Dyskinesia
Dyskinesia
Definition
Dyskinesias are a group of disorders characterized by involuntary movements of muscles.
Description
Dyskinesias are excessive abnormal movements that are involuntary. There are several different types of dyskinesias, and each has different clinical symptoms, causes, and treatments. Adults and children with certain chronic brain disorders often exhibit symptoms of dyskinesia. Movement can occur in the head, arms, legs, hand, feet, lips, or tongue. The dyskinesias can be categorized as chorea , dystonia , myoclonus , tremor, and paroxysmal tardive (late-onset type). Other forms of dyskinesia include athetosis, ballism, akathisia, tics, stereotypy, and restless legs. Dyskinesias can also be called hyperkinesia syndromes.
Chorea
Choreas are abnormal movements that are irregular, involuntary, nonrhymical, abrupt, rapid, and nonsustained jerking, which continuously flow from one body part to another. Movements are isolated, brief, and infrequent. Chorea can cause inability to maintain a sustained contraction, which causes affected persons to drop objects. Persons with chorea have an irregular dance-like gait. The cause of chorea is not completely understood.
Dystonia
Dystonia that occurs at rest may persist as the kinetic (clonic) form. Dystonias can be either focal or generalized. Focal dystonias are involuntary movements in a single body part, which commonly includes blepharospasm (upper facial), spasmodic torticollis (cervical), and writer's cramp. Dystonia affecting two or more body regions is called segmental dystonia. Generalized dystonia typically affects the trunk, one or both legs, and another body part. Other types of dystonias include Merge's syndrome (spasms of the jaw muscles when opening and closing of the mouth). Spasmodic dystonias can cause speech impairment due to spasms of laryngeal (throat) muscles. The intensity of muscular movements in patients with dystonia can fluctuate, and symptoms worsen during fatigue , stress, activity, and change in posture. In some cases, the bizarre symptoms of dystonia can be mistaken for psychological illness. Dystonias can be inherited or acquired due to another primary cause. Inherited diseases that exhibit dystonia are rare and include dopa-responsive dystonia, idiopathic tension dystonia, and x-linked dystonia-Parkinsonism (found among Ashkenazi Jews).
Myoclonus
Myoclonus refers to muscular contractions (positive myoclonus) that are brief, sudden, and severe, and shock-like movements or inhibitions (negative myoclonus). Myoclonus could be generalized or isolated. The movements consist of rhythmical irregular jerks or oscillatory jerks that occur abruptly and then fade. The abnormal jerks are associated with environmental stimuli such as light, sound, movement, and visual threat. The condition can be misdiagnosed for epilepsy . Myoclonus usually occurs at rest, but can also appear when the affected body part is subjected to voluntary activity, which is referred to as action myoclonus. Action myoclonus is more disabling than rest myoclonus.
Tremor
Tremors are rhythmic oscillatory movements that are regular, but may vary in rate, location, amplitude, and constancy, and depend on type and severity of the tremor. Tremors can occur with action, at rest, and with holding a position or posture. The tremor can be so rapid it is often described as a "flicker of light." Subtypes of tremors include tremors at rest, essential tremor, which is a postural tremor at either rest or activity and may be inherited, or tremor with movement (intention "kinetic" tremor). Resting tremors are usually slow, occur during an activity, and disappear when action is initiated (e.g., Parkinson's disease ). Essential tremor is usually benign, but can cause disability due to impairment of handwriting and limitations of activities related to daily living. Essential tremor may be inherited.
Paroxysmal dyskinesias
Paroxysmal dyskinesia is a group of disorders that includes paroxysmal kinesigenic dyskinesia, episodic ataxia , paroxysmal hypnogenic dyskinesia, paroxysmal exertion-induced dyskinesia, and paroxysmal non-kinesigenic dyskinesia. The paroxysmal dyskinesias are a hyperkinetic disorder characterized by intermittent involuntary movements consisting of symptoms from other movement disorders such as chorea, athetosis, dystonia, and ballismus. Episodes of paroxysmal dyskinesias can last from a few seconds to several days. Episodic ataxias are characterized by intermittent episodes of ataxia that can last seconds to hours. Paroxysmal dyskinesias may be triggered by prolonged exertion, sleep, stress, alcohol, coffee, tea, fatigue, sudden voluntary movement, heat, or cold.
Athetosis
Athetosis is a disorder characterized by movements that are continuous, slow, and writhing. The movements are commonly appendicular and frequently involve muscles in the face, neck, and tongue. The condition may occur at rest or when executing voluntary movement. The speed of movements in affected persons can sometimes increase and symptoms are similar to those of chorea (called choreoathetosis). Athetosis movements can blend with those of dystonia, if the muscular contractions are sustained and cause abnormal posturing.
Ballism
Ballismus are large choreic movements that are fast and usually affect the limbs. Affected individuals exhibit flinging and flailing movements. Commonly, ballismus affects one side of the body (unilateral), producing a condition called hemiballismus.
Akathisia
Akathisia refers to complex movements such as tics, compulsions, and mannerisms that are stereotypic and usually relieved when executing a motor act. Typically, when sitting, the akathitic persons may exhibit movements that include symptoms such as crossing and uncrossing the legs, squirming, pacing, stroking the scalp, or rocking the body. Patients may have burning sensations on the specific affected body part, and they may vocalize a continual moaning and groaning.
