Crohn’s disease associated Chronic Inflammatory Polyradiculopathy multifocal acquired demyelinating sensory and motor neuropathy (MADSAM)


Clinical Management

Poster Number: 3


Shri Kant Mishra, MD, Olive View-UCLA Medical Center, Shaweta Khosa, MBBS, Olive View-UCLA Medical Center

Acquired Inflammatory Polyradiculopathy (AIDP) and Chronic inflammatory demyelinating polyneuropathy (CIDP) are immune-mediated neuropathy disorders. Association of Crohn’s disease (CD) with AIDP and CIDP has been previously described but not with variants such multifocal acquired demyelinating sensory and motor neuropathy (MADSAM).

We report a 51-year-old man with CD who presented with weakness, pain, and numbness in bilateral upper and lower extremities. The symptoms started with numbness in his lower extremities and progressed to the upper extremities. He was not on any immunological treatment for CD. The patient had a similar episode 2 months ago which was diagnosed with AIDP and treated with IVIG and steroid taper. Patient reported another episode about a year ago.

Physical examination showed decreased muscle tone in the lower extremities. Reflexes were absent at triceps, knees, and ankles bilaterally. Sensory examination was remarkable for absent vibration and proprioception in lower extremities. Sensation to light touch was diminished in both upper and lower extremities. Laboratory examination including infectious, rheumatological, and deficiency disorders was unremarkable. MRI of the brain and spine were normal. Nerve conduction studies showed mild demyelination in tibial and peroneal nerves with prolonged F waves. Sural and superficial peroneal nerve sensory nerve action potential were absent. EMG of upper and lower extremities did not show any denervation. Lumbar puncture showed low protein, normal glucose, and pleocytosis with lymphocytic predominance. Patient was treated with IVIG for 3 days with significant improvements in his symptoms and no residual deficits.

Our patient had possible few episodes of Immune-mediated Neuropathy (AIDP and CIDP). The abnormal clinical presentation and CSF studies were nondiagnostic of CIDP with the most likely diagnosis of MADSAM. Our patient with CD presented with MADSAM and responded well to IVIG. This case highlights the unusual presentation of MADSAM associated with CD.