What is ataxia? This disorder involves dysfunction or damage in the brain’s cerebellum. The cerebellum controls muscle coordination. Ataxia can affect muscles throughout the body, such as those that control walking or swallowing.

It has several types, including some that worsen over time. It can’t be cured, but treatment may relieve symptoms.

Causes include:

• Genes (passed down from parents)
• Stroke
• Alcohol abuse
• Tumor
• Certain medications

Symptoms:

• Fine motor (movement) problems such as trouble writing
• Imbalance, which may lead to trouble walking
• Lack of coordination
• Rapid eye movement
• Slurred speech and difficulty swallowing

Treatments:

• Your ataxia may have an underlying cause, such as a tumor or reaction to a medication. Treating the underlying condition may ease symptoms.
• Therapy with OHSU’s neurologic rehabilitation team can improve quality of life and lessen symptoms.

More information:

• National Ataxia Foundation
• Ataxia, International Parkinson and Movement Disorders Society

What is blepharospasm? Blepharospasm is uncontrollable spasms of the eyelid muscles leading to extreme blinking. It’s rare a form of dystonia. It affects about 20,000 to 50,000 Americans, most of them older than 50.

Types: Benign essential blepharospasm is the most common type. Secondary blepharospasm is caused by other conditions, such as severe eye pain.

Causes: The causes of benign essential blepharospasm are unknown. Research shows that malfunctioning nervous-system cells may be the culprit.

Outlook: Symptoms often worsen over time. They can make reading, watching TV, driving and other activities difficult. In extreme cases, blindness may result. There is no cure, but treatment can relieve symptoms.

Symptoms:

• Excessive blinking
• Eye irritation
• Dry eyes
• Light sensitivity
• Twitching facial muscles

Treatments:

• Injections: Injections of botulinum toxin (such as Botox) can weaken eyelid muscles for up to three months, giving patients more control over blinking. This is the most common treatment. OHSU specialists in our injection clinic offer the advanced expertise needed for these precise injections.
• Medication: Pills such as clonazepam can block brain signals to the eyelid muscles. Relief may be short-term for many patients.
• Surgery: An operation called myectomy can remove some eyelid muscles and nerves.

More information:

• Resources of Possible Interest to Blepharospasm Patients, Benign Essential Blepharospasm Research Foundation
• Patient Support, Benign Essential Blepharospasm Research Foundation
• Facts About Blepharospasm, National Eye Institute

What is corticobasal degeneration? In this Parkinson’s-plus syndrome, also called CBD, the part of the brain that handles muscle movement shrinks. This causes difficulty with movement, speech and thinking. An estimated 2,000 to 3,000 people in the U.S. have this rare disorder.

Causes: A protein called tau, a normal part of the brain, acts abnormally in some cells, causing them to decline and die. The reasons this happens are unknown.

Outlook: Symptoms usually show up in people in their 60s. The disorder slowly worsens over time. There is no cure. CBD may become life-threatening after five to 10 years because of complications such as pneumonia or blood clots.

Symptoms:

• Stiff, shaky limbs, usually starting on one side of the body
• Difficulty talking, swallowing and controlling facial muscles
• Muscle spasms
• Loss of coordination
• Memory and cognitive problems
• Personality changes and compulsive behavior

Treatments: Drugs used to treat Parkinson’s disease, such as levodopa, may ease stiffness in some patients. Usually, they are not effective, though. Other drugs, such as clonazepam, may help with muscle spasms. Physical, speech and occupational therapy are the most common ways to manage symptoms.

More information:

• Corticobasal Degeneration, CurePSP
• Corticobasal Degeneration Information Page, National Institute of Neurological Disorders and Stroke

What is dystonia? This movement disorder is marked by involuntary muscle contractions (muscle movements you can’t control). Spasms can be mild or severe. They can affect one part of the body or several muscle groups. Spasms tend to worsen over time.

Types: Dystonia has many types. Cervical dystonia (spasmodic torticollis), for example, causes neck muscles to contract and results in head bobbing or twisting.

Causes: Dystonia may be passed down through genes, or it may be related to other conditions such as Parkinson’s disease or stroke.

Outlook: With treatment, people can often limit spasms and lead a normal life. OHSU treats more than 500 patients a year for dystonia. After Parkinson’s disease, it’s the condition our team treats most often.

Symptoms:

• Muscle spasms that typically begin in one or two body parts — usually the arm, neck and/or face
• Spasms that worsen with stress or fatigue
• Involuntary head twisting, tilting or shaking
• Uncontrollable blinking or eye closing
• Breathy or choked-sounding speech

Treatments:

• Injections: Therapeutic Botox injections over three or four months to weaken overcontracting muscles can reduce spasms.
• Medication: Options such as tetrabenazine, clonazepam and baclofen can decrease chemicals in the brain that tell muscles to move.
• Therapy: Therapists on OHSU’s neurologic rehabilitation team can help you improve your ability write, walk, talk and do other daily tasks.
• Deep brain stimulation (DBS) surgery uses an implantable device that generates electrical signals to help control muscle contractions. OHSU has world-class expertise in this procedure.

