Abstract
Background
The aim of this work is to investigate the facial distortion (dysmorphopsia) experienced by patients with homonymous paracentral scotomas and to analyze the interrelationship with the previously described “thin man” phenomenon.
Methods
Routine neuro-ophthalmological examination and brain MRI in three patients who suffered from small homonymous paracentral scotomas due to infarction or arteriovenous malformations of the occipital lobe. They all complained of distortion and shrinkage of their interlocutor’s face contralateral to the brain lesion. The phenomenon appeared some seconds after steady fixation on the interlocutor’s nose and was evident with both left and right homonymous scotomas. The patients did not notice a gap in the area corresponding to the scotoma and objects other than faces were perceived normally.
Results
Homonymous paracentral scotomas can lead to focal displacement of facial features towards the center of the field defect with resulting distortion of the face on the affected side. This so-called “dysmorphopsia” makes faces appear regionally narrower than they are in reality and may be induced even by visual field defects that remain undetected by conventional perimetry using 6° × 6° grids. Predilection for faces is probably associated with the superior location of scotomas or specific impairment of face processing abilities related to the lesion site.
Conclusions
Facial dysmorphopsia is most probably associated with cortical “filling-in” and spatial distortion, and can hence be regarded as a special entity of the “thin man” phenomenon.
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References
Safran AB, Landis T (1996) Plasticity in the adult visual cortex: implications for the diagnosis of visual field defects and visual rehabilitation. Curr Opin Ophthalmol 7:53–64
Nelles G, Widman G, de Greiff A, Meistrowitz A, Dimitrova A, Weber J, Forsting M, Esser J, Diener HC (2002) Brain representation of hemifield stimulation in poststroke visual field effects. Stroke 33:1286–1293
Ramachandran VS (1992) Blind spots. Sci Am 1992:89–91
Safran AB, Achard O, Duret F, Landis T (1999) The “thin man” phenomenon: a sign of cortical plasticity following inferior homonymous paracentral scotoma. Br J Ophthalmol 83:137–142
Blom JD (2010) A dictionary of hallucinations. Springer, Berlin Heidelberg New York
Ebata S, Ogawa M, Tanaka Y, Mizuno Y, Yoshida M (1991) Apparent reduction in the size of one side of the face associated with a small retrosplenial haemorrhage. J Neurol Neurosurg Psychiatry 54:68–70
Smith DL (1980) Micropsia: an uncommon pediatric symptom. Clin Pediatr 19:294–298
Golden GS (1979) The Alice in Wonderland syndrome in juvenile migraine. Pediatrics 63:517–519
Cooperman SM (1977) ‘Alice in Wonderland’ syndrome as a presenting symptom of infectious mononucleosis in children: a description of three affected young people. Clin Pediatr 16:143–146
Iruela L, Ibanez-Rojo V, Baca E (1993) Zolpidem-induced macropsia in anorexic woman. Lancet 342:443–444
Brust JC, Behrens MM (1977) "Release hallucinations" as the major symptom of posterior cerebral artery occlusion: a report of 2 cases. Ann Neurol 2:432–436
Cohen L, Gray F, Meyrignac C, Dehaene S, Degos JD (1994) Selective deficit of visual size perception: two cases of hemimicropsia. J Neurol Neurosurg Psychiatry 57:73–78
Frassinetti F, Nichelli P, di Pellegrino G (1999) Selective horizontal dysmetropsia following prestriate lesion. Brain 122:339–350
Safran AB, Rilliet B, De Tribolet ND, Landis T (2000) Perceptual distortion around homonymous scotomas is not restricted to defects located in the right hemifield. Br J Ophthalmol 84:803–804
Komatsu H (2006) The neural mechanisms of perceptual filling-in. Nat Rev Neurosci 7:220–231
Darian-Smith C, Gilbert CD (1994) Axonal sprouting accompanies functional reorganization in adult cat striate cortex. Nature 21:737–740
Cynader M (2000) Perspectives: neuroscience. Strengthening visual connections. Science 17:1943–1944
Spillmann L, De Weerd P (2003) Mechanisms of surface completion: perceptual filling-in of texture. In: Pessoa L, DeWeerd P (eds) Filling-in: From Perceptual Completion to Cortical Reorganization. Oxford University Press, Oxford, pp 81–105
Kölmel HW (1988) Die homonymen Hemianopsien – Klinik und Pathophysiologie zentraler Sehstörungen. Springer, Berlin Heidelberg New York
Taylor I, Scheffer IE, Berkovic SF (2003) Occipital epilepsies: identification of specific and newly recognized syndromes. Brain 126:753–769
Lance JW (1976) Simple formed hallucinations confined to the area of a specific visual field defect. Brain 99:719–734
Kolb M, Petersen D, Schiefer U, Kolb R, Skalej M (1995) Scotoma perception in white-noise-field campimetry and postchiasmal visual pathway lesions. Ger J Ophthalmol 4:228–233
Mavrakanas NA, Dang-Burgener NP, Lorincz EN, Landis T, Safran AB (2009) Perceptual distortion in homonymous paracentral scotomas. J Neuroophthalmol 29:37–42
Competing interests
None. The authors have full control of all primary data and they agree to allow Graefe’s Archive for Clinical and Experimental Ophthalmology to review the data upon request.
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Martin Ganssauge and Eleni Papageorgiou contributed equally to this work.
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Ganssauge, M., Papageorgiou, E. & Schiefer, U. Facial dysmorphopsia: a notable variant of the "thin man" phenomenon?. Graefes Arch Clin Exp Ophthalmol 250, 1491–1497 (2012). https://doi.org/10.1007/s00417-012-1958-z
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DOI: https://doi.org/10.1007/s00417-012-1958-z