Corticobasal Degeneration

Corticobasal Degeneration

1. Corticobasal Degeneration Ade Wijaya, MD – June 2019

2. Introduction Progressive neurodegenerative disease Mahapatra RK, Edwards MJ, Schott JM, Bhatia KP. Corticobasal degeneration. The Lancet Neurology. 2004 Dec 1;3(12):736-43.

3. Introduction The first clear description of corticobasal degeneration was given in 1968 in the report by Rebeiz and colleague Typically presents in the sixth to eighth decades of life, with onset of symptoms at mean age 63 years Woman predominance Sporadic disease Mahapatra RK, Edwards MJ, Schott JM, Bhatia KP. Corticobasal degeneration. The Lancet Neurology. 2004 Dec 1;3(12):736-43.

4. Clinical Features Mahapatra RK, Edwards MJ, Schott JM, Bhatia KP. Corticobasal degeneration. The Lancet Neurology. 2004 Dec 1;3(12):736-43.

5. Clinical Phenotypes Armstrong MJ, Litvan I, Lang AE, Bak TH, Bhatia KP, Borroni B, Boxer AL, Dickson DW, Grossman M, Hallett M, Josephs KA. Criteria for the diagnosis of corticobasal degeneration. Neurology. 2013 Jan 29;80(5):496-503.

6. Diagnostic Criteria Armstrong MJ, Litvan I, Lang AE, Bak TH, Bhatia KP, Borroni B, Boxer AL, Dickson DW, Grossman M, Hallett M, Josephs KA. Criteria for the diagnosis of corticobasal degeneration. Neurology. 2013 Jan 29;80(5):496-503.

7. Neuropathological Criteria Core features Focal cortical neuronal loss Substantia nigra neuronal loss Cortical and striatal Gallyas/tau-positive neuronal and glial lesions, especially astrocytic plaques and threads, in both white and grey matter Supportive features Cortical atrophy commonly with superficial spongiosis Ballooned neurons, typically many in atrophic cortices Tau-positive oligodendroglial coiled bodies Dickson DW, Bergeron C, Chin SS, et al. Office of Rare Diseases neuropathologic criteria for corticobasal degeneration. J Neuropathol Exp Neurol 2002 ; 61: 935–46

8. Treatment No cure; treat to manage symptoms Multimodality treatment Mahapatra RK, Edwards MJ, Schott JM, Bhatia KP. Corticobasal degeneration. The Lancet Neurology. 2004 Dec 1;3(12):736-43.

9. Summary Difficult to diagnose Similar to “atypical parkinsonism” Available treatments are moderately or poorly effective