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Diseases&Conditions

Ataxia

Ataxia-telangiectasia is a rare, childhood neurological disorder that causes degeneration in the part of the brain that controls motor movements and speech. Its most unusual symptom is an acute sensitivity to ionizing radiation, such as X-rays or gamma-rays. The first signs of the disease, which include delayed development of motor skills, poor balance, and slurred speech, usually occur during the first decade of life. Telangiectasias (tiny, red "spider" veins), which appear in the corners of the eyes or on the surface of the ears and cheeks, are characteristic of the disease, but are not always present and generally do not appear in the first years of life. About 20% of those with A-T develop cancer, most frequently acute lymphocytic leukemia or lymphoma. Many individuals with A-T have a weakened immune system, making them susceptible to recurrent respiratory infections. Other features of the disease may include mild diabetes mellitus, premature graying of the hair, difficulty swallowing, and delayed physical and sexual development. Children with A-T usually have normal or above normal intelligence.

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Chorea

Also known as Sydenham chorea (SD), is a neurological disorder of childhood resulting from infection via Group A beta-hemolytic streptococcus (GABHS), the bacterium that causes rheumatic fever. SD is characterized by rapid, irregular, and aimless involuntary movements of the arms and legs, trunk, and facial muscles. It affects girls more often than boys and typically occurs between 5 and 15 years of age. Some children will have a sore throat several weeks before the symptoms begin, but the disorder can also strike up to 6 months after the fever or infection has cleared. Symptoms can appear gradually or all at once, and also may include uncoordinated movements, muscular weakness, stumbling and falling, slurred speech, difficulty concentrating and writing, and emotional instability. The symptoms of SD can vary from a halting gait and slight grimacing to involuntary movements that are frequent and severe enough to be incapacitating. The random, writhing movements of chorea are caused by an auto-immune reaction to the bacterium that interferes with the normal function of a part of the brain (the basal ganglia) that controls motor movements. Due to better sanitary conditions and the use of antibiotics to treat streptococcal infections, rheumatic fever, and consequently SD, are rare in North America and Europe. The disease can still be found in developing nations.

Dystonia

A team of experts including renowned neurologists, neurosurgeons, researchers and a host of support personnel offers the latest proven treatments for people with movement disorders, including Parkinson’s disease, essential tremor and dystonia. Our surgical team is world-renowned for performing deep brain stimulation (DBS) surgeries and has experience with more than 1,200 DBS implants. The group also has expertise in the surgical management of spasticity. Various medication clinical trials as well as gene therapy surgical trial programs are under way.

Overview

Dystonia is a movement disorder in which forceful sustained muscle contractions cause twisting and repetitive movements or abnormal postures. These involuntary and sometimes painful movements may affect a single muscle; a group of muscles such as those in the arms, legs or neck; or the entire body.

Dystonia causes varying degrees of disability and pain, from mild to severe. It is a chronic disorder, but does not impact cognition or intelligence in the majority of patients. While there is presently no cure for dystonia, there are multiple treatment options available that reduce the severity of the symptoms.

What are the types of dystonias?

There are many different forms of dystonia and dozens of diseases and conditions that include dystonia as a major symptom. The forms of dystonia are classified by muscle location and distribution.

