From the Guidelines
Parkinson's plus syndrome is characterized by a group of movement disorders that extend beyond classic Parkinson's disease, including Progressive Supranuclear Palsy (PSP), Multiple System Atrophy (MSA), Corticobasal Degeneration (CBD), with clinical features such as poor response to levodopa therapy, early postural instability, and rapid disease progression 1.
Clinical Features
The clinical presentation of Parkinson's plus syndrome can vary depending on the specific condition, but common features include:
- Poor response to levodopa therapy
- Early postural instability with frequent falls
- Rapid disease progression
- Symmetric motor symptoms
- Autonomic dysfunction, such as orthostatic hypotension, urinary incontinence, and erectile dysfunction in MSA
- Cerebellar signs, such as ataxia, in MSA
- Stridor in MSA
- Asymmetric limb rigidity, apraxia, alien limb phenomenon, and cortical sensory loss in CBD
- Vertical gaze palsy, axial rigidity, and frontal lobe dysfunction in PSP
Diagnosis and Management
Correctly diagnosing a Parkinsonian syndrome on clinical features alone can be challenging, and imaging remains an essential diagnostic tool in the evaluation of a patient presenting with Parkinsonian symptoms 1. Management of Parkinson's plus syndrome focuses on supportive care, including physical therapy, occupational therapy, and treatment of specific symptoms. Levodopa trials may provide modest benefit in some patients, starting at 25/100mg carbidopa/levodopa three times daily, gradually increasing 1. Early recognition of these distinguishing features is crucial for accurate diagnosis, appropriate management expectations, and proper counseling of patients about disease progression.
From the Research
Clinical Features of Parkinson's Plus Syndrome
The clinical features of Parkinson's plus syndrome include:
- Symmetrical onset, infrequent or atypical tremor, prominent rigidity in axial musculature, bradykinesia, early postural instability, supranuclear gaze palsy, early autonomic failure, pyramidal affection, cerebellar involvement, alien limb phenomenon, apraxia and significant early cognitive dysfunction in some cases 2
- Nonmotor symptoms such as psychiatric, cognitive, autonomic, and gastrointestinal dysfunction, which appear to have a major impact on quality of life and disability 3
- Bradykinesia, tremor, rigidity, and postural instability, which are generally considered the cardinal signs of Parkinson's disease, but may be present in varying degrees in Parkinson's plus syndrome 4
Associated Disorders
Parkinson's plus syndrome is associated with several disorders, including:
- Progressive supranuclear palsy (PSP) 2, 3
- Multiple system atrophy (MSA) 2, 3, 5
- Dementia with Lewy body disease (DLB) 2
- Cortico-basal ganglionic degeneration (CBGD) 2, 3
- Frontotemporal dementia with chromosome 17 (FTDP-17) 2
- Pick's disease 2
- Parkinsonian-dementia complex of Guam 2
- Pallidonigral degeneration 2
- Wilson's disease 2
- A rigid variant of Huntington's disease 2
Diagnostic Challenges
The diagnosis of Parkinson's plus syndrome can be challenging due to: