| OCR Text |
Show Journal of Nemo- Ophthalmology 16( 2): 224- 233, 2996. i 1996 Lippincott- Raven Publishers, Philadelphia Visual Pathway Hemorrhage Associated with Alcohol- Induced Coagulopathy Deborah I. Friedman, M. D., S. Rao Aravapalli, M. D., and Michael C. Shende, M. D. Hemorrhage confined to the optic pathways is rare. We describe a patient with diffuse hemorrhage of the intracranial optic nerves, optic chiasm, and optic tracts, attributed to alcohol- induced coagulopathy. The hemorrhage resolved completely as the patient's vision improved, and subsequent neuroimaging studies showed atrophy of the affected structures. Key Words: Optic chiasm- Hemorrhage- Coagulopathy- Magnetic resonance imaging- Anterior visual pathways. Manuscript received February 3, 1995; accepted February 3, 1995. From the Departments of Neurology ( D. I. F.), Ophthalmology ( D. I. F.), Radiology ( Division of Neuroradiology) ( S. R. A.), and Neurosurgery ( M. C. S.), State University of New York Health Science Center at Syracuse, 750 East Adams Street Syracuse, New York, U. S. A. Presented at the North American Neuro- Ophthalmology Society, Park City, Utah, 1991. Address correspondence and reprint requests to Dr. Deborah I. Friedman, Department of Neurology, SUNY Health Science Center, 750 East Adams Street, Syracuse, NY 13210, U. S. A. CASE REPORT A 41- year- old man noticed blurred vision after ingesting approximately a dozen drinks ( beer and hard liquor). Two days later, he was unable to read or see the television set with either eye. He denied any contamination of his alcohol, methanol ingestion, or other toxic exposure. There was no pain on eye movement, headache, or other neurological complaints. The patient never smoked and had a sedentary lifestyle with no history of trauma. His medical history was notable for alcoholic cirrhosis with a coagulopathy and previous gastrointestinal bleeding. He took vitamin K daily, and sporadically took folic acid, multivitamins, and spironolactone. His visual acuity three months earlier was 20/ 20 OU without correction. Two weeks after the onset of symptoms, the visual acuity without correction was 20/ 400 OD and 20/ 200 ~ x OS, with marked impairment of color vision. The pupils were sluggishly reactive OU, with light- near dissociation. Tangent screen testing and Goldmann perimetry showed extensive central visual field loss ( Fig. 1). The ocular motility was normal. The fundus examination showed papillomac-ular nerve fiber bundle loss, and thinning of the remaining nerve fiber layer OU. A contrast- enhanced computed tomography ( CT) scan showed hyperdensity and enlargement of the intracranial optic nerves, optic chiasm, and optic tract ( Fig. 2). The scan was obtained prior to the patient's referral; noncontrast images were not 124 VISUAL PATHWAY HEMORRHAGE 125 • m % VJ » .' '•> JB&'>* •^; ' - > • • « • < * • : : : : : : " : . • • » » • * • FIG. 1. A: Goldmann visual fields demonstrate dense central and paracentral visual field loss in the left eye. B: There is profound central and peripheral field loss in the right eye. J Neuro- Ophthalmol, Vol. 16, No. 2, 1996 226 D. I. FRIEDMAN ET Ah. FIG. 2. Contrast- enhanced CT scan shows diffuse enlargement of the intracranial optic nerves, optic chiasm, and optic tracts. The hyperdensity is compatible with either hemorrhage or contrast enhancement. performed. The findings were compatible with either hemorrhage or enhancement. Magnetic resonance imaging ( MRI) using a 1.5- T superconducting magnet revealed a diffuse hyperintense signal on Tl- weighted images, with little change after contrast administration ( Fig. 3). There was mixed signal intensity on T2- weighted images, and high signal intensity on proton- density sequences in the same region ( Fig. 4). No abnormalities were present in the hypothalamus or the intraorbital optic nerves ( Figs. 5 and 6). The neuroimaging abnormalities were compatible with diffuse hemorrhage of the visual pathways. The cerebrospinal fluid contained no white blood cells, 460 crenated red cells; the protein