Guillain-Barré Syndrome After Primary Cytomegalovirus Infection in a Patient With a Heart Transplant

A 56-year-old man underwent cardiac transplantation in April 2018. His post-operative course was uncomplicated and he had normal allograft function. On December 2019 he was admitted for fever and diarrhea and was found to have cytomegalovirus infection. A few weeks later, he presented with Guillain-Barré Syndrome. (Level of Difficulty: Advanced.)


HISTORY OF PRESENTATION
In January 2020, a 56 year-old-man presented with 10 days of ascending sensory loss and proximal muscle weakness. Physical examination was notable for sensory loss in the lower extremities to just below the knees, in the hands and forearms, and on the anterior tongue. He had weakness of the deltoids, biceps, and intrinsic muscles of the hand, as well as weakness with hip flexion and dorsiflexion of the feet. He had a neuropathic gait. The remainder of his examination, including the cardiopulmonary examination, was unremarkable.

PATIENT MEDICAL HISTORY
The patient had a history of end-stage heart failure due to ischemic cardiomyopathy for which he underwent left ventricular assist device implantation as a bridge to cardiac transplantation in April 2018. Both he and the donor were seronegative for cytomegalovirus (CMV). He had normal allograft function without allograft rejection. He was on tacrolimus and mycophenolate mofetil for maintenance immunosuppression. In December 2019, he was hospitalized for CMV infection manifesting as fever and diarrhea.
His CMV DNA polymerase chain reaction (PCR) was 23,900 copies/ml, and he was treated with intravenous ganciclovir for 6 days and then transitioned to oral valganciclovir. By discharge, fever and diarrhea had resolved. On follow-up, CMV DNA was

LEARNING OBJECTIVES
To recognize CMV infection as a complication of heart transplantation. To recognize the association between CMV infection and GBS. To review the presentation and treatment of GBS.
undetectable. Three weeks after discharge he presented with progressive muscle and sensory loss (see Figure 1 for timeline).

INVESTIGATIONS
Laboratory investigations revealed a normal basic metabolic panel and complete blood count. His liver function tests were improving relative to his prior admission.
Creatine kinase was normal. Thyroid-stimulating hormone was 0.009 IU/ml (normal 0.4 to 4.2 IU/ml), free T4 was 1.58 ng/dl (normal 0.8 to 1.5 ng/dl), and free T3 was 6.56 pg/ml (normal 2.5 to 3.9 pg/ml). Magnetic resonance imaging of the entire spine showed possible enhancement of the cauda equina nerve roots at the L5-S1 level. Nerve conduction studies were suggestive of early to subacute Guillain-Barré Syndrome (GBS).

MANAGEMENT
The patient was given 3 days of intravenous immunoglobulin (IVIG), 30 g/day, and the valganciclovir was discontinued given the concern for valganciclovir toxicity. His hyperthyroidism was treated with methimazole.

FOLLOW-UP
Shortly after the initiation of IVIG therapy, he developed shortness of breath. Right heart catheterization showed borderline high filling pressures but a normal cardiac index. He was empirically treated for rejection with furosemide and intravenous steroids, which were tapered when biopsy was consistent with 1R allograft rejection. Computed tomography angiography showed a small subsegmental PE and he was begun on anticoagulation. GBS can also affect the myocardium leading to myocarditis and subsequent heart failure (5).
Only 6 prior cases of GBS due to CMV infection after heart transplantation have been described (Table 1)  The clinical characteristics of all 7 reported cases of GBS after CMV in patients with heart transplants are summarized. *Graded using the GBS syndrome disability (12).