Guillain-Barré syndrome in patients with Charcot-Marie-Tooth type 1A disease, probably a non-random association.

OBJECTIVE

To describe four cases of Charcot-Marie-Tooth disease type 1A (CMT1A) who developed Guillain-Barré syndrome (GBS), respectively the most frequent genetic and inflammatory neuropathies.

METHODS

We described the patients' clinical and electrodiagnostic characteristics.

RESULTS

Our CMT1A patients developed typical GBS at various ages (3 to 76 years). GBS-related clinical manifestations were different within patients, with various severity degrees of seve (weakness, respiratory failure). Nerve conduction studies revealed more severe demyelinating features than expected in patients with no CMT1A. High cerebrospinal fluid protein level was found in 3 patients. GBS outcome was mainly good, although some patients only slowly improved.

CONCLUSIONS

Our cases are close to the previously described cases of acute worsening in CMT1A, and presented with many electrophysiological features of GBS. Overall, GBS prognosis does not seem worse in CMT1A patients than in other patients. If GBS and CMT1A were independent, the expected frequency of co-occurrence of GBS and CMT1A in our two French regions should be 1 case every 137 years. As we observed 4 cases in only 5 years, we suspect that CMT1A is a risk factor of GBS.

SIGNIFICANCE: These cases bring further evidence for a non-random link between inflammatory and genetic neuropathies.