Title | Neurosyphilis Masquerading as Guillain-Barre Syndrome |
Creator | McNiel CL, Bhat N, Bindiganavile SH, Raviskanthan S, Lee AG. |
OCR Text | Show Clinical Correspondence Section Editors: Robert Avery, DO Karl C. Golnik, MD Caroline Froment, MD, PhD An-Guor Wang, MD Neurosyphilis Masquerading as Guillain–Barre Syndrome Christopher L. McNiel, Nita Bhat, MD, Shruthi Harish Bindiganavile, MD, Subahari Raviskanthan, MD, Andrew G. Lee, MD S yphilis is the great imitator and can mimic almost any pattern of neuro-ophthalmic disease. We present a case of neurosyphilis manifesting as Guillain–Barre syndrome (GBS) in which bilateral tonic pupils was the distinguishing neuro-ophthalmic sign. To our knowledge, this is the first such case in the English language ophthalmic literature. CASE REPORT A 30-year-old woman presented to the emergency department (ED) with a 2-week history of ascending weakness, which progressed from lower extremities to upper extremities bilaterally. She also had numbness of the lower lip, shoulders, and knees. She had an upper respiratory tract infection one week before presentation. Past medical, surgical, family, and social history were noncontributory. Neurologic exam confirmed moderate bilateral upper and lower extremity weakness causing difficulty walking and difficulty raising her arms above the horizontal with pain on passive motion. Sensation loss in the upper and lower extremities was also present. The reflexes, however, were intact. An electromyography (EMG) showed prolonged F waves without prolonged distal motor latencies or slowed motor conduction velocities with no decrease in motor amplitudes. The EMG findings were generalized in multiple nerve distributions but were more prominent in the upper extremities. These findings suggest an acute, demyelinating, polyneuropathy. MRI of the brain and spine were normal. A lumbar puncture was colorless and clear showing 0 red blood cells, 2 white blood cells (ref 0–5), 2 mononuclear cells (ref 0–5), glucose of 54 (40–70), and an eleWestern University of Health Sciences (CM), Lebanon, Oregon; Department of Ophthalmology (NB, SHB, SR, AGL), Blanton Eye Institute, Houston Methodist Hospital, Houston, Texas; The Houston Methodist Research Institute (AGL), Houston Methodist Hospital, Houston, Texas; Departments of Ophthalmology (AGL), Neurology, and Neurosurgery, Weill Cornell Medicine, New York, New York; Department of Ophthalmology (AGL), University of Texas Medical Branch, Galveston, Texas; University of Texas MD Anderson Cancer Center (AGL), Houston, Texas; Texas A and M College of Medicine (AGL), Bryan, Texas; Department of Ophthalmology (AGL), the University of Iowa Hospitals and Clinics, Iowa City, Iowa; and Baylor College of Medicine (AGL), Houston, Texas. The authors report no conflicts of interest. Address correspondence to Andrew G. Lee, MD, Blanton Eye Institute, Houston Methodist Hospital, 6560 Fannin Street Suite 450, Houston, TX 77030; E-mail: aglee@houstonmethodist.org e412 vated cerebrospinal fluid (CSF) protein of 89 (ref 15–45) which is consistent with albuminocytologic dissociation. She was diagnosed with GBS and started on intravenous immunoglobulin (IVIG) therapy 30 g/day for 5 days. Physical therapy reported an improvement in the patient’s ability to dress independently from more than 50% to more than 75% while still recommending a rolling walker, beside commode, and shower chair. She was discharged home, but then returned to the ED 2 months later with recurrent lower extremity weakness, associated with dyspnea. She was re-admitted for IVIG therapy, with marked improvement of her dyspnea. During the next few weeks, she experienced progressive numbness and weakness prompting her return to the ED. She was given a third loading dose of IVIG and scheduled for a maintenance dose every 3 weeks. During the next 4 months the patient’s condition was stable, but there was no return to baseline. She then returned with upper lip numbness followed by progressive weakness in her left side one week later. A few weeks later, the weakness extended to include her right side. She then developed altered taste sensation, dysphagia for solids, and left-sided visual disturbance, which she described as “blurriness.” On neurologic exam, she had