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Syphilitic Optic Perineuritis
404
AMERICAN JOURNAL OF OPHTHALMOLOGY
MARCH, 1981
SYPHILITIC OPTIC PERINEURITIS JAMES A. RUSH, M.D., AND EDWARD J. RYAN, M.D. Tampa, Florida
Fig. 2 (Gonvers and Zografos). Details of the gas-sterilized components: padded sleeve with Velcro tape and segment of cord with snap fasteners.
puts his forearms into the sleeves, which he then closes with Velcro tape. He connects the sleeves by a length of sterile cord to a cord suspended from the ceil ing. This latter passes through two pul leys and its end is weighted until the surgeon's arm is perfectly counterbal anced (about 2 to 3 kg). The surgeon feels as though he is working in a weightless environment; this permits great precision in all movements while requiring minimal muscular effort. Because the sterile cord is so long, the surgeon can easily disengage himself from the system at any time if he wants to leave the operating field temporarily.
We examined a patient who had syphilitic perioptic neuritis, a rarely diagnosed inflammation of the sheaths of the optic nerves that may be confused with papilledema caused by brain tumor or pseudotumor cerebri. The visual acuity was typically normal, and the only visual-field abnormality was enlargement of the blind Spots. After we excluded an intracranial mass lesion, the diagnosis was confirmed by cerebrospinal fluid sérologie findings positive for syphilis and normal intracranial pressure. Anti-inflammatory and antisyphilitic therapy resolved all the symptoms in our patient.
Syphilitic perioptic neuritis is a bilater al inflammation of the optic nerve sheaths that spares the subjacent optic nerves. 1 Visual acuity is unaffected, but the optic disks are swollen. This condition may be mistakenly diagnosed as papilledema. The accurate diagnosis of syphilitic peri optic neuritis depends upon positive sér ologie findings and normal cerebrospinal
From the Departments of Ophthalmology (Dr. Rush), and Neurology (Dr. Ryan), University of South Florida College of Medicine, Tampa, Florida. Reprint requests to James A. Rush, M.D., 12901 N. 30th St., Box 21, University of South Florida College of Medicine, Tampa, FL 33612.
VOL. 91, NO. 3
NOTES, CASES, INSTRUMENTS
fluid pressure. We recently treated a patient with syphilitic perioptic neuritis who responded to therapy. CASE REPORT A 34-year-old man was referred here on March 31, 1980, for evaluation of bilateral optic disk edema. A moderately severe, right-sided retro-orbital pain had developed one month previously. The frequent recurrences lasted several hours, and subsided spon taneously. Episodes of photopsia, resembling "bright lights and squiggly lines" and lasting a few seconds, had occurred sporadically in his right eye two weeks previously. On several occasions he had noted mo mentary blurring of the vision of his right eye. He had had typical, right-sided migraine head aches between the ages of 21 and 28 years, but his current peri-orbital pains were unlike them. He was taking no medication and, despite the headaches, continued to work as an interstate truck driver. He had had frequent heterosexual episodes and, in December 1979, had been treated with nystatin cream for a painless penile lesion. One month later, a brief nonpruritic chest rash had developed, but he had not sought treatment and denied having had syphilis. Visual acuity was 6/6 (20/20) in both eyes. Pupillomotor responses were brisk bilaterally, confrontation visual fields were full, and color vision was intact. Ocular movements were normal; there was no nys tagmus. Optokinetic reflexes were symmetrical. Re sults of biomicroscopy and applanation tonography were normal bilaterally. Each optic disk was moder ately elevated and had indistinct margins. A few peripapillary hemorrhages and dilated retinal veins were evident in each eye. An exudate was present at
405
the 8 o'clock position on the optic disk margin of the right optic nerve (Figure). The retina was otherwise normal, and there were no vitreous cells. Goldmann perimetry and tangent screen disclosed enlarged blind spots bilaterally. Results of a neurologic exami nation were noncontributory. A hemogram and routine serum chemistry profile were normal. A serum VDRL was positive in a 1:64 titer and an absorbed serum fluorescent-treponemal-antibody ab sorption (FTA-ABS) test was reactive. Results of a cranial computed tomogram were normal. A clear cerebrospinal fluid specimen was obtained; opening pressure was 140 mm Hg. The total protein was 35 mg/dl. Protein electrophoresis was normal. Cel lular analysis was not performed because of a lab oratory error. The cerebrospinal fluid VDRL was positive in a 1:4 dilution and a cerebrospinal fluid FTA-ABS test was reactive. An India ink preparation and bacterial and fungal cultures were negative. A short course of dexamethasone was administered while we were waiting for these results. After receiving the test results, we administered 9.6 million units of intramuscular benzathine penicillin during a four-week period. Six weeks after comple tion of therapy, the patient's visual acuity was 6/6 (20/20) in both eyes, visual fields were normal, and the optic disks were flat. DISCUSSION
A patient who has bilateral swollen disks and normal visual acuity is usually thought to have papilledema caused by increased intracranial pressure. If cere brospinal fluid pressure is normal, the appearance of papilledema in a patient
Figure (Rush and Ryan). Optic disks in the right (left) and left (right) eyes on March 31, 1980.