was 52 ng/ dl, and glucose was 72 ng/ dl. There was no anemia, and the red cell indices were normal. The white cell count was normal, and the platelet count was 80,000/ mm3. There was mild prolongation of the prothrombin time ( 13.4 s) and the activated partial thromboplastin time ( 29.8 s), with a normal bleeding time ( 7.0 min). The serum transaminases and total bilirubin were elevated. The total protein and albumin were normal, and the blood urea nitrogen was low ( 6 mg/ dl). Serum thiocyanate was undetectable. The patient was treated conservatively with parenteral thiamine and multivitamins. One week later the visual acuity was 20/ 100 OD and 20/ 80 OS, with residual hemorrhage visible on the CT scan ( Fig. 7). The patient continued to improve, despite intermittent drinking binges. Two months later, there was improvement of the hemorrhage on CT scan ( Fig. 8). At three months, the visual acuity was 20/ 50+ 2 OD with an incomplete temporal hemianopia. The visual acuity was 20/ 30 OS with central and paracentral scotomas. At five months, the visual acuity improved to 20/ 30+ 2 OD and 20/ 20 - 1 OS. He identified 9/ 15 H- R- R color plates OU, and the pupils reacted normally. The visual fields improved, but there were persistent central and bitemporal defects ( Fig. 9). Temporal pallor of both optic nerves had developed. MRI showed resolution of the acute hemorrhage and mild thinning of the anterior optic pathways ( Fig. 10). The hypoin-tensity on T2- weighted images suggests the presence of residual hemosiderin ( Fig. 11). No further improvement in the patient's vision occurred after four years. DISCUSSION Our patient had a hemorrhage of the visual pathways, a presumed complication of alcohol-induced coagulopathy. Chiasmal hemorrhage can occur with trauma ( 1), intrachiasmal vascular malformations ( 2), and cavernous angiomas ( 3). The MRI scan showed no vascular malformation in our patient. An angiogram was not performed, but the diffuse nature of the hemorrhage without a distinct focus suggests a microangiopathic process, rather than an occult vascular mass. There was no antecedent trauma. Although this patient had a coagulopathy, the propensity for hemorrhage to occur exclusively along the visual pathways is unexplained. Alcohol overuse may be a contributing factor. Alcoholism is an independent risk factor for nontraumatic intracerebral hemorrhage ( 4,5). Underlying pathology of the visual system caused by alcoholism, such as demyelination or ischemia, might lead to a localized hemorrhage in the setting of a coagulopathy. Other conditions associated with alcoholism, including Wernicke's encephalopathy and Marchi-afava- Bignami disease, can produce demyelination and hemorrhage in the brain. Although none of J Neuro- Ophthalmol, Vol. 16, No. 2, 1996 A- C | 1 ^ M .'• « '&£•' Y^ Sjar V. ^ C??\/> i k K1 '• 1 / ^ CUF FIG. 3. A- C: T1- weighted axial and coronal MRI shows diffuse enlargement of the intracranial optic nerves, optic chiasm, and optic tracts, showing high signal intensity. The signal was relatively lower in the central portion of the optic nerves, most prominent on the left. There is no hemorrhage in the hypothalamus. D- F: There was no significant enhancement after gadolinium- DTPA administration. FIG. 4. A: T2- weighted image demonstrates a heterogeneous signal in the optic pathways, with patchy areas of low signal intensity in the optic chiasm and most of the left optic tract. There is bright signal in the remaining optic pathways. B: The area is homogenous on the proton- density image. This pattern indicates diffuse acute and subacute hemorrhage within the optic pathways, representing intracellular and extracellular methemoglobin. 