A case of Sjögren's syndrome with brachial amyotrophic diplegia (BAD) is reported.
BAD was preceded by slowly progressive sensory neuronopathy from 2 years ago.
Rapidly progressive BAD was associated with anterior horn T2 hyperintensities.
Sensory neuronopathy was associated with posterior column T2 hyperintensities.
Rapidly progressive autoimmune BAD was refractory to immunotherapy.