EXTRAPYRAMIDAL DISORDER

Extrapyramidal disorder : defects in basal ganglia function; characterized by changes in muscle tone, poverty of voluntary movements (akinesia), or abnormal involuntary movements (dyskinesia).

More specific terms for Extrapyramidal disorder:

Chorea Dystonia Myoclonus Parkinsons disease Tardive Dyskinesia

Chorea
Definitions
Chorea is a neurological disorder characterized by involuntary spasmodic movements of the body. Movements are jerky and arrhythmic, and may involve the whole body.

Etiology

Huntington's disease Sydenham's chorea Drug-induced chorea Autoimmune disease Rheumatic fever Wilson's disease

Pregnancy Polycythemia SLE Hyperthyroidism Hypoparathyroidism Certain medications Oral contraceptives

Symptoms of Chorea

Jerky spasms Whole body spasms Leg spasms Arm spasms Facial spasms Twitching Facial twitch

Leg twitch Arm twitch Writhing spasm movements Muscle weakness Clumsiness Gait disturbances

Misdiagnosis of Chorea Alternative diagnoses list for Chorea:

Muscle twitches Tics Muscle spasms Seizures

Treatments for Chorea

Chorea may be difficult to treat. The mainstay of treatment in adults is the class of neuroleptics, including haloperidol and pimozide. These drugs selectively enhance the function of the indirect pathway by blocking the inhibitory effect of dopamine on this pathway. However, the incidence of side effects in children from neuroleptics has been reported to be as high as 20%. Therefore, it is often safer to start with an alternative medication, such as a benzodiazepine, particularly clonazepam, diazepam, or clobazam. According to reports, certain anti-epileptic medications, such as valproate and piracetam, may improve the symptoms of chorea. In particular, valproate may be very helpful in children with Sydenham's chorea.

Dystonia
Definitions Dystonia is involuntary, slow, repetitive, sustained muscle contractions that may cause freezing in the middle of an action, as well as twisting or turning of the trunk, the entire body, or part of the body.

Causes of Dystonia
Over activity in several areas of the brainthe basal ganglia, thalamus, cerebellum, and cerebral cortexseems to cause dystonia. Causes of dystonia include a severe lack of oxygen to the brain that occurs at birth or later in life, Parkinson's disease, multiple sclerosis, toxicity due to accumulation of certain metals (such as copper in Wilson's disease), and stroke. Antipsychotic drugs can cause various types of dystonia, including blepharospasm, spasmodic torticollis, tardive dyskinesia. Chronic dystonia usually has a genetic cause.

Types and Symptoms of Dystonia

Idiopathic torsion dystonia refers to dystonia that has no known cause. Episodes begin between the ages of 6 and 12. Early symptoms can be mild or severe. Muscles contract slowly and abnormally, causing twisting and turning. The dystonia commonly starts in one foot or leg. It may remain limited to the trunk or a leg, but sometimes it affects the whole body, ultimately confining the child to a wheelchair. When idiopathic torsion dystonia develops in adults, it usually begins in the face or arms and usually does not progress to other parts of the body.

Blepharospasm is a type of dystonia in which the eyelids are repeatedly and involuntarily forced shut. Occasionally, only one eye is affected at first, but ultimately, the other eye is also affected. It usually begins as excessive blinking, eye irritation, or extreme sensitivity to bright light. Spasmodic torticollis is dystonia involving the muscles of the neck.

Treatment
Correcting or eliminating the cause of dystonia, if known, usually reduces the dystonia. If the cause of dystonia is unknown, treatment is limited. Some people, especially children who have a hereditary form of dystonia (called dopa-responsive dystonia), improve dramatically when treated with levodopa. Benzodiazepines, a type of mild sedative, may be used. Baclofen, a muscle relaxant, may be given by mouth or by a pump implanted in the spinal canal. Anticholinergic drugs, such as trihexyphenidyl and diphenhydramine, are sometimes helpful, but they also cause side effects, such as drowsiness, dry mouth, blurred vision, dizziness, constipation, difficulty urinating, and tremor, especially in older people. The antipsychotics clozapine and olanzapine may be useful.