128 D. I. FRIEDMAN ET AL. FIG. 5. Sagittal sections clearly delineate the abnormality in optic chiasm from the hypothalamic structures. these disorders produces hemorrhage of the visual pathways, their neuroimaging characteristics are known, and their pathophysiology may provide insight into this patient's condition. Wernicke's encephalopathy, a syndrome of confusion, ophthalmoplegia, and ataxia, is caused by thiamine deficiency, usually in the setting of alcohol abuse. MRI is much more sensitive than CT for demonstrating abnormalities in the acute phase of Wernicke's encephalopathy. One patient had hy-perintensities on T2- weighted images in the periaqueductal gray and medial thalamus ( 6). Another had increased signal in the dorsal medial thalamus on T2- weighted images, that resolved as the patient improved clinically ( 7). In five additional patients, T2- weighted MRI showed hyperintense lesions surrounding the third ventricle and aqueduct ( 8). The high signal intensities resolved and were replaced by atrophic changes on subsequent scans. The MRI changes in Wernicke's encephalopathy are postulated to correlate with edema, demyeli-nation, and gliosis ( 8). However, MRI does not always demonstrate thalamic, periaqueductal, and brainstem abnormalities in Wernicke's encephalopathy ( 9). MRI does not show evidence of hemorrhage in these patients. Microscopic hemorrhagic foci are infrequent in autopsied cases and likely represent agonal change ( 10). Marchiafava- Bignami disease is a rare complication of alcoholism, producing demyelination of the corpus collosum. It is usually diagnosed at autopsy, and pathological examination may reveal demyelination of other structures, including the anterior commissure, cerebellar peduncles, optic tracts, and posterior columns of the spinal cord ( 11). Visual evoked potentials demonstrated a unilateral prechiasmal visual pathway disturbance in one patient with Marchiafava- Bignami disease, who was diagnosed antemortem with neuroimaging studies ( 12). Hemorrhage is not typically associated with this entity. A condition clinically resembling central pontine myelinolysis ( CPM) was described in four alcoholics with abnormal serum sodium levels. Hemorrhage and infarction occurred, rather than the typical demyelination of CPM ( 13,14). Our patient did not have abnormal sodium levels, but these cases raise the possibility of interactions between alcohol ingestion and other metabolic abnormalities. Tobacco- alcohol amblyopia is a frequent cause of visual loss in patients with alcohol abuse. This condition produces gradual bilateral visual loss with characteristic cecocentral scotomas. It is associated with alcohol abuse, tobacco abuse, and poor nutrition. The exact etiologic agent has not been clearly identified, but some patients have low serum and red cell folic acid levels ( 15). Alcohol may contribute by reducing the absorption of vitamin B12 ( 16). One autopsied case showed optic nerve atrophy, particularly in the temporal quadrant. There was no evidence of previous optic nerve hemorrhage, and hemorrhage is not generally associated with this disorder ( 17). The rapid onset of symptoms and the pattern of visual field loss in our patient were not typical of tobacco- alcohol amblyopia and suggested optic nerve and chiasmal involvement; neuroimaging studies demonstrated optic tract involvement as well. Although it may be a contributing factor, preexisting subclinical optic nerve damage due to alcohol does not completely explain the extensive visual pathway hemorrhage in this patient. Vasculitis and a coexisting coagulopathy could produce a focal hemorrhage confined to the visual pathways. There was no evidence of a central nervous system or systemic vasculitis in this patient. The MRI did not show any other evidence of vasculitis, and the patient did not develop any other medical problems to suggest a systemic disease. Without angiographic or pathological confirmation, the diagnosis of focal vasculitis is possible, but unproved. Acknowledgment: The authors thank George H. Collins, M. D., Robert Corona, M. D., and Edward G. Stopa, M. D., for their thoughtful review of the case and helpful comments. / Neuro- Ophthalmol, Vol. 16, No. 2, 1996 VISUAL PATHWAY HEMORRHAGE 129 FIG. 6. A, B: Routine T1- weighted images show no discernible abnormality of the intraorbital optic nerves. C: There is no enhancement with contrast administration. / Neuro- Ophthalmol, Vol. 16, No. 2, 1996 230 D. I. FRIEDMAN ET AL. FIG. 7. A: One week later, CT scan shows residual high density in the chiasmal region, that enhances with contrast ( B). FIG. 8. Two months after symptom onset, CT without ( A) and with ( B) contrast reveals improvement, with decreased thickness and hemorrhage of the optic tracts, and some residual enlargement and enhancement of the optic chiasm. / Neuro- Ophthalmol, Vol. 16, No. 2, 1996 VISUAL PATHWAY HEMORRHAGE 131 9- 2+ 44 O. S. 20tf5- l .