Journal of Neuro-Ophthalmology, September 1986, Volume 6, Issue 3

Ophthalmoplegia due to actinomycosis.

A patient with actinomycosis, involving the meninges and right cavernous sinus, presented with diplopia due to a total right external ophthalmoplegia and left abduction weakness. These was partial right ptosis but the pupils were spared. The diagnosis was made by biopsy of a neck mass. A complete recovery was made in response to intravenous penicillin.

'mIT/wi of C/illie,,1 NellrtJ'ol'i11lwl",olog.'l 6(3): 1S7-1S9, 1986. Ophthalmoplegia Due to Actinomycosis R. John Leigh, M.D., Edward F. Good, M.D., and R. Patrick Rudy, M.D. 'c, 19H6 Raven Press, New York A patient with actinomvcosis, involving the meninges and right cavernous sinus, presented with diplopia due to a total right external ophthalmoplegia and left abduc­tion weakness. There was partial right ptosis but the pupils were spared. The diagnosis was made by biopsy of a neck mass. A complete recovery was made in re­sponse to intravenous penicillin. From the Departments of Neurology (E.F.G.) and Medicine (R.P.R.), Humana Hospital, Clear Lake, Texas, and the Depart­ment of Neurology (R.}.L.), University Hospitals of Cleveland and Cleveland Veterans Administration Medical Center, Case Western Reserve University, Cleveland, Ohio. Address correspondence and reprint requests to R. John Leigh, M.D., Department of Neurology, University Hospitals of Cleveland, 2074 Abington Road, Cleveland, OH 44106. /57 Acute diplopia due to weakness of multiple ex­traocular muscles, accompanied by ptosis but with pupil sparing, suggests myasthenia gravis or con­ditions that involve multiple cranial nerves. The latter include Fisher syndrome, cavernous sinus lesions, and basal meningitis (1,2). We report a pa­tient with acute weakness of extraocular muscles in both eyes due to actinomycosis, involving both the meninges and cavernous sinus, which re­solved completely with antibiotic therapy. CASE HISTORY The patient was a lO-year-old Latin American male who presented with a 6-month history of in­termittent fever, headache, and vomiting. One week prior to being seen, he developed double vi­sion. His initial neurological examination revealed a total external ophthalmoplegia on the right with partial ptosis but sparing of the pupil. The left eye showed weakness of abduction, but adduction and vertical movements, lid, and pupil were pre­served. The rest of his neurological examination was normal. An edrophonium test was negative. Computed tomography (CT) of the head was read as normal. An initial lumbar puncture showed an opening pressure of 19 em of water, protein was 62 mg%, glucose was 68 mg%, and there were 98 red cells and 12 white cells, of which 75% were lymphocytes. The patient was treated with a short course of corticosteroids, which produced an improvement in his ocular motility and headaches. Two weeks later, his headaches returned and he developed fever with spikes to 103°F. A further 2 weeks later, a right-sided neck mass developed and was biop­sied; this demonstrated sulfur granules. A culture of the neck mass subsequently grew Actinomyces israelii. The patient also developed abdominal pain, and a CT scan was suggestive of a liver ab­scess. Open liver biopsy was performed but was R. f. LEIGH ET AL. 111\' 1'l'[)<',,1 CI "I till' Ill'"d showed an ab­"" '11.11 c,,"ll"II\' III 111<' rq';loll 01 Ihe right cav- ,I'lil', '1I1l1" Ih,ll"llh"lhl'd wilh l'lllllrasl (Fig. I). 111 .Ielcht'l"l, tlll'r,' \\'dS mild hvdroceph,llus. A re­1'<'. 11 11Imh'lr plllll'lll!'l' !'l'v"dled ,111 opelling pres­" lIl" 01 20,'m 01 w,ll<'r, proleill W,lS J]() mg1y" and Ihl'll' \\','rl' I,H2lJ <'l'lIs, 01 whidl 7()'Y, were poly­IlHlrphs ,1I1d .10'>; Wl'rl' Ivmphocytl's. A bone scan W,lS positi\'l' over Illl' righl greater wing of splll'IHlid, TIll' p,lliellt was trl',ltl'd d'lily with 20 millioll units of pl'nicillin intravl'nously for H \\'el'ks. In response to Ihis treatment, there was t,ll,ll resolution of Ihe ophthalmop!l'gia within {, weeks, '''ldiologic,ll resolution of till' hvdroceph­,1lus ,1ftl'r multiple t1ll'rapeutic lumb<H punctures, and normalization of the spinal fluid. DISCUSSION Acute diplopia, due to wl'akness of extraocular muscles in buth eyes, may be caused by many conditiuns (3). The findings of ptusis but pupil sparing initially suggested myasthenia gravis or Fisher syndrome in our patient. However, the per­sistence of headaches, fever, and the spinal fluid