Athetosis
Athetosis | |
---|---|
Bilateral athetosis | |
Specialty | Neurology |
Athetosis is a symptom characterized by slow, involuntary, convoluted, writhing movements of the fingers, hands, toes, and feet and in some cases, arms, legs, neck and tongue.[1] Movements typical of athetosis are sometimes called athetoid movements. Lesions to the brain are most often the direct cause of the symptoms, particularly to the
Signs and symptoms
Athetosis can vary from mild to severe motor dysfunction; it is generally characterized by unbalanced, involuntary movements of muscle and a difficulty maintaining a symmetrical posture. The associated motor dysfunction can be restricted to a part of the body or present throughout the body, depending on the individual and the severity of the symptom. One of the pronounced signs can be observed in the extremities in particular, as the writhing, convoluted movement of the digits.
Causes
Athetosis is a symptom primarily caused by the marbling, or degeneration of the basal ganglia.[citation needed] This degeneration is most commonly caused by complications at birth or by Huntington's disease, in addition to rare cases in which the damage may also arise later in life due to stroke or trauma.[citation needed] The two complications of particular interest are intranatal asphyxia and neonatal jaundice.
Asphyxia
Asphyxia directly causes basal ganglia damage due to lack of oxygen and therefore, insufficient nutrient supply.[
Neonatal jaundice
Neonatal jaundice is the other chief complication that leads to the basal ganglia damage associated with this condition. Jaundice is caused by hyperbilirubinemia, or abnormally high levels of bilirubin in the blood. Bilirubin is usually bound to albumin immediately and sent to the liver. However, in neonatal jaundice, the concentration of bilirubin overwhelms that of albumin and some of the bilirubin remains unconjugated and can enter the brain through the blood–brain barrier.[6] Normally bilirubin would not be able to diffuse across the blood–brain barrier, but in infants, the barrier is immature and has higher permeability. Bilirubin is toxic as it prevents the phosphorylation of many proteins, including synapsin I which binds vesicles in the presynaptic terminal.[7] Therefore, it directly inhibits the exocytosis of neurotransmitters and severely hinders the synapses it affects. In autopsies of children who suffered from neonatal jaundice, chronic changes of neuronal loss, gliosis and demyelination were observed in the basal ganglia and more specifically within the globus pallidus.[6]
Thalamic stroke
Another study was done where the onset of athetoid movement followed a thalamic stroke.[citation needed] The thalamus is part of a pathway that is involved with the cortical feedback loop in which signals from the cortex are relayed through the striatum, pallidus and thalamus before making it back to the cortex.[8] The striatum receives excitatory inputs from the cortex and inhibits the pallidum. By doing so it frees the thalamus from pallidal inhibition allowing the thalamus to send excitatory outputs to the cortex. Therefore, the lesions to the thalamus or any other part of this feedback loop can result in movement disorders as they can alter the reactivity of one towards the other.[8] Also, in a case of people with thalamic stroke, a majority suffered severe sensory deficits and ataxia. It is proposed that this loss of proprioception and the ensuing loss of synergic stabilization may also lead to abnormal movements, such as those dealt with in athetosis.[8]
Fahr's syndrome
Treatments
There are several different treatment approaches to dealing with athetosis. The most common methods are the use of drugs, surgical intervention, and retraining movements of the afflicted person. It is suggested that training a person to relearn movements can be helpful in select situations. Though, generally, this type of treatment will not work, in certain cases it can be found to be very helpful in treating the symptom of athetosis.[9]
Drugs can also be used in the treatment of athetosis, however their collective effectiveness is not very convincing.[10] There is not a single drug that is a standard among treatment. Many different medicines can be used, including:
- trihexyphenidyl
- benzatropine[11]
- Curare,[9] though not practical due to respiratory paralysis
- Tetrabenazine
- Haloperidol
- Thiopropazate
- Diazepam
Most instances of drug use where the symptoms seem to be lessened tend to be in more mild cases of athetosis.[10]
