Thread by @MedinaRiojaMD

Dementia with Lewy Bodies:

Part 1️⃣ Clinical features and Probable Diagnosis
🧠📚🔖

#Neuro #Neurotwitter #Teaching #Education

#NotEverythingIsMemory

#Dementia

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Introduction 🥇

DLB belongs to a family of disorders typically known as "synucleinopathies" 🔬

Other members are:

- MSA
- PD
- PDD

All of them have abnormal inclusions of α-Synuclein at a pathological level.

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⚠️⚠️⚠️
"Pathology is not pathogenesis", therefore we shouldn't assume that the presence of these pathological changes are a synonym of "protein toxicity".

Suggested reading ⬇️

jamanetwork.com/journals/jaman…

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Introduction

Lewy Body Dementia:

☂️ term, it includes:

- PDD
- DLB (today's topic)

🥔🍅

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Definition

DLB is a type of neurodegenerative dementia with histopathology characterized by progressive accumulation of alpha-synuclein as Lewy Bodies and Lewy Neurites in the brainstem, limbic and neocortical regions. 📖

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Main clinical features (there are more) 🤓

Dementia + 4 Core clinical symptoms:

1️⃣ Fluctuations
2️⃣ Hallucinations
3️⃣ RBD
4️⃣ Parkinsonism

We'll review each of them in order to find the main 🦪 and try to make our Dx more precise

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DLB

Distinct profile of cognitive dysfunction:

⚠️ Attention
📔 Executive function
🗻 Visospatial abilities

🦪 More impairment and more rapid decline of Executive function in PDD than DLB

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Fluctuations in DLB🤯

"Deficits in cognitive performance or daily functioning that alternate with periods of normal or close-to normal functioning."

Examples?
1️⃣ Daytime drowsiness and lethargy
2️⃣ Daytime 😴lasting for at least 2 h
3️⃣ Staring into space

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Fluctuations in DLB🤯

More examples

1️⃣ Episodes of disorganised speech
2️⃣ Brief interruptions of consciousness,
3️⃣ Periods of increased confusion
4️⃣ Episodes of diminished arousal
5️⃣ Periods of prolonged sleep

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Fluctuations in DLB🤯

Scores can help us identify fluctuations. ⬇️

🦪There is no perfect score/scale

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Fluctuations in DLB🤯

What causes fluctuations?

Disruption of thalamo-cortical or cortico-cortical connections? Maybe...

The real answer should be "weare not sure, yet". Nevertheless research using different biomarkes is being done and we may one day know the answer

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Hallucinatins in DLB 👻🪞

Present in 80% of Patients

VISUAL 👀
Complex
Well formed, include: 🤼 👶🐕

Other modalities? 👂
Auditory hallucinations have been reported in around 30% of pathologically confirmed DLB patients in one study.

🦪Always ask for Hallucinations

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RBD?

For more about RBD I highly suggest this 🧵

🤓

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https://twitter.com/1138982294633537536/status/1585058753489297409

Parkinsonism in DLB🕺💃

Rigidity and Bradykinesia more frequent

Tremor not so frequent

Limited response to Levodopa 💊

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Other features of DLB:

Severe sensitivity to antipsychotic agents 🤯

Postural instability, repeated falls🦯

Syncope or other transient episodes of unresponsiveness😴

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Other features of DLB:
🙇

Severe autonomic dysfunction:🦪

1️⃣Orthostatic hypotension
2️⃣Urinary incontinence
3️⃣Hypersomnia
4️⃣Constipation

👃Hyposmia

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Other features of DLB:
🧠

Systematized delusions
Apathy
Anxiety
Depression

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Diagnosis?

Most recent criteria published in 2017:

Probable:
Essential ➕ 2️⃣ Core clinical features

Possible
Essential ➕ 1️⃣ Core clinical feature

Biomarkers?
Topic for Part 2/2

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A prodromal DLB spectrum is now being recognized and research criteria have been proposed.

3️⃣ main phenotypes are present within this spectrum

🥇MCI-onset

🥈Delirium-onset

🥉Psychiatric-onset

For more about prodromal DLB 👇

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As many other neurodegenerative disorders, pathology will give the final diagnosis, however MRI and other biomarkers can help us reduce diagnostic uncertainty.

Part 2 will focus on biomarkers.

Hope you have enjoyed and wait for part 2!

20/20

Sources:
1.- Neuropsychologia. 2006;44(14):3011-5.
doi: 10.1016/j.neuropsychologia.2006.05.030
2.- Mol Neurodegener. 2021 Dec 18;16(1):83.
doi: 10.1186/s13024-021-00501-z
3.- Ther Adv Neurol Disord. 2021 Nov 23;14:17562864211057666.
doi: 10.1177/17562864211057666

More sources:
4.- Mol Neurodegener. 2019 Jan 21;14(1):5. doi: 10.1186/s13024-019-0306-8
5.- Aust N Z J Psychiatry. 2019 Apr;53(4):291-303.
doi: 10.1177/0004867419835029
6.- Neurology® 2020;94:1-e12. doi:10.1212/WNL.0000000000009434

Sources, again:
7.- Lancet Neurol. 2004 May;3(5):266. doi: 10.1016/S1474-4422(04)00728-8
8.- Brain. 2020 Jan 1;143(1):31-46. doi: 10.1093/brain/awz311
9.- Dis Mon. 2022 Jun 8;101441. doi: 10.1016/j.disamonth.2022.101441

Sources, sources:
10.- Neurology. 2017 Jul 4;89(1):88-100.
doi :10.1212/WNL.0000000000004058
11.- Neurology. 2020 Apr 28;94(17):743-755.
doi: 10.1212/WNL.0000000000009323