Quiz Form

Question Date: 06/08/2023

Question: 70 year old gentleman, presented with 2 month history of rapidly progressive cognitive decline, movement disorder- tremor, myoclonus, sleep disturbances- central sleep apnea, sleep parasomnias, periodic limb movements in sleep. He had severe shoulder pain, wasting and polyminimyoclonus. Skin changes like exfoliation present. He got seizures and status epilepticus<br><br>His MRI showed bilateral medial temporal hyperintense signal, and EEG showed bilateral temporal epileptiform discharges and slowing<br><br>Which antibody test will you send?

Options:

• LgI 1 and CASPR 2
• IgLON5
• GABA-B
• RT-QuIC

Correct Answer: LgI 1 and CASPR 2

Explaination: Rapidly progressive dementia have cognitive decline causing complete dependence in 1-2 years. Rapid Cognitive decline, movement disorder, sleep disorder, seizures, skin changes, joint inflammation, occur in LgI1 CASPR antibody disease. Autoimmune encephalitis is an important and treatable cause of rapidly progressive dementia. Timely Immunotherapy can satisfactorily reverse the cognitive dysfunction. Tumor screen is important.

Reference: Day, G. S. Rapidly Progressive Dementia. CONTINUUM Lifelong Learning in Neurology 28, 901 (2022).