Parkinsonism – Canadian Guideline 2012

Parkinson’s Dz: Extra Pyramidal Syndrome
Key Features: TRAP
  • Tremor: asymmetric 3-5hz “pill-rolling” resting or postural tremor, especially in hands
    • Tremor ↓ with movement
  • Rigidity: lead-piperegidity with cogwheeling due to superimposed tremor
    • Thoughtout the whole ROM, the rigidity affects both flexor and extensors
    • VS Spasticity – Pyramidal / corticalspinaltractdz
      1. velocity dependent
      2. Not thoughtout the whole movement
      3. affects flexor in upper extremities and extensor in lower extremities (posture of a stroke pt)
  • Akinesia / bradykinesia: slow small amplitude movements, fatigueing of rapid alt movements, 
    • affects face, eyes (less blinking – normal 15 blincs /min), slow motion
    • drooling (decreased swallowing causing pooling of saliva) – Tx by chewing gums (no anticholinergics)
    • freezing – difficulty initiating movement, lasts seconds
  • Postural instability: r/o HoTN
    • Test with Rhomberg – ready to catch pt
    • pt stand shoulder width & pushed in all al direction – normally steps 1-2 steps
  • Related findings:
    • masked facies
    • hypophonia, aprosody (monotonous speech),
    • dysarthria, micrographia
    • shuffling gait with decreased arm swing
    • Bradyphrenia – slow to think / respond & dementia (late finding)

Clinical Course:
  1. Early / Mild “honeymoon period” can last for years
  2. Mod/Non-fluctuating stage
    • ↑ disabling symptoms, ↑impairment of ADL, and ↓ response to 1st line Tx
  3. Advanced/fluctuating stage
    • Freeze – gait last few sec
    • Fluctuations in response to Tx
    • Dyskinesia
Definitions:
  • ON State – overall good function
  • OFF stage – Poor overall function
  • Motor Fluctuations – Alt ON & OFF
  • Dyskinesia – Involuntary, non-tremor movement
  • Troublesome Dyskinesia: painful, impairs balance, xs causing impairment

Dx:
  1. Clinical Hx + PEx
  2. R/o other etiologies
Dx Criteria:

1) Bradykinesia +

  1. resting tremor or
  2. muscle rigidity or
  3. postural instability – late finding presents as falls, festinating gait
    → NOT caused by visual, cerebellar, vestibular, or proprioceptive dysfunction

or

2) ≥ 3 of the following features:

  • Resting tremor
  • Unilateral onset
  • Persistent asymmetry, with side of onset most affected
  • Excellent response (70-100%) to levodopa
  • Severe levodopa-induced chorea
  • Response to levodopa for >5 yr
  • clinical course >10 yr
  • Progressive disorder
Exclusion
  • Repeated stroke with stepwise progression
  • Related head injury
  • Antipsychotic or dopamine-depleting drugs
  • Definite encephalitis
  • Sustained remission
  • negative response to large doses of levodopa
  • Strictly unilateral features after 3 years or symmetry at onset
  • Other neurological features: supranuclear gaze palsy, cerebellar signs, early severe autonomic
  • Involvement, Babinski sign, early severe dementia with disturbances of language, memory, or praxis
  • Exposure to known neurotoxin
  • Presence of cerebral tumor or communicating hydrocephalus
MRI if struggling to distinguish parkinsonian syndromes

r/o other etiologies – Parkinsonism ≠ Parkinson’s dz
  • Drug-induced – maxeran
  • Strokes – lacunar stroke in basal ganglia, multiple cerebral infarcts
  • Tumor
  • Parkinson-plus syndromes
    • Multisystem atrophy (MSA) syndrome
    • Dementia syndrome
      • Alzheimer’s, Pick’s dz, cortical Lewy body dz
    • Progressive supranuclear palsy (PSP)
  • Heredodegenerative dz
Clues NOT Parkinson’s dz
  • Motor –
    • early instability and falls,
    • rapid dz progression,
    • no response to Levodopa,
    • Pyramidal signs, cerebellar signs,
    • early dysphagia / dysarthria
  • Oculomotor – SNP (Supranuclear palsy): lack saccade vertical eye movement
    • frequent falls because pt not looking down – not characteristic of Parkinson’s dz early on
  • Cognitive / behaviour: early dementia, VH, ideomotor apraxia, sensory / visual neglect