406
AMERICAN JOURNAL OF OPHTHALMOLOGY
who has normal visual acuity suggests the possibility of perineuritis of the optic nerve. 2 Optic perineuritis is an inflammation of the meningeal sheaths of the optic nerve and usually occurs as an extension of a diffuse basal meningitis that spares the optic nerve substance. It is rarely diag nosed and has been reliably documented only in syphilitic meningitis. 3 Because syphilitic meningitis frequently causes papilledema or optic neuritis, the devel opment of optic perineuritis seems to depend on the vagaries of the inflamma tory process, and the diagnosis is provi sional until normal cerebrospinal fluid pressure and functionally intact optic nerves are demonstrated. Symptoms are few in optic perineuri tis. Retro-orbital headaches caused by inflamed méninges at the base of the brain occur, but meningismus is usually absent. Because optic nerve dysfunction does not occur in this condition, visual symptoms are nonspecific; the visual sen sations in our patient simulated those of papilledema caused by pseudotumor cerebri. 4 In these circumstances the clinical distinction between optic perineuritis and pseudotumor cerebri can be difficult, but we know that syphilitic meningitis occurs four times more frequently in men than in women, 5 whereas idiopathic pseu dotumor cerebri is usually a disorder of obese women. 6 The correct diagnosis of optic perineu ritis is achieved by finding a reactive cerebrospinal fluid VDRL test; cerebro spinal fluid cell counts and protein values
MARCH, 1981
can be normal. 7 Furthermore, cerebro spinal fluid FTA-ABS tests are sensitive more than 99% of the time 8 and give false-positive results in fewer than 1% of normal subjects. 9 Six to 9 million units of penicillin G is usually adequate treatment for symp tomatic neurosyphilis, 10 but there are no established dosages for syphilitic optic perineuritis. Our patient improved with 9.6 million units of penicillin, and Lorentzen 10 reported successful results with 6 million units of procaine penicil lin G for the treatment of syphilitic op tic neuritis. REFERENCES 1. Walsh, F. B.: Syphilis of the optic nerve. Trans. Am. Acad. Ophthalmol. Otolaryngol. 60:39, 1956. 2. : Clinical Neuro-ophthalmology. Balti more, Williams and Wilkins Co., 1947, p. 666. 3. : Clinical Neuro-ophthalmology, Balti more, Williams and Wilkins Co., 1947, p. 359. 4. Rush, J. A.: Pseudotumor cerebri. Clinical profile and visual outcome in 63 patients. Mayo Clin. Proc. 55:541, 1980. 5. Drake, R. L.: Ocular syphilis. III. Review of the literature and report of a case of acute syphilitic meningitis and meningo-encephalitis with special reference to papilledema. Arch. Ophthalmol. 9:234, 1933. 6. Wilson, D. H., and Gardner, W. J.: Benign intracranial hypertension with particular reference to its occurrence in fat young women. Can. Med. Assoc. J. 95:102, 1966. 7. Felman, Y. M., and Nikitas, J. A.: Syphilis serology today. Arch. Dermatol. 116:84, 1980. 8. Hooshmand, H., Escobar, M. R., and Kopf, S. W.: Neurosyphilis. A study of 241 patients. J.A.M.A. 219:726, 1972. 9. Goldman, J. N., and Lantz, M. A.: FTA-ABS and VDRL slide test reactivity in a population of nuns. J.A.M.A. 217:53, 1971. 10. Lorentzen, S. E.: Syphilitic optic neuritis. Acta Ophthalmol. 45:769, 1967.
