1 / 27

A 67-year-old female with acute mental status changes

A 67-year-old female with acute mental status changes. Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6. Clinical History. This was a 67-year-old female who presented with a fairly acute change in her mental status over the previous 6 months.

jlorene
Download Presentation

A 67-year-old female with acute mental status changes

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. A 67-year-old female with acute mental status changes Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6

  2. Clinical History • This was a 67-year-old female who presented with a fairly acute change in her mental status over the previous 6 months. • She noticed profound difficulty with her memory and confusion. • She also had ataxia suffering several falls. • Her family history was significant for some "form of brain stem atrophy" in her brother and sister, originally thought to be some form of spinocerebellar ataxia.

  3. Autopsy • An autopsy was performed and showed minimal generalized atrophy with no other gross abnormalities • Sections were taken for microscopic examination

  4. This is a section from the frontal lobe. What do you see? Frontal lobe. Click here to view H&E slide

  5. This is a section from the frontal lobe - cortex. What do you see?

  6. This is a section from the frontal lobe - cortex. What do you see? Astrogliosis Spongiform change

  7. This is a section from the frontal lobe - cortex. What do you see? Neuronal loss

  8. This is a section from the frontal lobe - cortex. What do you see? Normal cortex Neuronal loss

  9. This is a section from the frontal lobe – white matter. What do you see? The underlying white matter is relatively well-preserved

  10. Question: • The triad of: • Spongiosis • Neuronal loss • Astrogliosis • Is classic for what category of encephalopathies?

  11. Answer • Prion-related diseases • AKA spongiform encephalopathies

  12. Question: • Which are the human prion diseases?

  13. Answer • Creutzfeldt-Jakob disease (CJD) • New variant CJD (vCJD) • Gerstmann-Straussler-Scheinker disease (GSS) • Fatal familial insomnia (FFI) • Kuru

  14. Question • Is spongiform change diagnostic of prion diseases?

  15. Answer • No. Spongiform changes can be seen in: • Status spongiosus (coarse microvacuolation associated with neuronal loss) • Superficial microvacuolation of layers 2 and 3 – typical in FTLD • Metabolic diseases • Hypoxia

  16. Question • What is a helpful histologic feature in distinguishing different prion-related diseases?

  17. Answer • Plaques. There are 4 main types • Unicentric/Kuru • Multicentric • Associated with GSS • Florid • Associated with vCJD • Diffuse

  18. Kuru plaque Amyloid core with radiating spicules of amyloid Frontalcortex.com

  19. Multicentric plaque Several dense core regions of amyloid with radiating spicules which form multilobed structures

  20. Florid plaque Appear as unicentric amyloid deposits with radiating spicules of amyloid and a surrounding rim of spongiform change http://www.stn.ed.ac.uk/stn/vcjd.html

  21. This is a section of the cerebellum. What do you see? Click here to view cerebellum

  22. This is a section of the cerebellum. What do you see?

  23. This is a section of the cerebellum. What do you see? Multicentric plaques

  24. This is a section of the cerebellum. What do you see? Multicentric plaques

  25. Multicentric plaques - Immunohistochemistry

  26. Question • What is your final diagnosis?

  27. Answer • Gerstmann-Straussler-Scheinker disease

More Related