diffuse weakness in all extremities, decreased vibration, proprioception, and pin prick in all extremities, and trunk, and areflexia. A final dose of IVIG was administered and showed no improvement. Neuro-ophthalmology consultation was obtained. On eye exam, her vision was 20/20 in both eyes. She had anisocoria and the pupil in the right eye was 4 mm and left eye was 5 mm in the dark, poorly reactive to light in both eyes, with the left pupil being irregular. She demonstrated light-near dissociation in both eyes (Fig. 1A, B). MRI of the brain and spine was repeated and showed no lesion. A lumbar puncture showed increased CSF white blood cells at 28 (normal ,5) and an elevated CSF protein of 61 mg/ dL (less than 50). Antigangiolioside antibodies (GM1, GD1b, and GQ1) were negative but would be of little diagnostic use for the initial event that occurred one year earlier. Given the new bilateral tonic pupils serologic testing for syphilis was ordered. Syphilis total antibody was reactive. Rapid plasma reagin was positive to a titer of 1:2048. Venereal disease research laboratory testing in her cerebrospinal fluid was also positive to a titer of 1:2, confirming the diagnosis of neurosyphilis. A repeat EMG reading showed McNiel et al: J Neuro-Ophthalmol 2022; 42: e412-e414 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence left median and ulnar motor latencies, which were delayed, slowed velocities, and small amplitudes along with absent sensory responses. The records state that the study does not electro-diagnostically suggest compromise of the neuromuscular junction or chronic inflammatory demyelinating polyneuropathy (CIDP). She was treated with intravenous penicillin and improved over time. DISCUSSION The clinical presentation of an ascending paralysis (lower extremity to upper extremity weakness), bilateral ascending parasthesias, and CSF albumino-cytologic dissociation was initially suggestive of acute inflammatory demyelinating polyneuropathy (AIDP) also referred to by the eponym GBS. The diagnostic criteria for GBS include progressive weakness of lower extremities and hyporeflexia/areflexia. Reflexes provide little value in differentiating GBS from syphilis as GBS may not demonstrate areflexia early in the disease course, whereas neurosyphilis can also produce areflexia. The weakness of GBS is usually symmetric and progresses for less than 4 weeks. Other supporting symptoms include CSF protein elevation, autonomic dysfunction, sensory symptoms, and cranial nerve involvement (1). The initial EMG results also supported the diagnosis of AIDP. Although the patient had partial increase in functional independence after treatment with IVIG, she exhibited a resurgence of symptoms after a few weeks. Neurologic exams during follow-up visits showed areflexia along with progressing weakness. Given her recurrent and longstanding symptoms and appearance of areflexia, the diagnosis was then changed to “chronic inflammatory demyelinating polyneuropathy (CIDP).” The diagnostic criteria for CIDP which is often used in clinical practice was created by the European Federation of Neurological Societies and the Peripheral Nerve Society and has a sensitivity of 81% and specificity of 96%. The European Federation of Neurological Societies and the Peripheral Nerve Society criteria for diagnosing CIDP includes chronic, progressive, stepwise or recurrent symmetric proximal, and distal weakness and sensory deficits affecting all extremities. The symptoms usually develop over FIG. 1. A. Both pupils are irregular and do not react well to light. B. Both pupils constrict on focusing on a near target. McNiel et al: J Neuro-Ophthalmol 2022; 42: e412-e414 greater than 2 months and include hyporeflexia or areflexia in all extremities (2). Patients with CIDP tend to improve following IVIG infusion, but lack of improvement does not rule out the condition as 25% of patients with GBS continue to deteriorate after infusion (1). The later symptoms differed from the original symptoms because they did not improve with IVIG, the findings became asymmetric, the upper extremities were affected more than the lower, and the EMG no longer supported the diagnosis. The final lumbar puncture had a positive rapid plasma reagin .1:2048, venereal disease