Frontotemporal dementia: spectrum, criteria and clinical peculiarities. 🗣️📘🧠🧐
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Definition:
Spectrum of disorders without Alzheimer's pathology characterized by subcortical + frontal + temporal dysfunction.
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Spectrum of disorders without Alzheimer's pathology characterized by subcortical + frontal + temporal dysfunction.
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Many clinical SYNDROMES share Frontotemporal Lobar Degeneration Pathology (FTLD) and are grouped in this spectrum of disorders. 🔬
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Once again: clinical SYNDROMES can have different pathologies.
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Let's focus on the most frequent syndromes associated with FTLD pathology:
1⃣ Behavioural variant 🤬
2⃣ PPAnf: non fluent 🗣️
3⃣ PPAsv: semantic variant 🔖
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1⃣ Behavioural variant 🤬
2⃣ PPAnf: non fluent 🗣️
3⃣ PPAsv: semantic variant 🔖
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1⃣ Behavioural variant
6 main characteristics. 3 or more = possible diagnosis.
🤬Desinhibition
😴Apathy
👿Lack of empathy
🕺 Perseverations/stereotypies
🍖Diet habit changes/hyperorality
💭Executive deficits on Neuropsych
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6 main characteristics. 3 or more = possible diagnosis.
🤬Desinhibition
😴Apathy
👿Lack of empathy
🕺 Perseverations/stereotypies
🍖Diet habit changes/hyperorality
💭Executive deficits on Neuropsych
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1⃣ Behavioural variant
“It is more than possible; it is probable” – A. C. Doyle.
Possible dx + compatible MRI = probable dx
Probable dx + pathologically or genetically proven = definite dx
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“It is more than possible; it is probable” – A. C. Doyle.
Possible dx + compatible MRI = probable dx
Probable dx + pathologically or genetically proven = definite dx
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1⃣ Behavioural variant
🦪Diogenes Sx
Named for the Greek philosopher and cynic,it is accompanied by a lack of concern or awareness of their condition, a disheveled appearance, disregard of house and living invironment, and overt clutter and disorganization.
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🦪Diogenes Sx
Named for the Greek philosopher and cynic,it is accompanied by a lack of concern or awareness of their condition, a disheveled appearance, disregard of house and living invironment, and overt clutter and disorganization.
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2⃣PPAnf
At least one of the next:
-Agrammatism✍️
-Apraxia of speech🗣️
And at least two of three:
-Impaired comprehension of syntactically complex sentences📘
-Spared single-word comprenhension🤔
-Spared object knowledge🏀
Possible diagnosis
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At least one of the next:
-Agrammatism✍️
-Apraxia of speech🗣️
And at least two of three:
-Impaired comprehension of syntactically complex sentences📘
-Spared single-word comprenhension🤔
-Spared object knowledge🏀
Possible diagnosis
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2⃣PPAnf
"Man can believe the impossible but man can never
believe the improbable.” – Oscar Wilde
Possible dx + compatible MRI = probable dx
Probable dx + pathologically or genetically proven = definite dx
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"Man can believe the impossible but man can never
believe the improbable.” – Oscar Wilde
Possible dx + compatible MRI = probable dx
Probable dx + pathologically or genetically proven = definite dx
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2⃣PPAnf
🦪Binary reversals
Saying the opposite of what is meant
yes/no, hi/bye, good/bad
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🦪Binary reversals
Saying the opposite of what is meant
yes/no, hi/bye, good/bad
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3⃣ PPAsv
Both of the following:
-Impaired confrontation naming📘
-Impaired single word comprehension🤔
At least three of the next four:
-Impaired object knowledge🔑
-Surface dyslexia/dysgraphia✍️
-Spared repetition📞
-Spared speech🗣️
Possible diagnosis
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Both of the following:
-Impaired confrontation naming📘
-Impaired single word comprehension🤔
At least three of the next four:
-Impaired object knowledge🔑
-Surface dyslexia/dysgraphia✍️
-Spared repetition📞
-Spared speech🗣️
Possible diagnosis
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3⃣ PPAsv
“How often have I said to you that when you have eliminated the impossible, whatever remains, however improbable, must be the truth?” – A. Conan Doyle.
Possible dx + compatible MRI = Probable
Probable dx + pathologically or genetically proven = definite
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“How often have I said to you that when you have eliminated the impossible, whatever remains, however improbable, must be the truth?” – A. Conan Doyle.
Possible dx + compatible MRI = Probable
Probable dx + pathologically or genetically proven = definite
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3⃣ PPAsv
🦪Paradoxical Funcional Facilitation: damage to the left frontal and temporal lobes can release prior and ongoing inhibition of the right parietal lobe, thereby increasing activity in a brain region critically involved
in the visual creative process.
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🦪Paradoxical Funcional Facilitation: damage to the left frontal and temporal lobes can release prior and ongoing inhibition of the right parietal lobe, thereby increasing activity in a brain region critically involved
in the visual creative process.
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MRI patterns of atrophy in different FTD spectrum disorders
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Hope you had enjoyed this little thread and remember, accurate diagnosis is mainly done through detailed examination and history taking. 🧠🧑⚕️🩺🔨
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Sources:
1.- J Neurol Neurosurg Psychiatry. 2014Jun;85(6):692-8. doi: 10.1136/jnnp-2013-306285
2.- Am J Alzheimers Dis Other Demen. 2017 Nov;32(7):438-443. doi: 10.1177/1533317517717012
3.- Handb Clin Neurol. 2019;165:33-45.
doi: 10.1016/B978-0-444-64012-3.00003-4
1.- J Neurol Neurosurg Psychiatry. 2014Jun;85(6):692-8. doi: 10.1136/jnnp-2013-306285
2.- Am J Alzheimers Dis Other Demen. 2017 Nov;32(7):438-443. doi: 10.1177/1533317517717012
3.- Handb Clin Neurol. 2019;165:33-45.
doi: 10.1016/B978-0-444-64012-3.00003-4
More sources:
4.- Hodges' Frontotemporal Dementia, 2016.
5.- Lancet. 2015 Oct 24;386(10004):1672-82.
doi: 10.1016/S0140-6736(15)00461-4
6.- Semin Neurol. 2014 Apr;34(2):189-201.
doi: 10.1055/s-0034-1381735
4.- Hodges' Frontotemporal Dementia, 2016.
5.- Lancet. 2015 Oct 24;386(10004):1672-82.
doi: 10.1016/S0140-6736(15)00461-4
6.- Semin Neurol. 2014 Apr;34(2):189-201.
doi: 10.1055/s-0034-1381735
Even more sources:
7.- Neurology. 2011 Mar 15;76(11):1006-14.
doi: 10.1212/WNL.0b013e31821103e6
8.- Cortex. 2016 Sep;82:287-289.
doi: 10.1016/j.cortex.2016.05.017
9.- JAMA. 2018 Apr 3;319(13):1304-1306.
doi: 10.1001/jama.2017.19501
7.- Neurology. 2011 Mar 15;76(11):1006-14.
doi: 10.1212/WNL.0b013e31821103e6
8.- Cortex. 2016 Sep;82:287-289.
doi: 10.1016/j.cortex.2016.05.017
9.- JAMA. 2018 Apr 3;319(13):1304-1306.
doi: 10.1001/jama.2017.19501
Last one 🤓
10.- Nat Rev Neurol. 2017 Jul;13(7):406-419.
doi: 10.1038/nrneurol.2017.75
10.- Nat Rev Neurol. 2017 Jul;13(7):406-419.
doi: 10.1038/nrneurol.2017.75
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