Parkinson's and Movement Disorders

Types of Movement Disorders

The staff of the Parkinson’s and Movement Disorders Program evaluates and treats the entire range of movement disorders. More detailed information on these disorders is available from the National Institute of Neurological Disorders and Stroke and the National Parkinson Foundation.

Movement disorders include the following conditions:

Ataxias/gait-balance disorders

Spinocerebellar ataxia (SCA)
Normal pressure hydrocephalus (NPH)
Gait disorders/gait disturbance of any cause

Drooling

Dystonia

Adult-onset dystonia
Dopa-responsive dystonia/Segawa’s dystonia
Focal dystonia
- Cervical dystonia (spasmodic torticollis)
- Oromandibular dystonia
- Spasmodic dysphonia/vocal dystonia
- Writer’s cramp/task-specific dystonias

Essential tremor/familial tremor and other tremors

Hemifacial spasm/blepharospasm

Huntington’s disease (HD) and other choreas

Myoclonus

Parkinson’s disease and related conditions

Parkinson-plus syndromes (Parkinsonism)

Progressive supranuclear palsy (PSP)
Multiple system atrophy (MSA)
Corticobasal degeneration (CBD)
Diffuse Lewy body disease (DLBD)
Vascular parkinsonism

Psychogenic movement disorders

Spasticity

Tourette’s syndrome/Tics

Wilson’s disease

Other movement disorders

Painful legs and moving toes syndrome
Paroxysmal kinesigenic dyskinesia (PKD) and paroxysmal nonkinesigenic dyskinesia (PNKD)
Periodic limb movement disorder (PLMD)
Restless legs syndrome (RLS)

Ataxias/gait-balance disorders

Ataxias/gait-balance disorders are incoordination and impaired gait/balance, often the result of damage to malfunction of the cerebellum. Ataxia produces jerky movements and usually results from disease in the cerebellum of the brain. The term ataxia describes a set of symptoms and also refers to a family of disorders; it is not a specific diagnosis.

Spinocerebellar ataxia (SCA) — a degeneration of the spinal cord and the cerebellum, which coordinates body movements. The wasting away (atrophy) of the cerebellum results in a loss of muscle coordination. Atrophy in the spine can cause spasticity. There are many types of SCA.
Normal pressure hydrocephalus (NPH) — an increase in the amount of cerebrospinal fluid (CSF) in the brain without a corresponding increase in pressure. In NPH, CSF production seems to be normal, but there appears to be a problem with the absorption of CSF in the brain, causing CSF to build up. The buildup increases the size of the brain ventricles and causes tension on surrounding brain tissue, resulting in gait disturbance, dementia and impaired bladder control.
Gait disorders/gait disturbance — the slowing of gait speed (the pattern of how a person walks) or a change in the smoothness or symmetry of body movement. Gait disorders have many causes, including central nervous system disorders of the brain (e.g., multiple strokes, multiple sclerosis and cerebral palsy) that cause muscular problems and resulting gait disturbance.

Drooling

Drooling is a symptom associated with Parkinson’s disease. Excessive saliva is caused by a lack of spontaneous swallowing, and by slowness of the tongue, mouth and throat muscles. A flexed neck posture will cause saliva to accumulate at the front of the mouth, which leads to drooling.

Dystonia

Dystonia is a disorder that involves involuntary movements and prolonged muscle contractions, resulting in twisting and repetitive movements or abnormal postures. There are many forms of dystonia. These movements may affect the entire body, a single muscle or a group of muscles. Many cases of primary dystonias appear to be inherited. Symptoms may first appear in childhood, late adolescence or early adulthood.

Adult-onset dystonia — dystonia that begins in adulthood. The Parkinson’s and Movement Disorders Program treats many people with adult-onset dystonia as well as adults whose dystonia began in childhood.
Dopa-responsive dystonia (DRD) / Segawa’s dystonia — a type of dystonia that usually begins in childhood or adolescence with progressive difficulty walking. Symptoms mimic those of cerebral palsy, and spasticity may occur in some cases. Symptoms fluctuate from relative mobility in the morning to increasing disability later in the day. Usually symptoms are very responsive to low-dose levodopa treatment.
Focal dystonia is localized to a part of the body. Examples include:
- Cervical dystonia (spasmodic torticollis) — dystonia that affects the neck and sometimes shoulders. Muscles in the neck that control the position of the head are affected, causing the head to twist and turn to one side and possibly pull forward or backward. Most people first experience symptoms in middle age. More information is available from the National Spasmodic Torticollis Association or at www.spasmodictorticollis.org.
- Oromandibular dystonia — continuous spasms of muscles of the face, jaw, pharynx, tongue and, in severe cases, the neck, larynx and respiratory system.
- Spasmodic dysphonia/vocal dystonia — a voice disorder caused by involuntary movements of muscles of the larynx (voice box). People may have trouble saying a word or two, or they may experience more serious problems communicating. Spasmodic dysphonia causes the voice to break or to have a tight or strained quality. There are three different types of spasmodic dysphonia.
- Writer’s cramp and other task-specific dystonias — dystonia that occurs as a result of performing specific tasks, such as writing or playing the piano.

