week after the operation he suddenly began to experience visual hallucinations: he saw people, including his brother and Santa Claus, animals and other familiar objects. The hallucinations moved around in both visual fields. They lasted for three days, abating only in sleep. No provoking factors were identified. There were no auditory, olfactory or tactile accompaniments. Initially he was frightened by the hallucinations, and became angry when told that they were imaginary. There was no previous history of visual hallucinations. The child’s mental status appeared normal apart from the visual hallucinations, and neurological and fundoscopic examinations were unchanged apart from the sudden visual loss. He was receiving no. medication and was afebrile. There was no evidence of ‘electrolyte imbalance o r other pituitary dysfunction. An electroencephalogram was normal and a repeat C T scan showed a significant reduction in the size of the tumour, but no new abnormalities.
C. P. White J. E. Jan
’
Visual hallucinations are common manifestations of toxic confusional states and psychoses. They may also arise secondary to lesions of the visual pathway anywhere from the eye to the cortex in otherwise healthy people. They are most commonly reported in older individuals with gradually deteriorating vision from a variety of causes, and are thought of as release phenomena (Cogan 1973). Rarely, they may be a manifestation of seizure activity in the occipital or temporal regions (Russell and Whitty 1955, Lance 1976). Hallucinations with visual loss have not been reported previously in young children.
Case report At the age of I 1 months the patient presented with a four-week history of nysragmus. Pregnancy and delivery had been normal, as had been his development until that time. Initial cxamination revealed the presence of an involuntary, bilateral, conjugate nystagmus of similar amplitude in both eyes. The horizontal nature of the nystagmus was maintained in upgaze. Neurological examination was unremarkable and there was no evidence of neurofibromatosis. A C T scan revealed the presence of a large suprasellar tumour extending posteriorly along the optic radiations. Following stereotactic biopsy (confirming the presence o f a low-grade optic
881-889. Ornitz, E. M. (1985) ‘Neurophysiology of infantile autism.’ Journal o/ rhe Amerjcan Acadenty of Child Psychiatry, 24, 25 1-262. Reiss. A. L., Fcinstein, C., Rosenbaum, K . N. (1986) ‘Autism and genetic disorders.’ Schizophrenia Bulletin, 12, 724-738. Tomira, T.. McLone, D. G . (1985) ‘Brain tumors during the first twenty-four months of life.’ Neurosurgery, 17, 913-919.
Discussion In 1769 Charles Bonnet published an account of the visual hallucinations experienced by his grandfather, an 89year-old man with severely decreased visual ‘acuity as a result of bilateral cataracts, who otherwise was in excellent physical and mental health. Since then there have been a number of other reports of such phenomena occurring in individuals, especially the elderly, in the absence of psychiatric disorder (DamasMora el al. 1982, Olbrich et al. 1987), and
m v.
m-
259
-z v)
e cc: w d
260
the phenomenon is often referred to as Charles Bonnet syndrome. It has been recognised that visual hallucinations can occur after visual loss due to disease of other areas of the visual pathway (Cogan 1973) and with field defects in the presence of otherwise normal visual acuity (Lance 1976). In 1973 Cogan suggested that visual hallucinations occurring in the absence of psychiatric disorder be divided into two categories: those occurring in the presence of an irritative cerebral lesion and those occurring with deteriorating vision. Hallucinations occurring secondary to an irritative lesion are thought to be an ictal phenomenon resulting from epileptic discharges from lesions usually located in the occipital or temporal cortex (Penfield and Perot 1963, Lance 1976). When sensory deprivation occurs as a result of deteriorating vision, visual hallucinations are ‘released’ by the removal of normal visual input. Visual hallucinations may be simple or elementary phenomena consisting of photopsias-basic geometric or unformed shapes. More elaborate visions, such as people, faces and everyday objects, are classified as complex phenomena. Ictal visual hallucinations may be simple or complex, but are characteristically brief, intermittent and stereotyped. They most often are associated with sensory or motor symptomatology, suggesting that , the process is epileptic in nature (Lance 1976). The hallucinations ‘released’ by deteriorating vision may also be simple or complex: they are usually continuous, colourful and often moving, and while the theme may be fixed the ’mages are not repetitive or stereotyped (Cogan 1973). Release phenomena are more common than previously thought, because people are reluctant to report such experiences for fear of being thought disturbed. In a recent study of 104 consecutive cases with retinal or neural visual loss, Lepore (1990) found that 57 per cent of individuals had experienced elementary or complex visual phenomena. Although more commonly reported in the context of gradual visual loss, they have also been noted following sudden visual loss (Cogan 1973, Olbrich et al. 1987). In adults, gradual visual deterioration is far more common than
sudden visual loss. Both these situations are rare in childhood, where the most common causes of severe visual impairment are congenital and non-progressive (Robinson el al. 1987). Release visual hallucinations may arise in individuals with both ocular and cerebral pathology, involving the visual system anywhere from the eye to the occipital cortex, and do not help to locate the lesion (Weinberger and Grant 1940, Cogan 1973, Lepore 1990). The greater the decrease in visual acuity, and thus visual input, the more likely the emergence of hallucinations (Lepore 1990); improvement in vision may result in the cessation of the hallucinations (Olbrich et al. 1987). Similarly, when normal individuals are visually deprived they begin to experience visual hallucinations-initially simple, becoming complex later (Heron er al. 1956). These observations support the hypothesis that these are release phenomena. In this patient the hallucinations were complex, continuous and occurred in the context of sudden total visual lossfactors suggesting that they were release phenomena. There were no associated disorders of thought or personality and no other clinical explanation, such as fever, to account for the phenomena. Complex release visual hallucinations may be more common in children than previously supposed, since children may not be able clearly to articulate or describe their hallucinations, so their caregivers may misinterpret their complaints as being due to emotional or other causes. The knowledge that release visual hallucinations can occur in children with gradual and abrupt severe bilateral visual loss, and that they are benign in nature, is important to avoid misdiagnosis and inappropriate intervention. These children require only understanding and reassurance. Accepted for publicaiion 3rd Ociober 1991. Auiliors’ Appornrnienrs ‘C. P. White, M.B., B.S., M.R.C.P. (UK), Fellow; J. E. Jan, M.D., F.R.C.P. (C), Professor; Division of Child Neurology, University of British Columbia, British Columbia’s Children’s Hospital, 4480 Oak Street, Vancouver. ‘Correspondence 10 firsi auihor at Department of Neurology, Royal Liverpool Children’s Hospital, Eaton Road, Liverpool LIZ 2AP.
