Tobacco-alcohol amblyopia: a maculopathy?
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《英国眼科学杂志》
Neuroophthalmology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, USA
Keywords: tobacco-alcohol amblyopia; maculopathy
Tobacco-alcohol amblyopia or toxic-nutritional optic neuropathy is a condition characterised by papillomacular bundle damage, central or caecocentral scotoma, and reduction of colour vision in a patient who abuses tobacco and alcohol.1,2 There is consensus that nutritional deficiency has an important role as well.3,4 The appearance of the optic nerve is usually normal, but peripapillary dilated vessels and haemorrhages have been described.5,6 Testing with static perimetry often reveals central scotomas. Although this syndrome has been classified as optic neuropathy, the primary lesion has not actually been localised to the optic nerve and may possibly originate in the retina, chiasm, or even the optic tracts. We report two cases of tobacco-alcohol amblyopia and their electrophysiological findings after testing with multifocal electroretinography (MERG).
Case reports
Case 1
A 47 year old woman presented with gradual decrease in vision over 4 months. Her medical history showed that she has been in excellent health. She smoked one pack of cigarettes per week and had two to three beers daily. She denied any use of any medications in the past few months. She and her husband have been on a diet which contained fewer vegetables than their normal intake, for 4 months. Family history was unremarkable.
Visual acuity was 20/50 right eye and 20/100 left eye. Colour vision using the pseudoisochromatic plates was four of eight in right eye and two of eight in left eye. Intraocular pressure was 12 mm Hg right eye and 15 mm Hg left eye. She had normal anterior segment in both eyes. Her pupils were sluggish to direct stimulation of light with no afferent defect. Ocular motility was normal. Funduscopy showed anomalous optic nerves with no pallor, and normal maculas. Testing with 24-2 static perimetry revealed an inferior and nasal defect in the right eye; superonasal, inferior, and central defect in the left eye (fig 1A). Humphrey 10-2 static perimetry showed bilateral caecocentral scotomas (fig 1B). Magnetic resonance imaging (MRI) of the brain and orbit with and without contrast was normal. Serology tests for Lyme and antinuclear antibodies (ANA) were negative. Complete blood count, serum vitamin B12, and folate were within normal limits. MERG testing showed severe reduction in amplitude mostly centrally in both eyes (fig 2).
Figure 1 Static perimetry using (A) the 24-2 program shows an inferior arcuate defect in the right eye; a superonasal and an inferior defect in the left eye. (B) The 10-2 program shows bilateral central defects.
Figure 2 Trace arrays of multifocal ERG showing decreased amplitudes in both the right eye (A) and the left eye (B) with almost isoelectric responses centrally and improvement towards the periphery.
Case 2
A 55 year old woman presented with progressive decrease in vision of both eyes over 1 month. She had a history of multiple intracranial aneurysms that were clipped 15 years earlier. She was not using any medications. She smoked one pack of cigarette a day for 25 years and has five to eight drinks per week. Family history was positive for glaucoma in her mother. Visual acuity was counting fingers at 1 foot right eye and at 2 feet left eye. She could not identify any of the pseudoisochromatic colour plates in both eyes. She had normal anterior segment in both eyes. Pupillary reactions were sluggish to light stimulation with no afferent defect. Funduscopy showed mildly swollen optic nerves in both eyes (fig 3). Kinetic perimetry showed bilateral central scotomas. A CT scan (with and without contrast agent) of the brain and orbit was normal. Complete blood count, serum vitamin B12, and folate were within normal limits. Genetic testing of mitochondrial DNA for Leber’s hereditary optic neuropathy showed that the patient has the LHON 3460 G mutation. Multifocal ERG was performed and showed decreased amplitudes centrally with normal latency (fig 4).
Figure 3 Fundus photographs showing swollen discs with some telangiectatic vessels in both eyes. (A) Right eye, (B) left eye.
Figure 4 Trace arrays of patient 2 showing reduced amplitude in the central region of both eyes.
Comment
We describe two cases of "tobacco-alcohol amblyopia in patients who had a history of high alcohol intake (cases 1 and 2) and shortly after dietary alteration (case 1). In both cases, MERG testing showed decreased amplitudes in the central region, suggesting retinal dysfunction in the macula. The condition of the patient in case 2 may have been precipitated by a metabolic injury (tobacco, alcohol) to genetically "compromised" mitochondria. This shows the clinical overlap in conditions of inherited mitochondrial dysfunction and acquired ones such as tobacco-alcohol amblyopia.7
The clinical findings seen in tobacco-alcohol amblyopia can occur in any disease of anterior visual pathway from the retina to the optic tract and there is there is little evidence to suggest that the locus of pathology is restricted to the optic nerve. Hhistopathological studies on animal models of nutritional amblyopia showed lesions in the retina, optic nerve and tract,8 and the maculopapillary bundle.9 Electrophysiological abnormalities in animal models of tobacco-alcohol amblyopia showed reduced amplitudes with normal latencies using visual evoked potentials,10,11 and increased a-wave and b-wave implicit times and decreased b-wave amplitudes using full field electroretinograms.12
MERG signals are believed to arise from the outer retina (photoreceptor and bipolar cell layer) with only minimal contribution from the inner retina and optic nerve (ganglion cells and nerve fibre layer).13 Therefore, the severe reduction in amplitude in our patients suggests that the outer retina, particularly in the macula, is involved in this condition.