  • Focal dystonia forms during adulthood and affects the muscles in a specific area of the body, such as the eyes, mouth, vocal cords, neck, hands and feet.
  • Task specific dystonia is related to the occurrence of focal dystonia, but only during a specific task. For example, musicians’ dystonia is most often diagnosed in professional musicians and affects the mouth, cheeks, jaw and tongue. Similarly, writer’s cramp happens only in the hands and fingers.
  • Early-onset generalized dystonia is characterized by involuntary muscle contractions that begin in the limbs and eventually cause twisting contractions in other parts of the body. There are two main categories: DYT1 generalized dystonia, which is caused by a specific mutation in the DYT1 gene; and non-DYT1 generalized dystonia, which does not have the gene mutation.
  • Dopa-responsive dystonia is a form of dystonia in which the brain does not produce enough dopamine and is usually characterized by a progressive worsening of dystonia throughout the day. This form of dystonia responds to the medication levodopa, which relieves the symptoms.
  • Myoclonic dystonia is a genetic form of dystonia that affects the central part of the body, including the shoulders, arms, neck and trunk. It is characterized by rapid jerking movements in combination with the sustained muscular contractions and postures of dystonia.
  • Paroxysmal dyskinesias are episodic dystonias in which abnormal movements are only present during an actual attack with normal functionality between spells. This form of dystonia might be precipitated by movement (kinesigenic) or prolonged exercise (exercise induced). It can also occur spontaneously (nonkinesigenic).
  • X-linked dystonia-parkinsonism is a genetic form of dystonia and exhibits symptoms of both dystonia and slowness of movement as seen in Parkinson’s disease. It is one of the few degenerative forms of dystonia and primarily affects males of Filipino descent.
  • Rapid-onset dystonia-parkinsonism is a hereditary form of dystonia characterized by the abrupt onset of slowness of movement and dystonic symptoms.
  • Secondary dystonia is dystonia that develops as the result of other neurological conditions, certain medications or injury. Secondary dystonias may be produced by more than 50 neurological and metabolic diseases, many of which may be genetic.
  • Psychogenic dystonia refers to the uncommon situation where the dystonia is secondary to psychological disturbances. In the majority of cases, the process is subconscious, and the patient may not be aware of causing the abnormal movements.
What are the symptoms of dystonia?

Early symptoms of dystonia may include deterioration in handwriting, foot cramps or a dragging foot after running or walking a significant distance. Other possible symptoms include tremor and voice or speech difficulties. About half of the cases of dystonia have no connection to disease or injury and are called primary or idiopathic dystonia. Of the primary dystonias, many cases appear to be inherited. Dystonias can also be symptoms of other diseases, some of which may be hereditary. In some individuals, symptoms of dystonia appear in childhood. For others, symptoms may not appear until later in life.

Dystonias often progress through various stages. Initially dystonic movements are intermittent and appear only during voluntary movements or stress. Later, individuals may show dystonic postures and movements while walking or even while relaxed. Dystonic motions may lead to permanent physical deformities by causing the muscles or tendons to shorten.

What causes dystonia?

Dystonia typically occurs as a result of mutated genes, trauma, infection, stroke and certain medications. While an exact cause has yet to be determined, investigators believe that dystonia results from an abnormality in the area of the brain called the basal ganglia, where some of the messages that initiate muscle contractions are processed. Physicians and scientists continue to research this disorder to determine what exactly happens in the body that triggers the symptoms.

How is dystonia diagnosed?

At this time, there is no single test to confirm the diagnosis of dystonia. Instead, the diagnosis is determined by an analysis of the patient’s symptoms and a thorough review of his or her medical history by an experienced clinician. Videotaping either at home or in the clinic with an expert reviewing the movements is very important. In certain instances, tests may be ordered to rule out other associated conditions or disorders.

What treatment options are available for dystonia?

The medication response in children is marginally better than in adults. For primary dystonia with normal brain MRI studies, deep brain stimulation of the deeper part of the basal ganglia has been proven to be extremely beneficial when no other treatments work. Cleveland Clinic’s Center for Neurological Restoration has a dedicated team of experts with vast experience in deep brain stimulation for various neurological and psychiatric disorders including dystonia.

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Essential Tremor

A team of experts including renowned neurologists, neurosurgeons, researchers and a host of support personnel offers the latest proven treatments for people with movement disorders, including Parkinson’s disease, essential tremor and dystonia. Our surgical team is world-renowned for performing deep brain stimulation (DBS) surgeries and has experience with more than 1,200 DBS implants. The group also has expertise in the surgical management of spasticity. Various medication clinical trials as well as gene therapy surgical trial programs are under way.

What is essential tremor?

Essential tremor is a neurological disorder characterized by uncontrollable shaking, or "tremors," in different parts of the body. Areas affected often include the hands, arms, head, tongue, chin and other areas.

Essential tremor is not a life-threatening disorder. Mild cases which do not interfere with everyday activities like eating, dressing or writing often do not need to be treated. For more severe cases, both medications and surgical treatments are available.

What are the symptoms of essential tremor?

The primary symptoms associated with essential tremor include:

  • Uncontrollable shaking ("tremor")
  • Shaking voice
  • Shaking head
  • Shaking hands or arms
  • Rarely, shaking trunk or legs
  • Tremors that worsen during periods of emotional stress
  • Tremors that get worse with purposeful movement

The uncontrollable shaking associated with essential tremor is not unique to this condition. Many different factors or diseases can also cause tremors, including fatigue, emotional stress, Parkinson's disease, multiple sclerosis, caffeine, certain medications and alcohol or drug withdrawal.

