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Show Journal of Neuiv- Ophthalmology 18( 1): 32- 35, 1998. © 1998 LippincoU- Raven Publishers, Philadelphia Presumed Bilateral Occipital Neurosarcoidosis A Case Report Syndee J. Givre, M. D., Ph. D., and Joel S. Mindel, M. D., Ph. D. A 37- year- old man with a history of sarcoidosis, hypertension, asthma, depression and prior intravenous drug use presented with complaints of difficulty in finding his way around the house, headache, and blurred vision in both eyes. The symptoms had been increasing in severity over the prior several months. Physical examination showed normal visual acuity, pupil reactions, and fundi but severe, circumferential constriction of the visual fields bilaterally. The visual fields enlarged appropriately on increasing the distance from the patient to the tangent screen. Neuroimaging revealed bilateral, occipital meningeal involvement and parenchymal lesions consistent with sarcoidosis. Treatment with oral corticosteroids produced a mild subjective improvement in the patient's symptoms and stabilized the visual fields, without improving them. This case represents an unusual presentation of presumed neurosarcoidosis involving the visual pathways at the level of the occipital lobes. Key Words: Neurosarcoidosis- Occipital cortex- Macular sparing- Visual fields- Magnetic resonance imaging. CASE REPORT A 37- year- old man with a history of sarcoidosis, asthma, hypertension, prior intravenous drug use, and depression presented to the emergency department with complaints of two episodes of syncope in the 48 h before admission. He also complained of 2- 3 months of headache, dizziness, blurred vision bilaterally, right- sided facial numbness, and forgetfulness. The patient specifically noted a feeling of spatial " disorientation" and gave as an example getting lost in the hallway of his home despite intellectually knowing where he was. All of the symptoms had progressively increased in severity and frequency over the 6 weeks before admission. The patient denied any fevers, photophobia, or stiff neck. He was admitted for further workup and treatment. Three years before admission, the patient had pre- Manuscript received July 7, 1997; accepted July 25, 1997. From the Department of Ophthalmology, The Mount Sinai Medical Center, New York, New York, U. S. A. Address correspondence and reprint requests to Dr. Joel S. Mindel, Mount Sinai Medical Center, Department of Ophthalmology, Box 1183, One Gustave L. Levy Place, New York, NY 10029- 6574, U. S. A. sented to another institution with complaints of shortness of breath and headache. A transbronchial biopsy sample obtained via bronchoscopy was consistent with sarcoidosis. Before the present admission, he was maintained on 7 mg of prednisone daily. His other medications included enalapril, methadone, ranitidine, and trazadone. The patient was a well- developed, well- nourished man with a flat affect. Vital signs and results of the general physical examination were normal. Neurologic examination was significant for decreased sensation on the right side of the face and slow ambulation with normal gait. Ophthalmologic examination was significant for normal visual acuities, normal pupil reactions ( including light-near comparison), normal- appearing fundi, and optic nerve heads. Visual fields to confrontation were severely constricted bilaterally. Automated perimetry confirmed this impression ( Fig. 1). On tangent screen testing, the area of visual field increased appropriately when the distance from the patient to the screen was increased. Complete blood count, erythrocyte sedimentation rate, blood chemistries, and thyroid function tests were within normal limits. Testing for human immunodeficiency virus was negative. The blood angiotensin- converting enzyme level was within normal limits on two occasions. A lumbar puncture was performed. The cerebrospinal fluid ( CSF) levels of glucose and protein were 64 mg/ dl and 29 mg/ dl, respectively. The red blood cell and white blood cell counts in the CSF were 15/ mm3 and 14/ mm3, respectively. CSF cytology showed small, monomorphous lymphoid cells consistent with a reactive inflammatory process. CSF tests for syphilis VDRL and cryp-tococcal antigen were negative. Immunoelectrophoresis of the CSF was normal. CSF cultures and stains for bacteria, acid- fast bacteria, yeast, and fungi were negative. A radiograph of the chest was normal. Brain computed tomography ( CT) without contrast showed areas of hy-perdensity along the falx and tentorium and bilateral, occipital, and parenchymal hyperdensities. These areas enhanced after the administration of intravenous contrast ( Fig. 2). In addition, a focal area of hypodensity that did not enhance with contrast was also noted in the right occipital cortex. This area was thought to represent a focal region of infarction or edema. Tl- weighted brain magnetic resonance imaging ( MRI) performed after the 32 BILATERAL OCCIPITAL NEUROSARCOIDOSIS 33 FIG. 1. Automated visual fields performed on the Humphrey HFA II 1- 0- A5 field analyzer. The Central 24- 2 full- threshold test was used, with a white, size V stimulus. Graytone plots of three sets of visual fields obtained from the