Thread by @MedinaRiojaMD

Sporadic Creutzfeldt-Jakob disease: clinical variants 🧠

🥸🤓

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#CJD #Neurology #EndNeurophobia #Teaching

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⏬One of my favorite 📚 about the topic (non medical and it focuses on familial forms)

Introduction 🚨

As seen in other types of neurodegenerative disorders (AD), the main localization of the pathological process will give rise to the main symptoms.

"It's not about pathophysiology, it's about localization." 🧠🤓🥸

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1⃣ Heidenhain Variant

Posterior localization, think 🤔

Visual disturbances first: 👀👁‍🗨👁️

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2⃣ Brownell-Oppenheimer variant

"Pure cerebellar form" (at the beginning) 🧠

Progressive subacute cerebellar deficits🤔

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3⃣ Stern-Garcin variant

Thalamic and basal ganglia predominant involvement 🧠🕺

🤔Falls and severe parkinsonism are the main characteristics. 🧑‍🦯

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4⃣ Amyotrophic variant?

Most controversial

Think 🤔LMN involvement is prominent 🔨

ALS like features (fasciculations, weakness, atrophy, EMG changes, etc.)🍖

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5⃣ Classical variant🧠

"Rapidly progressive dementia syndrome" 🤔 +/-
ataxia at illness onset

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Careful clinical exam and history may help accurate diagnosis. 🥼

Unfortunately as of today, there is no effective treatment, nevertheless, advance care planning is an invaluable tool which may help not only the patients, but the families as well. ✍️

Sources:
1.- Br J Hosp Med (Lond). 2018 Dec 2;79(12):712-713. doi: 10.12968/hmed.2018.79.12.712
2.- Eur J Neurol. 2022 Aug;29(8):2412-2419. doi: 10.1111/ene.15380
3.- Arch Neurol. 2009 Feb;66(2):208-15. doi: 10.1001/archneurol.2008.533

Sources:
4.- Neuroradiology. 2001 Sep;43(9):746-9. doi: 10.1007/s002340100587
5.- Neurohospitalist. 2021 Jul;11(3):270-271. doi: 10.1177/1941874420977604
6.- Arch Neurol. 2000 Jan;57(1):33-8. doi: 10.1001/archneur.57.1.33