II. Epidemiology

  1. Onset after age 50 years
  2. Prevalence (U.S.)
    1. Overall: 0.3%
    2. Age over 60 years: 1%
    3. Age over 80 years: 4%
  3. Associated with positive Family History in >25% cases
  4. More common in men

III. Pathophysiology

  1. Slow degeneration of Substantia Nigra in Midbrain
  2. Dopaminergic Neurons degenerate
    1. Effects Extrapyramidal systems
      1. Start in Substantia Nigra pars compacta
      2. Continue via Striatum to Globus Pallidus
      3. Basal Ganglia project to cortex
    2. System regulates movement initiation and control
  3. Lewy bodies accumulate in residual DopamineNeurons
  4. Ascending process (Lower Brainstem affected first)
    1. Step 1: Medulla and later, Pons affected
      1. REM Sleep disorder
      2. Depression
      3. Dysautonomia
      4. Olfactory deficits
    2. Step 2: Midbrain and later basal forebrain affected
      1. Parkinsonian motor symptoms
    3. Step 3: Prefrontal cortex and ultimately neocortex affected diffusely
      1. Dementia

IV. Symptoms: Prodromal

  1. Non-specific symptoms may precede motor symptoms and Tremor by 20 years
  2. Non-specific symptoms
    1. Hyposmia
    2. Constipation
    3. Fatigue
  3. References
    1. Hawkes (2010) Parkinsonism Relat Disord 16(2): 79-84 [PubMed]

V. Signs

  1. Bradykinesia (PPV >4 when combined with rigidity)
    1. Smaller handwriting (micrographia) (PPV 2.8)
    2. Masklike stare
    3. Infrequent blink
    4. Slowed walking and dressing
    5. Soft Voice trails off
    6. Difficulty opening jars (PPV 6.1)
    7. Difficulty rolling over in bed (PPV 13)
  2. Impaired gait and mobility
    1. Change in stride
    2. Poor Heel to toe gait (tandem walking) (PPV 2.9)
    3. Short, shuffling steps (PPV 3.3)
  3. Postural Instability
    1. Imbalance while walking or standing
    2. Frequent falls
    3. Stooping forward to maintain center of gravity
  4. Resting Tremor (primarily, although also displays Action Tremor as well)
    1. Hands and feet considerably affected
      1. Begins as low frequency, pill-rolling finger motion
      2. Progresses to involve Forearm pronation and supination
      3. Then involves elbow flexion and extension
    2. Also affects head, face, lips, Tongue, jaw and neck
    3. Presenting Symptom in 50-75% of Parkinson's patients
    4. Regular Rhythm (3-6 beats/sec)
    5. Tremor absent in up to 20% of Parkinson's Disease
  5. Rigidity (PPV >4 with Bradykinesia)
    1. Affects breathing, eating, swallowing, and speech
    2. Cogwheel rigidity or lead-pipe rigidity
  6. Secondary Effects
    1. Akathisia
    2. Cognitive Impairment
    3. Depression
    4. Fatigue
    5. Freezing of movement (motor blocks)
    6. Impotence
    7. Increased Salivation
    8. Orthostatic Hypotension,
    9. Paroxysmal drenching sweats
    10. Seborrheic Dermatitis
    11. Urinary frequency
    12. Decreased olfaction
    13. Micrographia
  7. Abbreviations
    1. PPV: Positive Predictive Value
  8. References
    1. Rao (2003) JAMA 289:347-53 [PubMed]

VI. Diagnosis: Criteria

  1. Response to Levodopa or Dopamine Agonist challenge and
  2. Classic symptoms and signs
    1. Distal resting Tremor at 3-6 beats per second (Hz)
    2. Rigidity
    3. Bradykinesia
    4. Asymmetric onset
      1. Parkinsonism usually presents with one limb affected more than others
      2. Common pitfall in missed diagnosis is ruling-out Parkinsonism based on asymmetry

VII. Diagnosis: Findings that suggest alternative diagnosis

  1. Poor Levodopa response
  2. Falls in the early stages of disease
  3. Symmetric involvement at onset
  4. Rapid progression
  5. Absent Tremor
  6. Hallucinations
  7. Prominent and early Dementia
  8. Early postural instability
  9. Severe and early Autonomic Dysfunction
  10. Upward gaze paralysis
  11. Involuntary movements beyond Tremor
  12. Suchowersky (2006) Neurology 66(7): 968-75 [PubMed]

