Parkinsonism

Parkinsonism refers to multiple diagnoses that present with similar clinical signs to Parkinson’s Disease. These include: bradykinesia, rigidity, postural instability and tremor. About 80-85% of people with these cardinal features have idiopathic Parkinson’s Disease, however, the other 15% may have one of the alternate diagnoses that present similar to Parkinson’s Disease. In this blog post, we will discuss Parkinsonisms including Parkinson-Plus Syndromes and Secondary Parkinson’s.

Parkinsonism can be classified into multiple subgroups (see figure below) and each diagnosis has different responses to medications, various treatment approaches, and alterations in the progression of the disease.  It is important for someone who may be presenting with signs of Parkinson’s Disease to see a Movement Disorder Neurologist who would be able to best assist with an accurate diagnosis and treatment. 

The clinical signs that are common among Parkinson’s Plus syndromes include: 

• Decreased responsiveness to Sinemet (carbidopa-levedopa)

• Early onset dementia and postural instability

• Symmetrical symptoms at onset

• More trunk involvement at onset compared to arms or legs

• Visual impairments early on in diagnosis

• Significant autonomic signs early in diagnosis (such as orthostatic hypotension or a drop in BP and lightheadedness upon standing)

Parkinson-Plus syndromes:

• Progressive Supranuclear Palsy (PSP): PSP onset is usually between 50-60 years of age and is due to a build up of a protein called tau that affects the basal ganglia and brainstem. This disease is usually differentiated from idiopathic Parkinson’s due to the inability to gaze downwards and very prominent instability and falls early in the diagnosis. Dementia symptoms are also common early on in the disease process. People with this diagnosis may also have pseudobulbar signs such as dysarthria (difficulty speaking), dysphagia (difficulty swallowing) or emotional lability. This is the most common Parkinson-Plus syndrome. 

• Multiple System Atrophy (MSA): MSA onset is most commonly around 55 years of age, but can be seen anywhere from 33-78 years old. It is caused by a build up of alpha-synuclein in the brain. This syndrome is classified mainly by the early autonomic signs that are seen including orthostatic hypotension (68% of the time) and urinary dysfunction. People with this disease often have an asymmetric tremor that is irregular, bradykinetic (slowed) walking, and may have nystagmus (abnormal eye movements) while looking in certain directions. It is uncommon for someone with this diagnosis to have cognitive issues. There are some variants for this diagnosis with the most common one being Shy-Drager Syndrome. This is the second most common Parkinson-Plus syndrome.

• Corticobasal Syndrome (CBS): CBS symptoms start around 65 years of age and usually affect one side of the frontal lobe and the basal ganglia. The symptoms may present as incoordination of the arm or leg, aphasia (difficulty with understanding or expressing speech) or apraxia of speech (motor speech disorder). You may also see prominent sensory signs such as neglect of one side of the body, “alien limb” phenomenon, or difficulty with writing. Sometimes people with this disease have issues with looking side to side. It is the least common of the Parkinson-Plus syndromes.

• Lewy Body Dementia (LBD): LBD symptoms are very slow to be recognized and can be seen anytime between 50-85 years of age. This disease is caused by clumps of alpha-synuclein that form in the brain causing early and fluctuating cognitive issues as well as visual hallucinations. Other symptoms seen with this diagnosis are common to Alzheimer’s disease including difficulty with memory and thinking,  periods of unresponsiveness, and delusions. Usually cognitive and motor symptoms present together. People may also experience symmetrical motor symptoms and no resting tremor which is different than idiopathic Parkinson’s disease. This type of Parkinson-Plus syndrome may respond to levadopa/carbidopa for improving symptoms such as bradykinesia and rigidity.

Secondary Parkinsonism:

Secondary Parkinsonism occurs when there are symptoms similar to Parkinson’s Disease but it is caused by other factors besides neurodegeneration such as side effects from certain medications or a different neurologic diagnosis. Each of these diagnoses has an individual treatment approach that is different from idiopathic Parkinson’s Disease which makes an accurate diagnosis even more critical. The following 3 diagnoses are the most common types of secondary Parkinsonism. 

• Drug Induced: This is a Secondary Parkinsonism that can be caused as a side effect of taking certain medications. This is especially common with antipsychotics but can also be seen as a side effect from metoclopramide, reserpine, tetrabenazine, and certain calcium channel blockers. Usually these effects are reversible within weeks of stopping the medication. If you are starting to notice instability, tremor, or other signs associated with PD in conjunction with starting a new medication, talk to your doctor about these signs and symptoms before changing any medication.

• Vascular Parkinsonism: This Secondary type of Parkinsonism is caused by small strokes in the brain that cause signs and symptoms similar to idiopathic PD. Usually people with this diagnosis will have more difficulty with walking and have symptoms affecting their legs more than their arms, but are less likely to experience a tremor. Education is important for modifiable risk factors for stroke including managing high blood pressure and/or cholesterol, cessation of smoking, and assisting with lifestyle modifications to improve overall health and wellbeing.

• Normal Pressure Hydrocephalus (NPH): In individuals with Normal Pressure Hydrocephalus, there is increased fluid within the ventricles of the brain. This causes signs and symptoms that are very similar to idiopathic PD. In a study that compared PD and NPH, it was found that three of the four cardinal signs of idiopathic PD (bradykinesia, postural instability and rigidity) were found in both diagnoses. Resting tremor was less common in NPH. The diagnosis of NPH is done with a CT scan of the brain in which the ventricles are examined. Treatment for NPH usually involves the placement of a shunt to relieve the increased pressure and it has been shown that motor symptoms improve rapidly with this treatment approach.

Although the treatment approach for the many different types of Parkinsonism varies, the good news is that a physical therapist can assist with all of these conditions! Whether it is improving symptoms with an exercise based approach or providing education and family training to make the individual safer at home, physical therapists are trained in assisting with improving functional mobility and improving quality of life.

Sources:

K Mitra 1, P K Gangopadhaya, S K Das. Parkinsonism plus syndrome- a review. Neurol India. 2003 Jun;51(2):183-8.

M Höllerhage. Secondary parkinsonism due to drugs, vascular lesions, tumors, trauma, and other insults. Int Rev Neurobiol. 2019;149:377-418. doi: 10.1016/bs.irn.2019.10.010.

K Molde, L Soderstrom, K Laurell. Parkinsons in normal pressure hydrocephalus: a population-based study. J Neurol. 2017; 264(10): 2141–2148.