Treatment by surgical intervention can obviously have the most immediate impact, again however, it is not a cure-all. In patients that have cerebral palsy as the cause of their athetosis, it has been demonstrated that a
Related disorders
Choreoathetosis
Cerebral palsy
Athetosis is a commonly occurring symptom in the disease cerebral palsy.[14] Of all people with the disease, between 16%[15] and 25%[4] of them actually exhibit the symptom of athetosis. A component of this is the finding that most often the symptoms that involve athetosis occur as a part of choreoathetosis as opposed to athetosis alone.[16]
It is also noteworthy that the presence of athetosis in cerebral palsy (as well as other conditions) causes a significant increase in a person's basal resting metabolic rate. It has been observed that those who have cerebral palsy with athetosis require approximately 500 more Calories per day than their non-cerebral palsy non-athetoid counterpart.[15]
Pseudoathetosis
Social implications
Athetosis is characterized as a symptom that is present in many forms of central nervous system disorders that affect the body movement coordination, such as cerebral palsy. Children may struggle to engage in social communication, since the poor coordination of the tongue and mouth muscles can reduce their speech ability and hinder their social interaction to a greater degree.[19] The caregivers of the affected children are encouraged to closely monitor their nutrition and growth and to provide them with hearing aids in order to relieve their symptoms as well as support their academic plans.[20] A growing number of patients is shown to benefit from communication devices such as shorthand typing programs and computer speech devices, as well as simple picture boards.[19]
Patients living with the disorder into their adulthood often have trouble being involved in daily activities such as eating, walking, dressing, as well as performing everyday tasks. They are consistently faced with challenges that limit their ability to live on their own. They are more reluctant to be involved in social activities and romantic relationships and more likely to develop poor self-esteem and self-image related to their physical limitations as well as cognitive disabilities, though such habitual thinking is shown to decline when they feel they are accepted and supported by their peers.[21] Patients are also inclined to associate themselves with people who tend not to be engaged in physical activities, according to the September 2008 issue of "Journal of Physical Activity and Health".[22]
History
The first noted case of athetosis was discovered by
Research directions
As athetosis is relatively difficult to treat, efforts are being made to help those with the condition live and perform tasks more effectively and more efficiently. One such example of work that has been recently undertaken is a project to help those affected with athetosis to use a computer with more ease. Software for the control of the computer uses joysticks that perform linear filtering to aid in control.[24]
An additional possible treatment option for those afflicted with the symptom is neurostimulation. Studies have begun, and in cerebral palsy patients affected with dystonia-choreoathetosis, it has been demonstrated that neurostimulation has been an effective treatment in lessening symptoms in patients. There has not been a tremendous amount of experimentation, though, in this as a possible treatment option.[25]
See also
- Chorea
- Dyskinesia
- Dystonia
- Pupillary athetosis
References
- ^ PMID 21250235.
- ^ a b "Athetosis". Health Database - Medical Ailments & Diseases. Retrieved March 22, 2011.
- ^ ISBN 978-1-58890-514-7.
- ^ PMID 6341510.
- S2CID 42453096.
- ^ PMID 7611048.
- PMID 11803417.
- ^ PMID 19276093.
- ^ PMID 21433835.
- ^ PMID 1203674.
- ^ Polzin, Scott J., MS, and Teresa G. Odle. "Cerebral palsy." Gale Encyclopedia of Medicine. Thomson Gale, 2006. NA. Health Reference Center Academic. Gale.
- PMID 4918461.
- PMID 15356354.
- PMID 21096307.
- ^ PMID 8557940.
- PMID 18981805.
- PMID 21047218.
- S2CID 32393176.
- ^ a b Jeff Brody (2005). "Social Problems with Cerebral Palsy". Cerebral Palsy Lawyer - Birth Injury Attorney. Cerebral Palsy Source. Retrieved March 21, 2011.
- PMID 18981805.
- ^ Lehrman, Mary (10 May 2010). "The Social, Emotional, & Psychological Effects Of Adults With Cerebral Palsy". Demand Media, Inc. Retrieved March 22, 2011.
- PMID 18209260.
- ^ S2CID 13359301.
- .
- S2CID 24345609.