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Show LETTERS TO THE EDITOR Optic Neuropathy in Giant Cell Arteritis To the Editor: Even a patient free of visual symptoms and signs at the time high- dose corticosteroid treatment is implemented for newly- diagnosed giant cell arteritis may suffer visual loss within the first few days of treatment. In January 2001, a 73- year- old, 63- kilogram woman with inactive Crohn disease, coronary artery disease, and a supraventricular tachycardia developed fatigue and anorexia and a 4.5- kg weight loss. These manifestations, initially ascribed to a respiratory tract infection, persisted. One month later, scalp tenderness, jaw claudication, and headache supervened. She had no visual symptoms. On April 24, 2001, an erythrocyte sedimentation rate ( ESR) of 110 mm/ h was recorded. Prednisone 80 mg/ d, instituted the next day, promptly relieved all symptoms. Temporal artery biopsy showed giant cell arteritis. Prednisone therapy was continued at the same dose, but on April 27, with an ESR of 63 mm/ h, she first complained that the vision seemed " clouded" in her OS. An ophthalmologist found visual acuities of 20/ 20 OU and normal Humphrey automated visual fields, pupils, and fundi. Two days later, visual acuity was 20/ 25 OS with a nasal visual field defect, dyschromatopsia, a left relative afferent pupil defect, and a swollen optic disc OS. Examination of the OD was normal. On that day, she received two doses of 500 mg methylprednisolone intravenously 12 hours apart and prednisone 60 mg. The following day, with a ESR of 10 mm/ h, visual acuity fell to 20/ 200 OS. Thereafter she was treated with prednisone 60 mg/ d, but by May 3, visual acuity had declined to 5/ 200 OS. There have been no subsequent changes. It is well- recognized that in a minority of patients with acute or transient visual loss from giant cell arteritis, further loss of vision occurs despite the institution of appropriate corticosteroid therapy. ( 1) Hayreh and Zimmerman ( 1,2) have shown that when this occurs, it does so within six days of implementing treatment. This patient had neither transient nor persisting visual loss before treatment was initiated and yet developed anterior ischemic optic neuropathy forty- eight hours after starting prednisone 1.25 mg/ kg/ d. Judging from the paucity of well- documented examples, such a sequence must be rare in biopsy- proven giant cell arteritis. There is a report ( 3) of a patient who suffered a similar fate eight days after starting prednisone 80 mg/ d. In another report, ( 4) a corticosteroid- treated patient initially free of visual symptoms and signs lost vision much later. In that patient, ischemic optic neuropathy developed two months after diagnosis while she was being treated with prednisone 40 mg/ d. The laboratory findings and the patient's weight were not provided, but she went on to infarct the fellow eye and spinal cord despite doubling of the prednisone dose. In another report, ( 5) a patient who was initially free of vasculitic visual impairment at diagnosis of giant cell arteritis developed ischemic optic neuropathy five months after treatment was initiated; ischemic optic neuropathy developed while he was receiving prednisone 20 mg/ d, but his ESR and C- reactive protein levels were within normal limits. In summarizing 23 cases of giant cell arteritis in which there was permanent visual loss, Liozon et al ( 6) mention that there were three patients who developed it shortly after corticosteroid therapy was started, but details were not provided. Although I agree with Aiello et al ( 3) that the development of visual loss is rare after the initiation of corticosteroid therapy, physicians should nevertheless warn even those patients who are free of visual symptoms at the time of diagnosis and appropriate treatment of giant cell arteritis that they remain at risk for visual loss. Simmons Lessell, MD Massachusetts Eye and Ear Infirmary Boston, Massachusetts Simmons_ Lessell@ meei. harvard. edu REFERENCES 1. Hayreh SS, Zimmerman B. Visual deterioration in giant cell arteritis patients while on high doses of corticosteroid therapy. Ophthalmology 2003; 110: 1204- 15. 2. Hayreh SS, Zimmerman B. Management of giant cell arteritis. Our 27- year clinical study: new light on old controversies. Ophthalmo-logica 2003; 217: 239- 5. 