Pathophysiology
  1. Loss of dopaminergic neurons in pars compacta of substantia nigra → reduced dopamine in striatum → disinhibition of the indirect pathway and decreased activation of the direct pathway causing increased inhibition of cortical motor areas
  2. α-synucleinopathy: α-synuclein accumulates in Lewy bodies & cause neuritoxicity in substantia nigra
Risk factors
  • Age – strongest factor
  • Family Hx – second strongest factor
  • Male
  • head injury
  • rural living, exposure to certain enruo toxins
  • Some heroin addicts who accidentally used a meperidine analog MPTP – a potent neurotoxin that destroys dopaminergic neurons in a pattern similar to Parkinson’s dz
Protective:
  • coffee drinking, smoking
  • NSAIDs use, estrogen replacement in post-menopausal women

1  In patients with suspected Parkinson’s disease, accurately distinguish idiopathic Parkinson’s disease from atypical Parkinson’s disease (e.g., disease at a young age, drug-related disease), as treatment differs.

PD is suspected in pt with
  1. Rest tremor
  2. Slowness / stiffness
  3. balance problems
  4. Gait disorders

PD needs to be differentiated from other forms of parkinsonism and secondary causes such as drugs, neurotoxins, structural brain lesions as well as other causes of tremor.

Pt with suspected Pd should be referred quickly (seen within 6 weeks)

Atypical Parkinsonism – distinguish PD from other parkinsonian syndromes
  • Falls at presentation and early in the disease course
  • Poor response to levodopa
  • Symmetry at onset
  • Rapid progression
  • Lack of tremor
  • Prominent dysautonomia (urinary urgency / incontinence and fecal incontinence, urinary retention requiring catheterization, persistent ED or orthostatic HoTN)

Predictors of more benign course
  • Younger onset
  • Rest tremor as presenting symptom
Predictors of more rapid course
  • Older onset
  • rigidity / hypokinesia as an initial symptom
  • Postural instability / Freezing gait
  • Dementia
  • Associated comorbidities: stroke, auditory deficit, visual impairment
  • Male
  • Poor levodopa response
  • Postural instability / gate disturbance

2  In the care of all patients with Parkinson’s disease, involve other health care professionals to enhance the patient’s functional status. Speech, physiotherapy, and occupational therapy play an important role in managing motor problems.

PT for PD pt:
  • gait re-education, improvement of balance and flexibility 
  • enhancement of aerobic capacity 
  • improvement of movement initiation 
  • improvement of functional independence, including mobility and activities of daily living
  • provision of advice regarding safety in the home environment
OT for PD pt:
  • maintenance of work and family roles, home care and leisure activities
  • improvement and maintenance of transfers and mobility
  • improvement of personal self-care activities, such as eating, drinking, washing and dressing
  • environmental issues to improve safety and motor function
  • cognitive assessment and appropriate intervention.
Speech and language therapy
  • improvement of vocal loudness and pitch range, including speech therapy programs such as Lee Silverman Voice Treatment (LSVT)
  • teaching strategies to optimize speech intelligibility 
  • ensuring an effective means of communication is maintained throughout the course of the disease, including use of assistive technologies.
  • review and management to support safety and efficiency of swallowing and to minimize the risk of aspiration.

3  In an elderly patient with a deterioration in functional status, look for and recognize Parkinson’s disease when it is present, as it is a potentially reversible contribution to the deterioration.

S/Sx:

EARLY – asymmetrical / unilateral
  • Slowness + poverty of movement (bradykinesia + hypokinesia)
  • Loss of facial expression
  • Loss of arm swing
  • Difficult with fine movements, micrographia
  • Stiffness / rigidity
  • Shaking / resting tremor
  • Trouble turning in bed
  • Trouble opening jars
  • Shuffling gait
  • Trouble rising from a chair
  • Glabellar Tap (decrease blink in PD)
  • Difficult walking heel – toe
Later – progress to bilateral
  • Postural instability (use pull / push test) / Falls
  • Cognitive impairment / Dementia / Psychotic symptoms / Depression
  • Sleep disturbance – daytime hypersomnolence + nocturnal akinesia
  • Autonomic Dysfunction : GI, orthostatic HoTN, xs sweating
  • Pain

4  In a patient with a tremor, do an appropriate physical examination (e.g., observation, use of techniques to enhance the tremor) to distinguish the resting tremor of parkinsonism from other (e.g., essential) tremors.

