A previously healthy 37-year-old female presented to the emergency department with headache, rash, and bulbar weakness. Three months ago, she developed a gradual onset, constant, severe holocephalic, throbbing headache with photophobia. She presented to urgent care clinic for these symptoms and was prescribed symptomatic medications without resolution. One month later, she developed a pruritic rash on her chest, neck, palms, and soles. She again presented to urgent care and was given antihistamines for the rash. Two weeks later, she began to feel weakness in her arms as well as numbness in her arms, chest, and thighs. When her weakness progressed and she started to have difficulty swallowing two days ago, she came to the emergency department. She denied any fevers but reported intermittent chills for the past three months.
The examination was notable for bilateral cranial nerve V, VI and VII palsies. Bilateral optic nerve head edema was seen on fundoscopic exam. The motor exam showed weakness in neck flexion (4/5) as well as distal greater than proximal bilateral upper extremity weakness (3/5). Reflexes were absent throughout and there was no Babinski sign. Sensation in her extremities was significantly decreased to all modalities in a stocking glove distribution up to her knees and forearms. She also noted decreased sensation below her neck to her waist, circumferentially, though it did not follow a clear dermatome. The skin exam was notable for multiple morphologies including thin scaly papules on the upper chest and neck with more erythematous papules on the neck, back, flanks and extremities. Faint erythematous macules were present on her palms and soles (Fig. 1).
The patient’s history of headache, diffuse weakness and paresthesias, rash and exam findings of optic nerve head edema and multiple cranial neuropathies was suggestive of a systemic illness with likely central nervous system involvement. The weakness, numbness, and areflexia were suggestive of a polyradiculopathy, such as that as seen in GBS. Although cranial neuropathies can be seen in GBS, optic neuropathy is atypical in idiopathic GBS and prompted further investigation.
MR head imaging was obtained that was remarkable for contrast enhancement of cranial nerves V, VII, and VIII bilaterally (Fig. 2).
MRI of the spine was unremarkable. Lumbar puncture was significant for an elevated opening pressure of 36 cmH20 (normal: 6–25 cmH20) and CSF analysis revealed an RBC < 1 /mm3 (0), WBC of 6 /mm3 (0–5 /mm3), protein of 46 mg/dL (15–45 mg/dL), and glucose of 63 mg/dL (40–70 mg/dL). CSF Infectious PCR panel, a nucleic acid test for 14 bacteria, viruses, and yeast such as N. meningitidis, HSV1/2, Cryptococcus was negative. Coxsackievirus antibodies were negative. Anti-ganglioside and campylobacter jejuni antibodies were negative. HIV PCR was negative. Rheumatologic and systemic inflammatory etiologies that may present with skin findings, such as ANCA vasculitis and sarcoidosis, were also considered, but screening labs including Angiotensin-Converting Enzyme levels were within normal limits, as well as ANA, ANCA, and SSA/B. Serum RPR was positive in 1:128 titer, and CSF VDRL was positive with 1:1 titer, confirming neurosyphilis.
Due to atypical weakness presenting with neurosyphilis, inpatient electromyography (EMG) and a nerve conduction study (NCS) were performed. NCS of the left arm and leg showed low amplitude motor studies as well as prolonged distal motor latencies and slowing of conduction velocity. F-waves in the ulnar, median, and tibial nerves were absent (Table 1) (Fig. 3).
Table 1
Selected NCS findings. Motor Nerve Conduction
Nerve/Sites | Latency (ms) | Amplitude (mV) | Duration (ms) | Velocity (m/s) |
Left Median - APB |
Wrist | 15.1 (< 4.4) | 1.4 (> 4.0) | 13.4 | |
Elbow | 20.3 | 1.4 | 11.7 | 41.9 (> 49) |
Left Ulnar - ADM |
Wrist | 6.8 (< 3.6) | 1.0 (> 5.0) | 12.0 | |
Below Elbow | 10.6 | 0.9 | 11.7 | 47.2 (> 49) |
Above Elbow | 12.9 | 0.7 | 13.4 | 44.9 |
Left Peroneal - EDB - No Response |
Left Tibial - AH |
Ankle | 14.9 (< 6.0) | 0.1 (> 3.0) | 6.5 | |
Popliteal Fossa | 29.5 | 0.1 | 3.6 | 21.9 (> 39) |
F-wave |
Nerve | F Latency (ms) | M Latency (ms) | F-M Latency (ms) |
Left Ulnar - ADM | NR | | |
Left Median - APB | NR | NR | NR |
Left Tibial - AH | NR | NR | NR |
APB = Abductor Pollicis Brevis, ADM = Abductor Digit Minimi, EDB = Extensor Digitorum Brevis, AH = Adductor Hallicus, NR = No Response. EMG in the left tibialis anterior, vastus lateralis, and first dorsal interosseous muscles showed moderately decreased recruitment. Normal values are shown in parenthesis.
These findings suggested a demyelinating polyneuropathy affecting predominantly motor nerves consistent with an acute inflammatory demyelinating polyneuropathy variant of GBS. The clinical syndrome as well as electrodiagnostic testing provided support for treatment of both GBS as well as neurosyphilis.
The patient’s negative inspiratory force was − 15 cmH2O and she was electively intubated for airway protection. Plasma exchange was started every other day for five days as treatment of the GBS and penicillin was also started for treatment of the neurosyphilis. She was extubated on day four and continued to recover. She was eventually discharged to home on day 18 after completion of 14 days of IV penicillin therapy. The patient achieved almost complete recovery by day of discharge, though unfortunately is currently lost to follow up.