The clinical diagnosis of Parkinson's disease

Munhoz, Renato P.; Tumas, Vitor; Pedroso, José Luiz; Silveira-Moriyama, Laura

doi:10.1055/s-0043-1777775

Renato P. Munhoz Address for correspondence Renato P. Munhoz (email: renato.munhoz@uhn.ca)

conceptualization

formal analysis

project

writing – original draft

writing – review & editing

University Health Network, Toronto Western Hospital, Morton and Gloria Shulman Movement Disorders Centre and the Edmond J. Safra Program in Parkinson's Disease, Toronto, ON, Canada.

Krembil Research Institute, Toronto, ON, M5T 2S8, Canada.

https://orcid.org/0000-0002-4783-4067

Vitor Tumas

conceptualization

formal analysis

project

writing – original draft

writing – review & editing

Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto, Departamento de Neurociências e Ciências do Comportamento, Ribeirão Preto SP, Brazil.

https://orcid.org/0000-0003-2402-2709

José Luiz Pedroso

conceptualization

formal analysis

project

writing – original draft

writing – review & editing

Universidade Federal de São Paulo, Departamento de Neurologia, São Paulo SP, Brazil.

https://orcid.org/0000-0002-1672-8894

Laura Silveira-Moriyama

conceptualization

formal analysis

project

writing – original draft

writing – review & editing

Universidade Estadual de Campinas, Campinas SP, Brazil.

UCL Queen Square Institute of Neurology, London, United Kingdom.

https://orcid.org/0000-0003-2184-626X

Address for correspondence Renato P. Munhoz (email: renato.munhoz@uhn.ca)

Conflict of Interest

There is no conflict of interest to declare.

Figures (2)
Tables (2)

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Figure 1
DAT SPECT with ^99mTc-TRODAT-1. (A) shows a normal DAT SPECT from a healthy subject, while figure (B) discloses a marked decrease in dopamine in a patient with Parkinson's disease. This image is from the personal archive of the authors.

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Figure 2
Brain MRI with nigrosome and neuromelanin findings, respectively in healthy subjects, early stage of PD and advanced stage of PD. In healthy subjects there is a clear swallow tail appearance in nigrosome imaging and hyperintense signal in the substantia nigra in the neuromelanine sensitive MRI. On the other hand, in advanced stages of PD, there is absence of the swallow tail appearance in nigrosome imaging and decrease of the hyperintensity in neuromelanine imaging. Early stage of PD presents with intermediate findings between both conditions described above. This image was kindly supplied by Dr. Victor Hugo Rocha Marussi, from Beneficência Portuguesa, São Paulo, Brazil.

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Table 1
Comparison of the QSBB and the new MDS criteria.

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Table 2
Clinical features of the most common differential diagnoses of the syndrome.²⁷27 Munhoz RP, Werneck LC, Teive HA. The differential diagnoses of parkinsonism: findings from a cohort of 1528 patients and a 10 years comparison in tertiary movement disorders clinics. Clin Neurol Neurosurg 2010;112(05):431–435

28 Munhoz RP, Bertucci Filho D, Teive HA. Not all drug-induced parkinsonism are the same: the effect of drug class on motor phenotype. Neurol Sci 2017;38(02):319–324

29 Höglinger GU, Respondek G, Stamelou M, et al; Movement Disorder Society-endorsed PSP Study Group. Clinical diagnosis ofprogressive supranuclear palsy: The movement disorder society criteria. Mov Disord 2017;32(06):853–864. Doi: 10.1002/mds.26987
https://doi.org/10.1002/mds.26987...

30 Wenning GK, Stankovic I, Vignatelli L, et al. The Movement Disorder Society Criteria for the Diagnosis of Multiple System Atrophy. Mov Disord 2022;37(06):1131–1148

31 Yamada M, Komatsu J, Nakamura K, et al. Diagnostic Criteria for Dementia with Lewy Bodies: Updates and Future Directions. J Mov Disord 2020;13(01):1–10-³²32 Armstrong MJ, Litvan I, Lang AE, et al. Criteria for the diagnosis of corticobasal degeneration. Neurology 2013;80(05):496–503. Doi: 10.1212/WNL.0b013e31827f0fd1
https://doi.org/10.1212/WNL.0b013e31827f...