Essential Tremor/Familiar Tremor and Other Tremors

Essential tremor/familial tremor and other tremors are tremors are nerve disorders in which “shakes” occur when a person moves or tries to move. Essential tremor is most common in people over age 65. The cerebellum, a part of the brain that coordinates muscle movements, does not appear to work properly in people with essential tremor. If essential tremor occurs in more than one member of a family, it is called familial tremor. More information is available from the International Essential Tremor Foundation.

Hemifacial Spasm/Blepharospasm

Hemifacial spasm/blepharospasm involves sudden contractions of muscles on one side of the face that may be caused by a facial nerve injury, a tumor or a blood vessel pressing on the facial nerve. Sometimes, the cause is not known. Blepharospasm is an abnormal, involuntary blinking or spasm of the eyelids, associated with an abnormal function of the basal ganglia (a region of the brain). The condition may result in vision problems.

Huntington's Disease and Other Choreas

Huntington’s disease (HD) and other choreas are inherited disorders caused by the degeneration of certain nerve cells in the brain. The disease is genetic. HD causes uncontrolled movements (chorea) and dementia. The condition usually begins in mid-adulthood and progresses slowly to death. Other names are Huntington disease, Huntington chorea and Huntington’s chorea. More information is available from the Huntington’s Disease Society of America.

Myoclonus

Myoclonus is a sudden, involuntary jerking of a muscle or group of muscles usually caused by sudden muscle contractions or muscle relaxation. The twitching cannot be controlled. Myclonus occurs in simple forms (e.g., a hiccup) in healthy persons and causes no problems. When more widespread, myoclonus may involve continual and severe muscle contractions. Myoclonic jerking may develop in people with multiple sclerosis, Parkinson’s disease, Alzheimer’s disease, epilepsy or other disorders.

Parkinson's Disease and Related Conditions

Parkinson’s disease (PD) and related conditions are chronic and progressive disorders that occurs when certain nerve cells in the brain die or become impaired. Normally, these cells produce a chemical called dopamine, which allows coordinated function of muscles and movement. When most of the dopamine-producing cells are damaged, the symptoms of Parkinson’s disease appear.

According to the National Parkinson Foundation, researchers suspect that the cause of PD in most people is a combination of genetic factors and exposure to toxins in the environment. An estimated 1.5 million people in the United States have PD, and it affects about one in 100 Americans over age 60.

The four major symptoms of Parkinson’s disease are:

Resting tremor (trembling in hands, arms, legs, jaw and face), which occurs in about 70 percent of people with PD
Rigidity or stiffness of the limbs and trunk
Bradykinesia (slowness of movement)
Impaired balance and coordination

As symptoms progress, patients may have difficulty walking, talking or doing simple tasks. A diagnosis is based on a medical history, physical exam, blood tests and a brain scan. Usually, two of the four major symptoms must be present to consider a diagnosis of PD.

Parkinson-plus Syndromes

About 15 percent of patients originally diagnosed with PD begin to show signs of having “atypical” parkinsonism, also called “Parkinson-plus” syndromes. Parkinsonism refers to conditions that mimic PD, with symptoms such as tremor, slow movements, stiff muscles and balance and walking difficulties. These symptoms, however, are caused by another condition. Various conditions can cause parkinsonism, and diagnosis can be complex.

Other forms of parkinsonism include:

Progressive supranuclear palsy (PSP) — a rare brain disorder that causes serious and permanent problems with walking and balance control. Symptoms include difficulty aiming the eyes, caused by lesions in the part of the brain that coordinates eye movements. Other symptoms include mood and behavior changes, such as depression, apathy and mild dementia. PSP begins slowly and progresses, causing weakness (palsy) by damaging certain parts of the brain.
Multiple system atrophy (MSA) — a rare, progressive neurodegenerative disorder with symptoms that affect movement, blood pressure and other body functions. In MSA, cells are damaged in different areas of the brain that control body functions. Symptoms include a large drop in blood pressure (postural hypotension), which causes dizziness or brief blackouts when standing or sitting up. Other symptoms may include weakness, headaches, vision problems, speech problems, sensory changes, difficulties with breathing and swallowing, irregular heartbeat, inability to sweat and diarrhea. MSA affects people primarily in their 50s.

