From the Guidelines
Parkinsonism syndrome is a group of movement disorders characterized by motor symptoms of tremor, rigidity, postural instability, and bradykinesia, with Parkinson's disease being the most common cause, as well as other conditions such as progressive supranuclear palsy, multiple system atrophy, corticobasal degeneration, and vascular Parkinsonism 1.
Key Characteristics of Parkinsonism Syndrome
- Parkinsonian syndromes are neurodegenerative diseases and movement disorders that progressively degenerate the dopaminergic neurons in the substantia nigra/striatum 1
- The clinical presentation of Parkinson's disease is characterized by resting tremor, bradykinesia, and rigidity, related to progressive degeneration of the dopaminergic neurons in the substantia nigra projecting to the striatum 1
- Other features of Parkinson's disease include autonomic dysfunction, behavioral changes, and dementia 1
- Multiple system atrophy, progressive supranuclear palsy, and corticobasal degeneration are considered "Parkinson-plus" syndromes or atypical Parkinsonisms, demonstrating classic findings of Parkinson's disease with additional clinical features 1
Diagnosis and Evaluation
- 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
- The estimated interval between initial loss of dopaminergic neurons and the appearance of symptoms is approximately 5 years, after approximately 40% to 50% of the dopaminergic neurons in the substantia nigra have been lost 1
Types of Parkinsonian Syndromes
- Multiple system atrophy can be subdivided into three distinct clinical subtypes: MSA-P, MSA-C, and MSA-A, with the majority of cases exhibiting Parkinsonian symptoms at some stage of the disease 1
- Corticobasal degeneration initially presents with asymmetric limb clumsiness and progresses to include unilateral limb rigidity and dystonia, postural imbalance, and cortical features 1
- Progressive supranuclear palsy is the most common atypical Parkinsonism, with a prevalence of around 5/100,000, and patients classically present with a lurching gait and axial dystonia, manifested as unexplained falls 1
From the Research
Definition of Parkinsonism Syndrome
Parkinsonism syndrome refers to a group of neurological disorders with Parkinson disease-like movement problems, including:
Characteristics of Parkinsonism Syndrome
The syndrome is characterized by a broad differential diagnosis, involving a range of subtypes with different prognoses 4.
- Approximately 80% of akinetic-rigid syndromes are due to Parkinson's disease 3.
- Parkinsonism can be caused by secondary factors, such as drugs, and distinguishing levodopa-responsive idiopathic parkinson's disease from atypical parkinsonism is essential 4.
Symptoms of Parkinsonism Syndrome
Symptoms of parkinsonism syndrome include:
- Motor symptoms:
- Resting tremor
- Cogwheel rigidity
- Extreme slowness of movement
- Shuffling gait
- Impaired balance 5
- Nonmotor symptoms:
Diagnosis and Treatment of Parkinsonism Syndrome
Diagnosis of Parkinson disease is based on history and examination, and may involve dopamine transporter single-photon emission computed tomography to improve accuracy 2. Treatment of parkinsonism syndrome is symptomatic, focused on improving motor and nonmotor signs and symptoms, and may include:
- Dopamine-based therapies
- Nondopaminergic approaches (e.g. selective serotonin reuptake inhibitors for psychiatric symptoms)
- Rehabilitative therapy and exercise
- Advanced treatments (e.g. deep brain stimulation, levodopa-carbidopa enteral suspension) for complications such as worsening symptoms and functional impairment 2, 3, 5