References
Traquair HM. Toxic amblyopia, including retrobulbar neuritis. Trans Ophthalmol Soc UK 1930;50:351.
Golnik KC, Schaible ER. Folate-responsive optic neuropathy. J Neuro-ophthalmol 1994;14:163.
Victor M. Tobacco-alcohol amblyopia. A critique of current concepts of this disorder, with special reference to the role of nutritional deficiency in its causation. Arch Ophthalmol 1963;70:313.
Victor M, Mancall EL, Dreyfus PM. Deficiency amblyopia in the alcoholic patient: a clinicopathologic study. Arch Ophthalmol 1960;64:1.
Carroll FD. Nutritional amblyopia. Arch Ophthalmol 1966;76:406.
Frisén L. Fundus changes in acute nutritional amblyopia. Arch Ophthalmol 1983;101:577.
Cullom ME, Hayes KL, Miller NR, et al. Leber’s hereditary optic neuropathy masquerading as tobacco-alcohol amblyopia. Arch Ophthalmol 1993;111:1482.
Rodger FC. Degenerative changes in the rat visual pathway when thiamine and riboflavin deficiencies are combined. Br J Ophthalmol 1954;38:154.
Smiddy WE, Green WR. Nutritional amblyopia. A histopathologic study with retrospective clinical correlation. Graefes Arch Clin Exp Ophthalmol 1987;225:321–4.
Ikeda H, Tremain KE, Sanders MD. Neurophysiological investigation in optic nerve disease: Combined assessment of the visual evoked response and electroretinogram. Br J Ophthalmol 1978;62:227.
Kupersmith MJ, Weiss PA, Carr RE. The visual evoked potential in tobacco-alcohol amblyopia and nutritional amblyopia. Am J Ophthalmol 1983;95:307.
Pawlosky RJ, Bacher J, Salem N Jr. Ethanol consumption alters electroretinograms and depletes neural tissues of docosahexaenoic acid in rhesus monkeys: nutritional consequences of a low n-3 fatty acid diet. Alcohol Clin Exp Res 2001;25:1758–65.
Hood DC, Odel JG, Chen CS, et al. The multifocal electroretinogram. J Neuro-ophthalmol 2003;23:225–35.(R Behbehani, R C Sergott )
Keywords: tobacco-alcohol amblyopia; maculopathy
Tobacco-alcohol amblyopia or toxic-nutritional optic neuropathy is a condition characterised by papillomacular bundle damage, central or caecocentral scotoma, and reduction of colour vision in a patient who abuses tobacco and alcohol.1,2 There is consensus that nutritional deficiency has an important role as well.3,4 The appearance of the optic nerve is usually normal, but peripapillary dilated vessels and haemorrhages have been described.5,6 Testing with static perimetry often reveals central scotomas. Although this syndrome has been classified as optic neuropathy, the primary lesion has not actually been localised to the optic nerve and may possibly originate in the retina, chiasm, or even the optic tracts. We report two cases of tobacco-alcohol amblyopia and their electrophysiological findings after testing with multifocal electroretinography (MERG).
Case reports
Case 1
A 47 year old woman presented with gradual decrease in vision over 4 months. Her medical history showed that she has been in excellent health. She smoked one pack of cigarettes per week and had two to three beers daily. She denied any use of any medications in the past few months. She and her husband have been on a diet which contained fewer vegetables than their normal intake, for 4 months. Family history was unremarkable.
Visual acuity was 20/50 right eye and 20/100 left eye. Colour vision using the pseudoisochromatic plates was four of eight in right eye and two of eight in left eye. Intraocular pressure was 12 mm Hg right eye and 15 mm Hg left eye. She had normal anterior segment in both eyes. Her pupils were sluggish to direct stimulation of light with no afferent defect. Ocular motility was normal. Funduscopy showed anomalous optic nerves with no pallor, and normal maculas. Testing with 24-2 static perimetry revealed an inferior and nasal defect in the right eye; superonasal, inferior, and central defect in the left eye (fig 1A). Humphrey 10-2 static perimetry showed bilateral caecocentral scotomas (fig 1B). Magnetic resonance imaging (MRI) of the brain and orbit with and without contrast was normal. Serology tests for Lyme and antinuclear antibodies (ANA) were negative. Complete blood count, serum vitamin B12, and folate were within normal limits. MERG testing showed severe reduction in amplitude mostly centrally in both eyes (fig 2).
Figure 1 Static perimetry using (A) the 24-2 program shows an inferior arcuate defect in the right eye; a superonasal and an inferior defect in the left eye. (B) The 10-2 program shows bilateral central defects.
Figure 2 Trace arrays of multifocal ERG showing decreased amplitudes in both the right eye (A) and the left eye (B) with almost isoelectric responses centrally and improvement towards the periphery.