VIII. Differential Diagnosis: Secondary Parkinsonism

  1. Dementia with Lewy Bodies
    1. Resting Tremor often absent in Lewy Body Dementia
    2. Prominent Visual Hallucinations and signficant fluctuations in attention and cognition
    3. Poor response to Carbidopa/Levodopa
  2. Drug Induced Parkinsonism: Dopamine blocking drugs
    1. Metoclopramide (Reglan)
    2. Reserpine
    3. Antipsychotic (e.g. Haloperidol, Risperidone)
  3. Toxin-Induced Parkinsonism
    1. See Toxin-Induced Parkinsonism
    2. See Drug-Induced Tremor
  4. Vascular Parkinsonism
    1. Occurs in Cerebrovascular Disease (TIA, CVA) in step-wise progression with each vascular event
    2. Refractory to Carbidopa/Levodopa
    3. Focal neurologic deficits may be present
    4. Basal Ganglia or thalamic infarctions on CT Head or MRI Brain
  5. Structural lesions
    1. Cortical degeneration
    2. Brainstem infarction
    3. Multiple system atrophy (e.g. Shy-Drager syndrome)
  6. Hydrocephalus
    1. Normal Pressure Hydrocephalus
  7. Atypical Pakinsonism
    1. See Atypical Pakinsonism
  8. Essential Tremor
    1. Symmetric Postural Tremor of the distal extremities, head and voice
    2. Worse with movement and better with Alcohol, Beta Blockers
  9. Miscellaneous causes
    1. CNS Infection
    2. Other Tremor
      1. Rest Tremor

IX. Differential Diagnosis: Based on specific findings

  1. Stiff and slow without Tremor (seen in >20% of Parkinsonism)
    1. Progressive supranuclear palsy
  2. Isolated Tremor
    1. Essential Tremor
    2. Drug-Induced Tremor (e.g. Wellbutrin, Valproic Acid)
  3. Bradykinesia and gait change
    1. Advanced age
    2. Vascular Parkinsonism
  4. Dementia
    1. With mild Bradykinesia: Alzheimer's Disease
    2. With Hallucinations: Lewy Body Dementia
    3. With Incontinence: Normal Pressure Hydrocephalus
  5. Prominent autonomic symptoms
    1. Shy-Drager Syndrome

X. Imaging

  1. SPECT Imaging
    1. Consider in unclear cases of Parkinsonism to differentiate from other causes
    2. Visualizes integrity of CNS Dopaminergic pathways
    3. Vlaar (2007) BMC Neurol 7:27 [PubMed]
  2. MRI Head or CT Head
    1. Not routinely indicated
    2. Order if atypical presentation (see above)
    3. Evaluates for alternative diagnosis (e.g. progressive supranuclear palsy)

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Ontology: Parkinson Disease (C0030567)

Definition (MEDLINEPLUS)

Parkinson's disease (PD) is a type of movement disorder. It happens when nerve cells in the brain don't produce enough of a brain chemical called dopamine. Sometimes it is genetic, but most cases do not seem to run in families. Exposure to chemicals in the environment might play a role.

Symptoms begin gradually, often on one side of the body. Later they affect both sides. They include

  • Trembling of hands, arms, legs, jaw and face
  • Stiffness of the arms, legs and trunk
  • Slowness of movement
  • Poor balance and coordination

As symptoms get worse, people with the disease may have trouble walking, talking, or doing simple tasks. They may also have problems such as depression, sleep problems, or trouble chewing, swallowing, or speaking.

There is no lab test for PD, so it can be difficult to diagnose. Doctors use a medical history and a neurological examination to diagnose it.

PD usually begins around age 60, but it can start earlier. It is more common in men than in women. There is no cure for PD. A variety of medicines sometimes help symptoms dramatically. Surgery and deep brain stimulation (DBS) can help severe cases. With DBS, electrodes are surgically implanted in the brain. They send electrical pulses to stimulate the parts of the brain that control movement.