research laboratory .1:2 and a positive nonquantitative syphilis total antibody. When several aspects of a patient’s course are not following the usual presentation, keeping a broad differential diagnosis that includes mimickers such as syphilis is imperative. The challenge of this case is identifying whether the initial prestation was AIDP caused by syphilis, AIDP caused by the patient’s upper respiratory tract infection, or directly caused by neurosyphilis as in tabes dorsalis. We presented strong evidence for a diagnosis of AIDP, but because the symptoms reoccurred until syphilis was diagnosed and penicillin was administered, we think syphilis was the underlying cause. To our knowledge this is only the third case of AIDP due to syphilis to be reported. These patients presented 6 weeks after the onset of symptoms and did not display the classic albumino-cytologic disassociation. Both patients initially presented with hyporeflexia or areflexia possibly because of seeking medical attention later in the course of their disease. The cases were similar to ours in that our patient displayed areflexia during her follow-up visit and each patient presented with leg weakness along with sensory changes that lasted well beyond 6 weeks (3,4). Instead of an IVIG infusion, plasma exchange was attempted in one of the reports, with no improvement in symptoms. In our case, the development of bilateral, irregular, poorly reactive, and tonic pupils led to the syphilis serology testing. Although the Miller Fisher variant of GBS can present with anisocoria this patient never had ophthalmoplegia or diplopia. The classic Argyll Robertson pupil is small and constricts poorly to direct light but briskly when a reading distance target is viewed. Neurosyphilis can also produce any pupil finding including tonic pupils and light near dissociation with or without tonic response, and the typical or atypical Argyll Robertson pupil. Prompt treatment with neurosyphilis regimen penicillin improved the patient’s symptoms and signs. Syphilis remains the great imitator and clinicians should be aware that syphilis can mimic AIDP/GBS. To our knowledge this is the first such case to be described in the English language ophthalmic literature. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: C. McNiel, N. Bhat, S. H. Bindiganavile, and A. G. Lee; b. Acquisition of data: C. McNiel, N. Bhat, S. H. Bindiganavile, S. Raviskanthan, and A. G. Lee; c. Analysis and interpretation of data: C. McNiel, N. Bhat, S. H. Bindiganavile, S. Raviskanthan, and A. G. Lee. Category 2: a. Drafting the manuscript: e413 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence C. McNiel, N. Bhat, S. H. Bindiganavile, S. Raviskanthan, and A. G. Lee; b. Revising it for intellectual content: C. McNiel, N. Bhat, S. H. Bindiganavile, S. Raviskanthan, and A. G. Lee. Category 3: a. Final approval of the completed manuscript: C. McNiel, N. Bhat, S. H. Bindiganavile, S. Raviskanthan, and A. G. Lee. REFERENCES 1. Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barré syndrome. Lancet. 2016;388:717–727. e414 2. Chronic Inflammatory Demyelinating Polyneuropathy: Considerations for Diagnosis, Management, and Population Health. Available at: https://www.ajmc.com/journals/ supplement/2018/examining-therapies-cidp/chronicinfammatory-demyelinating-polyneuropathy-considerations-fordiagnosis-management-and-population-health. Accessed May 4, 2020. 3. Yost MD, Greenlund LS, Clin M. 62-year-old man with back pain and lower extremity weakness. Mayo Clin Proc. 2017;92:805–809. 4. Wasserman S, Vallie Y, Bryer A. The great pretender. Lancet. 2011;377:1976. McNiel et al: J Neuro-Ophthalmol 2022; 42: e412-e414 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. |
Date | 2022-03 |
Language | eng |
Format | application/pdf |
Type | Text |
Publication Type | Journal Article |
Source | Journal of Neuro-Ophthalmology, March 2022, Volume 42, Issue 1 |
Publisher | Lippincott, Williams & Wilkins |
Holding Institution | Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E SLC, UT 84112-5890 |
Rights Management | © North American Neuro-Ophthalmology Society |
ARK | ark:/87278/s6g24jbm |
Setname | ehsl_novel_jno |
ID | 2197501 |
Reference URL | https://collections.lib.utah.edu/ark:/87278/s6g24jbm |