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May 12 5 tweets 2 min read Twitter logo Read on Twitter
Atypical variants of motor neuron disease.



Does the phenotype matter?

Yes, as prognosis is different in some variants.

1/🧵 Image
a) Spinal-Onset ALS
b) Progressive Muscular Atrophy
c) Progressive Bulbar Palsy/Bulbar-onset ALS
d) Facial onset sensory and motor neuronopathy (FOSMN)
e) Flail-arm syndrome (Vulpian-Bernhardt syndrome): LMN upper limbs and UMN (usually only brisk reflexes in lower limbs)
f) Hirayama disease: monomelic amyotrophy
g) O’Sullivan-McLeod syndrome: slowly progressive distal amyotrophy of the hands and forearms extending over long periods of time
h) Flail-leg syndrome
i) Pseudopolyneuritic ALS: mimic of neuropathy 🚨
j) Primary lateral sclerosis
k) Mills’ syndrome: hemiplegic variant
l) FEWDON-MND syndrome: Finger Extensor Weakness with Downbeat nystagmus–motor neuron disease
Rev Neurol(Paris). 2019 Apr;175(4):221-232.
doi: 10.1016/j.neurol.2018.04.016.

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More from @MedinaRiojaMD

Jan 1
Midbrain Syndromes: Parinaud, Weber, Benedikt, Claude and Nothnagel.


#Neurology #Anatomy #Neurotwitter #History

Before we describe the main characteristics of these syndromes, it is improtant not to forget the main structures present in this region. ⚠️

Note: this is not a neuroanatomy 🧵

Structures not to forget at this level:

1️⃣ Corticospinal tract: motor function (contralateral)
2️⃣ Oculomotor nucleus: CN III👀
3️⃣ 🔴 Nucleus: rubrospinal tract 🦾 (arm flexion)
4️⃣ Subst Nigra: basal ganglia relay (more complex than that)
5️⃣ Inf Colliculus

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Dec 26, 2022
4⃣ core brain networks (there are more): expanding the localizationist approach 🧠 🥅🕸️

#Neuro #Neuroanatomy #Neurology #EndNeurophobia


1⃣ Default mode network

🥇 Endogenously mediated activities at rest
🥈 Self-referential and social cognitive processes,

🚫Not active during external goal-oriented processes

1⃣ Default mode network


💠 Posterior cingulate cortex
💠 Medial prefrontal cortex
💠 Precuneus
💠 Inferior parietal and medial temporal cortices

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Dec 23, 2022
Sporadic Creutzfeldt-Jakob disease: clinical variants 🧠



#CJD #Neurology #EndNeurophobia #Teaching


⏬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." 🧠🤓🥸

1⃣ Heidenhain Variant

Posterior localization, think 🤔

Visual disturbances first: 👀👁‍🗨👁️

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Dec 16, 2022
Idiopathic intracranial hypertension: MRI features.

#Neurology #Neurotwitter #MRI #educational #Tweetorial


1⃣ Empty sella turcica (🏹)

🚨Flattened appearance of the pituitary gland against the floor of the sella turcica (🔼)

2⃣ Enlarged Meckel caves bilaterally, filled with CSF (🏹)

Related to Temporal Lobe Epilepsy? Maybe

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Dec 12, 2022
Dementia with Lewy Bodies:

Part 2️⃣ Biomarkers

#Neuro #Neurotwitter #Teaching #Education




What is a biomarker? 🏷🧪️

"A characteristic that is objectively measured and evaluated as an indicator of normal biological processes, pathogenic processes, or pharmacologic responses to a therapeutic intervention." 🩸🧠


What is the use of biomarkers? 🧠

Diagnostic tool🪓

Tool for staging the disease 🥼

Indicator of prognosis📡

Predic or monitor of clinical response to an intervention🚨

Remember: "tools"should aid clinicians, not be the source of all truth.

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Dec 6, 2022
Dementia with Lewy Bodies:

Part 1️⃣ Clinical features and Probable Diagnosis

#Neuro #Neurotwitter #Teaching #Education



Introduction 🥇

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

Other members are:

- PD

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

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

Suggested reading ⬇️


Read 24 tweets

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