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Diagnosis and Management of Neurosyphilis: The Great Masquerader
I. Case History
A. Patient Demographics – 61 year old, white, male
B. Chief Complaint – Headaches x 1 month – diffuse head pain, then the left eye became
blurry and shortly thereafter, the right eye became blurry. Now right eye is blurrier than
left.
C. Ocular History: New to eye clinic. One month ago when this started, patient
went to ER and diagnosed with optic neuritis of unknown etiology – possibly due to high
blood pressure. No vision problems prior to this starting 1 month ago. Patient brought final
report from hospital with him.
1. ESR 1st day 50, 2nd day 21 (with no treatment in between)
2. Temporal Biopsy: negative
3. ANA: normal
4. Basic metabolic panel: normal
5. Visually evoked potential study: normal
6. CBC w/diff: normal
7. MRI and MRA of brain: normal
8. Chest X-ray: normal
9. CT scan of head: normal
10. Blood pressure 130/70, pulse 72 beats per minute, respirations 20,
temperature 96.9, no carotid bruit.
D. Medications: PCP prescribed Lisinopril 10mg – ½ tablet by mouth every day, for
elevated blood pressure
F. Smokes 1 Pack a day, denies alcohol usage
II. Pertinent Findings: My office visit – at literally end of the day on a Friday, lab closed
A. Vision: 20/40 OD, 20/30 OS, no improvement with pinhole in either eye
B. Pupils: RRL – APD
C. VF: FTFC OD, OS
D. Motility: FROM OD, OS no pain on eye movement
E. IOP@4:07pm, OD: 16mmHg, OS: 13mmHg
F. SLE: ALL Normal Findings
G. Red Cap Destauration Findings: Left eye 50% decreased when compared to right
H. DFE: Disc Hemorrhage at 7 o’clock OD; Disc Hemorrhage at 6 o’clock OS; Both Swollen
360 degrees
I. (-) weight loss, (-) fatigue, (-) pain when moving jaw, (+) has diffuse pain on scalp, (+)
left temporal hurts more than right temporal, but both hurt
J. Blood Pressure taken by primary care immediately prior to my visit: 180/113*
III. My Differentials1 for Bilateral Disc Swelling (in order of likelihood):
A. Papilledema - (+) bilaterally swollen hyperemic discs with blurring of disc
margins, some flame shaped hemes. Enlarged Physiological Blind Spot, normal pupil
response, normal color vision.
1. Possible Etiologies for Papilledema:
a. Malignant Hypertension – blood pressure diastolic 110-120, patient’s
blood pressure elevated at my visit but not malignant at previous ER visit or any
visits to come
b. Pseudotumor Cerebri – not typical “type” of patient, not obese,
(+) headaches, no nausea – will need LP to confirm
c. Meningitis (syphilis, bacterial, Lyme disease) – will need ESR, ACE, Lyme titer,
ANA, RPR, VDRL
d. Encephalitis – previous MRI negative for brain swelling
e. AV Malformation – previous MRI/MRA negative
B. Bilateral Ischemic Optic Neuropathy – usually unilateral with disc swelling, not
hyperemic.
1. Possible etiology for AION – GCA – patient had (+) scalp pain, (+) bilateral
temporal pain, but (-) weight loss,(-) fatigue, (-) jaw claudication
2. Bilateral NAION caused by high blood pressure
C. Infiltration of the optic disc by sarcoid, tuberculosis granuloma, leukemia,
metastasis, other inflammatory disease – lab tests need to be run to confirm or
rule out these diseases.
D. Tumor – previous MRI negative
E. Trauma – no history of this occurring to precipitate this episode
F. Leber’s Optic Neuropathy – usually occurs in 20-30 years old, initially
unilateral then rapidly becoming bilateral. Disc swelling with peripapillary
telangiectasias resulting in optic atrophy
IV. Laboratory Studies/Radiology Studies/Neurology Findings: Pt referred to the
Gainesville ER for ESR, ACE, RPR, VDRL, CBC w/diff, repeat MRI, and Lumbar Puncture.
Admitted to VA hospital for care.
A. Eye Clinic findings from ER Ophthalmologist – all findings the same. Additional testing
that was done, Ishihara color plates: normal – 10/10 plates OD, OS.