What are the treatment options for essential tremor?

Mild essential tremor may not require treatment. People with essential tremor should avoid caffeine, moderate their use of alcohol and discontinue use of medications that aggravate the condition - but only in consultation with the doctor who prescribed the medication.

However, if essential tremor is interfering with your ability to function or work or is causing severe social embarrassment, there are treatments that may improve symptoms. While there is no cure for essential tremor at present, oral medications can significantly reduce the severity of the tremor. Medications include Propanolol (Inderal), Primidone (Mysoline), Clonazepam (Klonopin), Topiramate (Topomax) and Gabapentin (Neurontin). If your tremor is not controlled with one medication, it might be more beneficial to take two drugs together.

If other medications fail, your health care provider may recommend injections of very small doses of highly purified botulinum toxin (Botox or Myoblock) into the muscles. Repeat injections may be needed. This treatment has been effective for vocal and head tremors.

Deep Brain Stimulation (DBS) is a surgical treatment option for those individuals who have severe tremor despite medical therapy. This involves surgical implantation of electrical leads into the thalamus. This is an area deep within the brain that coordinates muscle control that is thought to be affected in essential tremor.

Resources and organizations for the support of Essential Tremors.

Huntington's Disease

Huntington’s Disease (HD) is a disease that affects the brain and causes unsteady and uncontrollable movements in the hands, feet, and face, and mental illness symptoms. People who have HD develop abnormal movements that eventually affect such activities as walking, talking, and swallowing.

HD is an inherited disease; a parent passes it to his or her child.

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Myoclonus

Also known as Opsoclonus myoclonus, is a rare neurological disorder characterized by an unsteady, trembling gait, myoclonus (brief, shock-like muscle spasms), and opsoclonus (irregular, rapid eye movements). Other symptoms may include difficulty speaking, poorly articulated speech, or an inability to speak. A decrease in muscle tone, lethargy, irritability, and malaise (a vague feeling of bodily discomfort) may also be present. Opsoclonus myoclonus may occur in association with tumors or viral infections. It is often seen in children with tumors.

Parkinson's Disease

A team of experts including renowned neurologists, neurosurgeons, researchers and a host of support personnel offers the latest proven treatments for people with movement disorders, including Parkinson’s disease, essential tremor and dystonia. Our surgical team is world-renowned for performing deep brain stimulation (DBS) surgeries and has experience with more than 1,200 DBS implants. The group also has expertise in the surgical management of spasticity. Various medication clinical trials as well as gene therapy surgical trial programs are under way.

What is Parkinson’s disease?

Parkinson’s disease is a chronic, progressive neurological disease that affects a small area of nerve cells deep within the brain. These cells normally produce dopamine, a chemical that transmits signals between areas in the brain. Parkinson’s disease causes these cells to die, leading to a lack of dopamine in the brain. It is this loss of dopamine that causes nerve cells to fire out of control, leading to tremor, stiffness and loss of movement control.

Approximately 1.5 million Americans have Parkinson’s disease, including one out of every 100 people over the age of 60. More than 50,000 Americans are diagnosed with Parkinson’s disease each year.

What are the symptoms of Parkinson’s disease?

Common symptoms of Parkinson’s disease include tremor or a shaking that begins in a hand, leg or the jaw. In some cases this tremor will be confined to only one body part or side of the body. However, it may spread as the disease progresses and it can worsen with stress. Tremor rarely disables a person and often disappears during sleep and when the arm or leg is being moved.

Bradykinesia is another common symptom that includes generalized slowness of movement. Common activities such as getting dressed or bathing may take a person with Parkinson’s much longer to complete. Most people with Parkinson’s disease develop some degree of rigidity, or stiffness of limbs. This rigidity is caused by uncontrolled tensing of muscles and results in the person being unable to move about freely. People with Parkinson’s also may experience muscle aches or pains.

Balance and coordination problems are additional symptoms of Parkinson’s disease. People typically develop a forward or backward lean that makes them more likely to fall when bumped. Additionally, people with Parkinson’s often develop a posture in which the head is bowed and shoulders are slumped. Other symptoms include depression, feelings of fear and anxiety, decreased facial expressions, difficulty in swallowing and chewing, speech changes, cramped handwriting, urinary problems or constipation, skin problems, such as dandruff, and sleeping problems.