findings pointed to an infectious process, Cum­plete, isolated oculomotor nerve palsy has been described as a complication of purulent meningitis (4,5). Bilateral ophthalmoplegia has been reported with tuberculous meningitis and mucormycosis FIG. 1. CT scan showing an enhancing lesion in the fight cavernous sinus (indicated by the marker). (3), but in patients with these conditions, .the pupils were also involved. In our patient, the nght third nerve palsy was accompanied by involve­ment of the right fourth and sixth nerves. Taken with the radiological evidence, this would localize involvement to the region of the right cavernous sinus. Pupil-sparing third nerve palsy is a recog­nized feature when the disease process is in the cavernous sinus (6). The explanation for this is de­bated, but in our patient it was probably due to the sparing of the pupillomotor fibers rather than to the "apparent pupil-sparing" described by Meadows (7). The left abducens palsy may have been due to the meningitis or, possibly, to in­volvement of the left cavernous sinus. Actinomycosis is a chronic, suppurative inflam­mation characterized by fibrosis and the formation of granulation tissue. In humans, the principal eti­ological agent is ActillOlIlyces israel/i, a filamentous bacterium that lives as a commensal in the mouth (H-lO). What triggers the organism to become in­fective is unknown, though dental disease and trauma have been suggested. Actinomycosis most frequently affects the face and neck, but the lungs, abdomen, and pelvis may be infected (9,10). Its prevalence has declined with the widespread use of antibiotics (9). Actinomycosis of the central nervous system is rare, with estimates ranging from one to three percent of all actinomycosis cases (11,12). Brain abscess caused b~' hem~togenousspread from the lungs is more common (12-14). Patients with brain abscess may present with headache, low­grade fever, and focal neurological deficits. Less commonly, direct spread from the base of skull and nasal sinuses may cause osteomvelitis, sub­dural empyema (15), ~r meningitis (12), as in our patient. In addition, the optic nerve sheath may be involved by local spread of the infection, causing optic neuritis (16). Actinomycosis is a very rare cause of ophthal­moplegia; the mechanism may be meningitis, uncal herniation secondarv to abscess or orbital involvement (12,15-17) .. Actinomyc~sis of the central nervous system is overlooked as a possible diagnosis frequently. Features differentiating it trom other infections include a more indolent course and local manifestations, such as the neck mass in our patient. Diagnosis depends upon demonstrating the causative agent in clinical spec­imens. Pus should be examined for "sulfur granules"-tangled masses of the etiological agent-and cultured anaerobically on enriched media (9,10,12). In planning treatment, it is important to re- OPHTHALMOPLEGIA DUE TO ACTINOMYCOSIS 159 member that the label actinomycosis-Uray fungus"-is a misnomer and that the effective drugs are antibiotics (10,12). Penicillin is the drug of choice and should be given in high, intravenous doses during the acute illness and should be con­tinued until 3 months after resolution of all signs of the disease. For those patients who are allergic to penicillin, satisfactory alternatives are erythro­mycin and tetracycline. Surgical treatment of brain or epidural abscess may be necessary. Overall, the prognosis for actinomycosis of the nervous system is better than for other infections (12). Acknowledgment: We are grateful to Dr. Robert B. Daroff for his comments and to Ms. Susan Landgraf and Holly Stevens for editorial assistance. Dr. Leigh is sup­ported by the Veterans Administration and the Evenor Armington Fund. REFERENCES 1. Glaser JS. Intranuclear disorders of eye movements. In: Duane TO, Jaeger EA, eds. ClilliCtlI ol'hllra///lo!<'sy, 1'01. 2. 2nd ed. Philadelphia; Harper and Row, 1985:1-39. 2. Leigh RJ, Zee OS. The neurology of cyc //I01'CHIt'lItS. COIl tempo­rary neurology series. Philadelphia: FA Davis, 1983:173-5. 3. Keane JR. Acute bilateral ophthalmoplegia: 60 cases. Ncu­rology 1986;36:279-81. 4. Kilss MA, Kellner JL, Gay AJ. Total third nerve paralysis. Recovery in a case of meningococcic meningitis. Arch Oph­Ihll/ mo/1972;87:107-9. 5. Milll'r NR. Solitary oculomotor nerve palsy in childhood. A//I , Ol'htital//lol 1977;83:106-11. 6. Nadeau SE, Trobe jD. 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