Clinical Review & Education
Images in Neurology
Oculomotor Nerve Palsy as the Presenting Symptom of Gummatous Neurosyphilis and Human Immunodeficiency Virus Infection Clinical Response to Treatment Christopher W. Hess, MD; Steven S. Rosenfeld, MD; Stanley R. Resor Jr, MD
A 39-year-old man presented with 5 months of worsening right eyelid ptosis, fatigue, and diplopia, which progressed to include anorexia, low-grade fevers, and arthralgias. An examination revealed a subtle macular rash on his trunk, arms, and palms. The right pupil was dilated to 5 mm without direct or consensual response to light or accommodation with inferotemporal deviation of the eye. Extraocular movements were impaired in all directions except abduction, with near-complete ptosis of the eyelid. Serum studies showed a positive rapid plasma reagin test result (to 1:64) and reactive fluorescent treponemal antibodies and Lyme antibodies. Cerebrospinal fluid analysis demonstrated a total protein level of 0.084 g/dL (to convert to grams per liter, multiply by 10.0); a glucose level of 47 mg/dL (to convert to millimolesperliter,multiplyby0.055);awhitebloodcellcountof18/μL
(to convert to ×109 per liter, multiply by 0.001); red blood cell count of 3 × 106/μL (to convert to ×1012 per liter, multiply by 1.0); 91% lymphocytes; 9% monocytes; a polymerase chain reaction test result negative for Lyme disease; and a Venereal Disease Research Laboratory test result reactive to 1:2. Subsequent human immunodeficiency virus (HIV) antibody and Western Blot tests were positive with a CD4 lymphocyte count of 307 and a viral load of 30 000. Magnetic resonance imaging of his brain revealed an enlarged enhancing right third cranial nerve in the interpeduncular cistern. A focus of fluid-attenuated inversion recovery hyperintensity and enhancement was also noted in the right temporal lobe cortex (Figure 1). Neurosyphilis was diagnosed, and he was treated with a 2-week course of intravenous penicillin (24 million units daily).
Figure 1. Magnetic Resonance Imaging of the Brain at Presentation
Figure 2. Magnetic Resonance Imaging of the Brain at Follow-up
A
Postgadolinium T1
FLAIR
A
Postgadolinium T1
FLAIR
B
Postgadolinium T1
FLAIR
B
Postgadolinium T1
FLAIR
Axial views show right third cranial nerve lesion (A [arrowheads]), and coronal views show temporal lobe lesion (B [arrowheads]). FLAIR indicates fluid-attenuated inversion recovery.
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Axial views showing residual enhancement of the third cranial nerve (A [arrowhead]), and coronal views show resolution of the temporal lobe lesion (B). FLAIR indicates fluid-attenuated inversion recovery.
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At 2-month follow-up, pupillary reactivity had normalized, ptosis had improved, but impaired extraocular movements persisted. Another magnetic resonance imaging scan showed radiographic resolution of the temporal lobe abnormality with residual enhancement along the third right cranial nerve (Figure 2).
Discussion Clinical or radiographic neurosyphilis occurs in 5% to 10% of untreated patients,1 although cerebrospinal fluid treponemes can occur in approximately one-third of early infections.2 Syphilis is known to increase both the susceptibility of infection and the risk of transmitting HIV. In coinfected patients, the involvement of the central ARTICLE INFORMATION Author Affiliations: Department of Neurology, Neurological Institute of New York, Columbia University College of Physicians and Surgeons, New York, New York (Hess, Resor); University of Florida Center for Movement Disorders and Neurorestoration, Gainesville (Hess); Malcom Randall VA Medical Center, Gainesville, Florida (Hess); Rose Ella Burkhardt Brain Tumor and Neuro-Oncology Center, Cleveland Clinic, Cleveland, Ohio (Rosenfeld).
nervous system is more common and progresses more rapidly, and these patients have a less robust response to treatment with a high relapse rate; thus, treatment should be aggressive, and nontreponemal titers should be checked frequently.2 Neurosyphilis has been reported to affect multiple cranial nerves.1 Three prior cases have reported gummatous third cranial nerve meningeal neurosyphilis.1,3,4 One patient was coinfected with HIV,1 although none of the patients presented with an accompanying syphilitic rash. During follow-up, 1 of 3 patients had residual deficits.3 A hyperintense T2-weighted signal was noted in 2 patients, and contrast enhancement and cranial nerve enlargement were described in all 3 patients.