Resting tremor:
  • asymmetric 4-5hz “pill-rolling” tremor, especially in hands
  • can have postural tremor (rested on a object)
  • Tremor ↓ with movement

The combination of asymmetrical s/sx, a resting tremor, & a good response to levodopa differentiates Parkinson’s dz from parkinsonism due to other causes.

Tremor ddx:
  • Essential Tremor
  • Hyperthyroidism
  • Drug induced (beta agonist)
  • Cerebellar disorder

5  As part of the management of patients with Parkinson’s disease, identify anticipated side effects of medications, especially those with which you are unfamiliar.

Dopamine precursor: Levodopa – most effective Tx for PD
  • The clinical response is prolonged in the early stages, within few years, the duration of benefit from each dose may become progressively shorter
  • S/E:
    • Peripheral: N/V, orthostatic HoTN
    • Central: motor fluctuations, psychiatric disturbances (confusion, hallucinations, psychosis)
    • Dyskinesia: chorea-peak dose, dystonia-peak dose, diphasic-low phase
  • Long-term C/I:
    • Wearing off with unpredictable “ON-OFF”
    • delayed ON
    • Freezing
    • Akinesia in early AM
Dopamine Agonist: Bromocreptine / Domperidone (BF)
  • S/E:
    • ↑ sleepiness, ↑ sleep attacks (extreme form of somnolence) – issue with driving
    •  dopamine dysregulation syndrome (Impulse control disorder): eg. pathological gambling, ↑ sexual activty, stereotypic motor acts
    • Pyschosis
COMT inhibitor (block Levodopa conversion to 30MD): Entacapone
  • increase plasma and central levels of Levodopa
  • S/E: ↑ N/V/orthostatic HoTN, hallucination & Dyskinesia

6  As part of the ongoing follow-up care of patients with Parkinson’s disease:
– assess functional status.

People with PD should have regular access to the following:
  • clinical monitoring and medication adjustment
  • a continuing point of contact for support, including home visits, when appropriate
  • a reliable source of information about clinical and social matters of concern to people with PD and their caregivers which may be provided by a Parkinson’s disease nurse specialist.

More recently, non-motor symptoms have become recognized as a major source of disability in PD and treatment focus has shifted to quality of life and maintaining it in advanced disease.

– monitor them for medication side effects.

  • If using ergot-dopamine agonists – check renal function, ESR, CXR annually
  • Anti-parkinsonian medication should not be withdrawn abruptly or allowed to fail suddenly due to poor absorption (for example, gastroenteritis, abdominal surgery) to avoid the potential for acute akinesia or neuroleptic malignant syndrome.

– look for other problems (e.g., depression, dementia, falls, constipation), as they are more common.

Non-Motor Symptoms of PD
Mental Health
  • Depression – Clinical features of depression overlap with the motor features of PD can be managed in the same way as depression in those without Parkinson’s
    • can be managed in the same way as depression in those without Parkinson’s dz.
  • Psychotic symptoms – typically progression from illusions of presence, through pseudo hallucinations to true hallucinations. Paranoia is common.
    • Not all hallucinations require Tx
  • Dementia – increases with disease duration
    • Simplification of medications will minimize potential CNS effects that accentuate the cognitive dysfunction
Sleep Disorders –
  • insomnia, xs daytime somnolence, REM sleep behaviour disorder and restless legs syndrome
  • Advised about provincial legislation regarding driving in pt who are experiencing sleep attacks
Autonomic Disturbances
  • Urinary dysfunction – urgency, frequency, nocturia
    • BPH must be r/o in men
  • Constipation – dysmotility is caused by lower GI dysfunction and a slowing of transit time through the entire GI tract
  • Erectile Dysfunction – dysautonomia, mood dysfunction, motor disability, s/e of medications may contribute significantly.
    • Add sildenafil
  • Orthostatic HoTN – caused by poor intake of fluid, s/e of antihypertensives, antidepressants, diuretics
    • PD dysautonomia & s/e of all PD medications especially dopamine agonists

References:
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Posted in 71 Parkinsonism, 99 Priority Topics, FM 99 priority topics, Neuro

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