More information:

• Dystonia, U.S. National Library of Medicine
• Dystonia Medical Research Foundation

Essential tremor is marked by involuntary and rhythmic shaking, most often in the hands. It can also affect the arms, head, voice, torso and other body parts. It is a progressive condition, meaning it tends to worsen over time.  Learn more on our Essential Tremor page.

What is Huntington’s disease? In this genetic disorder, nerve cells in the brain break down over time, causing a loss of function. Huntington’s disease usually appears between ages 30 and 50.

Cause: A single genetic change causes this condition, which cannot be cured. OHSU provides genetic counseling and testing to help you understand your risk of developing or passing on Huntington’s disease. 

Symptoms:

• Muscle spasms or ataxia (balance or coordination problems; see more near the top of the page)
• Swallowing difficulties or slurred speech
• Slowed thinking
• Lack of impulse control or judgment
• Depression, mood swings or insomnia

Treatments:

• Medications: Tetrabenazine, haloperidol or amantadine can help control related movement disorders. Psychotherapy and mood-stabilizing medications can treat cognitive (thinking and perception) effects.
• Therapy: OHSU’s neurologic rehabilitation team offers physical, occupational and speech therapy to improve function.

More information:

• Huntington’s Disease, U.S. National Library of Medicine
• Huntington’s Disease Society of America

What is Lewy body dementia? This disorder happens when a buildup of Lewy bodies, a brain protein, leads to worsening dementia. It is a Parkinson’s-plus syndrome. Lewy body dementia affects behavior, thinking and movement. It is the second-most common form of dementia after Alzheimer’s disease. It affects an estimated 1.4 million people in the U.S.

Causes: The origins are unknown. Age, genes and environmental risks may play a role.

Outlook: Most people who develop this disorder are older than 50. People with Lewy body dementia live an average of five to eight years but can live much longer.

Symptoms: These get worse over time. They include:

• Memory and thinking problems, which may come and go
• Muscle tremors and stiffness
• Hallucinations, often visual
• Sleeping disorders, such as disrupted REM sleep
• Problems with depth perception and direction
• Irregular body functions, such as blood pressure, heart rate, digestion and the urinary tract
• Aggressive, depressed or apathetic behavior

Treatments: There is no cure, but some symptoms can be managed. Physical, occupational and speech therapy are often recommended. Some drugs may help:

• Memory and thinking: Drugs used to treat Alzheimer’s disease, called cholinesterase inhibitors, can help cognitive (thinking) problems.
• Movement: A Parkinson’s disease drug, carbidopa-levodopa, can help patients with motor (movement) skills.
• Hallucinations and behavior: These are difficult to treat because many patients are sensitive to antipsychotic drugs. Cholinesterase inhibitors may ease behavior problems and, in some patients, hallucinations. Antidepressants can treat depression and anxiety.
• Sleep disorders: Clonazepam and melatonin, used to treat insomnia, may help.

More information:

• 10 Things You Should Know about LBD, Lewy Body Dementia Association
• How to Care for a Person with Lewy Body Dementia, National Institute on Aging
• Portland Area LBD Support Group, Lewy Body Dementia Association (Choose OR in the search tool and click “Apply”)

What is multiple system atrophy? MSA is a Parkinson’s-plus syndrome. Over time, this rare disorder affects the body’s involuntary functions, such as digestion and blood pressure. MSA has no known cause. Symptoms typically develop when people are between ages 50 and 60.

Types: Depending on symptoms, MSA is classified as a parkinsonian type (MSA-P) or cerebellar type (MSA-C). MSA-P has Parkinson-like symptoms of tremor and muscle rigidity. MSA-C is marked by loss of balance or lack of coordination.

Symptoms:

• Dizziness or feeling lightheaded when standing up (due to low blood pressure)
• Parkinson’s disease symptoms of rigid muscles, slow movement, imbalance and tremor
• Ataxia-like symptoms including impaired coordination, imbalance and slurred speech (see more about ataxia near the top of the page)
• Changes to involuntary functions, possibly resulting in incontinence, impotence or abnormal sweat or tears

Treatments:

• Medications: Medications can raise your blood pressure to avoid lightheadedness or dizziness. Other medications can treat Parkinson-type symptoms. Learn more about Parkinson’s disease treatments.
• Therapy: OHSU experts in neurologic rehabilitation can help you improve or maintain your motor (movement) function.
• Injections: If dystonia (read more near the top of the page) is present, we offer therapeutic Botox injections to weaken overcontracting muscles.

More information:

• Multiple System Atrophy (MSA), CurePSP
• Multiple System Atrophy Fact Sheet, National Institute of Neurological Disorders and Stroke
• The Multiple System Atrophy (MSA) Coalition

What is myoclonus? Involuntary muscle movements called myoclonus result in sudden jerks. In healthy people, they can occur just before falling asleep or take the form of hiccups. These need no treatment. In others, a medication or a disorder such as epilepsy can cause severe twitches that benefit from treatment.

