Dyskinetic cerebral palsy

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Dyskinetic cerebral palsy
Other namesAthetoid cerebral palsy
Basal ganglia.png
The basal ganglia plays essential roles in voluntary motor function. Various forms of damage to the basal ganglia can cause a range of movement disorders.
Symptoms Dystonia, choreoathetosis
Usual onsetBirth
DurationLifelong
Causes Perinatal asphyxia, neonatal shock, hyperbilirubinaemia
TreatmentSupportive

Dyskinetic cerebral palsy (DCP), also known as athetoid cerebral palsy or ADCP, is a subtype of cerebral palsy that is characterized by dystonia, choreoathetosis, and impaired control of voluntary movement. [1] [2] Unlike spastic or ataxic cerebral palsies, dyskinetic cerebral palsy is characterized by both hypertonia and hypotonia, due to the affected individual's inability to control muscle tone. [3] [4] Clinical diagnosis of ADCP typically occurs within 18 months of birth and is primarily based upon motor function and neuroimaging techniques. [5] [6] While there are no cures for ADCP, some drug therapies as well as speech therapy, occupational therapy, and physical therapy have shown capacity for treating the symptoms.

Contents

Presentation

In dyskinetic cerebral palsy (DCP), both motor and non-motor impairments are present. Impaired regulation of muscle tone, lack of muscle control, and bone deformations are often more severe compared to the other subtypes of cerebral palsy (CP). [7] Severity of non-motor impairments is generally correlated with motor symptom severity.

The primary motor signs of DCP are dystonia and choreoathetosis. Dystonia is defined by twisting and repetitive movements, abnormal postures due to sustained muscle contractions, and hypertonia. Dystonia is aggravated by voluntary movements and postures, or with stress, emotion or pain. [8] [9] Choreoathetosis is characterized by hyperkinesia (chorea i.e. rapid involuntary, jerky, often fragmented movements) and hypokinesia (athetosis i.e. slower, constantly changing, writhing or contorting movements). [10] [11] Dystonia and choreoathetosis often occur concurrently in DCP. [11] Both increase with activity and are generalized over all body regions with a higher severity in the upper limbs than in the lower limbs. Dystonia has a significantly higher level of severity in the distal parts of the extremities, whereas choreoathetosis is more equally distributed. [2] Severity of dystonia but not choreoathetosis is correlated with higher levels of functional disability on the Gross Motor Function Classification System (GMFCS). [12] [2]

Approximately half of the individuals with DCP have severe learning disabilities. Epilepsy, visual impairments, and hearing impairments are reported in 51%, 45%, and 11% of individuals with DCP, respectively. Dysarthria or anarthria are also common. [7] [13]

Causes

Dyskinetic cerebral palsy could have multiple causes. The majority of the children are born at term and experience perinatal adverse events which can be supported by neuroimaging. Possible causes are perinatal hypoxic-ischaemia and neonatal shock in children born at term or near term. Hyperbilirubinaemia used to be a common contributing factor, [14] but is now rare in high-income countries due to preventive actions. Other aetiological factors are growth retardation, [15] brain maldevelopment, intracranial haemorrhage, stroke or cerebral infections. [7]

Diagnosis

Cerebral palsies have historically been diagnosed based on parental reporting of developmental motor delays such as failure to sit upright, reach for objects, crawl, stand, or walk at the appropriate age. [5] Diagnosis of ADCP is also based on clinical assessment used in conjunction with milestone reporting. [4] The majority of ADCP assessments now use the Gross Motor Function Classification System (GMFCS) or the International Classification of Functioning, Disability and Health (formerly the International Classification of Impairments Disease, and Handicaps), measures of motor impairment that are effective in assessing severe CP. [16] [4] The Barry-Albright Dystonia Rating Scale (BADS), the Burke-Fahn-Marsden Dystonia Rating Scale (BFMS) and the Dyskinesia Impairment Scale (DIS) are also commonly used to categorize clinical qualitative assessments of dyskinesia in DCP. [17] [18] [19]

Multiple classification systems using magnetic resonance imaging (MRI) have been developed, linking brain lesions to time of birth, cerebral palsy subtype and functional ability. [20] [21] [22] [23] Around 70% of patients with DCP show lesions in the cortical and deep grey matter of the brain, more specifically in the basal ganglia and thalamus. However, other brain lesions and even normal-appearing MRI findings can occur, for example white matter lesions and brain maldevelopments. [2] [22] [24] [25] Patients with pure basal ganglia and thalamus lesions are more likely to show more severe choreoathetosis whereas dystonia may be associated with other brain lesions, such as the cerebellum. [2] These lesions occur mostly during the peri- and postnatal period since these regions have a high vulnerability during the late third trimester of the pregnancy. [26] Unfortunately, contemporary imaging is not sophisticated enough to detect all subtle brain deformities and network disorders in dystonia. Research with more refined imaging techniques including diffusion tensor imaging and functional MRI is required. [9] [27]

