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Show Jtiiumil of Neiiro- Ophllmlmohjiy 18( 3): 192- 195. 199H. & 1998 l. ippincoll Williams & Wilkins, Philadelphia Microbial Keratitis as the Manifestation of Trigeminal Amyloidoma at Initial Presentation Ingeborg E. Kirch, M. D., Hilary A. Beaver, M. D., Andrew G. Lee, M. D., Linda K. Green, M. D., and Rosa A. Tang, M. D. Amyloidosis is characterized by the deposition of extracellular, insoluble proteinaceous, fibrillar material. It can be classified as primary, in cases without underlying disease, or secondary, in cases with an associated chronic disease ( e. g., multiple myeloma, rheumatoid arthritis, osteomyelitis, or tuberculosis). Isolated amyloidoma rarely involves the central nervous system but has been reported to occur in the orbit, spine, pituitary gland, jugular foramen, cerebellopontine angle, and cerebral white matter. In the literature, only five reports could be found of amyloidoma involving the trigeminal ganglion. This is a report of a case of trigeminal nerve amyloidoma that had initial symptoms of microbial keratitis secondary to trigeminal neuropathy. A review of the literature on amyloidoma of the trigeminal nerve is included. CASE REPORT A 34- year- old man had recurrent episodes of redness and pain in the left eye ( LE) for 5 years and had been diagnosed with " floppy eyelid" syndrome. In December 1994, pain and redness developed LE. The diagnosis was microbial keratitis LE, which was treated with topical antibiotics. After resolution of the corneal infection, he had a persistent nonhealing epithelial defect LE. He reported no ocular trauma, contact lens wear, or use of topical medication. His medical history was remarkable for noninsulin- dependent diabetes mellitus and hypertension. Manuscript received July 1997: accepted April 1998. From the Departments of Ophthalmology ( I. H. K, H. A. B.) and A. G. I..), Neurology and Neurosurgery ( A. G. L.), Baylor College of Medicine; Ihc Division of Neurosurgery, M. I). Anderson Cancer Center. University of Texas ( A. G. L.); the Department of Pathology. Veterans Affairs Medical Center ( I.. K. G.), Houston, Texas; and the Department of Ophthalmology, University of Texas Medical Branch ( R. A. T), Galveston, Texas, U. S. A. Supported in part by a grant from Research to Prevent Blindness, New York, New York, U. S. A. Presented in pari at I he April 15, 1997 meeting of the frank Walsh Society, Baltimore, Maryland, U. S. A.. Address correspondence and reprint requests to: Andrew G. I. ee M. D., Department of Ophthalmology. Baylor College of Medicine 6565 tannin Street, NC- 205, Houston, TX 77030 U. S. A. Physical examination showed a few cafc- au- lait spots on his trunk, bul no other stigmata of neurofibromatosis. Ophthalmic examination revealed best corrected visual acuity of 20/ 20 RE and 20/ 30 LE. There was mild upper-lid edema and ptosis LE, with no lagophthalmos. There was no other evidence of floppy eyelid syndrome. Hertel exophthalmometry measurements were 26 mm RE and 25 mm LE. The corneal sensation to the touch of the tip of a cotton swab was absent bilaterally. The sensation of light touch was markedly reduced in the cutaneous distribution of the first division of the trigeminal nerve bilaterally and moderately decreased in the cutaneous distribution of the second and third division of the trigeminal nerve bilaterally. The pupils were equal and reactive to light, with no afferent pupillary defect. The extraocular motility examination and visual field testing results were normal bilaterally. Slit lamp biomicroscopy showed a central epithelial defect with an underlying inactive stromal scar LE. The intraocular pressure measurement and ophthalmoscopic findings were normal bilaterally. A left lateral tarsorrhaphy was performed, and the epithelial defect LE improved. The patient was treated with topical lubrication. A magnetic resonance imaging ( MRI) scan of the head showed bilateral trigeminal masses that were slightly hypointense on Tl - weighted and proton- density images ( Fig. 1). The lesions demonstrated hypointense and isointense signal on T2- weighted MRI ( Fig. 2). There was homogeneous enhancement of the lesions after the administration of gadolinium-diethylenetriaminepentaacetic acid ( DTPA) ( Fig. 3). The patient underwent craniotomy and exploration of the trigeminal ganglion. The ganglion appeared to be infiltrated by a soft, brown tissue. Microscopic examination