UT Southwestern Medical CenterCharacterizing Lewy Pathology in 231 Essential Tremor (ET) Brains From the ET Centralized Brain Repository

Could the neuropathological overlaps between essential tremor and other degenerative diseases pave the way for innovative therapeutic approaches?

As physicians continue to explore the intricate relationships between neurological disorders, the Essential Tremor Centralized Brain Repository (ETCBR) provides significant insights into the intersection of essential tremor (ET), Lewy pathology (LP), and other comorbidities.

Key Points:

• The Essential Tremor Centralized Brain Repository (ETCBR), established in 2003, houses 231 prospectively collected ET brains.
• This study performed a comprehensive neuropathological assessment on each brain, including alpha-synuclein immunostaining.
• The results indicated that 25.1% of ET cases (58 out of 231) exhibited Lewy pathology (LP).
• 19.9% of these cases (46 out of 231) had early to late stages of Parkinson disease (PD).
• 24.1% of LP cases (14 out of 58) developed possible PD or PD after a latency of 5 or more years.

Additional Points:

• A 10-category classification scheme was created to encapsulate observed LP patterns.
• Four metrics of cerebellar pathology (Purkinje Cell, Torpedo (LH &E), Torpedo (Bielschowsky), Basket Rating) were quantified.
• ET cases exhibited a similar degree of cerebellar pathology irrespective of the presence or absence of LP.

Conclusion:

• A quarter of ET cases showed LP – a figure higher than expected based on control population studies. This hints at a clinical correlation between ET and PD, and ET with Alzheimer disease-type neuropathology.
• Cerebellar degeneration was apparent in ET cases with or without LP, indicating a connection of ET to other degenerative diseases.

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Did You Know?
Approximately 10 million people in the United States alone are impacted by essential tremor (ET), making it one of the most common neurological disorders.