Chapter 186: Parkinson’s Disease
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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.
CARDINAL MOTOR FEATURES | OTHER MOTOR FEATURES | NONMOTOR 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 |
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 |
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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.
CARDINAL MOTOR FEATURES | OTHER MOTOR FEATURES | NONMOTOR 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 |
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 |
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