Tics
Tics can be divided into two disorders: motor tics (abnormal movements) and/or vocal tics (abnormal sounds). Children can present with a chronic disorder of both motor and vocal tics (Gilles de la Tourette syndrome ). Movements of simple tics may be very similar to a choreic or myoclonic jerk (abrupt, single, sudden, isolated). Complex tics are movements that are distinctly coordinated patterns of sequential movements, but they may not be identical from occurrence to occurrence and they can occur in different body areas. Tics are rapid movements and, if contractions are sustained in affected body parts, they resemble dystonic movements.
One of the major clinical signs that help distinguish tics from other dyskinesias is the presence of involuntary ocular (eye) movement in persons affected with tics. The ocular manifestations of tics can include a brief jerk of the eyes or a sustained eye deviation. Two other dyskinesias, myoclonus and dystonia, can present with involuntary ocular manifestations.
With vocal tics, affected persons can exhibit grunts, throat-clearing sounds, or even the utterance of obscenities (coprolalia). Phonic tics (involving nasal and vocal muscles) can be divided into simple phonic tics such as throat-clearing or sniffing or complex phonic tics that include bark-like noises and verbalizations.
Stereotypies
Sterotypies are movements that are frequent and may last for minutes. These movements are repetitive and identical (continuous stereotypy.) The bizarre movements associated with mental retardation , autism , and schizophrenia are stereotypies. Continuous stereotypy is characteristic of another type of dyskinesia called tardive dyskinesia, which results from treatment with neuroleptic and antipsychotic medications.
Tardive dyskinesia
Tardive (late-onset) dyskinesia refers to a group of movement disorders that are characterized by hyperkinetic involuntary movements, consisting of mixed manifestations of orofacial dyskinesia, chorea, tics, and/or athetosis. Abnormal movement can affect muscles in the lips, face, trunk, tongue, and extremities, which can interfere with eating and dexterity. The most characteristic symptom of tardive dyskinesia is orofacial dyskinesia, which usually starts with slow, mild tongue movements followed by exaggerated movements of lips and tongue. Affected individuals can have symptoms that may progress to chewing movements, blinking, bulging cheeks, grimacing, arching eyebrows, and blepharospasms.
Tardive dyskinesias are commonly seen in patients taking certain medications such as neuroleptics and antipsychotic medication that are prescribed for schizophrenia, schizoaffective disorder, or bipolar disorder. Other types of tardive dyskinesias include tardive akathisia, tardive dystonia, tardive myoclonus, tardive Tourettism, tardive tremor, and blepharospasm. Approximately 50% of patients taking dopamine receptor blocker medication will develop a form of tardive dyskinesia.
Tardive akathisia refers tapping, squirming, and marching movements that are repetitive. Movements associated with tardive dystonia can include a fixed posturing of face and neck, trunk, and extremities. Persons affected with tardive myoclonus, which is a rare disorder, exhibit brief jerky movements of muscles in the face, neck, trunk, arms, and legs. Symptoms of tardive Tourettism usually begins in persons older than 21 years of age and include frequent, multiple tics that are both vocal and motor. This disorder should not be confused with Tourette syndrome, which commonly presents by seven years of age.
Tardive tremors often present as involuntary rhythmical, wave-like, and persistent movements of the head, neck, limbs, or voice. Tardive tremors are present both at rest and during voluntary movement.
Early myoclonic encephalopathy
Early myoclonic encephalopathy is a rare disorder, in which the incidence is approximately one in 40,000 children. It is characterized by brief and abrupt myoclonic jerks (common occurrence in 90% of patients) and seizures . The onset of symptoms usually occurs within the first three years of life. Treatment and management depends on the underlying cause of seizures. Typically, patients receive antiepileptic medications, and improvement of symptoms is usually associated with a good prognosis. If symptoms do not improve with antiepileptic medication(s), the prognosis is not favorable.
Resources
BOOKS
Goetz, Christopher G., et al. (eds). Textbook of Clinical Neurology. 1st ed. Philadelphia: W.B. Saunders Company, 1999.
Goldman, Lee, et al. Cecil's Textbook of Medicine. 21st ed. Philadelphia: W.B. Saunders Company, 2000.
Noble, John, et al, (eds). Textbook of Primary Care Medicine. 3rd ed. St. Louis: Mosby, Inc., 2001.
PERIODICALS
Brasic, James R. "Tardive Dyskinesia." eMedicine Series (December 2003).
Jankovic, J., and M. Demirkiran. "Paroxysmal Dyskinesias: An Update." Annals Medical Science 10 (2001).
Jenner, Peter. "Avoidance of Dyskinesia: Preclinical Evidence for Continuous Dopaminergic Stimulation." Neurology 62:1 (January 2004).
WEBSITES
Gardos, G., and J. O. Cole. The Treatment of Tardive Dyskinesias. (May 20, 2004). <http://www.acnp.org/g4/GN401000145/CH142.html>.
ORGANIZATIONS
American College of Neuropsychopharmacology. 320 Centre Building 2014 Broadway, Nashville, TN 37203. (615) 322-2075; Fax: (615) 343-0662. [email protected].
Laith Farid Gulli, MD
Nicole Mallory, MS, PA-C