NIH: National Institute of Neurological Disorders and Stroke

Definition (MSHCZE) Onemocnění nervového systému bazálních ganglií mozku, jehož nápadným příznakem je převážně klidový třes (tremor). Současně je zvýšená svalová ztuhlost (rigidita), pohybová chudost (hypokineze), maskovitý obličej, změněná řeč a chůze (s nebezpečím pádu). Často bývají rovněž vegetativní příznaky (zácpa, poruchy spánku) či psychické obtíže. Kromě neurologického postižení může být přítomna podkorová demence, zpomalení psychických procesů, deprese. Úbytek kognitivních funkcí je spíše pomalý. Morfologicky lze nalézt zejm. Lewyho tělíska, bývají rovněž depigmentace substantia nigra. Příčina není známa, na vzniku se podílí např. oxidační poškození buněk. Podstatou je porucha metabolismu některých látek v mozku (nedostatek dopaminu v bazálních gangliích a relativní převaha acetylcholinu). V léčbě se používají antiparkinsonika. Ochranný vliv na zbylé buňky produkující dopamin se někdy připisuje selegilinu. Zkouší se transplantace nervových buněk lidského zárodku, které by nahradily nedostatečnou tvorbu dopaminu. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI_NCI-GLOSS) A progressive disorder of the nervous system marked by muscle tremors, muscle rigidity, decreased mobility, stooped posture, slow voluntary movements, and a mask-like facial expression.
Definition (NCI) A progressive degenerative disorder of the central nervous system characterized by loss of dopamine producing neurons in the substantia nigra and the presence of Lewy bodies in the substantia nigra and locus coeruleus. Signs and symptoms include tremor which is most pronounced during rest, muscle rigidity, slowing of the voluntary movements, a tendency to fall back, and a mask-like facial expression.
Definition (MSH) A progressive, degenerative neurologic disease characterized by a TREMOR that is maximal at rest, retropulsion (i.e. a tendency to fall backwards), rigidity, stooped posture, slowness of voluntary movements, and a masklike facial expression. Pathologic features include loss of melanin containing neurons in the substantia nigra and other pigmented nuclei of the brainstem. LEWY BODIES are present in the substantia nigra and locus coeruleus but may also be found in a related condition (LEWY BODY DISEASE, DIFFUSE) characterized by dementia in combination with varying degrees of parkinsonism. (Adams et al., Principles of Neurology, 6th ed, p1059, pp1067-75)
Definition (PSY) A disease characterized as a progressive motor disability manifested by tremors, shaking, muscular rigidity, and lack of postural reflexes.
Definition (CSP) progressive, degenerative disorder of the nervous system characterized by tremors, rigidity, bradykinesia, postural instability, and gait abnormalities; caused by a loss of neurons and a decrease of dopamine in the basal ganglia.
Concepts Disease or Syndrome (T047)
MSH D010300
ICD9 332.0, 332
ICD10 G20
SnomedCT 49049000, 192825001, 154999006, 155002003, 192831003, 155000006
LNC MTHU020807
English Paralysis Agitans, Parkinson's Disease, PARKINSON'S SYNDROME, PARALYSIS AGITANS, Parkinson's disease NOS, PARALYSIS AGITANS <PARKINSONISM>, PARKINSONS DIS, PARKINSON DIS, PARKINSONS DIS IDIOPATHIC, IDIOPATHIC PARKINSON DIS, IDIOPATHIC PARKINSONS DIS, LEWY BODY PARKINSON DIS, PARKINSON DIS IDIOPATHIC, PARKINSONS DIS LEWY BODY, Parkinson's disease, NOS, Parkinson's disease (diagnosis), Parkinson's dis, Disease Parkinson's, Palsy shaking, PARALYSIS AGITANS PARKINSONISM, Lewy Body Parkinson Disease, Lewy Body Parkinson's Disease, Parkinson's Disease, Lewy Body, Idiopathic Parkinson Disease, Idiopathic Parkinson's Disease, Parkinson Disease, Idiopathic, Parkinson's