AMERICAN JOURNAL OF OPHTHALMOLOGY
MARCH, 1981
SYPHILITIC OPTIC PERINEURITIS JAMES A. RUSH, M.D., AND EDWARD J. RYAN, M.D. Tampa, Florida
Fig. 2 (Gonvers and Zografos). Details of the gas-sterilized components: padded sleeve with Velcro tape and segment of cord with snap fasteners.
puts his forearms into the sleeves, which he then closes with Velcro tape. He connects the sleeves by a length of sterile cord to a cord suspended from the ceil ing. This latter passes through two pul leys and its end is weighted until the surgeon's arm is perfectly counterbal anced (about 2 to 3 kg). The surgeon feels as though he is working in a weightless environment; this permits great precision in all movements while requiring minimal muscular effort. Because the sterile cord is so long, the surgeon can easily disengage himself from the system at any time if he wants to leave the operating field temporarily.
We examined a patient who had syphilitic perioptic neuritis, a rarely diagnosed inflammation of the sheaths of the optic nerves that may be confused with papilledema caused by brain tumor or pseudotumor cerebri. The visual acuity was typically normal, and the only visual-field abnormality was enlargement of the blind Spots. After we excluded an intracranial mass lesion, the diagnosis was confirmed by cerebrospinal fluid sérologie findings positive for syphilis and normal intracranial pressure. Anti-inflammatory and antisyphilitic therapy resolved all the symptoms in our patient.
Syphilitic perioptic neuritis is a bilater al inflammation of the optic nerve sheaths that spares the subjacent optic nerves. 1 Visual acuity is unaffected, but the optic disks are swollen. This condition may be mistakenly diagnosed as papilledema. The accurate diagnosis of syphilitic peri optic neuritis depends upon positive sér ologie findings and normal cerebrospinal
From the Departments of Ophthalmology (Dr. Rush), and Neurology (Dr. Ryan), University of South Florida College of Medicine, Tampa, Florida. Reprint requests to James A. Rush, M.D., 12901 N. 30th St., Box 21, University of South Florida College of Medicine, Tampa, FL 33612.
VOL. 91, NO. 3
NOTES, CASES, INSTRUMENTS
fluid pressure. We recently treated a patient with syphilitic perioptic neuritis who responded to therapy. CASE REPORT A 34-year-old man was referred here on March 31, 1980, for evaluation of bilateral optic disk edema. A moderately severe, right-sided retro-orbital pain had developed one month previously. The frequent recurrences lasted several hours, and subsided spon taneously. Episodes of photopsia, resembling "bright lights and squiggly lines" and lasting a few seconds, had occurred sporadically in his right eye two weeks previously. On several occasions he had noted mo mentary blurring of the vision of his right eye. He had had typical, right-sided migraine head aches between the ages of 21 and 28 years, but his current peri-orbital pains were unlike them. He was taking no medication and, despite the headaches, continued to work as an interstate truck driver. He had had frequent heterosexual episodes and, in December 1979, had been treated with nystatin cream for a painless penile lesion. One month later, a brief nonpruritic chest rash had developed, but he had not sought treatment and denied having had syphilis. Visual acuity was 6/6 (20/20) in both eyes. Pupillomotor responses were brisk bilaterally, confrontation visual fields were full, and color vision was intact. Ocular movements were normal; there was no nys tagmus. Optokinetic reflexes were symmetrical. Re sults of biomicroscopy and applanation tonography were normal bilaterally. Each optic disk was moder ately elevated and had indistinct margins. A few peripapillary hemorrhages and dilated retinal veins were evident in each eye. An exudate was present at
405
the 8 o'clock position on the optic disk margin of the right optic nerve (Figure). The retina was otherwise normal, and there were no vitreous cells. Goldmann perimetry and tangent screen disclosed enlarged blind spots bilaterally. Results of a neurologic exami nation were noncontributory. A hemogram and routine serum chemistry profile were normal. A serum VDRL was positive in a 1:64 titer and an absorbed serum fluorescent-treponemal-antibody ab sorption (FTA-ABS) test was reactive. Results of a cranial computed tomogram were normal. A clear cerebrospinal fluid specimen was obtained; opening pressure was 140 mm Hg. The total protein was 35 mg/dl. Protein electrophoresis was normal. Cel lular analysis was not performed because of a lab oratory error. The cerebrospinal fluid VDRL was positive in a 1:4 dilution and a cerebrospinal fluid FTA-ABS test was reactive. An India ink preparation and bacterial and fungal cultures were negative. A short course of dexamethasone was administered while we were waiting for these results. After receiving the test results, we administered 9.6 million units of intramuscular benzathine penicillin during a four-week period. Six weeks after comple tion of therapy, the patient's visual acuity was 6/6 (20/20) in both eyes, visual fields were normal, and the optic disks were flat. DISCUSSION
A patient who has bilateral swollen disks and normal visual acuity is usually thought to have papilledema caused by increased intracranial pressure. If cere brospinal fluid pressure is normal, the appearance of papilledema in a patient
Figure (Rush and Ryan). Optic disks in the right (left) and left (right) eyes on March 31, 1980.
