Disclosures: Chin-Hen Chang, MD: No financial relationships or conflicts of interest

Case Diagnosis: A case presented with trunk ataxia and limb incoordiaiton that was diagnosed with Sjögren syndrome associated sensory ganglionopathy after months of workup.

Case Description or Program Description: Patient with medical history of coronary artery disease, hypertension and type 2 diabetes lived independently until one year prior to admission when she spontaneously developed burning and decreased sensations of her hands and feet. Over months, she developed arm and leg incoordination, falls and worsening ADL function leading to her rehabilitation admission. Inpatient physical exams showed limb and trunk ataxia, dysmetria, markedly impaired proprioception and relatively preserved strength in all 4 extremities. NCS performed during her rehabilitation stay diagnosed sensory axonal polyneuropathy vs. sensory ganglionopathy of unknown etiology. Electromyography was normal. Extensive imaging, lab work up including paraneoplastic antibodies, ganglioside antibodies, toxic exposures, rheumatologic work up (including SSA and SSB) and lumbar puncture results were unrevealing. After intensive rehabilitation, patient showed limited degree of improvement and remained non-ambulatory and dependent in ADL function. During her outpatient neurology follow-up, genetic testing could not be obtained due to lack of saliva. This symptom suggested Sjögren’s syndrome. Lip biopsy was performed revealing chronic sialadenitis and a focus score of 2, confirming the diagnosis of Sjögren’s syndrome.

Setting: Acute rehabilitation hospital

Assessment/Results: Patient was started on pulse steroid therapy and mycophenolate mofetil based on previous case reports of Sjögren sensory ganglionopathy. Clinical improvement was limited after the treatment.

Discussion (relevance): This is a rare case of Sjögren sensory ganglionopathy presenting with progressive and persistent impaired limb coordination associated with sensory ataxia causing marked impairment in function and disability. This atypical clinical presentation can lead to delayed diagnosis and treatment with poor functional outcomes.

Conclusions: Physiatrists should consider Sjögren’s syndrome when studying patients presenting with sensory ganglionopathy. Lip biopsy and clinical symptoms can help clinicians establish the diagnosis of Sjögren’s syndrome when laboratory findings are negative.

Level of Evidence: Level V

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PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/sjogren-syndrome-associated-sensory-ganglionopathy-diagnosed-after-going-through-inpatient-neurologic-rehabilitation-a-case-report/