Thread by @MedinaRiojaMD

Atypical variants of motor neuron disease.

🧠🏥🏃

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Does the phenotype matter?

Yes, as prognosis is different in some variants.

1/🧵

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)
2/🧵

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
3/🧵

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
4/4

Reference:
Rev Neurol(Paris). 2019 Apr;175(4):221-232.
doi: 10.1016/j.neurol.2018.04.016.