In a recent issue of the “Handbook of Clinical Neurology,” a chapter is devoted to the four atypical parkinsonism disorders — dementia with Lewy bodies (DLB), multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). The authors — both movement disorder specialists at the University of Florida — indicate that “diagnosis is critical as the atypical parkinsonisms herald symptom profiles and prognoses distinct” from Parkinson’s Disease (PD). They also indicate it’s important to identify the suspected underlying pathology — whether it be alpha-synuclein (for DLB and MSA) or tau (for PSP and CBD). Identifying the underlying pathology influences clinical trial enrollment.
Here’s a link to an abstract on PubMed about the chapter:
https://www.ncbi.nlm.nih.gov/pubmed/31753139
Recognizing and treating atypical Parkinson disorders.
Armstrong MJ, McFarland N.
Handb Clin Neurol. 2019;167:301-320.
(There is a $32 fee to download the full article.)
The authors, Drs. Melissa Armstrong and Nikolas McFarland, point out that one reason to know whether you are dealing with an atypical parkinsonism disorder rather than PD is that the survival time is less than 10 years on average and that most die from disease-related issues such as falls, aspiration pneumonia, and failure to thrive.
This statement of concern about caregivers is interesting:
“Given patients’ mounting disability over time and the need for increasing support for both instrumental activities of daily living and basic activities of daily living, caregiver support is critical. In some circumstances, clinicians will need to assist couples in identifying backup caregivers to prepare for the possibility that older caregivers may themselves experience health concerns.”
PHARMACOLOGIC TREATMENT
Pharmacologic treatment is entirely symptomatic, and includes:
MOTOR
* Parkinsonian symptoms: levodopa
* Dystonia: dopaminergic agents; botulinum toxin injections; anticholinergic agents (in younger people with MSA)
* Myoclonus: valproic acid, levetiracetam, clonazepam
The authors note that deep brain stimulation is “rarely helpful” to those with atypical parkinsonism.
NON-MOTOR
* Depression: avoid tricyclic antidepressants in those with orthostatic hypotension
* Depression and Pain: duloxetine
* Depression, Sleep, and Weight Loss: mirtazapine
NONPHARMACOLOGIC TREATMENT
Nonpharmacologic treatment is focused on dysphagia (swallowing problems) and fall prevention. Treatment also includes physical therapy, occupational therapy, and speech-language pathology.
“The role of exercise in the atypical parkinsonisms is not well-researched to date, but increasing evidence of the benefits of exercise in people with PD suggests that there may be an important role for exercise in the atypical parkinsonisms, as well. Safety is a priority and guides selection of appropriate physical interventions.”
The authors encourage neurologists to discuss advance care directives with patients and families early on so that personal preferences are known. These directives should be re-discussed along the way. Palliative care and hospice can be helpful.
The chapter includes a helpful table for physicians on “Red flags suggesting an atypical parkinsonism.” That’s copied below.
Robin
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Recognizing and treating atypical Parkinson disorders.
Armstrong MJ, McFarland N.
Handb Clin Neurol. 2019;167:301-320.
Table 16.1
Red flags suggesting an atypical parkinsonism (a)
| Red flag | Suggested atypical parkinsonism |
| Rapid disease progression | Any atypical parkinsonism |
| Lack of a robust levodopa response | Any atypical parkinsonism |
| Bilateral symmetric parkinsonism | DLB, PSP |
| Early gait impairment, falls | Any atypical parkinsonism (b) |
| REM sleep behavior disorder | DLB, MSA |
| Early bulbar dysfunction | PSP |
| Irregular, jerky tremor | MSA, CBD |
| Myoclonus | MSA, CBD (less common in DLB, PSP) |
| Supranuclear gaze palsy | PSP, CBD |
| Dysautonomia | DLB, MSA |
| Cerebellar signs | MSA |
| Laryngeal stridor | MSA |
| Perioral/facial levodopa-induced dyskinesias | MSA |
| Early dementia | DLB, PSP, CBD (b) |
| Apraxia of speech or progressive nonfluent aphasia | PSP, CBD |
| Apraxia | CBD, PSP |
| Alien limb phenomenon | CBD |
| Higher cortical findings (e.g., agraphesthesia) | CBD |
(a) Most commonly associated diagnoses are listed but this does not exclude the possibility that an unlisted atypical parkinsonism could be associated with the red flag (for example, RBD is described as occurring in people with PSP, but it is more commonly associated with the synucleinopathies).
(b) Specific details of impairments may suggest a specific atypical parkinsonism.
CBD, corticobasal degeneration; DLB, dementia with Lewy bodies; MSA, multiple system atrophy; PSP, progressive supranuclear palsy.