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.