3. Aiello PD, Trautmann JC, McPhee TJ, et al. Visual prognosis in giant cell arteritis. Ophthalmology 1993; 100: 550- 5. 4. Galetta SL, Balcer LI Lieberman AP, et al. Refractory giant cell arteritis with spinal cord infarction. Neurology 1997; 49: 1720- 3. 5. Kim N, Trobe JD, Flint A, et al. Late ipsilateral recurrence of ischemic optic neuropathy in giant cell arteritis. J Neuro- Ophthalmol 2003; 23: 122- 6. 6. Liozon E, Herrmann F, Ly K, et al. Risk factors for visual loss in giant cell ( temporal) arteritis: a prospective study of 174 patients. Am J Med 2001; 111: 211- 7. Uveitis as a Presenting Sign of Giant Cell Arteritis The relevance of uveitis as an ocular manifestation of giant cell arteritis ( GCA) is controversial. Vedrine et al ( 1) emphasized its occurrence as a presenting feature of GCA. In contrast, Salvarani et al ( 2) argued that the reported cases J Neuro- Ophthalmol, Vol. 25, No. 3, 2005 247 J Neuro- Ophthalmol, Vol. 25, No. 3, 2005 Letters to the Editor of uveitis and GCA could merely represent a chance association. We present a patient with biopsy- proven GCA who had developed an acute uveitis some weeks previously, for which the GCA can be considered the cause. A 69- year- old hypertensive man complained of anorexia and a dull widespread headache. One week later he noted blurred vision, conjunctival hyperemia, and photophobia in the left eye. Examination elsewhere disclosed a visual acuity of 20/ 20 OD and 20/ 40 OS. Thin folds of Descemet's membrane were noted OS, together with keratic precipitates and flare in the anterior chamber. Intraocular pressures were 14 mm Hg OD and 13 mm Hg OS. There was no relative afferent pupillary defect. The fundus examination was unrevealing. The erythrocyte sedimentation rate ( ESR) was not measured at that time. Acute anterior uveitis in the left eye was diagnosed. The patient was treated with topical corticosteroids to which his ocular symptoms initially responded. Anorexia and headache, however, persisted. Three weeks later, the visual acuity in his OS suddenly dropped to no light perception, preceded by one week of fatigue, jaw claudication, and scalp tenderness. Physical and neurological examinations were normal but for tenderness of both temporal arteries. Neuro- ophthalmological examination did not reveal any abnormality OD. There was a left relative afferent pupillary defect. No signs of anterior uveitis were seen. The optic disc OS showed pallid swelling with a few peripapillary hemorrhages. Ocular motility was normal. Fluorescein angiography demonstrated diffuse hyper-fluorescence of the left optic disc. Laboratory tests revealed a hypochromic microcytic anemia, an erythrocyte sedimentation rate ( ESR) of 90 mrn/ hr and a C- reactive protein of 28.1 mg/ 1 ( normal 0- 5 mg/ 1). The angiotensin converting enzyme level, VDRL, thyroid function studies, autoantibody profile ( including anticardiolipin antibodies), and computed tomography scan of head and orbits were all normal. Doppler imaging showed mild stenosis of the right internal carotid artery. The patient was treated with intravenous methylpred-nisolone 250 mg every six hours for three days followed by oral prednisone 75 mg/ day, with a dramatic and complete relief of the constitutional symptoms but no improvement in vision. The ESR decreased to 20 mm/ hr after three days. A left temporal artery biopsy showed evidence of giant cell arteritis ( Figure 1). When last examined six months later, there was no recovery of vision in the OS, whose optic disc appeared pale. There were no keratic precipitates in the anterior chamber. Intraocular pressures were 15 mm Hg OD and 14 mm Hg OS. Uveitis as a presenting feature of GCA is uncommon. To the best of our knowledge, there are only two reported patients ( 3,4). Unlike the patient of Rajesh and Cole ( 3), FIG. 1. Temporal artery biopsy showing a diffuse mixed inflammatory infiltrate with multinucleated giant cells ( arrow). who suffered from a subacute bilateral panuveitis, our patient had a unilateral anterior uveitis, occurring only three weeks before GCA diagnosis was made. The patient of Dasgupta et al ( 4) had an acute anterior and posterior uveitis OS which preceded by two months the diagnosis of