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Show PHOTO ESSAY Susac Syndrome in a Patient With Hepatitis C Anand Chawla, MBChB, Sivakumar Sathasivam, MBChB, MRCP, Rakhee Nayar, MBChB, and Mark Doran, MBChB, FRCP FIG. 1. A. Coronal FLAIR MRI shows high signal abnormalities in the basal ganglia { arrow) and corpus callosum { arrowhead). B. Precontrast T1 sagittal MRI shows low signal in the corpus callosum { arrowhead) corresponding to the high signal area on the FLAIR study ( A). C. Fundus photograph of the left eye shows retinal infarctions. Abstract: A 38- year- old woman seropositive for hepatitis C developed headache, sensorineural hearing loss, encephalopathy, and retinal arteriolar occlusions. Brain MRI showed signal abnormalities in the basal ganglia and corpus callosum. These features are consistent with Susac syndrome, a multifocal central nervous system disorder of uncertain etiology. This is the first reported case of Susac syndrome in a patient with hepatitis C. ( J Neuro- Ophthalmol 2007; 27: 55- 56) Walton Centre for Neurology and Neurosurgery, Lower Lane, Fazakerly, Liverpool, United Kingdom. Address correspondence to Dr. Anand Chawla, MBChB, Senior House Officer in Neurology, 23A Central Avenue, Eccleston Park, Liverpool, L34 2QL, UK; E- mail: anand001@ hotmail. com A38- year- old woman presented with a 3 week history of worsening frontal headaches. The headache was worse in the morning and improved throughout the day. There were no precipitating factors and she had never had similar episodes of headache. A few days later she developed bilateral deafness that progressed over 2 weeks. The patient noticed that her speech had become slurred. She also gave a history of having memory loss over the preceding 2 weeks. Furthermore, she also reported mood disturbance, fluctuating confusion, agitation, and a feeling of unsteadiness while walking. She had no visual symptoms. She had suffered from eczema and anxiety and was an intravenous drug abuser. She was taking regular medications and had no allergies. She smoked 20 cigarettes per day and reported minimal alcohol intake recently, although she had previously been an alcoholic. She lived with a male partner and her 5- year- old child. J Neuro- Ophthalmol, Vol. 27, No. 1, 2007 55 J Neuro- Ophthalmol, Vol. 27, No. 1, 2007 Chawla et al A general physical examination was unremarkable. However, on neurologic examination, she was vague, confused, and dysarthria Her Mini- Mental State Examination score was 14/ 30. She was disoriented to time, place, and person and had impaired delayed recall. Visual acuity was normal at 20/ 20 in both eyes. She had obvious deafness, but because of her confusion, it was not possible to do formal testing to determine whether the hearing loss was conductive or sensorineural. Speech was slurred. Limb coordination was normal, but gait was unsteady. The remaining neurologic examination was normal. Hematologic and biochemical profiles were normal. Autoantibody, VDRL, and human immunodeficiency virus ( HIV) tests were also negative. Antistreptococcal antibody titers were minimally elevated, and cytomegalovirus IgG was elevated. She was hepatitis C- positive with a high viral load and a high level of replication. The liver profile was normal. An electroencephalogram showed generalized slowing of background rhythm to the delta/ theta range. Brain MRI showed high signal abnormalities on FLAIR and T2 sequences in the cerebral white matter involving the corpus callosum and basal ganglia ( Fig. 1). A lumbar puncture was normal except for a protein level of 81 mg/ dL. Ophthalmologic consultation later yielded the finding of multiple bilateral retinal arteriolar occlusions ( Fig. 1). An audiogram demonstrated mild bilateral sensorineural hearing loss. Therapy was initiated with 75 mg aspirin once daily and a 3 day course of 1 g intravenous methylprednisolone daily. She showed only moderate improvement and was therefore given a 5 day course of intravenous immunoglobulin, but still showed little immediate improvement. She is taking 75 mg aspirin daily on a long- term basis. After 1 month, the gait