SPASMODIC TORTICOLLIS

Spasmodic torticollis (cervical dystonia) is a disorder characterized by painful intermittent or continuous contractions or spasms of the neck muscles, forcing the head to rotate or tilt forward, backward, or sideways. The disorder can occur at any age but usually develops between the ages of 25 and 55. Usually, the cause is unknown. Dysfunction within the basal ganglia (collections of nerve cells located at the base of the cerebrum, deep within the brain) may be the cause. Sometimes it is caused by injury to the neck muscles during pregnancy or during a difficult delivery. This type of spasmodic torticollis is called congenital torticollis. Imbalanced eye muscles and bone or muscle deformities of the upper spine can cause torticollis in children.

Symptoms and Diagnosis

Initially, symptoms may be mild, but they may become severe. They include involuntary turning of the head, muscle pains, and slight tremor of the neck muscles. Usually, only one side of the neck is affected. Sharp, painful neck muscle spasms may start suddenly and occur intermittently or continuously. Imaging procedures, such as x-rays, CT, and MRI, are sometimes used to look for specific causes of neck muscle spasms, although such causes are not commonly identified.

Treatment and Prognosis When a cause, such as bone or muscle deformities, is identified, torticollis can usually be treated successfully. However, when the cause is unknown, treatment is less likely to control the spasms. Sometimes the spasm can be temporarily relieved by physical and occupational therapy, which may include biofeedback, electrical stimulation, massage, cold packs, heat, and deep heat with ultrasonography.

Certain drugs help reduce muscle spasms and involuntary movements in about one third of adults with spasmodic torticollis. Commonly used are anticholinergic drugs (such as trihexyphenidyl and benztropine), which block specific nerve impulses, and benzodiazepines (particularly clonazepam), which are mild sedatives. Less frequently, muscle relaxants (such as baclofen) and antidepressants (such as amitriptyline) are used.

Myoclonus
Definitions

Myoclonus is a term that refers to brief, involuntary twitching of a muscle or a group of muscles. It describes a symptom and, generally, is not a diagnosis of a disease. Involuntary shock-like contractions, irregular in rhythm and amplitude, followed by relaxation, of a muscle or a group of muscles. This condition may be a feature of some central nervous system diseases (e.g., epilepsy, myoclonic). Nocturnal myoclonus may represent a normal physiologic event or occur as the principal feature of the nocturnal myoclonus syndrome.

Causes of Myoclonus

Infection Head injury Spinal cord injury Stroke Brain tumors Kidney failure Liver failure Lipid storage disease Chemical poisoning Drug poisoning Hypoxia - causing posthypoxic myoclonus. Other causes of symptoms seizures or spasms

Symptoms of Myoclonus

The myoclonic twitches or jerks are usually caused by sudden muscle contractions; they also can result from brief lapses of contraction. Myoclonic jerks may occur alone or in sequence, in a pattern or without pattern. They may occur infrequently or many times each minute. Muscle twitches - involuntary, with or without a pattern Muscle twitches when trying to move

Treatments for Myoclonus

Clonazepam Barbiturates Phenytoin Primidone Sodium valproate

Parkinson's diseases

Parkinson's disease is a movement disorder that mainly affects people after 50 years of age, but can present as juvenile Parkinson's that affects people before their 40's . Components in the brain involved with regulating movement are faulty due to a lack of neurotransmitter called dopamine. Deregulation of movement presents as a resting tremor, rigidity of arm movement, a shuffling gait and loss of facial expression. The disease is influenced by genes, age, and environment.

Causes
Parkinson's and related disorders are the result of the loss of dopamineproducing brain cells. Dopamine is a chemical messenger responsible for transmitting signals within the brain. Parkinson's disease occurs when certain nerve cells, or neurons, die or become impaired. Normally, these neurons produce dopamine. Loss of dopamine causes the nerve cells to fire out of control, leaving patients unable to direct or control their movement in a normal manner. Following is a list of underlying conditions that could possibly cause Parkinson's Disease includes: Encephalitis Brain tumor Traumatic brain injury Carbon monoxide poisoning Hydrocephalus Certain toxins or medications

Symptoms
The four primary symptoms of Parkinson's are tremor or trembling in hands, arms, legs, jaw, and face; rigidity or stiffness of the limbs and trunk; bradykinesia, or slowness of movement; and postural instability or impaired balance and coordination. Patients may also have difficulty walking, talking, or completing other simple tasks. The disease is both chronic and progressive. Parkinson's is not usually inherited. Early symptoms begins subtle and occur gradually.