„•!<- x7M ** y 111 . . ! • • - .-. • . - : /• •' * 1 7 " ? : ' " i ' / il i = a i. a F;.. h ::. » ;.. i! „ J a^" 5 » p t i>' ii'f.' h^ ^*' ,_- LLI : : | I -. il., u.| ' •• y' ' • f i'i - i - J I i ••: • • •••: : ^* S?&* . . I » | - . : . . I,:...:....- . yU... . / " • frrt^ alb: FIG. 9. A: At five months, there is improvement of the field OS, but the temporal central and paracentral regions are still affected. B: Visual field testing in the right eye shows a persistent inferotemporal field defect encroaching on fixation. J Neuro- Ophthalmol, Vol. 16, No. 2, 1996 232 D. I. FRIEDMAN ET Ah, ;- U- SEF- S » H: 53i5ff FIG. 10. A, B: T1- weighted MRI shows resolution of the visual pathway hemorrhage, with minimal hyperintensity of the optic chiasm and optic tract. The optic chiasm and optic tract appear somewhat thinner than normal ( arrows). No vascular malformation is present. REFERENCES FIG. 11. The optic chiasm is hypointense on T2- weighted images, likely due to hemosiderin deposition from the previous hemorrhage. 10. 11. 12, Crowe NW, Nickles TP, Troost T, et al. Intrachiasmal hemorrhage: A cause of delayed post- traumatic blindness. Neurology 1989; 39: 863- 5. Hankley CJ, Khangure MS. Chiasmal apoplexy due to in-trachiasmatic vascular malformation rupture. Aust NZ } Med 1987; 17: 444- 6. Hassler W, Zentner J, Wilhelm H. Cavernous angiomas of the anterior visual pathways. J Clin Neuro- Ophthalmol 1989; 9: 160- 4. Weisberg LA. Alcoholic intracerebral hemorrhage. Stroke 1988; 19: 1565- 9. Calandre L, Arnal C, Ortega JF, et al. Risk factors for spontaneous cerebral hematomas. Case control study. Stroke 1986; 17: 1126- 8. Drayer BP. Imaging of the aging brain. Part II. Pathological conditions. Radiology 1988; 166: 797- 806. Donnal JF, Heinz ER, Burger PC. MR of reversible thalamic lesions in Wernicke syndrome. AJNR 1990; 11: 893- 4. Gallucci M, Bozzao A, Splendiani A, et al. Wernicke encephalopathy: MR findings in five patients. AJNR 1990; 11: 887- 92. Charness ME, DeLePaz RL. Mammillary body atrophy in Wernicke's encephalopathy: antemortem identification using magnetic resonance imaging. Ann Neurol 1987; 22: 595- 600. Victor M. MR in the diagnosis of Wernicke- Korsakoff syndrome. Am J Neuroradiol 1990; 11: 895- 6. Brion S. Marchiafava- Bignami syndrome. In: Vinken PJ, Bruyn GW, eds. Handbook of clinical neurology, vol 28. Amsterdam: North Holland, 1976: 317- 29. Baron R, Heuser K, Marioth G. Marchiafava- Bignami disease with recovery diagnosed by CT and MRI: demyelina-tion affects several CNS structures. / Neurol 236: 364- 6. / Neuro- Ophthalmol Vol. 16, No. 2, 1996 VISUAL PATHWAY HEMORRHAGE 133 13. Gianetti AV, Pitella JE. Ischemic and hemorrhagic necrosis of the pons with anatomical location similar to that of central pontine myelinolysis in a chronic alcoholic patient. Clin Neuropathol 1993; 12: 156- 9. 14. Adamson DJ, Laing RB, Nathwani D. Alcoholism, hypona-traemia, and central neurological damage: more than pontine myelinolysis? Scott Med J 1992; 37: 83- 4. 15. Knox DL, Chen MF, Guilarte TR, et al. Nutritional amblyopia. Folic acid, vitamin B- 12, and other vitamins. Retina 1982; 2: 288- 93. 16. Lindenbaum J, Lieber CS. Alcohol- induced malabsorption of vitamin B12 in man. Nature 1969; 224: 806. 17. Quigley HA, Addicks EM, Green WR. Optic nerve damage in human glaucoma. III. Quantitative correlation of nerve fiber loss and visual field defect in glaucoma, ischemic neuropathy, papilledema, and toxic neuropathy. Arch Ophthalmol 1982; 100: 135- 46. / Neuro- Ophthalmol, Vol. 16, No. 2, 2996 |