left ( left) and right eyes ( right) are shown. The uppermost set was obtained on the second day of admission, 1 day after the initiation of high- dose corticosteroids. The middle set was obtained 13 days after the initiation of high- dose corticosteroids. The lowermost set was obtained 42 days after the onset of high- dose corticosteroids. administration of gadolinium showed marked posterior fossa dural ( including tentorial) enhancement, bilateral occipital gyral enhancement, and bilateral cerebellar fo-lial enhancement. Figure 3A demonstrates the abnormalities seen in occipital cortex on MRI. Both T2 and proton density images showed bilateral occipital parenchymal changes ( Fig. 3B and 3C). These abnormalities were interpreted to be consistent with a diagnosis of neurosarcoidosis. The patient was treated with 60 mg of prednisone daily. He subsequently noted an improvement in his symptoms and was discharged. During the next 14 months, his visual fields remained stable on a slowly tapered dose of prednisone. DISCUSSION Sarcoidosis is a systemic, inflammatory disorder. Because the etiology remains unknown, diagnosis is based on a constellation of radiologic, pathologic, and clinical data. Classical chest radiographic findings include bilateral hilar and mediastinal adenopathy. The pathologic hallmark of sarcoidosis is the noncaseating or non-necrotizing granuloma. The most common clinical presentation of sarcoidosis is as an incidental finding on chest film ( 1). The frequency of clinically evident, neurologic involvement in sarcoidosis is estimated to be 5% ( 2- 6). Neurosarcoidosis also has been demonstrated in the absence of clinical neurologic manifestations ( 7). In a large study of 694 patients with sarcoidosis, 5.1% were found to have neurosarcoidosis, and of these, 48% initially presented with neurologic complaints ( 3). Ninety- seven percent of the patients with neurosarcoidosis in this study had other systemic manifestations of the disorder. All portions of the nervous system, both peripheral and central, may be affected by sarcoidosis. Cranial neuropathy is the most common neurologic deficit, with the facial nerve being most frequently involved ( 2,3,5). Other central nervous system ( CNS) manifestations include meningeal infiltration, parenchymal infiltration or mass, hydrocephalus, vasculopathy, diffuse encephalopathy and seizures. Our patient had a prior diagnosis of sarcoidosis. His complaints on the present admission were of spatial disorientation and " blurred" vision. However, on neuro-ophthalmologic examination, he was found to have normal visual acuities, normal pupil reactions and normal appearance of the optic nerve heads but extremely constricted visual fields bilaterally. This combination of FIG. 2. Brain CT obtained after the administration of intravenous contrast. A section at approximately the level of the occipital cortex demonstrating dural and parenchymal enhancement. The right occipital cortex also shows a focal area of hypodensity that does not enhance with contrast. This may represent a localized infarction or edema. J Neuro- Ophlhalmol, Vol. IS, No. I, 1998 34 S. J. GIVRE AND J. S. MINDEL : < 4 Ik* fr - • • ( r i . < : k\ h y7 FIG. 3. Transverse MRI sections at approximately the level of the occipital cortex. T1- weighted image obtained after the administration of gadolinium demonstrating marked meningeal enhancement, as well as parenchymal involvement ( A). T2- weighted ( B) and proton density images ( C) demonstrating bilateral occipital cortex hypodensities. findings is consistent with bilateral occipital ( visual) cortical lesions with macular sparing or psychogenic visual loss ( 8) The first clinical evidence supporting organic CNS disease was that the patient did not exhibit tunnel vision on tangent screen testing. Although a biopsy specimen was not obtained, neu-roimaging supported a diagnosis of CNS sarcoidosis by demonstrating involvement of the meninges around and parenchyma of both occipital lobes. To our knowledge, this patient is unique in having bilateral involvement of the occipital meninges and parenchyma without evidence of any other involvement of the visual system. ./ Neiim- Ophthalmol, Vol. IS, No. I, 1998 BILATERAL OCCIPITAL NEUROSARCOIDOSIS 35 REFERENCES 1. DeRemee RA. Sarcoidosis. Mayo Clin Proc 1995; 70: 177- 81. 2. Delaney P. Neurologic manifestations in sarcoidosis: review of the literature, with a report of 23 cases. Ann Intern Med 1977; 87: 336- 45. 3. Stern BJ, Krumholz A, Johns C, Scott P, Nissim J. Sarcoidosis and its neurological manifestations. Arch Neurol 1985; 42: 909- 17. 4. Heck AW, Phillips LH III. Sarcoidosis and the nervous system. Neurol Clin 1989; 7: 641- 54. 5. Scott TF. Neurosarcoidosis: progress and clinical aspects. Neurology 1993; 43: 8- 12. 6. Chapelon C, Ziza JM, Piette JC, et al. Neurosarcoidosis: signs, course and treatment in 35 confirmed cases. Medicine 1990; 69: 261- 76. 7. Ricker W, Clark M. Sarcoidosis: a clinical pathologic review of 300 cases, including 22 autopsies. Am J Clin Pathol 1949; 19: 725- 49. 8. Burde RM, Savino PJ, Trobe JD. Clinical Decisions in Neuro-ophthalmology. St. Louis: Mosby- Year Book, 1992: 31, 104- 16. J Neuw- Oplulmlmol, Vol. 18, No. I, 1998 |
