Objective: The objective of this case study is to highlight a rare neurological manifestation of Sjogren’s disease.

Background: The neurological manifestations of Sjogren’s disease can affect both the central and peripheral nervous system. We present a case of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) leading to severe pseudoataxia and pseudoathetosis.

Method: This is a case study with video.

Results: A 73-year-old male presented to clinic with lower extremity weakness and generalized pain. His surgical history included C3-C5 laminectomy and carpal tunnel release, and medical history included hypothyroidism, arthritis, and cataracts. He described his legs would “fly” out sometimes or would suddenly give out. He also experienced pain upon standing that improved after a few steps. His electrophysiologist reported dyskinesias along with REM behavior disorder type symptoms. Spine surgery was considered to correct his symptoms. The movement disorder examination was significant for choreiform movements, dystonic posturing, and moderate to severe axial and extremity ataxia. It was subsequently noted that these symptoms worsened significantly when his eyes were closed. In addition, there was 4- to 4+ weakness of all extremities proximally and distally and severely diminished sensation to all modalities, predominantly in the distal distribution. Joint position sense was impaired at the ankles and wrists.  He also had absent lower extremity deep tendon reflexes.  

MRI lumbar spine revealed advanced multilevel degenerative changes, multilevel spinal canal stenosis, and advanced multilevel facet arthrosis with grade one anterolisthesis of L4 on L5. EMG revealed left C6 and C7 motor radiculopathies, left L5 and S1 motor radiculopathies, and severe peripheral polyneuropathy with both segmental demyelination and axonal loss. Needle EMG was normal in thirty-eight tested muscles.

Conclusion: We believe his unusual presentation was most consistent with severe pseudoathetosis and pseudoataxia secondary to CIDP likely related to Sjogren’s disease. His case demonstrates the importance of a thorough sensory exam and recognizing movement disorder symptoms secondary to impaired sensation and that spine pathology may often be a distractor in this diagnosis.

« Back to 2025 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/an-atypical-case-of-ataxia-and-hyperkinetic-movements/