406
AMERICAN JOURNAL OF OPHTHALMOLOGY
who has normal visual acuity suggests the possibility of perineuritis of the optic nerve. 2 Optic perineuritis is an inflammation of the meningeal sheaths of the optic nerve and usually occurs as an extension of a diffuse basal meningitis that spares the optic nerve substance. It is rarely diag nosed and has been reliably documented only in syphilitic meningitis. 3 Because syphilitic meningitis frequently causes papilledema or optic neuritis, the devel opment of optic perineuritis seems to depend on the vagaries of the inflamma tory process, and the diagnosis is provi sional until normal cerebrospinal fluid pressure and functionally intact optic nerves are demonstrated. Symptoms are few in optic perineuri tis. Retro-orbital headaches caused by inflamed méninges at the base of the brain occur, but meningismus is usually absent. Because optic nerve dysfunction does not occur in this condition, visual symptoms are nonspecific; the visual sen sations in our patient simulated those of papilledema caused by pseudotumor cerebri. 4 In these circumstances the clinical distinction between optic perineuritis and pseudotumor cerebri can be difficult, but we know that syphilitic meningitis occurs four times more frequently in men than in women, 5 whereas idiopathic pseu dotumor cerebri is usually a disorder of obese women. 6 The correct diagnosis of optic perineu ritis is achieved by finding a reactive cerebrospinal fluid VDRL test; cerebro spinal fluid cell counts and protein values
MARCH, 1981
can be normal. 7 Furthermore, cerebro spinal fluid FTA-ABS tests are sensitive more than 99% of the time 8 and give false-positive results in fewer than 1% of normal subjects. 9 Six to 9 million units of penicillin G is usually adequate treatment for symp tomatic neurosyphilis, 10 but there are no established dosages for syphilitic optic perineuritis. Our patient improved with 9.6 million units of penicillin, and Lorentzen 10 reported successful results with 6 million units of procaine penicil lin G for the treatment of syphilitic op tic neuritis. REFERENCES 1. Walsh, F. B.: Syphilis of the optic nerve. Trans. Am. Acad. Ophthalmol. Otolaryngol. 60:39, 1956. 2. : Clinical Neuro-ophthalmology. Balti more, Williams and Wilkins Co., 1947, p. 666. 3. : Clinical Neuro-ophthalmology, Balti more, Williams and Wilkins Co., 1947, p. 359. 4. Rush, J. A.: Pseudotumor cerebri. Clinical profile and visual outcome in 63 patients. Mayo Clin. Proc. 55:541, 1980. 5. Drake, R. L.: Ocular syphilis. III. Review of the literature and report of a case of acute syphilitic meningitis and meningo-encephalitis with special reference to papilledema. Arch. Ophthalmol. 9:234, 1933. 6. Wilson, D. H., and Gardner, W. J.: Benign intracranial hypertension with particular reference to its occurrence in fat young women. Can. Med. Assoc. J. 95:102, 1966. 7. Felman, Y. M., and Nikitas, J. A.: Syphilis serology today. Arch. Dermatol. 116:84, 1980. 8. Hooshmand, H., Escobar, M. R., and Kopf, S. W.: Neurosyphilis. A study of 241 patients. J.A.M.A. 219:726, 1972. 9. Goldman, J. N., and Lantz, M. A.: FTA-ABS and VDRL slide test reactivity in a population of nuns. J.A.M.A. 217:53, 1971. 10. Lorentzen, S. E.: Syphilitic optic neuritis. Acta Ophthalmol. 45:769, 1967.