It is important to note that the symptoms of Parkinson’s disease vary among people, sometimes making it hard to diagnose. In fact, as many as 40 percent of people are not diagnosed and 25 percent are misdiagnosed.

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Understanding Parkinson’s Disease

Hubert Fernandez, MD, Cleveland Clinic Movement Disorders Specialist

 
Restless Legs Syndrome

Restless Legs Syndrome (RLS) is a sleep disorder that causes an intense, often irresistible urge to move your legs, often accompanied by other sensations in the legs such as tingling, pulling, creeping, or pain. This sensation is brought on by lying down in bed or by sitting for prolonged periods of time, such as while driving or at a theater. RLS typically occurs in the evening, making it difficult to fall asleep. Often, people with RLS want to walk around and shake their legs to help relieve the uncomfortable sensation.

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Tourette Syndrome

Tourette Syndrome is an abnormal neurological condition characterized by motor and vocal tics. Tics are involuntary, rapid, sudden repetitive movements.

There are a wide variety of different tics.

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Chronic Pain

For more than a decade, our neurosurgical surgeons have provided surgical management of chronic pain conditions including failed back surgery syndrome, RSD, CRPS, facial pain, stroke pain, peripheral nerve cluster headaches and migraines and other chronic pain disorders. Various surgical procedures including lesioning and the implantation of intrathecal infusion pumps, spinal cord stimulators, peripheral nerve stimulators, cranial nerve stimulators and brain stimulators are performed for patients.

Chronic pain is pain that persists after the resolution of the acute injury. There is no longer any ongoing tissue injury. In chronic pain, signals of pain remain active in the nervous system for weeks, months, or years.

Chronic pain can lead to a chronic stress reaction involving an increase in blood pressure and heart rate. This stress reaction can contribute to adverse health effects such as lowered immunity and an increased risk for conditions such as heart disease. Chronic pain’s physical effects include tense muscles, limited mobility, a lack of energy, and changes in appetite. Emotional effects include depression, anger, anxiety, and fear of re-injury. Such a fear might hinder a person’s ability to return to normal work or leisure activities.

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Rehabilitation & Sports Therapy
Depression

The Center for Neurological Restoration’s team of neurosurgeons, psychiatrists and psychologists have been involved in studies using DBS for treating obsessive-compulsive disorder and major depression for the past seven years with promising outcomes. Patients with disabling OCD and depression are being actively enrolled in these studies. The center also performs vagal nerve stimulation and electroconvulsive therapy for treatment refractory depression.

What is depression?

Nearly everyone has felt depressed, sad, or blue at one time or another. A depressed mood is a normal reaction to loss, life's struggles, or injured self-esteem. Sometimes, however, depression becomes intense, lasts for long periods, and prevents a person from leading a normal life.

More than 10 million Americans suffer from major depressive disorder each year, but most never get treatment. When left untreated, depression can become worse, last for years, and cause untold suffering.

If you suffer from depression, it is important to remember that depression is a medical disorder that can be treated successfully.

What are the treatment options for depression?

The most common treatment for depression includes the combination of antidepressant medicine and psychotherapy.

Psychotherapy is used to treat mild and moderate forms of depression. A licensed mental health professional helps people with depression by focusing on behaviors, emotions and ideas that contribute to depression. They also help the depressed person identify and understand life problems that contribute to their illness in order to enable them to regain a sense of control. Psychotherapy can be done on an individual or group basis and can include family members and spouses. It is most often the first line of treatment for depression.

Medicines are commonly used to treat depression. Your family doctor or a psychiatrist can prescribe the medications, which are chosen based on your symptoms. The cost of medicines and potential side effects are important considerations when choosing this type of treatment for depression.

Electroconvulsive therapy (ECT) is a procedure in which an electric current is used to produce a seizure in the patient. It is believed that ECT results in the release of chemicals in the brain that aid communication between nerves. It is one of the fastest and most effective ways to relieve symptoms in severely depressed or suicidal patients who are suffering from mania. ECT is used when severe depression is unresponsive to other forms of therapy. It also is used when medication is considered unsafe. ECT may only be temporary and therefore requires continued psychotherapy and medication under a psychiatrist’s care. On occasion, ECT is prescribed on a regular basis (monthly to quarterly) to prevent further episodes of depression.