Criteria	Queen Square Brain Bank Criteria (Gibb & Lees, 1988)¹⁰10 Gibb WR, Lees AJ. The relevance of the Lewy body to the pathogenesis of idiopathic Parkinson's disease. J Neurol Neurosurg Psychiatry 1988;51(06):745–752	MDS criteria for Parkinson's disease (Postuma et al., 2015)²⁵25 Postuma RB, Berg D, Stern M, et al. MDS clinical diagnostic criteria for Parkinson's disease. Mov Disord 2015;30(12):1591–1601
Chore findings	STEP 1: identification of Parkinsonian syndrome. Defined as bradykinesia and at least one of the following: Muscular rigidity. 4-6 Hz rest tremor. Postural instability not caused by primary visual, vestibular, cerebellar, or proprioceptive dysfunction.	The first essential criterion is parkinsonism, which is defined as bradykinesia, in combination with at least 1 of rest tremor or rigidity.
Negative features used as	Step 2: Exclusion Criteria	Absolute exclusion criteria or Red flags (when combined with supportive criteria do not exclude PD)
Positive features used as	Step 3: Supportive Criteria	Supportive Criteria
Ancillary tests	Imaging used to exclude differential diagnosis (Step 2)	Olfactory loss or cardiac sympathetic denervation on MIBG scintigraphy are supportive criteria Normal functional neuroimaging of the presynaptic dopaminergic system is an exclusion criteria
Certainty levels	Definite PD (three or more positive supportive findings)	Clinically Established PD: Absence of absolute exclusion criteria At least two supportive criteria, and No red flags Clinically Probable PD: Absence of absolute exclusion criteria Presence of red flags counterbalanced by supportive criteria red flag requires at least 1 supportive criterion red flags require at least 2 supportive criteria no more than 2 red flags are allowed for this category

	PD	DIP	VP	PSP	MSA-P	LBD	CBD
Mean age of onset (SD)	59.4 (11.5)	60.6 (13.4)	70.6 (6.4)	66.9 (7.6)	55.5 (6.5)	67.8 (9.2)	63 (7.7)
Tremor	Pure rest (30%), pure action (20%), mixed (20%)	Pure rest (35%), pure action (10%), mixed (30%)	Pure rest (4%), pure action (10%), mixed (2%)	Pure rest (10%)^a a) Notes: PSP-P variant presents with asymmetric features, rest tremor, levodopa response and LID; , pure action (20%), mixed (20%)	Rest (5%), Action (80%)^f f) jerky postural tremor / polyminimyoclonus; , mixed (10%)^f f) jerky postural tremor / polyminimyoclonus;	Pure rest (3%), pure action (7%), mixed (24%)	Rest (2%), Action (10%)^g g) jerky action tremor / myoclonus. , mixed (55%)^g g) jerky action tremor / myoclonus.
Postural instability	Common but late feature	Rare	Prominent / early or presenting sign	Prominent / early or presenting sign	Prominent / early	Prominent / early	Prominent / early
Asymmetry	+++	0	+	0^a a) Notes: PSP-P variant presents with asymmetric features, rest tremor, levodopa response and LID;	+	0	+++
Survival – Mean (SD)	Variable^b b) widely dependent on age of onset, ranging from 38 (5) years for early onset (25-39 years old) to 5 (4) for late onset (≥ 65 years old);	N/A	8 (4.1)	8.6 (5.7)	7.5 (4)	4.1 (4.1)	8 (0.7)
Levodopa response	Marked / sustained	None to moderate^c c) may be sustained in responders;	None to moderate^c c) may be sustained in responders;	Mild to moderate^d d) typically in early stages, not sustained;	Mild to moderate^d d) typically in early stages, not sustained;	Mild to moderate^d d) typically in early stages, not sustained;	Mild^d d) typically in early stages, not sustained;
LID ^e e) in levodopa responders under long term treatment;	++ + +	0	+	+^a a) Notes: PSP-P variant presents with asymmetric features, rest tremor, levodopa response and LID;	++	+	+
Dementia	Common in advanced stages	0	Very common, presenting as VD	Very common, early, fast decline	Less common than PD	Part of diagnostic criteria; may fluctuate	Common, may be early, fast decline
RBD	Very common	0	0	Unusual	Very common	Very common	0
Additional clinical features	Slower progression compared to other degenerative forms.	Onset during treatment with offending drug; improvement / resolution after withdrawal.	Pyramidal and pseudobulbar signs; lower body predominant.	Supranuclear gaze palsy; disproportional axial (nuchal) rigidity; photophobia / blepharospasm;	Profound early dysautonomia; anterocollis; pseudobulbar affect; pyramidal signs.	Early well-formed visual hallucinations; neuroleptic sensitivity; dysautonomia.	Limb dystonia; apraxia; cortical sensory loss; alien limb phenomena.
Brain MRI findings	No specific findings on standard imaging.	No change	Periventricular white matter lesions, lacunar infarcts in BG, ventricular dilation.	Predominant midbrain atrophy; superior cerebellar peduncle atrophy.	Putaminal atrophy; OPCA and "hot cross bum sign" in advanced stages.	Global atrophy.	Asymmetric fronto-parietal atrophy.

[1] Address for correspondence Renato P. Munhoz (email: renato.munhoz@uhn.ca)

Brasil

Brasil

The clinical diagnosis of Parkinson's disease

O diagnóstico clínico da doença de Parkinson

Abstract