Chapter 186: Parkinson’s Disease

CLINICAL FEATURES

Parkinsonism is a general term used to define a syndrome of bradykinesia (slowness of voluntary movements) with rigidity and/or tremor (Table 186-1); it has a wide differential diagnosis (Table 186-2). Parkinson’s disease (PD) is idiopathic parkinsonism without evidence of more widespread neurologic involvement. PD afflicts >1 million individuals in the United States. Mean age of onset is about 60 years; course progressive over 10–25 years. Tremor (“pill rolling” of hands) at rest (4–6 Hz). Presentation with tremor confined to one limb or one side of body is common. Other findings: rigidity (“cogwheeling”—increased ratchet-like resistance to passive limb movements), bradykinesia, fixed expressionless face (facial masking) with reduced frequency of blinking, hypophonic voice, drooling, impaired rapid alternating movements, micrographia (small handwriting), reduced arm swing, and flexed “stooped” posture with walking, shuffling gait, difficulty initiating or stopping walking, en-bloc turning (multiple small steps required to turn), retropulsion (tendency to fall backwards). Nonmotor aspects of PD include depression and anxiety, cognitive impairment, sleep disturbances, sensation of inner restlessness, loss of smell (anosmia), and disturbances of autonomic function. Normal muscular strength, deep tendon reflexes, and sensory examination. Diagnosis based on history and examination; neuroimaging, EEG, and CSF studies usually normal for age.
TABLE 186-1: Clinical Features of Parkinson’s Disease
CARDINAL MOTOR FEATURESOTHER MOTOR FEATURESNONMOTOR FEATURES
Bradykinesia
Rest tremor
Rigidity
Postural instability
Micrographia
Masked facies (hypomimia)
Reduced eye blinking
Drooling
Soft voice (hypophonia)
Dysphagia
Freezing
Anosmia
Sensory disturbances (e.g., pain)
Mood disorders (e.g., depression)
Sleep disturbances (e.g., RBD)
Autonomic disturbances
 Orthostatic hypotension
 Gastrointestinal disturbances
 Genitourinal disturbances
 Sexual dysfunction
Cognitive impairment/Dementia
Abbreviation: RBD, rapid eye movement behavior disorder.
TABLE 186-2: Differential Diagnosis of Parkinsonism
Parkinson’s disease
 Sporadic
 Genetic
Dementia with Lewy bodies
Atypical parkinsonism
 Multiple-system atrophy (MSA)
  Cerebellar type (MSA-c)
  Parkinson type (MSA-p)
Progressive supranuclear palsy
  Parkinsonism
  Richardson variant
Corticobasal syndrome
Frontotemporal dementia
Secondary parkinsonism
 Drug-induced
 Tumor
 Infection
 Vascular
 Normal-pressure hydrocephalus
 Trauma
 Liver failure
 Toxins (e.g., carbon monoxide, manganese, MPTP, cyanide, hexane, methanol, carbon disulfide)
Neurodegenerative disorders and other forms of parkinsonism
 Wilson’s disease
 Huntington’s disease
 Neurodegeneration with brain iron accumulation
 SCA 3 (spinocerebellar ataxia)
 Fragile X–associated ataxia-tremor-parkinsonism
 Prion disease
 X-linked dystonia-parkinsonism
 Alzheimer’s disease with parkinsonism
 Dopa-responsive dystonia
Abbreviation: MPTP, 1-methyl-4-phenyl-1,2,5,6-tetrahydropyridine.

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