Abstract Details

Title Concomitant Chorea, Head Tremor and Cerebellar Degeneration; a Rare Combination in a Case of CRMP-5 Encephalitis

Topic Autoimmune Neurology

Presentation(s) P4 - Poster Session 4 (5:30 PM-6:30 PM)

Poster/Presentation
Number 15-003

Objective We present an unusually complex case of paraneoplastic CRMP-5 antibody positive autoimmune encephalitis, associated with small cell lung carcinoma.

Background Antibodies against collapsin-response mediator protein-5 (CRMP-5) are associated with rare paraneoplastic syndrome, often presenting as encephalomyelitis, cerebellar degeneration, loss of olfaction and taste, optic neuropathy, other cranial neuropathies, chorea, peripheral polyradiculoneuropathy or rarely, myasthenic syndrome. It is rare to see all these manifestations in a single patient. The most common associated neoplasms are small cell lung carcinoma and thymoma.

Design/Methods Case report

Results A 73-year-old right-handed woman with 40 pack-year smoking history initially presented with binocular diplopia, followed by proximal leg weakness, and sensory changes. A few weeks later, this was followed by asymmetric bilateral choreatic arm movements along with horizontal nodding head tremor. She was also found to have nystagmus, dysmetria, scanning speech, ataxia and loss of olfaction. Behavioral and personality changes were also noted. Her MRI brain with/without contrast and CSF analysis were unremarkable. EMG/NCV revealed axonal polyneuropathy. Her initial lab results were positive for low titers of voltage gated calcium channel (VGCC) antibodies and later, her autoimmune encephalitis panel revealed positive CRMP-5 antibody. Malignancy workup showed inflamed mediastinal lymph nodes and biopsy revealed small cell lung cancer. With chemotherapy, no significant improvement was noted, but she responded well to plasma exchange, especially with respect to her chorea, mental status and cerebellar symptoms.

Conclusions Although chorea is commonly seen with CRMP-5 encephalitis, our patient was unique in the sense that she also had cerebellar degeneration, head tremor, neuropathy, and possibly neuromuscular junction dysfunction, manifesting as diplopia, proximal muscle weakness and positive VGCC antibodies. Thus, multiple neurological manifestations in an elderly patient should warrant further workup for paraneoplastic etiologies to ensure early diagnosis and management with immunosuppressive therapies.

Authors/Disclosures
Harsh Patel, MBBS (Atrium Health Cabarrus) PRESENTER	No disclosure on file
Hina Aslam, MD, MBBS (Houston Methodist Hospital)	No disclosure on file
Robert G. Smith, MD, PhD, FAAN (Methodist Neurological Institute)	Dr. Smith has nothing to disclose.

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