Corresponding Author: Christopher W. Hess, MD, University of Florida Center for Movement Disorders and Neurorestoration, 3450 Hull Rd, Gainesville, FL 32607 (christopher.hess@neurology .ufl.edu).
clinicians with an emphasis on HIV coinfection. Mayo Clin Proc. 2007;82(9):1091-1102.
Conflict of Interest Disclosures: None reported.
4. Vogl T, Dresel S, Lochmüller H, Bergman C, Reimers C, Lissner J. Third cranial nerve palsy caused by gummatous neurosyphilis: MR findings. AJNR Am J Neuroradiol. 1993;14(6):1329-1331.
REFERENCES 1. Corr P, Bhigjee A, Lockhat F. Oculomotor nerve root enhancement in meningovascular syphilis. Clin Radiol. 2004;59(3):294-296.
3. Seeley WW, Venna N. Neurosyphilis presenting with gummatous oculomotor nerve palsy. J Neurol Neurosurg Psychiatry. 2004;75(5):789.
2. Zetola NM, Engelman J, Jensen TP, Klausner JD. Syphilis in the United States: an update for
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JAMA Neurology December 2013 Volume 70, Number 12
Copyright 2013 American Medical Association. All rights reserved.
Downloaded From: by a Göteborgs Universitet User on 01/21/2019
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Images in Neurology
Oculomotor Nerve Palsy as the Presenting Symptom of Gummatous Neurosyphilis and Human Immunodeficiency Virus Infection Clinical Response to Treatment Christopher W. Hess, MD; Steven S. Rosenfeld, MD; Stanley R. Resor Jr, MD
A 39-year-old man presented with 5 months of worsening right eyelid ptosis, fatigue, and diplopia, which progressed to include anorexia, low-grade fevers, and arthralgias. An examination revealed a subtle macular rash on his trunk, arms, and palms. The right pupil was dilated to 5 mm without direct or consensual response to light or accommodation with inferotemporal deviation of the eye. Extraocular movements were impaired in all directions except abduction, with near-complete ptosis of the eyelid. Serum studies showed a positive rapid plasma reagin test result (to 1:64) and reactive fluorescent treponemal antibodies and Lyme antibodies. Cerebrospinal fluid analysis demonstrated a total protein level of 0.084 g/dL (to convert to grams per liter, multiply by 10.0); a glucose level of 47 mg/dL (to convert to millimolesperliter,multiplyby0.055);awhitebloodcellcountof18/μL
(to convert to ×109 per liter, multiply by 0.001); red blood cell count of 3 × 106/μL (to convert to ×1012 per liter, multiply by 1.0); 91% lymphocytes; 9% monocytes; a polymerase chain reaction test result negative for Lyme disease; and a Venereal Disease Research Laboratory test result reactive to 1:2. Subsequent human immunodeficiency virus (HIV) antibody and Western Blot tests were positive with a CD4 lymphocyte count of 307 and a viral load of 30 000. Magnetic resonance imaging of his brain revealed an enlarged enhancing right third cranial nerve in the interpeduncular cistern. A focus of fluid-attenuated inversion recovery hyperintensity and enhancement was also noted in the right temporal lobe cortex (Figure 1). Neurosyphilis was diagnosed, and he was treated with a 2-week course of intravenous penicillin (24 million units daily).
