A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology...
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Transcript of A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology...
![Page 1: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/1.jpg)
A 67-year-old male with behavioral and language problems
Leonidas Arvanitis, M.D.
Neuropathology Fellow, PGY-6
![Page 2: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/2.jpg)
History• The patient was a 67-year-old male with a long
standing history of progressive difficulty with language and thinking.
• His wife stated that his overall problems began approximately 17 years ago during which time he was drinking approximately 2 cases of beer per week.
• Approximately at that time he first started having "trouble placing words in sentences."
![Page 3: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/3.jpg)
History• Further cognitive evaluation revealed marked
problems with language function and suggested that he had primary progressive aphasia.
• An MRI showed extensive temporal and frontal lobe atrophy.
• His MMSE was 20/30. • The patient further declined and eventually died
![Page 4: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/4.jpg)
Autopsy• An autopsy was performed and showed
the following (describe):
http://library.med.utah.edu/WebPath/TUTORIAL/CNS/CNSDG008.html
![Page 5: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/5.jpg)
Autopsy• An autopsy was performed and showed
the following (describe):
http://library.med.utah.edu/WebPath/TUTORIAL/CNS/CNSDG008.html
Lobar atrophy involving the frontal (mostly) and temporal lobes. “Knife-like” gyri.
![Page 6: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/6.jpg)
This is a section from the frontal lobe. What do you see?
Frontal lobe. (Click here for H&E)
![Page 7: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/7.jpg)
This is a section from the frontal lobe. What do you see?
![Page 8: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/8.jpg)
This is a section from the frontal lobe. What do you see?
Eosinophilic cytoplasmic inclusions
Neuronal loss
![Page 9: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/9.jpg)
This is a section from the frontal lobe. What do you see?
Severe gliosis
![Page 10: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/10.jpg)
This is a section from the frontal lobe. What do you see?
![Page 11: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/11.jpg)
This is a section from the frontal lobe. What do you see?
Vacuolization of the superficial cortical layer
![Page 12: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/12.jpg)
This is a section from the frontal lobe. What do you see?
![Page 13: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/13.jpg)
This is a section from the frontal lobe. What do you see?
Balloon cell
![Page 14: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/14.jpg)
Question:
• In the work up of a neurodegenerative disease what immunohistochemical stains are helpful in highlighting intracellular inclusions?
![Page 15: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/15.jpg)
Answer• Tau
– Alzheimer’s disease– Pick’s disease– Progressive Supranuclear Palsy– Corticobasal degeneration
• A-synuclein– Parkinson’s disease
• TDP-43– FTLD-TDP
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Question:
• The intracellular inclusions on this case were negative for a-synuclein and TDP-43, but Tau showed the following staining pattern:
– Click here to view Tau stain
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Tau stain
![Page 18: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/18.jpg)
How are these spherical, intraneuronal inclusions known as?
![Page 19: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/19.jpg)
Pick bodies
![Page 20: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/20.jpg)
Answer
• Pick bodies are spherical intraneuronal cytoplasmic inclusions
• They are most frequently found in the frontal and temporal lobes and limbic cortex
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Question
• What is the most likely diagnosis of a patient with behavioral and language difficulties that showed these associated pathologic features?
![Page 22: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/22.jpg)
Answer
• FTLD-Tau: Pick’s disease
![Page 23: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/23.jpg)
Question
• What are the clinical features of Frontotemporal Lobar Degeneration (FTLD)?
![Page 24: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/24.jpg)
Answer• The 3 main clinical syndromes are
1.Behavioral variant– Cognitive decline and changes in social and
personal conduct
2.Progressive nonfluent aphasia (PNFA)– Problems in word retrieval but with preservation of
comprehension
3.Semantic dementia– Patients have impairment of the realm of memory
that relates to the meaning of verbal and visual inputs
![Page 25: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/25.jpg)
Question
• What are the pathologic subtypes of FTLD?
![Page 26: A 67-year-old male with behavioral and language problems Leonidas Arvanitis, M.D. Neuropathology Fellow, PGY-6.](https://reader033.fdocuments.us/reader033/viewer/2022051619/56649d965503460f94a7f702/html5/thumbnails/26.jpg)
Anwser
• FTLD-Tau
• FTLD-TDP
• FTLD-FUS
• FTLD-UPS
• FTLD-ni (no inclusions)