Parkinsonism and Parkinson’s Plus Syndromes

Parkinsonism

Parkinsonism is a group of conditions that are characterized by the four typical motor symptoms of tremor, rigidity, postural instability (impaired balance), and bradykinesia (gradual loss of spontaneous movement). Parkinson’s disease (PD) is the most common form of parkinsonism.1

Parkinson’s Plus Syndromes, or Parkinson’s-plus, are a group of conditions that cause symptoms like Parkinson’s disease as well as other symptoms.1

Parkinson’s Plus Syndromes

Multiple System Atrophy

Multiple system atrophy (MSA) is the name given to a group of disorders that progressively affect the central and autonomic nervous systems. (The central nervous system is made up of the brain and spinal cord, while the autonomic nervous system controls automatic functions like breathing, heart beating, and the movements of the intestines.) Atrophy means wasting or the decrease in size, strength or function of an organ or system. In MSA, the supporting cells in the brain called oligodendrocytes are damaged by abnormal accumulations the protein alpha-synuclein.

There are 2 types of MSA. One type, MSA-P, is characterized by Parkinson’s like symptoms such as slowness of movement, rigid muscles and lack of facial expression. Initially, the same treatments for PD can alleviate symptoms in MSA-P. However, MSA-P tends to progress much more rapidly than typical PD. MSA-C, also known as cerebellar MSA, is characterized by lack of coordination and balance, tremor when they reach for an object and slurred speech and trouble swallowing.

Problems with the autonomic system characterize MSA such as orthostatic hypotension which is feeling dizzy when going from a supine position to standing up, constipation, increased sweating, sleep apnea, and sexual dysfunction.2

Dementia with Lewy Bodies

Dementia with Lewy bodies (DLB) is named for the presence of abnormal protein deposits of alpha-synuclein (Lewy bodies) that are also seen in people with PD. DLB is the second most common type of dementia after Alzheimer’s disease, and it is a progressive disorder. In people with DLB, the Lewy bodies are found throughout the brain. DLB is characterized by cognitive impairments, visual hallucinations, inability to focus, inflexible thinking and paranoia. DLB patients initially have these cognitive symptoms but also develop motor symptoms similar to PD such as bradykinesia, tremor, rigidity, and shuffling walk. Similar to PD, DLB patients also have REM sleep behavior disorder in which they act out their dreams. DLB is treated with cholinesterase inhibitors and atypical antipychotics. However, there is no cure for DLB.

Progressive Supranuclear Palsy

Progressive supranuclear palsy (PSP) is a rare, chronic disorder causing difficulties with balance, walking, muscle stiffness, and abnormal eye movements. A defining symptom is the inability to aim the eyes properly, caused by lesions in the area of the brain that controls eye movement. In addition, many patients with PSP experience alterations of mood and behavior, such as depression, apathy, and progressive dementia. Unlike PD, patients with PSP stand very straight and tend to fall backward (PD patients tend to stoop forward). Tremor is rare in PSP. Patients with PSP do not respond to typical PD treatments such as levodopa.

Corticobasal Degeneration

In corticobasal degeneration (CBD), multiple areas of the brain sustain nerve cell loss and atrophy. Symptoms generally begin around age 60 and may initially affect only one side of the body (unilateral) before progressing to both sides of the body. Symptoms are similar to PD (poor coordination, rigidity, and impaired balance), such as the loss of ability to make purposeful movements, hesitant and halting speech, stiffness, muscle jerks, and difficulty swallowing. There are also cognitive problems and problems with language, such as the ability to remember a word.  There are also personality changes, problems with inappropriate behavior and compulsive behaviors. There is currently no treatment to slow the course of progression in CBD. Symptoms of CBD are generally not affected by drug therapy, although occupational, physical, and speech therapy can help patients manage their disability.1,3

Written by: Emily Downward | Last reviewed: March 2017
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