Abstract: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.
“…In these cases, electrodiagnostics were not consistent with GBS or were not performed (9)(10)(11). More recently McNiel and Berger describes cases of suspected GBS from syphilis, though each are confounded by other infections including a preceding upper respiratory infection, COVID, or COVID vaccination (12,13). In each of the above cases, it remains unclear whether the patient's polyradiculopathy was demyelinating in nature, or even whether syphilis was the primary inciting factor.…”
Background
Syphilis is associated with a wide variety of different systemic presentations, earning it the moniker “The great mimicker”. Neurosyphilis is classically associated with meningovasculitis in the acute-subacute stage and tabes dorsalis and dementia paralytica in later stages. However, one of the less well described presentations includes Guillain-Barre Syndrome. This case presents a patient with neurosyphilis who developed with an ascending polyneuropathy with electrodiagnostic testing consistent with demyelination, supporting a diagnosis of neurosyphilis associated Guillain-Barre Syndrome.
Case presentation
A 37-year-old female presented to the emergency department with a weakness and difficulty swallowing. She described a three-month history of symptoms, initially starting with a persistent headache followed by one month of a pruritic rash on her chest, palms, and soles. Two weeks ago, she developed weakness in her arms, numbness in her arms and chest, and for the last two days, difficulty swallowing. Neurological exam was notable for multiple cranial neuropathies, distal greater than proximal upper and lower extremity weakness, length-dependent sensory loss, and hyporeflexia. Investigation revealed a positive Venereal Disease Research Laboratory in her cerebrospinal fluid without pleocytosis, contrast enhancement in cranial nerve V, VII, and VIII on MRI, and a demyelinating polyneuropathy on electrodiagnostic testing. She was diagnosed with Guillain-Barre syndrome, secondary to neurosyphilis. The patient acutely declined and required intubation, and ultimately made a full recovery after treatment with plasmapheresis and penicillin.
Conclusions
This case describes a clinical entity of syphilitic Guillain-Barre Syndrome and highlights the importance of including syphilis in the differential of a patient presenting with ascending polyradiculopathy, especially given the resurgence of syphilis.
“…In these cases, electrodiagnostics were not consistent with GBS or were not performed (9)(10)(11). More recently McNiel and Berger describes cases of suspected GBS from syphilis, though each are confounded by other infections including a preceding upper respiratory infection, COVID, or COVID vaccination (12,13). In each of the above cases, it remains unclear whether the patient's polyradiculopathy was demyelinating in nature, or even whether syphilis was the primary inciting factor.…”
Background
Syphilis is associated with a wide variety of different systemic presentations, earning it the moniker “The great mimicker”. Neurosyphilis is classically associated with meningovasculitis in the acute-subacute stage and tabes dorsalis and dementia paralytica in later stages. However, one of the less well described presentations includes Guillain-Barre Syndrome. This case presents a patient with neurosyphilis who developed with an ascending polyneuropathy with electrodiagnostic testing consistent with demyelination, supporting a diagnosis of neurosyphilis associated Guillain-Barre Syndrome.
Case presentation
A 37-year-old female presented to the emergency department with a weakness and difficulty swallowing. She described a three-month history of symptoms, initially starting with a persistent headache followed by one month of a pruritic rash on her chest, palms, and soles. Two weeks ago, she developed weakness in her arms, numbness in her arms and chest, and for the last two days, difficulty swallowing. Neurological exam was notable for multiple cranial neuropathies, distal greater than proximal upper and lower extremity weakness, length-dependent sensory loss, and hyporeflexia. Investigation revealed a positive Venereal Disease Research Laboratory in her cerebrospinal fluid without pleocytosis, contrast enhancement in cranial nerve V, VII, and VIII on MRI, and a demyelinating polyneuropathy on electrodiagnostic testing. She was diagnosed with Guillain-Barre syndrome, secondary to neurosyphilis. The patient acutely declined and required intubation, and ultimately made a full recovery after treatment with plasmapheresis and penicillin.
Conclusions
This case describes a clinical entity of syphilitic Guillain-Barre Syndrome and highlights the importance of including syphilis in the differential of a patient presenting with ascending polyradiculopathy, especially given the resurgence of syphilis.