GCA. However, although their patient fulfilled the clinical criteria for GCA, her temporal artery biopsy was not indicative of arterial inflammation. In order to avoid delays in treatment and devastating visual consequences, clinicians should be reminded that, although unusual, uveitis in elderly patients can be a presenting feature of GCA. Fabio Bandini, MD Luana Benedetti, MD Paola Ceppa, MD Guido Corallo, MD Laboratory of Neuro- ophthalmology Department of Neurosciences, Ophthalmology, and Genetics Department of Pathology University of Genoa Genoa, Italy fbandini@ neurologia. unige. it REFERENCES 1. Salvarani C, Cantini F, Boiardi L, Hunder GG. Polymyalgia reumatica and giant- cell arteritis. N EnglJ Med 2002; 347: 261- 71. 2. Vedrine L, Algayres JP, Coutant G. Giant- cell arteritis. N Engl J Med 2003; 348: 1497- 8. 3. Rajesh Cy Cole M. Panuveitis as a presenting feature of giant cell arteritis. Br J Ophthalmol 2000; 84: 340. 4. Dasgupta B, Pitzalis C, Panayi GS. Inflammation of the uveal tract as a presenting feature of temporal arteritis. Ann Rheum Dis 1989; 48: 964- 5. 248 J Neuro- Ophthalmol, Vol. 25, No. 3, 2005 Letters to the Editor J Neuro- Ophthalmol, Vol. 25, No. 3, 2005 Burkitt Lymphoma Presenting with Gingival Pain and a Cavernous Sinus Syndrome in an Adult We report an unusual case of adult- onset Burkitt lymphoma presenting with gingival pain and a right cavernous sinus syndrome. A 65- year- old man noted left gingival pain and progressive painful binocular diplopia, followed by right upper lid ptosis and a right fixed dilated pupil. The gingival pain was elicited when he inserted his dentures at the site where he had had a left cuspid tooth extracted three months earlier. Visual acuity was 20/ 30 OD and 20/ 20 OS. Confrontational visual fields were full OU. The pupil OD measured 6 mm and did not constrict to direct light; the pupil OS measured 4 mm and constricted normally. There was no relative afferent pupillary defect. There was complete ophthalmoplegia and complete ptosis OD and normal ductions and lid position OS. There was no proptosis. Slit lamp and funduscopic examinations were normal and facial motor function was normal. Trigeminal examination revealed a numb lower lip and chin more on the right side. Dental examination showed a smooth mass extruding from the extraction site of a left cuspid tooth. Brain MRI showed a 2.2 X 1.1 cm mass in the right cavernous sinus ( Fig. 1). CT of the head and neck showed cervical lymphadenopathy with maxillary and mandibular lesions ( Fig. 2). CT of the chest showed nodular pleural thickening and a large left pleural effusion and supraclavicular adenopathy. A lumbar puncture showed 22 white blood cells ( 87% polymorphonuclear, 4% mononuclear) and 11,850 red blood cells in the cerebrospinal fluid ( CSF). CSF glucose was 80 mg/ dL and CSF protein was elevated at 77 mg/ dL. Biopsy of the left mandibular mass showed diffuse infiltration of lymphoid cells with a high rate of mitotic activity and prominent nuclei suggestive of Burkitt lymphoma ( BL) ( Fig. 3). Immunohistochemical studies, including CD- 20, CD- 10, and BCL2, were compatible with BL. Epstein- Barr virus ( EBV) IgG antigen titers were positive. HIV testing was not obtained because of the lack of risk factors. A positron emission tomographic ( PET) scan showed uptake in both humeral shafts, right clavicle, right scapula, spine, left Departments of Ophthalmology, Neurology, and Neurosurgery. University of Alabama Birmingham, Alabama ( Dr. Vaphiades) Departments of Ophthalmology, Neurology, and Neurosurgery University of Iowa Hospitals and Clinics Iowa City, Iowa. ( Dr. Lee) Email: vaph@ uab. edu. Address correspondence and reprint to: Michael S. Vaphiades, D. O. UAB Department of Ophthalmology, Suite 601, 700 South 18th Street, Birmingham, AL 35233, ( 205) 325- 8620, Email: vaph@ uab. edu lung, left adrenal, celiac plexus and head and neck; these findings were considered indicative of disseminated BL. The patient was treated with eight cycles of cyclophosphamide, doxorubicin, vincristine, and prednisone ( CHOP) as well as rituximab. Four months after treatment, clinical examination improved in that now the right lid was halfway open, and he had slight eye movement OD in all directions except for abduction, which was now 