Atypical parkinsonism

Disease Description (Causes and Symptoms)

Atypical Parkinsonism is a group of degenerative disorders that show motor symptoms similar to Parkinson’s disease (bradykinesia, muscle rigidity, gait disturbance, tremor, etc.), but in which additional features—including abnormalities of the autonomic nervous system, cognition, and brainstem function—appear early in the clinical course, and which respond poorly to dopaminergic agents such as levodopa. The main subtypes and features are as follows.

• Progressive supranuclear palsy (PSP): Postural instability and frequent falls from the early stage, vertical gaze palsy (especially difficulty looking downward), muscle rigidity in the neck, and findings of midbrain atrophy. Pathologically, it is primarily associated with accumulation of tau protein.
• Multiple system atrophy (MSA): Autonomic dysfunction (orthostatic hypotension, urinary and bowel dysfunction) and cerebellar symptoms (ataxia), symmetrical and rapid progression, and often little resting tremor. Pathologically, it is associated with accumulation of alpha-synuclein. MRI may show the “hot cross bun” sign in the pons.
• Corticobasal degeneration (CBD): Markedly asymmetric symptoms (difficulty using tools and performing movements on one side, non-articulated movements, involuntary movements), frontal and parietal cognitive decline, and findings of unilateral cerebral atrophy and asymmetric hypometabolism. It is primarily associated with tau-related pathology.
• Dementia with Lewy bodies (DLB): Cognitive decline that progresses rapidly at the same time as or before motor symptoms, fluctuating consciousness and attention, recurrent visual hallucinations, and coexisting parkinsonism and dementia. Alpha-synuclein pathology.

Common clinical points: Compared with Parkinson’s disease, progression is faster, and autonomic dysfunction, falls, cognitive impairment, eye-movement disorders, and cerebellar symptoms often appear early. Findings on imaging (brain MRI, FDG-PET, etc.) can help with diagnosis.

Treatment Methods and Precautions

The goals of treatment are symptom relief, prevention of complications, and maintenance of function rather than fundamentally stopping the disease.

Medication Treatment

• Dopaminergic agents such as levodopa: Worth trying, but the response is often limited. If symptoms improve, treatment is continued; if not, management is adjusted to focus on other symptomatic treatments.
• Autonomic symptoms: For orthostatic hypotension → increased fluid and salt intake, compression stockings, and medications such as midodrine and fludrocortisone. For urinary dysfunction → medications (e.g., beta-3 agonists), and if necessary, intermittent catheterization or an indwelling catheter. Medications for urinary incontinence may worsen cognitive function and should be used with caution.
• Cognitive and psychiatric symptoms: When dementia with Lewy bodies is present, a cholinesterase inhibitor (rivastigmine) may be considered. Antipsychotics may be necessary for hallucinations or severe psychiatric symptoms, but typical antipsychotics (e.g., haloperidol) can worsen parkinsonism and should be used cautiously, with relatively safer medications such as quetiapine or clozapine selected.
• REM sleep behavior disorder: Small doses of melatonin or clonazepam may be used.
• Dysphagia: Swallowing training and dietary modification; enteral feeding through a gastrostomy tube (PEG) may be considered when the risk of aspiration is high.

Non-medication Treatment and Rehabilitation

• Physical therapy (balance and gait training), occupational therapy (assistance with daily activities), speech and swallowing therapy, and creation of an environment that prevents falls.
• Nutritional management and oral and airway care (to prevent aspiration pneumonia).
• Early consultation and palliative care planning for end-of-life and symptom management (pain, weakness, breathing problems, etc.).

Surgery/Other

• Deep brain stimulation (DBS) generally has limited effectiveness and is not recommended for atypical parkinsonism.

Precautions (Risk of Major Complications)

• When selecting medications, consider cognitive function, autonomic dysfunction, and cardiovascular status.
• Typical antipsychotics carry a risk of worsening symptoms → use the minimum dose and monitor closely if they are used.
• Major complications include aspiration pneumonia, injuries caused by repeated falls, and loss of function due to advanced cognitive impairment.

Prognosis

• In general, the disease progresses more rapidly than Parkinson’s disease, and a shorter survival period and faster functional decline may occur. Differences exist depending on the subtype, initial symptoms, and overall health.

Checklist Before an Outpatient Visit (Items to Prepare and Sample Questions)

Items/Information to Prepare

• A list of all medications currently being taken (including prescription drugs, nonprescription drugs, and supplements) and the dosing times.
• A record of recent symptom changes: when symptoms began, the rate of progression, frequency of falls (when and how many times), whether orthostatic dizziness occurs, changes in urination and bowel movements, difficulty swallowing, and sleep problems (dream-related behaviors), etc.
• Copies or CDs of recent test results (brain MRI, DAT SPECT, FDG-PET, etc.).
• Blood pressure measurements (when lying down and after standing) and records of weight changes.
• Bringing a family member or caregiver is recommended (as this can help observe symptoms and cognitive status).

Information to Tell the Doctor in Advance During the Visit (Examples)

• The number and circumstances of recent falls (when and where the person fell).
• Whether any medications have recently been started or discontinued.
• Whether visual or auditory hallucinations, changes in consciousness, daytime sleepiness, or sleep disorders are present.
• Whether there are foods that are difficult to swallow or whether coughing occurs.

General Sample Questions to Ask the Doctor

• What are the expected effects and side effects of this medication (or this combination therapy)?
• If we try levodopa, how long will its effectiveness be evaluated, and according to what criteria?
• What should be done in daily life to prevent falls, orthostatic hypotension, and dysphagia?
• What treatments and support are available if cognitive function continues to worsen?
• What is the future follow-up schedule, and what tests will be needed?

Examples of Disease-Specific Questions

• Are my symptoms (e.g., early falls, gaze abnormalities, or rapid cognitive decline) more consistent with PSP, MSA, CBD, or DLB? What additional tests are specifically needed?
• If my symptoms are not adequately controlled with the current medications, what is the next treatment step (e.g., management of autonomic symptoms, a rehabilitation plan, or a palliative care consultation)?