Clinical features and prognosis of patients with Guillain-Barré and acute transverse myelitis overlap syndrome

Objectives: Patients who present with Guillain-Barré syndrome (GBS) and acute transverse myelitis (ATM), either simultaneously or consecutively, are defined as having GBS/ATM overlap syndrome. As this syndrome has been underinvestigated, we performed a literature review to evaluate case reports of GBS/ATM overlap syndrome to facilitate its early diagnosis.

Patients and methods: We searched four scientific literature databases (PUBMED, EMBASE, ELSEVIER and WEB OF SCIENCE) for cases that presented as GBS/ATM overlap syndrome. Eighteen articles that described 23 cases were included, and the clinical and prognostic data were analyzed.

Results: Initially, only five (29.4%) patients were diagnosed with GBS/ATM overlap syndrome. Patients with GBS/ATM overlap syndrome presented as four clinical features. First, some of the patients displayed abnormal pyramidal signs, including 29.4% of patients who had positive pyramidal signs or a negative plantar reflex and 17.6% of patients who had signs of areflexia or hyporeflexia combined with positive pyramidal signs. Second, patients suffered pain and respiratory failure at a high rate (43.5% with pain at the onset of the disease, 43.5% with ventilator support, and 47.8% shown with respiratory failure). Third, patients had a partial clinical recovery of immunomodulators, 56.5% of patients had a favorable outcome, 46.2% of patients who received intravenous immunoglobulin (IVIG) combined steroids responded well to the treatment. Four, acute axonal polyneuropathy seemed to be associated with poor outcomes (odds ratio = 3.00, 95% CI = 1.35-6.68, P = 0.01). Abnormalities in spinal cord magnetic resonance imaging (MRI) were detected in all patients, and the most frequently involved segments were the cervical cord (69.6%) and the thoracic cord (69.6%). In addition to spinal cord lesions, in three patients lesions in the medulla and cerebral hemisphere were also observed. The most common prescriptions included a high dose of methylprednisolone at 1 g/day for 3-5 days that was followed by a 6-week course of oral prednisone and IVIG at 0.4 g/kg/day for 5 days.

Conclusion: While it is difficult to make an early diagnosis of GBS/ATM overlap syndrome, electrophysiology is helpful in the diagnosis of GBS and spinal cord MRIs are key to identifying ATM. Brain MRIs are also recommended to detect subclinical lesions. The combined use of IVIG and steroids was the most frequent treatment. However, less than half of the patients responded positively to treatment. Acute axonal neuropathy may be a risk factor for a poor prognosis.