Abstract Details

Title A Rare Case of Severe Sensory Ganglionopathy in a Patient With a Thymoma

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P3 - Poster Session 3 (5:00 PM-6:00 PM)

Poster/Presentation
Number 9-002

Objective To describe a case of sensory ganglionopathy and discuss its clinical management.

Background Sensory ganglionopathies are unique in their presentation from length-dependent peripheral neuropathies in that they have significant ataxia without weakness, most affect large nerve fibers, are often associated with dysautonomia, and are rapidly progressive. Possible etiologies of sensory ganglionopathies include paraneoplastic, autoimmune, infectious, drug-related, or idiopathic. While around half of sensory ganglionopathies are idiopathic, many are paraneoplastic. The proposed mechanism for this is cytotoxic T cells being aggravated by the antigens in the tumors that cross-react with epitopes on sensory ganglia. Early recognition is the most critical component for treatment with immunotherapies as once the cell body of the sensory neuron is destroyed, it cannot be regenerated.

Design/Methods NA

Results A 49-year-old female presented with six months of progressive worsening numbness ascending from legs to arms, difficulty walking, difficulty holding items, and unintentional weight loss. Exam was notable for intact strength, pseudoathetosis, diminished bulk, glove and stocking pattern of sensation loss to pinprick and temperature, diminished vibratory sense in bilateral upper and lower extremities, almost complete loss of proprioception in bilateral lower extremities (up to the hip) and bilateral upper extremities. EMG revealed a non-length dependent sensory neuropathy or neuronopathy. CT Chest revealed a soft tissue mass in the anterior mediastinum that biopsy then revealed to be a thymoma. CSF and serum paraneoplastic panels were negative. Patient was treated with a short course of steroids and 7 days of plasmapheresis with minimal improvement. There are plans for possible surgical removal of the thymoma.

Conclusions Sensory ganglionopathies have a distinct presentation that needs to be recognized quickly for effective management. Increased clinical awareness is imperative for swift treatment of these patients.

Authors/Disclosures
Sanjana Nayak, MD PRESENTER	Dr. Nayak has nothing to disclose.
Bahar Saber, MD (Baylor Scott and White)	Dr. Saber has nothing to disclose.
Raleigh Sorbonne, Medical Student	Mr. Sorbonne has nothing to disclose.

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