Neurology nerve conduction, Lecture notes of Neurology

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Approach to motor to motor

neuron disease

Hiroyuki Nodera, MD （野寺裕之）野寺裕之） University of Tokush to motor ima

Electrodiagnosis

of ALS

Oth to motor er motor

neuron diseases

ultrasound

EDx of ALS is EASY! of ALS is EASY!

 Affects motor only  Active denervation  Reinnervation

Really??

Diagnosis of ALS

relies on clinical basi

s

 (^) Gradual onset Progressive course  (^) Upper motor neuron  (^) Lower motor neuron

Widespread involvement

(to rule out focal conditions)

Cranial segment Cervical segment Th to motor oracic segment Lumbosacral segment

● UMN and LMN signs ● widespread involvement Definite （野寺裕之） clinical findings only ） Probable ●clinical (clinical only ） ● laboratory-supported （野寺裕之） clinical + EMG EMG ） Possible

Revised El Escorial criteria

Revised El Escorial criteria

atroph to motor y-weakness tongue, h to motor and, leg h to motor yperreflex of ALS is EASY!ia jaw, arm, leg UMN + EMG LMN signs In ３ segments ⇒ Definite ALS UMN + EMG LMN signs In ２ segments ⇒ Clinically Probable ALS atroph to motor y-weakness tongue, h to motor and h to motor yperreflex of ALS is EASY!ia jaw, arm

Revised El Escorial criteria

LMN assessment by EMG active denervation

• EMG reinnervation Cervical ２ muscles Lumbosacral ２ Cranial １ Th to motor oracic １

EMG findings in ALS

Revised El Escorial Edx of ALS is EASY! criteria

Neuronal dysfunction Abnormal ex of ALS is EASY!citability Neuronal death to motor years asymptomatic Fasciculation Active denervatio n Too late to await Fib/positive sh to motor arp wave Low diagnostic sensitivity Failure to find early

Awaji EDx of ALS is EASY! criteria (2008)

Referenced 267 times

Awaji EDx of ALS is EASY! criteria

●definition of ongoing denervation ： Fibrillation Positive sh to motor arp wave Fasciculation potential (accompanied by reinnervation ）

Detection of fasciculation potential

Sonoo (2014) ●remove h to motor ands from electrode ●confirm quiet background (no muscle activity) ●observe >60 sec Multiple appearance