El Escorial Revisited: Revised Criteria for the Diagnosis of Amyotrophic Lateral Sclerosis

TABLE 1
Lower Motor Neuron and Upper Motor Neuron Signs in Four CNS Regions

  Brainstem Cervical Thoracic Lumbosacral
Lower motor neuron signs
weakness,
atrophy,
fasciculations
jaw, face,
palate,
tongue,
larynx
neck, arm,
hand,
diaphragm
back,
abdomen
back, abdomen,
leg, foot
Upper motor neuron signs
pathologic spread of reflexes, clonus, etc.
clonic jaw
gag reflex
exaggerated snout reflex
pseudobulbar features
forced yawning
pathologic DTR's
spastic tone
clonic DTR's Hoffman reflex
pathologic DTR's
spastic tone

preserved reflex in weak wasted limb
loss of superficial abdominal reflexes
pathologic DTR's
spastic tone
clonic DTR's - extensor plantar response
pathologic DTR's
spastic tone

preserved reflex in weak wasted limb

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