Lewis-Sumner syndrome: contribution of diffusion tensor imaging in its differential diagnosis

Abstract

Lewis-Sumner syndrome (LSS) is an atypical variant of chronic inflammatory demyelinating polyneuropathy characterized by an asymmetric sensory-motor neuropathy with multifocal distribution. The diagnosis is typically clinical and electrophysiological but in some cases might be challenging causing a significant therapeutic delay. Diffusion tensor imaging (DTI) has been progressively used for the in vivo assessment of peripheral nerves integrity. In this study, we aimed to elucidate if DTI was able to detect the specific nerve damage in a patient with suspected LSS, and determine if DTI presented a specific pattern that could be useful in its differential diagnosis. A 38-year-old male with a right foot drop was studied. Physical examination, electrodiagnostic, and MRI studies were performed. MRI of the lower limb was acquired in a 3-T scanner and included T1-wi, T2-TSE-SPIR, and PD-TSE-SPAIR images in axial and coronal planes. Axial DTI was acquired using a single-shot EPI sequence with diffusion encoding in 32 directions. The electrodiagnostic tests suggested a demyelinating sensorimotor neuropathy with conduction blocks. Conventional MRI was normal. DTI showed pathological results in Tibial and Peroneal nerves consisting of thinning and discontinuities along both nerves but more significant in the Peroneal. Compared with MRI, DTI offered a significant improvement to detect the specific nerve damage and its characteristics. The observed nerve damage in DTI suggested polyneuropathy and was compatible with the electrophysiological findings, endorsing the LSS diagnosis. This is the first report in the literature presenting the DTI findings in LSS.