Disease, Idiopathic, Parkinsonism, Primary, Primary Parkinsonism, Parkinson Disease [Disease/Finding], disease disorders parkinson's, diseases parkinson, Paralysis;agitans, diseases parkinsons, idiopathic parkinson disease, parkinson s disease, syndrome parkinson's, Disease;Parkinsons, disease parkinson s, Parkinson disease (PD), disease idiopathic parkinsons, palsy shaking, parkinson disease lewy body, parkinson's syndrome, bodies disease lewy parkinson's, disease parkinsons's, diseases parkinson's, idiopathic parkinson's disease, Parkinsons disease, Shaking Palsy, Parkinson's disease NOS (disorder), Shaking palsy, Paralysis agitans, Parkinson's disease, Idiopathic parkinsonism, Primary parkinsonism, Idiopathic Parkinson's disease, PD - Parkinson's disease, Parkinson disease, Parkinson's disease (disorder), shaking palsy, palsy; shaking, paralysis; agitans, parkinson's, agitans; paralysis, shaking; palsy, Parkinson Disease, Parkinsons Disease, Parkinson's syndrome, Syndrome Parkinson's, Parkinson`s disease, paralysis agitans
Portuguese DOENCA DE PARKINSON, PARALISIA AGITANTE, Síndrome de Parkinson, Doença de Parkinson NE, Paralisia agitante, Mal de Parkinson, Doença de Parkinson com Corpos de Lewy, Doença de Parkinson Idiopática, Doença de Parkinson, Paralisia Agitante, Parkinsonismo Primário
Spanish PARALISIS AGITANTE, Parálisis temblorosa, Síndrome de Parkinsosn, Síndrome de Parkinson, Parálisis agitante, Enfermedad de Parkinson NEOM, SINDROME DE PARKINSON, enfermedad de Parkinson, SAI, Parkinson's disease NOS, enfermedad de Parkinson, SAI (trastorno), enfermedad de Parkinson (trastorno), enfermedad de Parkinson, parkinsonismo idiopático, parkinsonismo primario, parálisis agitante, parálisis temblorosa, Enfermedad de Parkinson, Enfermedad de Parkinson con Cuerpos de Lewy, Enfermedad de Parkinson Idiopática, Parálisis Agitante, Parkinsonismo Primario
Dutch Parkinson-syndroom, Parkinsons ziekte, ziekte van Parkinson NAO, schuddende verlamming, paralysis agitans, agitans; paralyse, palsy; shaking, paralyse; agitans, shaking; palsy, ziekte van Parkinson, Idiopathische ziekte van Parkinson, Lewy-body-ziekte, Paralysis agitans, Parkinsonisme, primair, Ziekte van Parkinson
French Mal de Parkinson, Maladie de Parkinson SAI, Syndrome de Parkinson, Paralysis agitans, PARALYSIE AGITANTE, SYNDROME DE PARKINSON, Maladie de Parkinson, Maladie de Parkinson idiopathique, Maladie parkinsonienne à corps de Lewy, Paralysie agitante, Parkinsonisme primitif
German Krankheut Parkinson, Syndrom Parkinson, Schuettellaehmung, Parkinson Syndrom NNB, Parkinson-Syndrom, PARALYSIS AGITANS, PARKINSON SYNDROM, Primaeres Parkinson-Syndrom, Morbus Parkinson, Paralysis agitans, Parkinson-Krankheit, Idiopathische Parkinson-Krankheit, Lewy-Body-Parkinson-Krankheit, Primärer Parkinsonismus, Primäres Parkinson-Syndrom
Italian Sindrome di Parkinson, Parkinsonismo primario, Paralisi agitante, Malattia del Parkinson idiopatico, Malattia di Parkinson da corpi di Lewy, Morbo di Parkinson NAS, Morbo di Parkinson, Malattia di Parkinson
Japanese パーキンソン症候群, パーキンソン病NOS, パーキンソンショウコウグン, パーキンソンビョウNOS, パーキンソンビョウ, シンセンマヒ, Parkinson病, パーキンソン病, 振戦麻痺, パーキンソン氏病, 振顫麻痺
Swedish Parkinsons sjukdom
Czech Parkinsonova nemoc, Parkinsonův syndrom, Třaslavá obrna, Parkinsonova nemoc NOS, Syndrom Parkinsonův
Finnish Parkinsonin tauti
Russian PARKINSONA BOLEZN', PARALICH DROZHATEL'NYI, ПАРАЛИЧ ДРОЖАТЕЛЬНЫЙ, ПАРКИНСОНА БОЛЕЗНЬ
Korean 파킨슨병
Croatian PARKINSONOVA BOLEST
Polish Choroba Parkinsona, Drżączka poraźna
Hungarian Parkinson-os remegés, Parkinson kór, Parkinson-kór k.m.n., Parkinson syndroma, Parkinson-syndroma, Paralysis agitans, Parkinson-kór, Parkinson-betegség
Norwegian Parkinsons sykdom

