Abstract Details

Title Diffuse White Matter Disease and Rapidly Progressive Dementia Secondary to Non-Paraneoplastic Voltage-Gated Calcium Channels Antibody

Topic Autoimmune Neurology

Presentation(s) P2 - Poster Session 2 (5:30 PM-6:30 PM)

Poster/Presentation
Number 15-018

Objective N/A

Background Rapidly progressive dementias secondary to non-paraneoplastic autoimmune encephalopathies are rare but there has been increasing recognition due to recent technological progress.

Design/Methods Here we present a case of a young patient with white matter disease and rapidly progressive dementia associated with voltage-gated calcium channels (VGCC) antibody in the absence of detected malignancy.

Results A 47-year-old right handed man with no past medical history presented to our clinic for evaluation of progressive cognitive decline over 4 months. He presented with a broad spectrum of cognitive impairment with particular involvement in the domains of memory, visuospatial function, executive function, and attention. Brain MRI revealed abnormal non-enhancing FLAIR signal involving the frontal and occipital lobes as well as subcortical and periventricular white matter with involutional changes. Cerebrospinal fluid (CSF) analysis showed normal cell count, protein (48) and glucose level (73). CSF cultures and meningitis/encephalitis panel were negative. A paraneoplastic antibody panel in blood revealed a positive VGCC (>30) and high ds DNA antibody and anti-nuclear antibody titer. Malignancy work-up was negative. Brain biopsy demonstrated white matter non-specific T-cell involved gliosis. Based on the patient’s medical history, seropositive voltage-gated calcium channel antibody, and positive oligo-band in CSF, the diagnosis of autoimmune dementia was made. The patient was started on immunosuppressive treatment with IV methylprednisolone 1 gram for 5 days. Following initial treatment, the patient’s symptoms markedly improved, as well as his MoCA performance. Partial resolution of white matter disease was shown in post-treatment brain MRI.

Conclusions We reported an unusual association of rapidly progressive dementia with positive VGCC antibody. We showed this patient had positive response to immunotherapy. Clinical recognition of autoimmune dementia and initiation of immunotherapy are critical for favorable neurological outcome in these patients.

Authors/Disclosures
PRESENTER	No disclosure on file
Daniel Garbin Di Luca, MD (Washington University in St. Louis)	Dr. Garbin Di Luca has nothing to disclose.
Melissa R. Ortega, MD (University of Miami)	An immediate family member of Dr. Ortega has received personal compensation for serving as an employee of Aptitude. An immediate family member of Dr. Ortega has received personal compensation for serving as an employee of Oncocyte. An immediate family member of Dr. Ortega has received personal compensation for serving as an employee of Total Health Information.
Sakir H. Gultekin, MD (Memorial Sloan Kettering Ctr)	No disclosure on file
Xiaoyan Sun, MD, PhD (University of Miami Miller School of Medicine)	The institution of Dr. Sun has received research support from NIH. The institution of Dr. Sun has received research support from Dr. M. Lee Pearce Foundation.

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