There are three subtypes of MSA:
- Parkinsonsian-predominant (striatonigral degeneration), caused by a disruption in the connection between two areas of the brain — the striatum and the substantia nigra. These two areas work together to allow balanced and movement. Symptoms include tremor, rigidity, instability, impaired speech and slow movements.
- Autonomic-predemoninant with parkinsonian features (Shy-Drager syndrome), a progressive disorder of the central nervous system and the autonomic nervous system, which regulates certain body functions.
- Cerebellar parkinsonism (olivopontocerebellar atrophy), a mixture of parksinsonian and cerebellar features.
Corticobasal degeneration (CBD), is a progressive disorder related to Parkinson’s disease. CBD involves involves nerve cell loss and atrophy (shrinkage) of many areas of the brain including the cerebral cortex and the basal ganglia. The disease progresses gradually. Symptoms, which usually begin around age 60, may first appear on one side of the body. As the disease progresses, both sides are affected. Symptoms are similar to those found in Parkinson’s disease, and other symptoms may occur.
Diffuse Lewy body disease (DLBD) is a spectrum of diseases involving dementia and motor symptoms, and is the second most common cause of dementia. Because the dementia is similar to that of Alzheimer’s, and other symptoms mimic Parkinson’s disease, the disease can be difficult to diagnose. However, patients with DLBD have hallucinations and are very sensitive to antipsychotic medications. In addition, their daily symptoms vary. DLBD is more common in men than women.
Vascular parkinsonism — a neurological disorder in which the symptoms of parkinsonism (tremor, rigidity, slow movement and difficulty walking) are a result of small strokes vs. a loss of nerve cells. When one or more strokes occur in the basal ganglia (the part of the brain that controls movements) on one side of the brain, a person may develop parkinsonism on the opposite side of the body. Symptoms may progress gradually and resemble the progression of Parkinson’s disease.

Psychogenic Movement Disorders

Psychogenic movement disorders are increased or decreased movements that originate in the mind. The movements cannot be controlled and are not associated with a known neurological problem.

Spasticity

Spasticity is a condition in which certain muscles contract continuously, causing stiffness or tightness. Spasticity may interfere with movement, speech and walking. It is usually caused by damage to the portion of the brain that controls voluntary movement. People who suffer a stroke or brain injury, or who have multiple sclerosis or cerebral palsy may experience spasticity.

Tourette's Syndrome/Tics

Tourette’s syndrome/Tics is an inherited disorder of the nervous system characterized by repeated involuntary movements and uncontrollable vocal sounds called tics. The most common symptoms are facial tics, such as eye-blinking, nose-twitching, facial grimacing, shoulder shrugging and head/shoulder jerking. In a few patients, these tics include using inappropriate words. Some people experience inattention, hyperactivity, impulsivity and symptoms such as intrusive thoughts and repetitive behaviors. Symptoms usually appear before age 18, and men are affected more often than women.

Wilson's Disease

Wilson’s disease is a rare inherited disorder in which excess amounts of copper build up in the body, beginning at birth. Symptoms appear later in life. The disease can cause neurological symptoms (tremor, rigidity, drooling and speech problems), personality/behavioral problems, depression and damage to the liver, kidneys, brain and eyes. If treatment begins early, symptoms can be greatly reduced, and a person can expect a normal length and quality of life. Without proper treatment, Wilson’s disease is fatal.

Other Movement Disorders

Other movement disorders include:

Painful legs and moving toes syndrome — a rare disorder marked by pain in the foot or lower leg and involuntary toe movements. One or both legs may be affected. Discomfort, which may be mild or severe, intensifies with activity and usually stops during sleep. In most cases, the cause is unknown.
Paroxysmal kinesigenic dyskinesia (PKD) and paroxysmal nonkinesigenic dyskinesia (PNKD) — sudden attacks of dystonia. PKD consists of brief attacks of dystonia — lasting from seconds to 5 minutes — that affect muscles of the arms and legs, but may also involve muscles of the face, neck and/or legs and trunk. PNKD involves attacks of dystonia or chorea that last from minutes to hours. A person may have many attacks daily, or there may be weeks or months between attacks.
Periodic limb movement disorder (PLMD) — involuntary leg twitching or jerking movements frequently during sleep that typically occur every 10 to 60 seconds. The cause is unknown.
Restless legs syndrome (RLS) — a neurological disorder with unpleasant sensations in the legs and an uncontrollable urge to move when at rest to relieve these feelings. Symptoms are often described as burning, creeping, tugging or like insects crawling inside the legs. Lying down and trying to relax activates the symptoms; as a result, most people with RLS have difficulty falling asleep and staying asleep. Most people who are severely affected are middle-aged or older, and the severity of the disorder appears to increase with age. Some researchers estimate that RLS affects as many as 12 million Americans. However, others estimate this number is higher because RLS can be underdiagnosed and, in some cases, misdiagnosed.

Although many people with RLS also develop PLMD, most people with PLMD do not experience RLS.

Author: Marla Fraunfelder

Date: Feb. 25, 2008

Online Editor(s): Rich Petre