Case
A 53-year-old African American man presented to the emergency room with acute dyspnea, anxiety, muscle spasms and severe pain in his mid-thoracic spine. He also reported paresthesia of his arms and legs. Past medical history was notable for recent open heart surgery with aortic valve replacement, mitral and tricuspid valve repair, and left atrial appendage exclusion 12 days prior to admission. Cardiovascular history was significant for a bicuspid aortic valve with resultant severe aortic insufficiency, severe mitral regurgitation, and mild tricuspid regurgitation with severe pulmonary hypertension and a markedly dilated tricuspid annulus. He had no significant coronary atherosclerosis. Additional medical history included end stage renal disease on hemodialysis, hypertension, chronic tophaceous gout, and hyperlipidemia. Physical exam revealed BP 176/94 mmHg, pulse 80 beats/min, temperature 97.9°F, respiratory rate 20, and BMI 23.89 kg/m².
Cardiovascular exam revealed a healing sternotomy scar without evidence of infection and artificial aortic valve closure on cardiac auscultation. Cardiac echocardiography showed preserved ejection fraction with a well-functioning bioprosthetic aortic valve. There was no aortic insufficiency, no mitral regurgitation, and trace to mild tricuspid regurgitation. Mean gradient for the aortic prosthesis was 10, mean mitral gradient was 2, and mean tricuspid gradient was 1. There was no pericardial effusion.
Breath sounds were diminished in the left lung base. He had preserved motor strength in the upper and lower extremities. Initial CXR revealed an elevated left hemi-diaphragm despite no intraoperative topical hypothermia or internal mammary harvest. Initial laboratory studies demonstrated sodium 133 mmol/L, potassium 4.4mmol/L, bicarb 24 mmol/L, chloride 94 mmol/L, BUN 28 mg/dl, Cr 5.9 mg/dl, and blood glucose 93 mg/dl. Complete blood count showed WBC 11.6 with neutrophilia and lymphopenia, Hb 7.0, Hct 20.8, and platelet count 317,000.
On hospital day two he subsequently developed progressive weakness and numbness of both lower extremities. Physical exam revealed symmetric bilateral lower extremity weakness, areflexia and a sensory level around T 10. Computed tomography scan of cervical, thoracic and lumbar spine demonstrated degenerative changes and osteophytes with no evidence of compression fracture, hematoma, abscess, or transverse myelitis. Lumbar puncture revealed normal opening pressure with clear fluid, protein 315 mg/dL, WBC 5 cells/mm3, RBC 0 cells/mm3, and glucose 62 mg/dL. Cerebrospinal fluid albumin was 133 mg/dl (0-35), and oligoclonal bands were negative. Acetylcholine receptor antibodies were 0.0 nmol/L, and a nasopharyngeal swab for respiratory viruses was negative.
Electrodiagnostic studies showed diffuse sensory-motor peripheral neuropathy with axonal degeneration and demyelinative changes consistent with acute inflammatory demyelinating polyneuropathy, the hallmark for Guillain-Barré Syndrome. Neurology was consulted and recommended transfer to ICU and initiation of IVIG. Stat IgA level was normal 228 mg/dl (70-400). On hospital day six the patient’s respiratory status decompensated requiring intubation and mechanical ventilation. Plasmapheresis was initiated, and IVIG was discontinued. The patient’s hospital stay was complicated by pneumonia, but he was extubated after 7 days. Motor strength gradually improved, he was subsequently transferred to acute rehab, and he was discharged home and ultimately regained full motor strength. Several months later the patient was able to receive a cadaveric heterotopic renal transplant without complication.