Atypical Asymmetric Guillain–Barré Syndrome With Acute Rhabdomyolysis
A 54-year-old man presented to an outside emergency department with aching proximal leg pain for 1 day. He was discharged with orthopedic follow-up but returned with lower extremity weakness and shortness of breath. He was transferred to our emergency department for further evaluation.
Review of systems was notable for lower extremity paresthesias and aching back pain. No recent illness was reported. Physical examination revealed 2/5 strength in the lower extremity hip flexors with the left being weaker than the right. Dorsiflexion and plantarflexion of the ankles was 4/5 with the left ankle being weaker as well. The patient was areflexic only at the knees. Negative inspiratory force and forced vital capacity were nonconcerning. Serum creatine kinase was noted to be elevated at 1134 IU/L.
Within 7 hours, the patient became completely areflexic and had progressive weakness of the lower extremities. Facial and left, more than right, proximal upper extremity weakness was now noted. Hand strength remained unaffected. Negative inspiratory force and forced vital capacity declined to −25 cm-H2O/1.37 L. Given the patient's rapid loss of reflexes and progressive lower before upper extremity weakness, the diagnosis of Guillain–Barré syndrome (GBS) was made, and plasma exchange was initiated. Cytomegalovirus IgM/IgG levels were consistent with remote infection. Anti-GM1, GD1a, DC1b, and GQ1b antibodies were found to have no detectable titer. Campylobacter was not detected in the patient's stool sample. Lumbar puncture was without cytoalbuminologic dissociation.
The patient's weakness progressed, and he required mechanical ventilation on day 3. Neurophysiologic studies done that day revealed prolonged distal latencies, slowed conduction velocities without conduction block, and absent F-waves consistent with acute inflammatory demyelinating polyradiculoneuropathy. A contrasted cervical and thoracic spine magnetic resonance imaging on hospital day 7 and revealed enhancement of the nerve roots. The patient's strength improved, and he was successfully extubated on day 12. By day 35, the patient was discharged to an inpatient rehabilitation facility. His strength was 4/5 throughout except for 5/5 handgrip strength at discharge.
GBS is a common neurologic disorder and has a classic presentation with symmetric ascending weakness; however, not every patient with GBS follows this rule. Our patient is unique because his weakness was noted to be asymmetric, started proximally in every extremity, and was accompanied by myopathy. Patients with GBS presenting with progressive asymmetric,1,2 proximal before distal weakness,3 and concurrent myopathy4,5 have been individually described in the literature, but we present a patient with all 3 as a reminder that a classic syndrome may have many different presentations.