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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