Sanjana Nayak1, Bahar Saber3, Raleigh Sorbonne2
1Neurology, Baylor Scott & White Medical Center - Temple, 2Baylor Scott & White Medical Center - Temple, 3Baylor Scott and White Medical Center - Temple
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.
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.
Disclaimer: Abstracts were not reviewed by Neurology® and do not reflect the views of Neurology® editors or staff.