Vagal Nerve Stimulation (VNS) has been FDA-approved for use as a treatment for depression. For many years it has been used to treat refractory epilepsy. VNS involves a simple surgical procedure in which a pacemaker-like device is implanted in the chest. Connected to the device is a wire, which is run through the neck into the large vagus nerve. Through the lead, the device sends electricity to the nerve, which relays information to and from the brain. Through these electrical impulses, the symptoms of depression may be relieved.

Alternative treatments

Alternative treatments can sometimes provide relief that traditional western medicine cannot. While some alternative treatments have become accepted as part of modern health care practice, others still have not been proven safe and effective.

Experimental treatments

Investigation is currently being done regarding the use of deep brain stimulation (DBS) to treat depression. During a DBS surgery, electrodes are implanted within the brain to deliver electrical impulses. In the case of depression, it blocks the signals within the brain that cause the symptoms of these disorders. The success of DBS surgery is directly related to finding the specific area in the brain for stimulation. The stimulation can be adjusted as a patient’s condition changes over time.

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Obsessive-Compulsive Disorder

The Center for Neurological Restoration’s team of neurosurgeons, psychiatrists and psychologists have been involved in studies using DBS for treating obsessive-compulsive disorder and major depression for the past seven years with promising outcomes. Patients with disabling OCD and depression are being actively enrolled in these studies. The center also performs vagal nerve stimulation and electroconvulsive therapy for treatment refractory depression.

What is obsessive-compulsive disorder?

Obsessive-compulsive disorder (OCD), one of the anxiety disorders, is a potentially disabling illness that traps people in endless cycles of repetitive thoughts and behaviors. People with OCD are plagued by recurring and distressing thoughts, fears or images (obsessions) they cannot control. The anxiety (nervousness) produced by these thoughts leads to an urgent need to perform certain rituals or routines (compulsions). The compulsive rituals are performed in an attempt to prevent the obsessive thoughts or make them go away; not performing the rituals increases the anxiety.

Although the ritual might make the anxiety go away temporarily, the person must perform the ritual again when the obsessive thoughts return. This OCD cycle can progress to the point of taking up hours of the person’s day and significantly interfering with normal activities. People with OCD might be aware that their obsessions and compulsions are senseless or unrealistic, but they cannot stop themselves.

What are the treatment options for obsessive-compulsive disorder?

OCD will not go away by itself, so it is important to seek treatment. The person might be referred to a psychiatrist or psychologist, health care professionals who are specially trained to diagnose and treat mental illnesses.

The most effective approach to treating OCD combines medications with cognitive-behavior therapy.

  • Cognitive-behavior therapy — This approach teaches people with OCD to confront their fears and reduce anxiety without performing the ritual behaviors. It also focuses on reducing the exaggerated or catastrophic thinking that often occurs in people with OCD
  • Medication therapy — Selective serotonin reuptake inhibitors (SSRIs) work by increasing and sustaining the levels of serotonin in the brain, thus improving the movement of electrical signals from neuron to neuron. The SSRIs approved in the United States for the treatment of OCD include fluoxetine (Prozac), fluvoxamine (Luvox), Paroxetine (Paxil), sertraline (Zoloft) and citalopram (Celexa). Another antidepressant medication, clomipramine (Anafranil), also is approved for treating OCD.

In severe cases of OCD and in people who do not respond to treatment, surgery might be used to treat OCD.

Investigation is currently being done regarding the use of deep brain stimulation (DBS) to treat OCD. During a DBS surgery, electrodes are implanted within the brain to deliver electrical impulses. In the case of OCD, it blocks the signals within the brain that cause the symptoms of these disorders. The success of DBS surgery is directly related to finding the specific area in the brain for stimulation. The stimulation can be adjusted as a patient’s condition changes over time.

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Spasticity

Muscle movements are normally controlled by a complex system that allows some muscles to contract (tighten) while others relax. Damage to nerves in the central nervous system can disrupt the pattern. As a result, many muscles may contract all at once. This condition can be found in cerebral palsy, traumatic brain injury, stroke, spinal cord injury, multiple sclerosis, and other conditions that affect the brain and/or spinal cord. Spasticity mostly occurs in the legs but can occur in the arms.

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