Dyskinetic cerebral palsy: a population‐based study of children born between 1991 and 1998

Abstract

The aim of this study was to describe the epidemiology, aetiology, and clinical findings in dyskinetic cerebral palsy (CP)in a population-based follow-up study of children born between 1991 and 1998. Age range at ascertainment was 4 to 8 years and prevalence was 0.27 per 1000 live-births. Fortyeight children were examined (27 males, 21 females; mean age 9y, range 5-13y). Thirty-nine had dystonic CP and nine a choreo-athetotic subtype. Primitive reflexes were present in 43 children and spasticity in 33. Gross Motor Function Classification System levels were: Level IV, n=10 and Level V, n=28. The rate of learning disability (n=35) and epilepsy (n=30) increased with the severity of the motor disability. Thirty-eight children had anarthria. Peri-or neonatal adverse events had been present in 34 of 42 children born at ≥ ≥34 weeks' gestation. Motor impairment was most severe in this group. Placental abruption or uterine rupture had occurred in 8 participants and 19 of the 42 near-term/term children required assisted ventilation, compared with 1% and 12% respectively in other CP types. Neuroimaging in 39 children born at ≥ ≥34 weeks revealed isolated, late third trimester lesions in 24 and a combination of early and late third trimester lesions in seven. Dyskinetic CP is the dominant type of CP found in term-born, appropriate-for-gestational-age children with severe impairments who have frequently experienced adverse perinatal events.Cerebral palsy (CP) is the most common cause of motor disability in children and affects about 2 per 1000 live-births. 1,2 Spastic CP is by far the most common, constituting 80 to 85% of most cases, while 15% of the children in the latest Swedish study had dyskinetic CP. 1 In that study, the overall prevalence of CP had decreased, but the prevalence of dyskinetic CP had not. Motor disability in dyskinetic CP is often severe and is characterized by changes in muscle tone and posture, with a varying element of involuntary movement. Primitive reflexes persist and spasticity is often present, but not as a dominating feature. Accompanying impairments such as learning disability* and epilepsy are frequent. 3 Anarthria and dysarthria are also common, necessitating alternative communication aids. Growth is often hampered due to feeding difficulties and enhanced energy costs. Kyllerman et al. described 116 children with dyskinetic CP born between 1959 and 1970. 4 In 70% of the children, dystonic features dominated, while 30% were predominantly choreoathetotic. The main aetiological factors were found to be perinatal in two-thirds of the children and prenatal in about one in five. Modern magnetic resonance imaging (MRI) has improved the ability to visualize and date brain lesions in CP, which in dyskinetic CP are typically located in the basal ganglia. [5][6][7] Adverse events in the peri-or neonatal period or late gestation in the term or near-term infant may cause injury to the basal ganglia, which are vulnerable due to high metabolic demands at this stage of development. 9 Kernicterus is known t...

Deep brain stimulation for the treatment of childhood dystonic cerebral palsy

“…[13][14][15] Dystonia is more prevalent than the choreoathetotic subtype 19 and commonly manifests as dystonic hyper tonia of the upper extremities, neck, and face with concomitant * The Activa RC neurostimulator system was used in all cases. Lead location was calculated in reference to the midpoint of the anterior commissureposterior commissure (AC-PC) line.…”

“…These areas are particularly vulnerable given the high metabolic demands of the peri-and neonatal developmental periods. 5,13,14 In a study to investigate the MRI correlates of CP, the European Cerebral Palsy Study found that basal ganglia and thalamus damage was associated with dystonic CP in 75.6% (34 of 45 cases) of the study cohort. 5 The posteroventrolateral globus pallidus internus (GPi) has become a well-established target for deep brain stimulation (DBS) in both primary and secondary dystonia.…”

Correlation between temporal-spatial parameters and manual ability of adults with dyskinetic cerebral palsy

“…It is important to consider the interference of involuntary movements, which is a characteristic of the disease, on the variability of performance, due to the inability to exclude the unwanted components from the movement trajectory 1 . Therefore, motor behaviors that do not follow an execution pattern for the same task may result in interference in the variables of velocity an time of execution; these are compared with the MACS score, and they may reflect a poor relation.…”

“…The dyskinetic cerebral palsy (DCP) is characterized by the presence of involuntary, uncontrolled and recurring movements, which are occasionally stereotyped, representing one of the most incapacitating forms of the disease due to severe motor compromise 1 . The involuntary movement can lead to discomfort, interfere in voluntary movements, limit and even prevent upper limb (UULL) functions, which results in worse performance of activities of daily living (ADL), such as clothing, diet and personal hygiene, once the reach, the prehension and the manipulation are damaged 2 .…”

Clinico-anatomical correlations in asphyxiated babies who developed spastic quadriparesis at one year of age

“…There were associated recurrent chest infections, feeding problems due to pseudobulbar palsy and refractory seizures. The child also developed choreoathetoid movements with dystonia possibly due to deep nuclear variety of neuronal necrosis involving the Thalamoputaminalregion 4 . Thus he exhibited Mixed spastic dyskinetic features.…”