Anti-Ma-2 associated encephalitis presenting as slowly progressive dementia and cerebellar atrophy

Abstract

Abstract Cognitive impairment can be a manifestation of a neurodegenerative disease or may have a reversible etiology. Among reversible causes, metabolic, nutritional and autoimmune conditions are commonly evaluated. We report 43-year-old lady who presented with history of personality changes first noted three years back. Subsequently, she developed slowness in speech and activities of her daily living, hypersomnolence, dream enactment, unexplained weight gain and memory impairment. Cognitive assessment revealed poor delayed recall, impaired copying, calculation deficits and bradyphrenia. Mild bradykinesia and rigidity of upper limbs were also noted. MRI brain showed minimal cerebral atrophy and gross cerebellar atrophy. Blood investigation revealed strongly positive anti Ma-2 antibodies. Whole body PET-CT was negative. Ma-2 encephalitis is characterised by diencephalic, brainstem and /or limbic involvement. Presence of Ma-2 antibodies has strong link with malignancies, commonest being the testicular cancer. Anti- neuronal antibodies in association with progressive cognitive impairment in the absence of encephalitis like picture is increasingly being recognized. Although autoimmune work up is generally advocated for rapidly progressive dementia, this case highlights the need to consider antineuronal antibody evaluation in a young individual with longer duration of symptoms.