B. Blood Pressure 156/94, HR 92, Respirations 18, Temp 98.4
C. Lab Findings:
1. ESR: 13, normal
2. CBC w/diff: normal
3. ACE: normal
4. RPR and VDRL not ordered by ER doctor as I requested
D. Radiological Findings: MRI/MRA of head/neck normal
E. Gainesville Eye Clinic:
1. VA: OD: 20/50, OS: 20/40, Last Visit OD: 20/40, OS: 20/30
2. Visual Field: Enlarged blindspot in both eyes – both poor in reliability
3. All other findings the same as above - pt sent to Neurology
F. Neurological Findings:
1. Mental Status: AO x 3, language fluent, comprehension intact. Attention,
concentration, short term and long term memory demonstrated during the
history and physical exam
2. Cranial Nerve: VFF, EOMI, facial strength and sensation symmetric, raises palate,
turns head versus resistance, protrudes tongue midline
3. Sensory: Intact to light touch, sharp touch, vibration, and position x 4 extremities
4. Reflexes: all normal
5. Cerebellar: normal finger-nose, palm-dorsum-palm, heel-knee-shin
6. Gait: normal stride, stance and turning
G. Differentials in Neurology Notes:
1. Suspect benign HA syndrome w/congenital abnormality of disks
2. Possible papillitis related to chronic meningitis, infectious, or neoplastic
a. Order LP for opening pressure
b. Check CSF for VDRL
H. Lumbar Puncture Results:
1. Opening pressure 220
2. Lab of CSF: VDRL 1:16
I. Additional LAB Tests then Ordered by Neurology:
1. Serum RPR 1:32
2. HATTS – positive
3. HIV - negative
V. Final Diagnosis – Neurosyphilis
A. Patient had syphilis a long time ago – was allergic to Penicillin – never finished
required dosage per patient
B. Admitted for a desensitization of the penicillin first, then high dose penicillin for a 2
week course
VI. Follow – up Visits - Significant Findings:
A. Follow Up 1 - 3 Days after starting PCN
1. Vision improved to OD: 20/30 OD, 20/30 OS (previously 20/50)
2. Resolving ONH swelling ou and hemes still present at 7 o’clock OD and 6 o’clock OS
3. Visual Field –OD: Enlarged blindspot same, OS: Enlarged blindspot
B. Follow Up 2 – 3 days after completion of PCN
1. Vision improved: OD: 20/30 OD, 20/30 OS No Improvement with Pinhole
2. Resolving ONH swelling ou and hemes still present at 7 o’clock OD and 6 o’clock OS
3. Visual Field – OD: Enlarged blind spot same, OS: Enlarged blindspot slightly improved
–but both fields much more reliable
C. Follow Up 3 – 2 weeks after completion of PCN
1. Vision improved: OD: 20/20 OD, 20/30 OS No Improvement with Pinhole
2. OD: ONH swelling with distinct superior rim and heme still resolving
3. OS: ONH with mild swelling, now with distinct rim and surrounding water line
D. Follow Up 4 – 2 ½ months after completion of PCN
1. Best Corrected Vision: OD: 20/20 OD, 20/20 OS
2. OD: (-) ONH swelling and (-) pallor; distinct rim
3. OS: (-) ONH swelling and (-) pallor; distinct rim and surrounding water line
4. Both Visual Fields are full, blindspot normal; fair reliability of both eyes
VII. Syphilis and Neurosyphilis
A. 3 Stages of Syphilis2:
1. Primary – chancre (ulcerated painless lesion), regional lymphadenopathy (can occur on
eyelid or conjunctiva)
2. Secondary – Sore throat, fever, generalized lymphadenopathy; ocular – uveitis, optic
neuritis, retinal vasculitis, conjunctivitis, active Chorioretinitis, monocular interstitial
keratitis – “The Great Masquerader”
3. Latent – no clinical manifestations (usually following disappearance of symptoms after
secondary stage)
4. Tertiary – Tabes dorsalis, general paresis, - CNS disease, cardiovascular disease;
ocular - optic Atrophy, old chorioretinitis, interstitial keratitis, chronic iritis, Argyll
Robertson pupil
B. Criteria for Diagnosis of Syphilis3: (See Chart for visualization of reactivity of each
serological test based on stage)
1. Primary – usually the presence of a chancre, positive RPR or VDRL confirmation with
FTA-ABS. The MHA-TP is less sensitive than the FTA-ABS test; 30% will have a
