Parkinsonism



Parkinsonism





Characterized by any combination of: tremor at rest, rigidity, bradykinesia (slowing of movements), hypokinesia (less frequent spontaneous movements), flexed posture, loss of postural reflexes, “freezing” (motor block).

Categories: idiopathic, symptomatic, Parkinson-plus syndromes, heredodegenerative diseases with parkinsonism (Table 115.1).

Core biochemical pathology: decreased dopaminergic neurotransmission in basal ganglia, leading to disinhibition of subthalamic nucleus and medial globus pallidus.









Table 115.1 Classification of Major Parkinsonian Syndromes










Idiopathic Parkinsonism
Parkinson disease
Symptomatic Parkinsonism
Drug-induced: dopamine antagonists and depleters
Hemiatrophy-hemiparkinsonism
Hydrocephalus; normal pressure hydrocephalus
Hypoxia
Infectious; postencephalitic
Metabolic; parathyroid dysfunction
Toxins: manganese, carbon monoxide, MPTP, cyanide
Trauma
Tumor
Vascular; multi-infarct state
Parkinson-plus Syndromes
Cortical-basal ganglionic degeneration
Dementia syndromes
  Alzheimer disease
  Diffuse Lewy body disease (dementia with Lewy bodies)
Lytico–Bodig (Guamanian parkinsonism–dementia–ALS complex)
Multiple system atrophy syndromes
  Striatonigral degeneration
  Shy-Drager syndrome
  Sporadic olivopontocerebellar degeneration
  Motor neuron disease-parkinsonism
Progressive pallidal atrophy
Progressive supranuclear palsy
Heredodegenerative Diseases
Neurodegeneration with brain iron accumulation (NBIA; Hallervorden-Spatz disease)
Huntington disease
Lubag (X-linked dystonia-parkinsonism)
Mitochondrial cytopathies with striatal necrosis
Neuroacanthocytosis
Wilson disease
ALS, amyotrophic lateral sclerosis; MPTP, methyl-4-phenyl-1,2,3,6-tetrahydropyridine



  • Rest tremor: 4 to 5 Hz; usually involves limbs, especially distally; also lips, chin, tongue. Disappears with action, but reemerges with maintained posture. Worsened by stress, walking.


  • Rigidity: increased resistance to passive movement; equal in all directions; often with palpable “cogwheeling.”



  • Flexed posture: commonly begins in arms, spreads to body.


  • Bradykinesia: slow movements, difficulty starting movement, loss of automatic movements (arm-swing).


  • Hypokinesia: reduced amplitude of movement, particularly repetitive movements (“decrementing” finger or toe-tapping).


  • Akinesia: reduced frequency of movements.


  • Examples of bradykinesia, hypokinesia, akinesia: hypomimia (decreased facial expression), decreased blinking, loss of gestures, setting motionless, hypophonia, dysarthria, tachyphemia (rapid speech), aprosody (monotonous speech; loss of rhythm of speech), drooling (impaired swallowing), micrographia, impaired fine motor skills, short stride (festinating gait), difficulty rising from seat. Advanced bradykinesia—major impediment in activities of daily living. If feet involved, should stop driving.


  • Freezing phenomenon (motor block): transient inability to perform active movements. Manifest in legs (gait freezing), eyelids (apraxia of lid opening), speech (palilalia), writing. Lasts several seconds. Triggers: starting to walk, turning, approaching a destination, revolving doors, elevator doors, crossing heavily trafficked streets. Often overcome by visual clues.


Parkinson Disease (PD)

Also known as idiopathic Parkinson disease or primary parkinsonism.


Pathology

Degeneration of neuromelanin-containing neurons in pars compacta of substantia nigra, locus ceruleus; Lewy bodies in surviving neurons.


Epidemiology

About 80% of cases of parkinsonism outlined in Table 115.2. Mean onset age 55, range 20 to 80. M:F ratio 3:2. Prevalence 160:100,000; incidence 20:100,000/year.









Table 115.2 Clues Indicating the Likely Type of Parkinsonism































































































































Clinical Most likely form of parkinsonism
Never responded to levodopa Other than PD
Predominantly unilateral PD; HP-HA syndrome; CBGD
Symmetric onset PD; most forms of parkinsonism
Presence of rest tremor PD; secondary parkinsonism
Lack of rest tremor Parkinson-plus syndromes
History of encephalitis Postencephalitic parkinsonism
History of toxin exposure Parkinsonism caused by the toxin
Taking neuroleptics Drug-induced parkinsonism
Severe unilateral rigidity CBGD
Cortical sensory signs CBGD
Unilateral cortical myoclonus CBGD
Unilateral apraxia CBGD
Alien limb CBGD
Early dementia CBGD
Psychotic sensitivity to levodopa Diffuse Lewy body disease; AD
Early loss of postural reflexes Progressive supranuclear palsy
Impaired downgaze Progressive supranuclear palsy
Deep nasolabial folds Progressive supranuclear palsy
Furrowed forehead and eyebrows (procerus sign) Progressive supranuclear palsy
Nuchal dystonia Progressive supranuclear palsy
Abducted arms when walking Progressive supranuclear palsy
Square wave jerks Progressive supranuclear palsy
Pure freezing Progressive supranuclear palsy
Meaningful orthostatic hypotension Shy-Drager syndrome
Urinary or fecal incontinence Shy-Drager syndrome
Cerebellar dysarthria and dysmetria Olivopontocerebellar degeneration
Laryngeal stridor (vocal cord paresis) Striatonigral degeneration
Lower motor neuron findings Multiple system atrophy
Upper motor neuron findings Multiple system atrophy
Laboratory   
Fresh blood smear: acanthocytes Neuroacanthocytosis
Grossly elevated creatine kinase Neuroacanthocytosis
MRI: many lacunes Vascular parkinsonism
MRI: “tiger’s eye” in pallidum Hallervorden-Spatz disease
MRI: caudate atrophy HD; neuroacanthocytosis
MRI: decreased T2 signal in striatum Multiple system atrophy
MRI: midbrain atrophy Progressive supranuclear palsy
MRI: huge ventricles Normal pressure hydrocephalus
Abnormal autonomic function tests Shy-Drager syndrome
Denervation on sphincter EMG Shy-Drager syndrome
AD, Alzheimer disease; CBGD, corticobasal ganglionic degeneration; HP-HA, hemiparkinsonism-hemiatrophy; PD, Parkinson disease.

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Jul 27, 2016 | Posted by in NEUROLOGY | Comments Off on Parkinsonism

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