Prevention

Prevention strategies have been developed for the different risk factors of the specific cerebral palsy subtypes. Primary prevention consists of reducing the possible risk factors. However, when multiple risk factors cluster together, prevention is much more difficult. Secondary preventions may be more appropriate at that time, e.g. prevention of prematurity. Studies showed a reduced risk of cerebral palsy when administering magnesium sulfate to women at risk of preterm delivery. [28] [29] Cooling or therapeutic hypothermia for 72 hours immediately after birth has a significant clinical effect on reducing mortality and severity of neurodevelopmental disabilities in neonates with perinatal asphyxia. This has been documented for newborns with hypoxic-ischemic encephalopathy. [30] [31]

Management

Dyskinetic cerebral palsy is a non-progressive, irreversible disease. The current management is symptomatic, since there is no cure. The main goal is to improve daily activity, quality of life and autonomy of the children by creating a timed and targeted management. The many management options for patients with DCP are not appropriate as standalone treatment but must be seen within an individualized multidisciplinary rehabilitation program. Medical treatment consists of oral medication and surgery. Before using oral drugs, it is important to differentiate between spasticity, dystonia, and choreoathetosis since each motor disorder has a specific approach. In general, many oral drugs have low efficacy, unwanted side-effects and variable effects. [32]

Oral baclofen and trihexyphenidyl are commonly used to decrease dystonia, although its efficacy is relatively low in most patients. Adverse effects of the latter can include worsening of choreoathetosis. [9] Since dystonia predominates over choreoathetosis in most patients, reducing dystonia allows the possibility of a full expression of choreoathetosis. This suggests that the discrimination of dystonia and choreoathetosis is crucial, since misinterpretations in diagnosing can contribute to the administration of inappropriate medication, causing unwanted effects. [9] [33] Intrathecal baclofen pumps (ITB) are often used as an alternative to reduce side-effects of the oral dystonic medication over the whole body. [34] Botulinum toxin injections may be applied to decrease dystonia in specific muscles or muscle groups. [9] [35] [36]

Deep brain stimulation (DBS) in patients with DCP has shown to decrease dystonia. [37] [38] However, the responsiveness is less beneficial and the effects are more variable than in patients with inherited or primary dystonia. [39] The effects on choreoathetosis have not been investigated.

Orthopedic surgery is performed to correct musculoskeletal deformities, but it is recommended that all other alternatives are considered first. [9]

The previous management options need to be combined with rehabilitation programs, adapted to the specific needs of each individual. Typically, the team of caregivers can consist of physiotherapists, occupational therapists and speech/communication therapists. The therapy mainly focusses on the motor problems by using principles of neuroplasticity, patterning, postural balance, muscle strengthening and stretching. [35] Non-motor impairments such as epilepsy require specific treatment.

Prevalence

Dyskinetic cerebral palsy is the second most common subtype of cerebral palsy after spastic CP. A European Cerebral Palsy study reported a rate of 14.4% of patients with DCP [40] which is similar to the rate of 15% reported in Sweden. [41] The rate appeared lower in Australia, where data from states with full population-based ascertainment listed DCP as the predominant motor type in only 7% of the cases. [42] The differences reported from various registers and countries may relate to under-identification of dyskinetic CP due to a lack of standardization in definition and classification based on predominant type. [43] [10]

References

  1. ↑ Rosenbaum P, Paneth N, Leviton A, Goldstein M, Bax M, Damiano D, et al. (February 2007). "A report: the definition and classification of cerebral palsy April 2006". Developmental Medicine & Child Neurology. 49 (6): 8–14. doi: 10.1111/j.1469-8749.2007.tb12610.x .
  2. 1 2 3 4 5 Monbaliu E, de Cock P, Ortibus E, Heyrman L, Klingels K, Feys H (February 2016). "Clinical patterns of dystonia and choreoathetosis in participants with dyskinetic cerebral palsy". Developmental Medicine & Child Neurology. 58 (2): 138–144. doi: 10.1111/dmcn.12846 . PMID   26173923.
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  5. 1 2 "Athetoid Dyskinetic". Swope, Rodante P.A. Retrieved 31 October 2012.
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  20. ↑ Krägeloh-Mann I, Horber V (February 2007). "The role of magnetic resonance imaging in elucidating the pathogenesis of cerebral palsy: a systematic review". Developmental Medicine & Child Neurology. 49 (2): 144–151. doi:10.1111/j.1469-8749.2007.00144.x. PMID   17254004.
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  42. ↑ Report of the Australian Cerebral Palsy Register Birth years 1995-2012 (Report). The Australian Cerebral Palsy Register Group. November 2018.
  43. ↑ Cans C (February 2007). "Surveillance of cerebral palsy in Europe: a collaboration of cerebral palsy surveys and registers". Developmental Medicine & Child Neurology. 42 (12): 816–824. doi: 10.1111/j.1469-8749.2000.tb00695.x .
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