nonreactive RPR, VDRL
2. Secondary – 100% Sensitivity of MHA-TP, FTA-ABS
3. Latent – Diagnosis made by combination of several tests and history: RPR is more
reactive in the early stage of latency, but becomes less reactive the longer the disease
is latent. History of negative RPR/VDRL the year prior, a fourfold increase in titer
compared with the most recent test for people with a history of syphilis or a history of
symptoms compatible with syphilis left untreated
4. Tertiary – 30% of patients RPR may be nonreactive, so be sure to check one of the
confirmatory tests: MHA-TP, FTA-ABS, HATTS
C. Criteria for Diagnosis of Neurosyphilis4: A positive MHA-TP,FTA-ABS, or HATTS and
meeting one of the CSF criteria: 1) a reactive VDRL or 2)more than 10 white blood cells
per cubic millimeter in a nontraumatic tap or 3) protein greater than 46 mg/dl (in a
nondiabetic patient)
D. Treatment5: Aqueous Penicillin G, 2 to 4 milling units intravenously every 4 hours for 10
to 14 days. If patient allergic to penicillin there is no substitution for treatment. Pt is first
desensitized by a low dose treatment of penicillin, the treated as above
E. Recommended follow – Patient to be reexamined at 3,6,12 and 24 months. Titers should
decline to less than 1:8, if increasing titers then reinfection should be considered. CSF
testing/evaluation should be repeated at least once more. Retreatment should be
considered if CSF not normalized in 2 years
F. Prognosis - 80-90% of asymptomatic neurosyphilis patients remained asymptomatic with
improvement or reversal of serology in 75-85%.6
VII. Conclusion - Clinical Pearls – Lessons Learned
A. Run all serological tests that are warranted by your differential diagnoses. Do not forget
“The Great Masquerader – Syphilis”
As in this case, the diagnosis could have been found more quickly had a RPR, VDRL,
FTA-ABS, MHA-TP or HATTS had been run by the initial examiner.
B. Be thorough when eliciting a medical history from the patient. Remember the history is
ongoing. This patient was never asked if he had, had syphilis. We also may have found
out that he was allergic to penicillin and never completed his required dosage of it,
thereby diagnosing the patient more efficiently.
IX. References
1, 2. Rhee DJ, Pyfer MF. The Wills Eye Manual. Lippincott Williams and Wilkins.
Third Edition 1999
3. Larsen SA, Steiner BM, Rudolph AH. Laboratory Diagnosis and Interpretation of
Tests for Syphilis. Clinical Microbiology Reviews
1995: 8:1-21.
4. Danesh-Meyer H, Kubis K, Sergott R Not So Slowly Progressive Visual Loss.
Survey of Ophthalmology 1999; 44:247-252
5. Chao JR, Khurana RN, Fawzi AA, et al. Syphilis: Reemergence of an Old
Adversary. Ophthalmology 2006; 113: 2074-20798.
6. Stefanis L, Rowland L. H. Houston Meritt and Neurosyphilis, Then and Now. P&S
Medical Review 1995; 2:1-10.
7. Primary and Secondary Syphilis – United States, 2003-2004. MMWR 2006; 55
(10); 269-273
8. Browning, DJ. Posterior Segment Manifestations of Ocular Syphilis, Their
Response to a Neurosyphilis Regimen of Penicillin Therapy, and the Influence of
Human Immunodeficiency Virus Status on Response. Ophthalmology 2000;
107:2015-2023.
5 Questions for CE:
1) What standard tests should be considered when trying to determine the etiology of
bilateral papilledema?
a. Lyme titer
b. blood pressure
c. RPR
d. ESR
*e. all of the above
2) What percentage of patients with Tertiary Syphilis will the RPR be negative?
a. 10%
b. 20%
*c. 30%
d. 40%
e. none of the above
3) Which one is an abnormal opening pressure on lumbar puncture?
a. 190
b. 200
c. 210
d.220
*e. none of the above
4) What is considered a malignant diastolic blood pressure?
a. 110
b.120
c.130
d.140
*e. all of the above
5) What additional serological test should be run if neurosyphilis is the diagnosis?
*a. HIV
b. Lyme titer
c. ANA
d. ACE
e. none of the above