Table 1 Percentage of pathologically confirmed PD, DLB, MSA, PSP and CBD with each item of MDS Diagnostic Criteria

Clear and dramatic beneficial response to dopaminergic therapy. During initial treatment patient returned to normal or near normal.

84.1 (69–100)^{10,11,14,27,28,29,30,31,32}

n = 917

ND

35.9 (16–54)^{10,31,32,33,34,35}

n = 293

15 (7–23)^10,14

n = 63

NA

Marked improvement with dose increases or marked worsening with dose decreases (Objective eg > 30% UPDRS III with change of treatment) or subjective from history). Or unequivocal and marked on/off with predictable end-of-dose wearing off. Fluctuations

57.1 (33–84)^{10,27,31,35,36,37,38}

n = 788

ND

29 (24–39)^10,31,35

n = 266

36¹⁰

n = 18

NA

Presence of levodopa-induced dyskinesia(s)

61.7 (55– 80.8)^{10,27,31,38,39}

n = 560

NA

36.4 (27–39.5)^10,31,34,35

n = 289

28¹⁰

n = 18

NA

Rest tremor of a limb (in past or current examination)

68 (43–91)^{11,14,27,30,31,32,37,38,40,41}

n = 675

39⁴¹

n = 39

28 (26–30)^32,41

n = 256

22 (7–37)^14,41

n = 45

NA

Presence of olfactory loss

94.6¹¹

n = 39

NA

NA

NA

NA

Presence of Cardiosympathetic denervation (MIBG SPECT)

ND

ND

ND

ND

ND

 Gait Ataxia

NA

11⁴¹

36 (23–49)^35,41

43⁴¹

ND

 Limb Ataxia

NA

6⁴¹

47.5 (47–48)^35,41

43⁴¹

ND

 Non-Specific Ataxia

1 (0–1)^31,32

n = 134

ND

n = 18

47.9 (32–64)^31,32,42

n = 231

ND

n = 14

ND

Downward Supranuclear gaze palsy or selective slowing of downward vertical saccades (*no differentiation of direction)

6.8*¹⁵

n = 146

ND

20.6⁴³

Downgaze only 21.5⁴³

n = 160

66.9 (61–75)^15,16,43

Downgaze only 37.5⁴³

n = 51

ND

Behavioural Variant frontotemporal dementia /progressive aphasia

ND

NA

19 (13–25)^42,43

n = 176

8 (8)^16,43

n = 37

ND

Parkinsonian features restricted to lower limbs for more than 3 years

ND

ND

ND

ND

ND

Absence of observable response to high dose levodopa despite at least moderate severity of disease

1.7 (0–2.7)^14,28,44

n = 247

ND

29 (27–31.2)^33,44

n = 27

75⁴⁴

n = 16

ND

Unequivocal Cortical sensory loss; limb apraxia; progressive aphasia

ND

ND

ND

ND

33¹⁶

Normal functional of the presynaptic dopaminergic system

0¹²

n = 47

NA¹²

2.4¹²

n = 42

6.8¹²

n = 73

40¹²

n = 10

Rapid progression of Gait impairment requiring wheelchair within 5 years of disease onset

8.7 (3–14.4)^28,38

n = 266

7⁴⁵

n = 14

30.1 (13–53)^43,45

n = 165

33 (20–46)^43,45

n = 37

8⁴⁵

n = 13

Complete lack of progression within 5 years, unless related to treatment

0.8%¹⁴

n = 116

NA

ND

ND

ND

Early Bulbar dysfunction, severe dysphonia or dysarthria (speech unintelligible most of the time or severe dysphagia (requiring soft food, NG or PEG within first 5years)

2.5¹⁴

n = 116

ND

46⁴³

n = 160

23⁴³

n = 13

ND

Inspiratory stridor

0.8¹⁴

n = 116

NA

21 (10–31.8)^35,43

n = 363

0³⁵

n = 13

ND

Severe autonomic failure within first 5 years with Orthostatic hypotension (see definition)

4.7 (0–9.4)^14,32

n = 151

NA

47 (18 – 68.7)^{14,32,33,35,44}

n = 438

0⁴⁴

n = 16

ND

Severe urinary retention /incontinence (associated with erectile dysfunction in men)

0⁴⁶

n = 11

15⁴⁶

n = 14

46.3 (31–87)^{31,33,43,46,47}

n = 246

45⁴⁶

n = 13

ND

Recurrent falls (>1 per year) within first 3 years

3.8 (0–7.7)^14,41

n = 124

28⁴¹

n = 19

28.8 (13–42.5)^41,43,47

n = 274

33.6 (29–38.5)^41,43

n = 28

22¹⁶

n = 27

Disproportionate (dystonic) anterocollis or contractures of hands/feet within first 10 years

NA

NA

21 (10–32.5)^32,35

n = 380

15.4³²

n = 17

ND

Absences of common non-motor symptoms: despite 5 years of disease duration: sleep disorder: autonomic dysfunction, hyposmia, psychiatric symptoms

ND

ND

ND

ND

ND

Otherwise, unexplained Pyramidal tract signs

7 (6.9–7)^14,31

n = 108

11⁴¹

n = 18

40.9 (31.3–52)^{31,32,35,37,41,42}

n = 536

18.6 (14–23.1))^32,41

n = 31

ND

Bilateral symmetric Parkinsonism

17.6 (0.02–28)^14,31,44

NA

33.9 (24–43.7)^31,44

84 (81–87)^16,44

48¹⁶