Fixed facial expression Lack of blinking Tremor or trembling - various uncontrolled movements typically occurring in fingers, hands, arms, legs, jaw, and face

Tremor in one little finger - a common early symptom Hand tremor Tremor in one hand , in one leg, in one arm, in one side of the body and involuntary head nodding

Walking symptoms

Difficulty walking Stooping posture Loss of postural reflexes Rigid back Shuffling gait Postural instability Impaired balance and coordination Falling forwards Falling backwards

Rigidity - stiffness of the limbs and trunk Bradykinesia - slowness of movement Difficulty swallowing Speech changes

Difficulty talking Voice changes Softer voice Monotonous voice Stammering Fixed mask-like expression Tight facial muscles Facial muscle spasm

Facial symptoms

Intellectual symptoms - usually late in the progression of symptoms; most people suffer physical symptoms without early mental changes.

Treatments for Parkinson's Diseases

Levodopa is most effective in reducing tremor and muscle rigidity and in improving movement. Levodopa, taken by mouth, is converted to dopamine in the basal ganglia, thus compensating for the decrease in dopamine production. Levodopa is given with carbidopa. Carbidopa prevents levodopa from being converted to dopamine before it reaches the brain.

Levodopa-carbidopa is the mainstay of treatment for Parkinson's disease. Doctors must balance control of the disease with the development of certain side effects, these side effects include involuntary movements of the mouth, face, and limbs; nightmares; hallucinations; and changes in blood pressure. After taking levodopa for 5 or more years, more than half of the people begin to alternate rapidly between a good response to the drug and no responsean effect called the on-off phenomenon.

Dopamine agonists (pramipexole and ropinirole), which mimic the action of dopamine, may be useful at any stage of the disease. Selegiline, a type of antidepressant called MAOI prevents the breakdown of dopamine, thereby prolonging dopamine's action in the body. Tolcapone and entacapone also prevent the breakdown of dopamine and appear to be useful supplements to levodopa. Anticholinergic drugs (benztropine and trihexyphenidyl) effective in reducing the severity of a tremor and can be used in the early stages of Parkinson's disease. They can also be used in the later stages to supplement levodopa. Anticholinergic drugs may reduce tremor because they block the action of acetylcholine, and tremor is thought to be caused by an imbalance of acetylcholine (too much) and dopamine (too little). Other anticholinergic drugs, including some antihistamines and tricyclic antidepressants, are mildly effective and are used to supplement levodopa. Amantadine, a drug sometimes used to treat influenza, may be used alone to treat mild disease or as a supplement to levodopa. Propranolol, a beta-blocker, may be prescribed to reduce the severity of a tremor.

Tardive Dyskinesia
Tardive dyskinesia is a neurological syndrome caused by the long-term use of neuroleptic drugs. Neuroleptic drugs are generally prescribed for psychiatric disorders, as well as for some gastrointestinal and neurological disorders.

Symptoms of Tardive Dyskinesia

Tardive dyskinesia is characterized by repetitive, involuntary, purposeless movements. Features of the disorder may include grimacing, tongue protrusion, lip smacking, puckering and pursing, and rapid eye blinking. Rapid movements of the arms, legs, and trunk may also occur. Impaired movements of the fingers may appear as though the patient is playing an invisible guitar or piano.

Symptoms of Tardive Dyskinesia

Involuntary movements - repetitive and purposeless Jaw movements Chewing-like movements Facial grimacing Tongue movements Lip smacking Lip puckering Lip pursing Rapid eye blinking Rapid movements - of the arms, legs, and trunk.

Misdiagnosis of Medication Causes of Tardive Dyskinesia

Neuroleptic drugs Chlorpromazine Ormazine Chlorprothixine Taractan Thorazine Acetophenazine Maleate Tindal

Treatments for Tardive Dyskinesia

Discontinue neuroleptic drug Substituting another drug for the neuroleptic drug Benzodiazepines Adrenergic antagonists Dopamine agonists