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Table 2 Comparison of the diagnostic guidelines

From: Late-onset neuromuscular disorders in the differential diagnosis of sarcopenia

Disease Stage Criteria
Sarcopenia
(EWGSOP2)
probable sarcopenia Low muscle strength only
sarcopenia Low muscle strength AND
Low skeletal muscle mass
severe sarcopenia Low muscle strength AND
Low skeletal muscle mass AND
Low physical performance
ALS
(rEEC)
suspected ALS Affection of UMN + LMN in 1 region OR
Affection of UMN in ≥ 2 regions
probable ALS (laboratory supported) Signs of denervation in the EMG in ≥ 2 limbs AND
Affection of UMN + LMN in 1 region OR
Affection of UMN in ≥ 2 regions
possible ALS Affection of UMN + LMN in 2 regions
definite ALS Affection of UMN + LMN in 2 regions + bulbar OR
Affection of UMN + LMN in 3 regions
IBM
(ENMC)
possible IBM Weakness of finger flexion > shoulder abduction OR
Weakness of knee extension ≥ hip flexion AND
Age > 45a + disease duration > 1a + elevated CK ≤ 15 × ULN AND
At least one, not all pathological features* detectable
clinically defined-IBM Weakness of finger flexion > shoulder abduction AND
Weakness of knee extension ≥ hip flexion AND
Age > 45a + disease duration > 1a + elevated CK ≤ 15 × ULN AND
at least one, not all pathological features* detectable
clinico-pathologically defined IBM Weakness of finger flexion > shoulder abduction OR
Weakness of knee extension ≥ hip flexion AND
Age > 45a + disease duration > 1a + elevated CK ≤ 15 × ULN AND
All pathological features* 1–3 detectable
DM2
(ENMC)
ensured DM2 Weakness of iliopsoas muscles, head flexors,
myalgia, cataracts, muscle stiffness
Molecular genetic testing of DM2 tetranukleotid-repeat expansion
  1. References: [1, 7,8,9, 13]
  2. a = years; ALS = amyotrohic lateral sclerosis; DM2 = myotonic dystrophy type 2; rEEC = revised El Escorial criteria; ENMC = European Neuromuscular Centre; EMG = Electromyography; EWGSOP2 = revised European Working Group on Sarcopenia and Older People; LMN = lower motoneuron; CK = serum creatine kinase; IBM = inclusion body myositis; ULN = upper limit of normal; UMN = upper motoneuron
  3. *pathological features in muscle biopsy: 1. Endomysial inflammatory infiltrates 2. Rimmed vacuoles 3. Protein accumulation or 15-18 nm Filaments 4. Upregulation of major histocompatibility complex class 1