Several Atypical Presentations of TS-HDS Associated Neuropathies (P6.269)
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Abstract
OBJECTIVE:To present 3 cases of TS-HDS associated peripheral neuropathies which he atypical presentations. Background: TS-HDS versus IgM antibody has been reported in the literature to predominantly cause painful distal sensory > motor peripheral neuropathy. The common presentation is usually distal neuropathic pain affecting the hands or feet, predominantly. This is not extremely well-defined syndrome, to this point. Methods:Chart review of 3 cases of TS-HDS associated peripheral neuropathies was performed. Results: Three cases of atypical, slowly progressing disease were found in review of University of Missouri-Columbia health system medical record. One of the cases presented with severe intractable facial pain, initially diagnosed as trigeminal neuralgia, which was followed after about 5 years with a painful upper extremity peripheral neuropathy. This neuropathy was initially primarily affecting the ulnar nerve, with excessive pain in and around the left ulnar groove. It progressed to distal greater than proximal muscle wasting about 3 years after diagnosis. A second case started with intractable occipital pain bilaterally, initially diagnosed as new daily persistent headache, and then progressed to a peripheral neuropathy and painful proximal myopathy and severely elevated CK levels. A third case presented with distal, painless muscle wasting and profound weakness with loss of pinprick sensation, but was associated with diplopia and ptosis on fatigue. Conclusions:It may be reasonable to consider TS-HDS associated pathology in cases of intractable pain syndromes, particularly trigeminal neuralgia, or new daily persistent headache. Cranial nerve lesions other than pain, such as diplopia may be associated with TS-HDS versus IgM antibody pathology. Immune modulation, such as plasmapheresis or rituximab, may be an intervention to treat cases such as those presented which were previously thought intractable.
Disclosure: Dr. Lucchese has nothing to disclose.
Thursday, April 21 2016, 8:30 am-5:30 pm
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