Log In

Forgot Password?

OR

Not a member? Continue as a nonmember.

Become a Member

By becoming a member of the AAN, you can receive exclusive information to help you at every stage of your career. Benefits include:

Join Now See All Benefits

Loading... please wait

Abstract Details

Cryoglobulinemia and Neuromyelitis optica: a case of progressive weakness and neurocognitive decline
Autoimmune Neurology
P10 - Poster Session 10 (8:00 AM-9:00 AM)
6-026
The disease expression of mixed cryoglobulinemia vasculitis caused by HCV infection triggers the downstream B-cell arm of autoimmunity.
Describe a case of a woman with mixed cryoglobulinemia who presented with progressive weakness and biopsy proven systemic vasculitis in the setting of Hepatitis C virus (HCV) negative serology.
A 61 year old woman with a history of HCV infection, mixed cryoglobulinemia, mononeuritis multiplex secondary to nervous system vasculitis affecting peripheral nerves and hypertension presented with weakness that progressed to quadriparesis and neurocognitive decline over a period of eight months.

MRI examination of the brain showed bilateral ventral pontine lesions extending into the middle cerebellar peduncles, ventrolateral midbrain and cerebral peduncles as well as bilateral cortical frontal lobe lesions. Also present were intrinsic mixed intensity DWI/ADC lesions of undetermined etiology and extensive subcortical and periventricular white matter hyperintensities. CT angiography of her head and neck showed large vessel intracranial vasculopathy involving the bilateral MCA branches. Conventional angiogram, however, did not show any evidence of angiographic vasculitis. MRI spectroscopy was non-diagnostic. MRI of the neuroaxis showed a small focus of linear non-enhancing hyperintense lesion at the level of C3. EMG demonstrated severe generalized sensory-motor polyneuropathy with severe axonal loss. EEG showed mild to moderate diffuse slowing. CSF evaluation was negative, HCV viral load was undetectable, connective tissue markers (Anti-dsDNA, Anti-SCL70, Anti-CENTR, Anti-RNP, Anti-Smith, Anti-SSA/SSB, ANCA) were negative and the remaining serology was significant for elevated ESR, CRP, low C4 complements, positive anti-AQP4 antibodies titers (1:2560) and positive myelin basic protein. Brain biopsy showed perivascular lymphocytic inflammation, confirming vasculitis. Our patient completed courses of high dose steroids, IVIG and Rituximab with mild improvement in motor strength.

Although an atypical feature of NMO, cryoglobulinemia with production of anti-AQP4 antibodies may, in part, contribute to development of a demyelinating process.

Authors/Disclosures
Aimalohi Esechie, MD, PhD (SLUH)
PRESENTER
Dr. Esechie has nothing to disclose.
Ahmed Khalid Salman Harazeen, MBBS Dr. Harazeen has nothing to disclose.
Nelitza Ivemaris Rivera Vega, MD Dr. Rivera Vega has nothing to disclose.
Neeharika Thottempudi, MD Dr. Thottempudi has nothing to disclose.
Arun Singh Chhabra, MD Dr. Chhabra has stock in Pfizer. Dr. Chhabra has stock in Biogen. Dr. Chhabra has stock in Johnson and Johnson. Dr. Chhabra has stock in Medtronic.
Hashem M. Shaltoni, MD Dr. Shaltoni has nothing to disclose.
Muhammad Zeeshan Memon, MD (UTMB) Dr. Memon has nothing to disclose.
Anand Vilaschandra Patel, MD (University of Texas Medical Branch) Dr. Patel has nothing to disclose.
Laura J. Wu, MD, PhD (UTMB Neurology) Dr. Wu has nothing to disclose.