Background:
Anti-carbonic anhydrase-related protein VIII (CARPVIII) is reported to be associated with paraneoplastic cerebellar degeneration. Our case extends the spectrum of anti-CARPVIII-associated disease to severe cognitive impairment.
Methods:
We present the case of a 75-year-old woman who presented to our Department of Psychiatry and Psychotherapy with a dementia syndrome. The diagnostic approach included magnetic resonance imaging (MRI), cerebrospinal fluid analysis (CSF) analysis involving autoantibody determination, and neuropsychological examination.
Results:
Neuropsychological examination revealed severe cognitive impairment meeting the criteria for dementia. MRI showed evidence of moderate cerebral microangiopathy. CSF analysis revealed mild pleocytosis, and serum analysis revealed anti-CARPVIII autoantibodies. Based on the dementia syndrome entailing signs of CNS inflammation such as pleocytosis and the repeated detection of anti-CARPVIII autoantibodies in serum, we diagnosed autoimmune dementia as a component of mixed dementia with additional vascular dementia components.
Conclusion:
Our finding adds severe cognitive impairment to the spectrum of anti-CARPVIII-associated disease. However, detecting anti-CARPVIII antibodies may also be an incidental finding in conjunction with typical mixed dementia. Further studies are needed to evaluate the relevance of these clinical findings.