“…While initial improvement with IVIG suggested a sole diagnosis of GBS, such improvement has been documented before in a case report detailing NS treated with IVIG prior to definitive treatment with PCN G [11]. Typically, serum RPR will rise in early syphilis infection and peak during the secondary stage with a decline over time, even in the absence of treatment [26].…”
Section: Discussionmentioning
confidence: 99%
“…One case was treated with PCN and plasmapheresis with initial improvement; however, the patient was also lost to followup. The remaining two cases were treated with fever therapy, and both showed moderate recovery [3][4][5][6][7][8][9][10][11]. Additionally, recent literature has shown GBS associated with coronavirus disease 2019 (COVID-19) vaccinations from multiple pharmaceutical companies [12].…”
We present a 51-year-old male, with a past medical history of type 2 insulin-dependent diabetes mellitus (T2IDDM) without neuropathy, coronavirus disease 2019 in April 2020 without residual symptoms, Raynaud's, and recent occupational outdoor exposure to insects as a construction manager who came to the emergency room complaining of a three-week history of bilateral progressive numbness and weakness beginning in his lower extremities and ascending toward his pelvis. Notably, he received the second dose of his Moderna COVID-19 vaccine one week prior to symptom onset and four weeks prior to admission. He also reported a recent appearance of a maculopapular rash on his upper extremities and flanks. Physical exam was remarkable for bilateral distal motor weakness in the upper and lower extremities with associated paresthesia and decreased reflexes in the lower extremities. The patient had slight ataxia and difficulty with heel walk and toe walk. Notably, the cranial nerve exam was normal, and the patient was afebrile. Intravenous immune globulin (IVIG) was started empirically for the treatment of Guillain-Barre syndrome (GBS), and doxycycline 100mg intravenous twice a day and ceftriaxone 2g intravenous daily were started for possible tick-borne disease. Subsequently, rapid plasma reagin (RPR) returned reactive at 1:64, and cerebral spinal fluid (CSF) venereal disease research laboratory (VDRL) test was reactive at 1:2 with markedly elevated protein and pleocytosis. Human immunodeficiency virus (HIV) testing was negative. Lyme disease testing was negative. Nerve conduction studies (NCS) and electromyography (EMG) showed a sensorimotor polyneuropathy with mixed demyelinating and axonal features. IVIG was continued for a total of five days, and antibiotics were changed to penicillin G (PCN G) for a total of 14 days for definitive treatment of early neurosyphilis (NS). While both clinical and laboratory findings confirm a positive diagnosis of NS, the patient's CSF composition showed very elevated total protein levels and pleocytosis. Additionally, his early peripheral neuropathy and EMG findings are not characteristics of a single disease and, instead, suggested a mixed pathology. We postulate that this patient had confirmed secondary syphilis with early NS associated with, and possibly correlated with, a simultaneous episode of acute inflammatory demyelinating polyneuropathy (AIDP) and/or a vaccine-related phenomenon.
Background
Syphilis is associated with a wide variety of systemic presentations, earning it the moniker “The great mimicker”. Neurosyphilis is classically associated with meningovasculitis in the acute-subacute stage and tabes dorsalis and dementia paralytica in later stages. However, one of the less well described presentations include Guillain–Barre Syndrome. This case presents a patient with an ascending polyneuropathy suspicious for Guillain–Barre Syndrome who also had other atypical findings including a truncal sensory loss, optic disc swelling, and rash ultimately found to have neurosyphilis. Electrodiagnostic testing was consistent with demyelination, supporting a diagnosis of neurosyphilis associated Guillain–Barre Syndrome.
Case presentation
A 37-year-old female presented to the emergency department with a weakness and difficulty swallowing. She described a three-month history of symptoms, initially starting with a persistent headache followed by one month of a pruritic rash on her chest, palms, and soles. Two weeks prior to presentation, she developed progressive weakness in her arms, numbness in her arms and chest, and difficulty swallowing. Neurological exam was notable for multiple cranial neuropathies, distal predominant weakness in all extremities, length-dependent sensory loss, and hyporeflexia. Investigation revealed a positive Venereal Disease Research Laboratory in her cerebrospinal fluid without significant pleocytosis, contrast enhancement in cranial nerves V, VII, and VIII on MRI, and a demyelinating polyneuropathy on electrodiagnostic testing. She was diagnosed with Guillain–Barre syndrome, secondary to neurosyphilis. The patient acutely declined and required intubation, and ultimately made a full recovery after treatment with plasmapheresis and penicillin.
Conclusions
This case describes a clinical entity of syphilitic Guillain–Barre Syndrome and highlights the importance of including syphilis in the differential of any patient presenting with ascending polyradiculopathy, especially given the resurgence of syphilis.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.