disturbance had resolved, hearing had improved, and confusion had lessened, but a formal psychometric assessment demonstrated that her IQ remained subnormal relative to an expected level. After 2 months, she still had a mild cognitive deficit, and her hearing was still improving. She still had evidence of retinal arteriolar occlusions, but no new lesions had developed. In 1979, Susac, Hardman, and colleagues ( 1) described two young women who presented with a triad of encephalopathy, retinopathy ( arteriolar occlusions), and hearing loss. Subsequent brain biopsy showed multifocal microinfarction, and the authors suggested that the condition represented a microangiopathy of the brain, retina, and cochlea. The condition is thought to mainly affect women between the ages of 18 and 40 years ( 2- 4). The etiology, however, remains unclear. Our patient presented with the triad of manifestations described in Susac syndrome ( 1,5). The diagnosis was made by the recognition of the clinical and MRI findings ( 1- 7). In other cases, brain biopsy is needed for diagnostic confirmation; the biopsy should show microangiopathic infarcts without evidence of vasculitis. The hearing loss and visual disturbance are often asymmetrical, as in our patient, and there have also been reports of patients in whom not all components of the clinical triad are found ( 2,3). Approximately 70 cases of Susac syndrome have been reported ( 1- 11). The connection to viral disease is not well developed. Nicolle and McLachlan ( 8) described a 24- year-old woman who had prodromal symptoms of fever, cough, and coryza before developing the symptoms of Susac syndrome. Coppeto et al ( 9) described a patient who had developed a sore throat and photophobia before developing the syndrome. Altogether, there have been six reports in which a viral illness has been thought to precipitate this syndrome, although no viral agent has been identified ( 10). Our case is the first report of Susac syndrome in a patient seropositive for hepatitis C. The relevance of this finding to the neurologic manifestations is unclear, but the absence of any known etiologic factor, together with the previous reports suggesting a viral link to this syndrome, invites the consideration that it may play a causative role. Acknowledgments We thank Mr. C. S. Ng ( Consultant Ophthalmologist, HM Stanley Hospital, St. Asaph) for his help in providing us with ophthalmic photographs. REFERENCES 1. Susac JO, Hardman JM, Selhorst JB. Microangiopathy of the brain and retina. Neurology 1979; 29: 313- 6. 2. Papo T, Biousse V, Lehoang P, et al. Susac syndrome. Medicine ( Baltimore) 1998; 77: 3- 11. 3. Susac JO. Susac's syndrome: the triad of microangiopathy of the brain and retina with hearing loss in young women. Neurology 1994; 44: 591- 3. 4. Barker RA, Anderson JR, Meyer P, et al. Microangiopathy of the brain and retina with hearing loss in a 50 year old woman: extending the spectrum of Susac's syndrome. J Neurol Neurosurg Psychiatry 1999; 66: 641- 3. 5. O'Halloran HS, Pearson PA, Lee WB, et al. Microangiopathy of the brain, retina and cochlea ( Susac syndrome). A report of five cases and a review of the literature. Ophthalmology 1998; 105: 1038^ 4. 6. Susac JO, Murtagh FR, Egan RA, et al. MRI findings in Susac's syndrome. Neurology 2003; 61: 1783- 7. 7. Vila N, Graus F, Blesa R, et al. Microangiopathy of the brain and retina ( Susac's syndrome): two patients with atypical features. Neurology 1995; 45: 1225- 6. 8. Nicolle MW, McLachlan RS. Microangiopathy with retinopathy, encephalopathy and deafness ( RED- M) and systemic features. Semin Arthritis Rheum 1991; 21: 123- 8. 9. Coppeto JR, Currie JN, Monteiro ML, et al. A syndrome of arterial-occlusive retinopathy and encephalopathy. Am J Ophthalmol 1984; 98: 189- 202. 10. Petty GW, Engel AG, Younge BR, et al. Retinocochleocerebral vasculopathy. Medicine ( Baltimore) 1998; 77: 12^ 0. 11. Bogousslavsky J, Gaio JM, Caplan LR, et al. Encephalopathy, deafness and blindness in young women: a distinct retinocochleocerebral arteriolopathy? J Neurol Neurosurg Psychiatry 1989; 52: 43- 6. 56 © 2007 Lippincott Williams & Wilkins |
