Tremor seen here, has the same phenomenology as Holmes (Rubral 🤔❓)Tremor. (3–4 Hz flexorextension oscillation, present at rest and exacerbated with posture and additionally intensified with action)
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So, who described this peculiar type of tremor first?
📜🧠
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Answer:
Benedikt (1899), Gordon Holmes published his description in 1904.
🤔
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🥇 Benedikt (1889)
Rubral❓
Is it always due to a 🔴 N lesion?
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Answer:
In a case-series published in 2016 in #Neurology, the most Fx lesion localizations that caused "Holmes" tremor were:
1️⃣Midbrain
2️⃣Thalamus
3️⃣ Other (including cortical localizations)
Never forget that brainstem anatomy is complex, simplification is a 🔑 asset for the clinician🥼, oversimplification may not be useful. I hope I have achieved the first one ⚖️
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)
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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
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"A characteristic that is objectively measured and evaluated as an indicator of normal biological processes, pathogenic processes, or pharmacologic responses to a therapeutic intervention." 🩸🧠
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Introduction
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.
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".