showed ganglion cells that were entrapped and widely separated by large amounts of eosinophilic material ( Fig. 4). This acellular material stained mildly with trichrome and sulfonated alcian blue, and showed birefringence under polarized light ( Fig. 5). These histochemical findings were consistent with the presence of amyloid. Electron microscopy showed characteristic straight and un-branched fibrils measuring 8 to 10 nm in diameter, consistent with the diagnosis of amyloid ( Fig. 6). Unfortunately, the patient was lost to further follow- up. No further information was available. No diagnosis was made 192 MICROBIAL KERATITIS SIGNALING TRIGEMINAL AMYLOIDOMA 193 FIG 1. T1- weighted coronal magnetic resonance image of the head shows a slightly hy-pointense signal intensity in bilateral trigeminal nerves ( arrow). FIG 2. T2- weighted axial magnetic resonance image shows hypointense and isointense signals in the trigeminal nerves bilaterally ( arrow). FIG 3. T1- weighted axial magnetic resonance image shows homogenous enhancement of the lesions after administration of gadolinium-diethylenetriaminepentaacetic acid ( DTPA; arrow). J Neitm- Oplillmlmol. Vol. IS. No. . i. IWH 194 1. E. KIRCH ET AL. FIG 4. Hematoxylin- eosin- stained section of trigeminal ganglion biopsy reveals scattered ganglion cells entrapped within large amounts of eosinophilic material. FIG 6. Electron microscopy shows straight and unbranched fibrils consistent with the diagnosis of amyloid. of systemic amyloidosis because the medical evaluation was limited. DISCUSSION Amyloid is a fibrous protein that may be stored in the extracellular space in one or more organs ( 1). Amyloidosis is classified as primary when there is no underlying disease present or secondary when there is associated underlying disease, such as multiple myeloma, rheumatoid arthritis, osteomyelitis, or tuberculosis. Isolated amyloidoma rarely involves the central nervous system ( 1). Although amyloidoma has been reported in the orbit, spine, pituitary gland, jugular foramen, cerebellopontine angle, and cerebral while matter, there are only five previous reports ( 7 cases) of isolated amyloidoma that involved the gasserian ganglion causing trigeminal neuropathy ( 2- 6). Only one of these cases involved the gasserian ganglion bilaterally ( 6). Of the seven previously reported patients, five were women and two were men. Their ages ranged between 32 and 59 years. None of the patients had systemic amyloidosis. Hypesthesia in two or more divisions of the trigeminal nerve was present in all seven patients, trigeminal pain was present in five, and weakness or atrophy of the masticatory muscles was present in four. In one patient, trigeminal nerve dysfunction developed after an anesthetic injection for a dental procedure and in another patient, trigeminal pain, and later, trigeminal hypesthesia developed after infection by herpes zoster virus. To our knowledge, this is the first case of trigeminal amyloidoma that had manifestations of microbial keratitis and a nonhealing epithelial defect. Patients with microbial keratitis without a risk factor ( e. g., trauma, contact lens wear) should be evaluated for evidence of trigeminal dysfunction. Assessment of the corneal reflex and facial sensation in these cases may lead to the appropriate diagnosis. Unexplained absence of the corneal FIG 5. Birefringence of eosinophilic material under polarized light ( arrow). J Neitiv- OphlhciliHol, Vol. IK No. .1. 1998 MICROBIAL KERATITIS SIGNALING TRIGEMINAL AMYLOIDOMA 195 reflex or trigeminal neuropathy should prompt neuroim-aging of the trigeminal nerve. REFERENCES 1. Kyle RA, Bayi'd ED. Amyloidosis: review of 236 Cases. Medicine 1975; 54: 271- 99. 2. Daly DD, Love JG, Dockerty MB. Amyloid tumor of the Gasserian ganglion. J Neurosurg 1957; 14: 347- 52. 3. Borghi G, Tagliabuc G. Primary amyloidosis of the Gasserian ganglion. Ada Neurol Scant! 1961; 37: 105- 10. 4. DeCastro S, Sparks . IT, Lapcy . ID, Freidbcrg SR. Amyloidoma of the Gasserian ganglion. Surg Neurol 1976; 6: 357- 9. 5. Bornemann A, Bohl J, Hey O, el al. Amyloidoma of the Gasserian ganglion as a cause of symptomatic neuralgia of ( he trigeminal nerve: report of three cases. ./ Neurol 1993; 241: 10- 4. 6. O'Brien TJ, McKclvic PA, Vrodos N. Bilateral trigeminal amyloidoma: an unusual case of trigeminal neuropathy with a review of the literature. ./ Neurosurg 1994; 81: 780- 3. ./ Neum- Oplillmlnml. Vol. IS. No. .(. I'MS |