90% of normal. The right pupil was still fixed and dilated. A repeat PET scan revealed almost complete disappearance of tumor in the head, neck, both humeral shafts and abdomen. There was persistent pleural uptake, however. Four months later, more lymphomatous areas ( confirmed by biopsy) were detected, one in the right anterior chest wall and one in the left inner thigh area. He then received salvage chemotherapy. BL is a type of non- Hodgkin's lymphoma ( NHL), a heterogenous group of lymphoproliferative malignancies that usually originates in the lymphoid tissues. ( 1) Denis Burkitt ( 1959) described " a sarcoma involving the jaws" in East African children. ( 2) The two classic subtypes of BL are endemic and sporadic, but a third subtype is associated with AIDS. Although the classic subtypes are indistinguishable histologically, they differ in their epidemiological, clinical, and biologic features. The endemic form of BL is the most common childhood malignancy in Africa, making up more than 50% of childhood neoplasms. In contrast, BL in the United States represents only 2% of all childhood neoplasms but FIG. 1. Enhanced coronal T1- weighted MRI shows an area of focal abnormal high signal in keeping with a right cavernous sinus mass. J Neuro- Ophthalmol, Vol. 25, No. 3, 2005 249 J Neuro- Ophthalmol, Vol. 25, No. 3, 2005 FIG. 2. Non- contrast axial CT scan at the level of the mandible shows a right sublingual mass ( arrow). 35%- 45% of NHL ( 3). In endemic BL, 80%- 90% of tumor cells contain the EBV genome; in sporadic BL, only 20% have high EBV antigen titers ( 4). Facial bone involvement ( especially of the jaw) is a common finding in endemic BL. Endemic BL is rare in adults ( 2). The sporadic form of BL ( 5) often presents with intra- abdominal or retroperitoneal lymphadenopathy, but 250 Letters to the Editor extranodal disease also occurs in 10%- 30%. Facial bone involvement, as seen in our patient, is rare, but orbital involvement may occur. ( 6) Intracranial involvement, as exemplified by our patient's cavernous sinus lesion, is usually an extension of sino- orbital involvement. Treatment of NHL consists of chemotherapy. CHOP remains the best available standard therapy, but it is curative in less than 50% of patients. ( 1) Monoclonal antibodies have recently been added to the regimen. Rituximab, the agent used in our patient, is an anti- CD20 monoclonal antibody. ( 7) Radiation plays a limited role because B- cell lymphoma is often disseminated. Prognosis varies with age, histology, stage of disease, and early response to therapy. ( 1) Our case emphasizes the importance of recognizing the jaw as a potential site for BL even in adults. The combination of new oral symptoms and diplopia could suggest BL. Michael S. Vaphiades, DO Departments of Ophthalmology, Neurology, and Neurosurgery University of Alabama Birmingham, Alabama vaph@ uab. edu Andrew G. Lee, MD Departments of Ophthalmology, Neurology, and Neurosurgery University of Iowa Hospitals and Clinics Iowa City, Iowa ACKNOWLEDGMENT This work was supported in part by an unrestricted grant from the Research to Prevent Blindness, Inc. N. Y, N. Y. REFERENCES 1. Gajra A, Vajpayee N, Grethlein S. Lymphoma. B- cell. eMedicine Journal, [ serial on the Internet]. 2001 Aug [ cited 2001 Aug 2] 2( 8): Available from: http:// emedicine. com. 2. Burkitt DP, Nelson CL, Williams EH. Some geographical variations in disease patterns in East and Central Africa. East Afr Med J1963 ; 40: 1- 6. 3. Ries LAG, Smith MA, Gurney JG et al. ( eds), Cancer Incidence and survival among children and adolescents: United States SEER program 1975- 1995, National Cancer Institute, SEER Program. NIH Pub. No. 99^ 649. Bethesda, MD, 1999. 4. Gaffey MJ, Weiss LM. Association of Epstein- Barr virus with human neoplasia. Pathol Annu 1992; 21: 55- 1 A. 5. Tsui SH, Wong MH, Lam WY. Burkitt's lymphoma presenting as mandibular swelling - report of a case and review of publications. Br J Oral Maxillofacial Surgery 2000; 38: 8- 11. 6. Lee AG, Quick SJ, Liu GT, et al. A childhood cavernous conundrum. Surv Ophthalmol 2004; 49: 231- 6. 7. Plosker GL, Figgitt DP. Rituximab: a review of its use in non- Hodgkin's lymphoma and chronic lymphocytic leukaemia. Drugs 2003; 63: 803^ 3. J Neuro- Ophthalmol, Vol. 25, No. 3, 2005 |