Figure 1. Magnetic Resonance Imaging of the Brain at Presentation
Figure 2. Magnetic Resonance Imaging of the Brain at Follow-up
A
Postgadolinium T1
FLAIR
A
Postgadolinium T1
FLAIR
B
Postgadolinium T1
FLAIR
B
Postgadolinium T1
FLAIR
Axial views show right third cranial nerve lesion (A [arrowheads]), and coronal views show temporal lobe lesion (B [arrowheads]). FLAIR indicates fluid-attenuated inversion recovery.
1582
Axial views showing residual enhancement of the third cranial nerve (A [arrowhead]), and coronal views show resolution of the temporal lobe lesion (B). FLAIR indicates fluid-attenuated inversion recovery.
JAMA Neurology December 2013 Volume 70, Number 12
Copyright 2013 American Medical Association. All rights reserved.
Downloaded From: by a Göteborgs Universitet User on 01/21/2019
jamaneurology.com
Images in Neurology Clinical Review & Education
At 2-month follow-up, pupillary reactivity had normalized, ptosis had improved, but impaired extraocular movements persisted. Another magnetic resonance imaging scan showed radiographic resolution of the temporal lobe abnormality with residual enhancement along the third right cranial nerve (Figure 2).
Discussion Clinical or radiographic neurosyphilis occurs in 5% to 10% of untreated patients,1 although cerebrospinal fluid treponemes can occur in approximately one-third of early infections.2 Syphilis is known to increase both the susceptibility of infection and the risk of transmitting HIV. In coinfected patients, the involvement of the central ARTICLE INFORMATION Author Affiliations: Department of Neurology, Neurological Institute of New York, Columbia University College of Physicians and Surgeons, New York, New York (Hess, Resor); University of Florida Center for Movement Disorders and Neurorestoration, Gainesville (Hess); Malcom Randall VA Medical Center, Gainesville, Florida (Hess); Rose Ella Burkhardt Brain Tumor and Neuro-Oncology Center, Cleveland Clinic, Cleveland, Ohio (Rosenfeld).
nervous system is more common and progresses more rapidly, and these patients have a less robust response to treatment with a high relapse rate; thus, treatment should be aggressive, and nontreponemal titers should be checked frequently.2 Neurosyphilis has been reported to affect multiple cranial nerves.1 Three prior cases have reported gummatous third cranial nerve meningeal neurosyphilis.1,3,4 One patient was coinfected with HIV,1 although none of the patients presented with an accompanying syphilitic rash. During follow-up, 1 of 3 patients had residual deficits.3 A hyperintense T2-weighted signal was noted in 2 patients, and contrast enhancement and cranial nerve enlargement were described in all 3 patients.
Corresponding Author: Christopher W. Hess, MD, University of Florida Center for Movement Disorders and Neurorestoration, 3450 Hull Rd, Gainesville, FL 32607 (christopher.hess@neurology .ufl.edu).
clinicians with an emphasis on HIV coinfection. Mayo Clin Proc. 2007;82(9):1091-1102.
Conflict of Interest Disclosures: None reported.
4. Vogl T, Dresel S, Lochmüller H, Bergman C, Reimers C, Lissner J. Third cranial nerve palsy caused by gummatous neurosyphilis: MR findings. AJNR Am J Neuroradiol. 1993;14(6):1329-1331.
REFERENCES 1. Corr P, Bhigjee A, Lockhat F. Oculomotor nerve root enhancement in meningovascular syphilis. Clin Radiol. 2004;59(3):294-296.
3. Seeley WW, Venna N. Neurosyphilis presenting with gummatous oculomotor nerve palsy. J Neurol Neurosurg Psychiatry. 2004;75(5):789.
2. Zetola NM, Engelman J, Jensen TP, Klausner JD. Syphilis in the United States: an update for
jamaneurology.com
JAMA Neurology December 2013 Volume 70, Number 12
Copyright 2013 American Medical Association. All rights reserved.
Downloaded From: by a Göteborgs Universitet User on 01/21/2019
1583