SUMMARY A four-year-old boy developed complex visual hallucinations consisting of moving figures, animals and other familiar objects one week after becoming totally blind. This followed a six-month period of gradually deteriorating vision and was precipitated by the debulking of a large optic nerve glioma. Although visual hallucinations are well recognised in adults with visual loss, secondary to both ophthalmological and neurological causes, they have not been reported in young children. RESUME Hallucina,ions visuelles apres line perte aigue de la vision chez un jeirne enfani L’article decrit un garcon d e quatre ans qui presenta des hallucinations visuelles complexes, consistant en figures mobiles, animaux et autres objets familiers, une semaine apres Ctre devenu totalement aveugle. Cela survint apres une periode de perte partielle d e la vision de six mois et une aggravation apres ablation d’un gliome d e nerf optique. Les hallucinations visuelles apres perte visucllc, sccondaire a des causes ophtalmologiques ou nrurologiques, sont bien connues chez I’adulte mais n’avaient pas encore ete decrites chez le jeune enfant.
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ZUSAMMENFASSUNG Visuelle Hallazinationen nach akiiiern Sehverlust bei einem Kleinkind Ein vierjahriger Junge cntwickelte eine Woche nach totaler Erblindung komplexe visuelle Halluzinationen, bestehend aus bewegten Figuren, Tieren und anderen vertrauten Objekten. Dies trat nach einer Zeitspanne von seche Monaten auf, in der sich das Sehvermogen nach und nach verschlechtert hatte, und war bedingr durch ein grones Gliom des N. opticus. Obwohl visuelle Halluzinationen bei Erwachsenen rnit Sehverlust sowohl aufgrund ophthalmoligscher als auch neurologischer Ursachen gut bekannt sind, sind sie bei Kindern noch nicht beschrieben worden. RESUMEN Alircinaciones visuales despues de la perdida de la vision en un niiio pequeito Un muchacho de cuatro aiios de edad, una semana despues de quedar totalmente ciego, sufrio unas alucinaciones visuales complcjas consistentes en figuras moviles, animales y otros objetos familiares. Esto occurio despues d e un period0 de deterioracion gradual d e la vision y se precipito por el crccimiento de un gran glioma del nervio optico. Si bien las alucinaciones visuales son bien conocidas en adultos con perdida de vision, secundariamente a causas oftalmologicas y neurologicas, no se habian comunicado en el caso de niiios pequeiios. References Bonnet, C. (1769) Essai Analytique sur les Facultes de I’Ame, 2nd Edn., Vol. 2. Copenhagen and Geneva: Philibert. Cogan, D. G. (1973) ‘Visual hallucinations as release phenomena.’ Craefe’s Archive f o r Clinical and Experimental Ophthalmology. 188, 139-1 50. Damas-Mora, J., Skelton-Robinson, M., Jenner, F. A . (1982) ‘The Charles Bonnet syndrome in perspective.’ Psychological Medicine, 12, 25 1-261. Heron, W., Doane, B. K . , Scott, T. H. (1956) ‘Visual disturbances after prolonged perceptual isolation.’ Canadian Journal of Psychology. 10, 13-18, Lance, J . W. (1976) ‘Simple formed hallucinations confined to the area of a specific visual field defect.’ Brain, 99, 719-734. Leporc, F. E. (1990) ‘Spontaneous visual phenomena with visual loss: 104 patients with
lesions of the retinal and neural afferent pathways.’ Neurology, 40, 441-447. Olbrich, H . M.,Engclmeier, bl. P., Pauleikhoff, D., Waubke, T. (1987) ‘Visual hallucinations in ophthalmology.’ Graefe’s Archive f o r Clinical and Experimental Ophthalmology, 225, 2 17-220. Penfield, W., Perot. P. (1963) ‘The brain’s record of auditory and visual experience.’ Brain, 86, 595-696. Robinson, G . C., Jan, J. E., Kinnis, C. (1987) ‘Conaenital ocular blindness in children. 1945 to 1984.’ American Journal of Diseases of Children, 141, 1321-1324. Russell, W. R . , Whitty, C. W. M. (1955) ‘Studies in traumatic eoilemv. . . . 111: Visual fits.’ Journal o f Neurology, Neurosurgery and Psychiatry, I a, 79-96. Weinberger, L. M., Grant, F. C. (1940) ‘Visual hallucinations and their neuro-optical correlates.’ Archives of Ophthaltnology, 23, 166-199.
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