Ontology: Parkinsonian Disorders (C0242422)

Definition (MSH) A group of disorders which feature impaired motor control characterized by bradykinesia, MUSCLE RIGIDITY; TREMOR; and postural instability. Parkinsonian diseases are generally divided into primary parkinsonism (see PARKINSON DISEASE), secondary parkinsonism (see PARKINSON DISEASE, SECONDARY) and inherited forms. These conditions are associated with dysfunction of dopaminergic or closely related motor integration neuronal pathways in the BASAL GANGLIA.
Definition (PSY) Clinical state, usually drug induced, characterized by tremors, muscle rigidity, postural reflex dysfunction, and akinesia. Compare PARKINSONS DISEASE.
Concepts Disease or Syndrome (T047)
MSH D020734
SnomedCT 394962002, 32798002
English Parkinsonian Syndrome, Parkinsonism, PARKINSONISM, PARKINSONIAN DIS, parkinsonism, parkinsonism (diagnosis), Parkinsonian Diseases, Parkinsonian Disorders, Parkinsonian Syndromes, Parkinsonian Disorders [Disease/Finding], parkinsonian disease, parkinsonian disorders, parkinsonian syndrome, parkinsonian syndromes, parkinsonisms, Parkinsonism (finding), Hypokinetic-rigid syndrome, Parkinsonism (disorder), Parkinsonism, NOS
Swedish Parkinsonism
Japanese パーキンソニズム, 実験的パーキンソニズム, パーキンソン症候群-家族性若年性, 実験的パーキンソン症候群, 若年性パーキンソニズム, パーキンソン症候群, パーキンソニズム-家族性若年性, Parkinson症候群, パーキンソニズム, 若年性パーキンソン症候群, パーキンソニズム-実験的, パーキンソン障害, パーキンソン症候群-若年性, 家族性若年性パーキンソン症候群, 家族性若年性パーキンソニズム, パーキンソニスム, パーキンソン症, パーキンソン症候群-実験的, ウンドウゲンショウコシュクショウコウグン, 運動減少−固縮症候群
Czech parkinsonský syndrom, parkinsonské poruchy, Parkinsonismus, Hypokineticko-rigidní syndrom
Finnish Parkinsonismit
Russian PARKINSONIZM EKSPERIMENTAL'NYI, PARKINSONIZM IUVENIL'NYI, PARKINSONOPODOBNYE RASSTROISTVA, RAMZAIA KHANTA PARALICHA SINDROM, PARKINSONIZM SEMEINYI IUVENIL'NYI, PARKINSONIZM, PARKINSONIZMA SINDROM, ПАРКИНСОНИЗМ, ПАРКИНСОНИЗМА СИНДРОМ, ПАРКИНСОНИЗМ СЕМЕЙНЫЙ ЮВЕНИЛЬНЫЙ, ПАРКИНСОНИЗМ ЭКСПЕРИМЕНТАЛЬНЫЙ, ПАРКИНСОНИЗМ ЮВЕНИЛЬНЫЙ, ПАРКИНСОНОПОДОБНЫЕ РАССТРОЙСТВА, РАМЗАЯ ХАНТА ПАРАЛИЧА СИНДРОМ
French PARKINSONISME, Syndrome hypokinétique-hypertonique, Parkinsonisme, Troubles parkinsoniens, Syndromes parkinsoniens
Portuguese PARKINSONISMO, Síndrome rígida hipocinética, Síndrome Paralítica de Ramsay Hunt, Parkinsonismo, Síndrome Parkinsoniana, Transtornos Parkinsonianos
Spanish PARKINSONISMO, Síndrome rígido hipercinético, parkinsonismo (trastorno), parkinsonismo, Parkinsonismo, Síndrome Parkinsoniano, Trastornos Parkinsonianos
German PARKINSONISMUS, Hypokinetisch-rigides Syndrom, Parkinson-Syndrom, Parkinsonismus, Parkinsonstörungen
Croatian Not Translated[Parkinsonian Disorders]
Polish Zaburzenia parkinsonowskie, Parkinsonizm młodzieńczy rodzinny
Hungarian Parkinsonismus, Hypokinetikus-rigid syndroma
Italian Sindrome parkinsoniana, Parkinsonismo, Sindromi parkinsoniane, Sindrome ipocinetica rigida, Malattie parkinsoniane
Norwegian Parkinsonisme
Dutch hypokinestisch-rigide syndroom, parkinsonisme, Parkinsonachtig syndroom, Parkinsonachtige stoornis, Parkinsonachtige stoornissen, Parkinsonisme, Stoornis, parkinsonachtige, Stoornissen, parkinsonachtige