Case 2
A 55 year old woman presented with progressive decrease in vision of both eyes over 1 month. She had a history of multiple intracranial aneurysms that were clipped 15 years earlier. She was not using any medications. She smoked one pack of cigarette a day for 25 years and has five to eight drinks per week. Family history was positive for glaucoma in her mother. Visual acuity was counting fingers at 1 foot right eye and at 2 feet left eye. She could not identify any of the pseudoisochromatic colour plates in both eyes. She had normal anterior segment in both eyes. Pupillary reactions were sluggish to light stimulation with no afferent defect. Funduscopy showed mildly swollen optic nerves in both eyes (fig 3). Kinetic perimetry showed bilateral central scotomas. A CT scan (with and without contrast agent) of the brain and orbit was normal. Complete blood count, serum vitamin B12, and folate were within normal limits. Genetic testing of mitochondrial DNA for Leber’s hereditary optic neuropathy showed that the patient has the LHON 3460 G mutation. Multifocal ERG was performed and showed decreased amplitudes centrally with normal latency (fig 4).
Figure 3 Fundus photographs showing swollen discs with some telangiectatic vessels in both eyes. (A) Right eye, (B) left eye.
Figure 4 Trace arrays of patient 2 showing reduced amplitude in the central region of both eyes.
Comment
We describe two cases of "tobacco-alcohol amblyopia in patients who had a history of high alcohol intake (cases 1 and 2) and shortly after dietary alteration (case 1). In both cases, MERG testing showed decreased amplitudes in the central region, suggesting retinal dysfunction in the macula. The condition of the patient in case 2 may have been precipitated by a metabolic injury (tobacco, alcohol) to genetically "compromised" mitochondria. This shows the clinical overlap in conditions of inherited mitochondrial dysfunction and acquired ones such as tobacco-alcohol amblyopia.7
The clinical findings seen in tobacco-alcohol amblyopia can occur in any disease of anterior visual pathway from the retina to the optic tract and there is there is little evidence to suggest that the locus of pathology is restricted to the optic nerve. Hhistopathological studies on animal models of nutritional amblyopia showed lesions in the retina, optic nerve and tract,8 and the maculopapillary bundle.9 Electrophysiological abnormalities in animal models of tobacco-alcohol amblyopia showed reduced amplitudes with normal latencies using visual evoked potentials,10,11 and increased a-wave and b-wave implicit times and decreased b-wave amplitudes using full field electroretinograms.12
MERG signals are believed to arise from the outer retina (photoreceptor and bipolar cell layer) with only minimal contribution from the inner retina and optic nerve (ganglion cells and nerve fibre layer).13 Therefore, the severe reduction in amplitude in our patients suggests that the outer retina, particularly in the macula, is involved in this condition.
References
Traquair HM. Toxic amblyopia, including retrobulbar neuritis. Trans Ophthalmol Soc UK 1930;50:351.
Golnik KC, Schaible ER. Folate-responsive optic neuropathy. J Neuro-ophthalmol 1994;14:163.
Victor M. Tobacco-alcohol amblyopia. A critique of current concepts of this disorder, with special reference to the role of nutritional deficiency in its causation. Arch Ophthalmol 1963;70:313.
Victor M, Mancall EL, Dreyfus PM. Deficiency amblyopia in the alcoholic patient: a clinicopathologic study. Arch Ophthalmol 1960;64:1.
Carroll FD. Nutritional amblyopia. Arch Ophthalmol 1966;76:406.
Frisén L. Fundus changes in acute nutritional amblyopia. Arch Ophthalmol 1983;101:577.
Cullom ME, Hayes KL, Miller NR, et al. Leber’s hereditary optic neuropathy masquerading as tobacco-alcohol amblyopia. Arch Ophthalmol 1993;111:1482.
Rodger FC. Degenerative changes in the rat visual pathway when thiamine and riboflavin deficiencies are combined. Br J Ophthalmol 1954;38:154.
Smiddy WE, Green WR. Nutritional amblyopia. A histopathologic study with retrospective clinical correlation. Graefes Arch Clin Exp Ophthalmol 1987;225:321–4.
Ikeda H, Tremain KE, Sanders MD. Neurophysiological investigation in optic nerve disease: Combined assessment of the visual evoked response and electroretinogram. Br J Ophthalmol 1978;62:227.
Kupersmith MJ, Weiss PA, Carr RE. The visual evoked potential in tobacco-alcohol amblyopia and nutritional amblyopia. Am J Ophthalmol 1983;95:307.
Pawlosky RJ, Bacher J, Salem N Jr. Ethanol consumption alters electroretinograms and depletes neural tissues of docosahexaenoic acid in rhesus monkeys: nutritional consequences of a low n-3 fatty acid diet. Alcohol Clin Exp Res 2001;25:1758–65.
Hood DC, Odel JG, Chen CS, et al. The multifocal electroretinogram. J Neuro-ophthalmol 2003;23:225–35.(R Behbehani, R C Sergott )