Symptoms: Sudden or brief spasms or jerks

Treatments:

• Treating the underlying cause: This is the best way to reduce or eliminate myoclonus.
• Medications: When we can’t find the cause, we may prescribe medications such as tranquilizers or anti-seizure drugs.
• Injections: Therapeutic Botox injections may provide relief by blocking the chemical messages that trigger muscle movement.

More information:

• Myoclonus Information Page, National Institute of Neurological Disorders and Stroke

What is progressive supranuclear palsy? This rare disorder is a Parkinson’s-plus syndrome. It occurs when brain cells that control movement are damaged. Researchers suspect protein buildup.

Symptoms:

• Problems with balance while walking, which may lead to falls
• Vision problems such as blurring, difficulty with bright lights, or inability to focus
• Difficulty swallowing or speaking
• Memory problems, lack of impulse control or other cognitive problems
• Sleep disorders such as insomnia

Treatments:

• Medication: Dopamine replacement may ease or control symptoms. Dopamine is a brain chemical that plays a part in muscle movement.
• Injections: Injecting therapeutic Botox into the muscles around the eyes may block muscle spasms for better eye control. 
• Therapy: OHSU’s neurologic rehabilitation specialists can help you improve balance and swallowing.

Learn more about OHSU's expertise treating PSP.

More information:

• Progressive Supranuclear Palsey (PSP), CurePSP

What is restless legs syndrome? Leg discomfort causes an uncontrollable urge to move your legs, especially when they are at rest. The cause is unknown, but it may be related to a reduction in a brain chemical, dopamine, that affects muscle control.

Symptoms:

• Pain that leads to a desire to move when legs are at rest, when you are seated or lying down
• Symptoms that worsen as the day goes on
• Involuntary leg movement or twitching during sleep

Treatment:

• Medications: Medicine can affect muscle movement by changing your body’s use of dopamine. Your doctor may prescribe a muscle relaxant to help control nighttime symptoms. Gabapentin (Neurontin), pregabalin (Lyrica) or opioids may ease pain.

More information:

• Restless Legs Syndrome Fact Sheet, National Institute of Neurological Disorders and Stroke
• Restless Legs Syndrome Foundation

What is spasticity? Spasticity is muscle tightening and stiffness. It’s caused by damage to the part of the brain and spinal cord that control movement. Muscles continuously contract, affecting movement and speech. It can occur with other diseases such as multiple sclerosis.

Symptoms:

• Involuntary muscle spasms
• Discomfort

Treatments:

• Medications: Muscle relaxers such as tizanidine (Zanaflex) and diazepam (Valium) or anticonvulsants such as gabapentin (Neurontin) may relieve symptoms.
• Therapy: Physical, occupational or speech therapists on OHSU’s neurologic rehabilitation team can help you improve or maintain muscle movement.
• Injections: Therapeutic Botox injections may decrease muscle activity to ease spasms.
• Implanted pump: For people with severe spasticity, we may recommend an intrathecal pump implant to deliver baclofen, a muscle relaxant, into the spinal cord.

More information:

• Spasticity Information Page, National Institute of Neurological Disorders and Stroke
• Spasticity, American Association of Neurological Surgeons

What are tics and Tourette syndrome? Tics are unintended sounds or movements. They usually begin in childhood and may be a sign of Tourette syndrome, a common but severe form of vocal and physical tics. Tics are more likely to develop into Tourette syndrome in males. The cause of Tourette syndrome is unknown.

Symptoms:

• Eye blinking, nose twitching or head jerking
• Throat clearing or grunting
• Repeating a gesture that was just observed or using obscene gestures
• Swearing, making vulgar statements or repeating the phrases of others

Treatments:

• Medications: Medications can decrease dopamine, the brain chemical that plays a part in muscle movement. Doctors may also control symptoms with medications used to treat attention deficit hyperactivity disorder, seizures and high blood pressure.
• Therapy: Physical and occupational therapy can help you manage tics by identifying patterns and urges.

More information:

• Tourette Association of America
• Tic and Tourette Syndrome, International Parkinson and Movement Disorders Society

What is Wilson disease? In this rare disorder, the body doesn’t eliminate copper properly. Without treatment, copper can build up in organs to life-threatening levels.

Cause: You must inherit a genetic change from both parents to have Wilson disease. We offer genetic counseling and testing to help you understand your risk of inheriting or passing on this condition.

Symptoms:

• Belly pain and lack of appetite
• Jaundice (yellowing of the skin and whites of the eyes)
• Fluid buildup in legs or belly
• Difficulty with speech or swallowing
• Problems with coordination, involuntary movements and muscle stiffness

Treatments:

• Medication: Medication can manage symptoms throughout your life. Penicillamine and trientine prevent copper buildup. Zinc acetate decreases the amount of copper you absorb from food.
• Liver transplant: If copper builds up in the liver to dangerous levels, a liver transplant may be recommended.

More information:

• Wilson Disease Information Page, National Institute of Neurological Disorders and Stroke
• Wilson Disease Association