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Developing fetal or infant brain: the lesion occurs before, at, or within 2 years of birth
Associated impairments:
Sensation: vision, hearing and other sensory modalities may be affected
Cognition: both global and specific cognitive processes may be affected,
including attention
Communication: expressive and/or receptive communication and/or social
interaction skills may be affected
Perception: the capacity to incorporate and interpret sensory and/ or cognitive
information may be impaired
Behaviour: behavioural problems in the context of psychiatric disorders, such as
features of autism, ADHD, mood disorders and anxiety disorders may be
included
Seizure disorder: every seizure type and many epileptic syndromes may be seen
in CP
II. Incidence
Overall, the rate of CP is between 2 and 3 per 1000 live births. This rate increases to 40100 per 1000 live births among babies born very early or with very low birth weight.
(Cans 2000) During the past 20 years, there have been increases in the incidence and
prevalence of cerebral palsy that may be related to improved documentation of cases by
national registries, advances in neonatal care and other factors. (Koman et al. 2004)
III. Etiology
Cerebral Palsy is not caused by a uniform neurological abnormality. Risk factors are
frequently categorized according to the developmental period in which they are
considered to exert their etiological effect, leading to the categorization of etiology as
pre-, peri or postnatal.
Prenatal causes include congenital brain malformation, infection, exposure to
teratogens (e.g. tobacco, alcohol,), intrauterine vascular accident.
Perinatal
preterm
incompatibility.
Postnatal causes include infection and trauma, which are responsible for most of
the cases of postnatal/acquired CP, but there are many other possible causes like
cerbrovascular accident e.g. after cardiac surgery/ problems
uncontrolled,
recurring,
occasionally
stereotyped
(Cans 2000)
(http://www-rheop.ujf-grenoble.fr/scpe2/site_scpe/index.php)
Figure 1 shows the classification tree for the different subtypes of Cerebral Palsy
according to the SCPE.
Figure 1. Classification tree (SCPE, 2000)
C2. The Manual Ability Classification System (MACS) is designed to classify how
children with CP use their hands when handling objects in daily activities. The MACS is
not an assessment of each hand separately, but looks for the childrens manual ability to
handle objects in daily life. That means those activities that are relevant and age
appropriate for the child including for example: eating, dressing, playing, writing,
(Eliasson et al. 2006)
Figure 3 gives the MACS level description and explanation of the 5 different levels.
D. Classification according to the causation and the timing of the brain injury
The human brain undergoes complex organizational changes during development, in
and ex utero. Pathogenic events affecting the developing brain can cause abnormalities
or lesions. The pattern of those abnormalities or lesions depends on the stage of brain
development (Krgeloh-Mann et al. 2007). CP thus results from an injury in the
developing central nervous system, which can occur in utero, during delivery, or during
the first years of life.
Lesions can be divided in four main categories:
1. Malformations develop during the first and second trimester of gestation and include
predominantly cortical maldevelopments in bilateral distribution such as schizencephaly
(abnormal slits, or clefts, in the cerebral hemispheres), lissencephaly (literally smooth
brain, lack of development of brain folds (gyri) and grooves (sulci)) or polymicrogyria
(excessive number of small convolutions (gyri) on the surface of the brain).
2. Periventricular lesions (PVL-group) occur during the late second and early third
trimester and comprise periventricular venous infarction and periventricular leukomalacia
(white-matter brain injury, characterized by the necrosis of white matter near the lateral
ventricles)
3. Congenital cortical-subcortical lesions (CSC-group) occur in the late third trimester
or around birth and involve lesions of the gray matter structures, e.g. cortico-subcortical
infarctions, lesions of the basal ganglia and/ or thalamus.
4. Acquired brain lesions (ACQ-group) reflect all non-progressive brain lesions
occurring after the first 28 days of life and before the age of two or three, e.g. as a result
of trauma or infectious diseases.
(Steinlin et al. 1993; Cioni et al. 1999; Truwit et al. 2000)
co-contractions
b. Dyskinesia
Dyskinetic CP occurs in around 15% of the children with cerebral palsy (Odding et
al. 2006). Dyskinesia may include dystonia and/ or choreo-athetosis. Often, both
clinical phenomena are simultaneously present.
Dystonia is characterised by involuntary sustained or intermittent muscle
contractions causing twisting and repetitive movements, abnormal postures or both
(Sanger et al. 2003). It is dominated by abnormal postures, which may give the
impression of hypokinesia and tone fluctuation with easily tone increase.
Choreo-athetosis is characterised by hyperkinesia (e.g. increased activity)
and hypotonia (SCPE 2000). Choreo-athetosis can in turn be subdivided into
chorea and athetosis.
Chorea is characterised by rapid, involuntary, jerky, often fragmented
movements. Chorea is a ongoing random-appearing sequence of one or
more discrete involuntary movements or movement fragments.
axial hypotonia
asymmetric postures
c. Ataxia
Ataxia is a feature of central nervous system motor dysfunction, particularly
cerebellar abnormality, and is present in around 4% of people with cerebral palsy
(Bax et al. 2006).
Ataxia is defined as an inability to activate the correct pattern of muscles during
movement (Sanger et al. 2006), resulting in an abnormal trajectory of movement
and inaccurate limb placement.
Characteristics of children with ataxia that are observed are
-
tremor
visual problems
d. Hypotonia
Hypotonia is more common in young children, and particularly in children with
quadriplegic CP. The prevalence of this form is relatively low (1% according Blair
and Stanly 1997). More recent reports indicate that pure hypotonia is not a feature
of cerebral palsy (Krgeloh-Mann et al. 2009). Like those with hypertonia, children
with hypotonia have a reduced ability to respond to movement demands.
Characteristics of children with hypotonia
-
Stability or balance
Children with CP also experience problems with their equilibrium or balance and
frequently show impaired trunk control, which can affect performances of activities of
daily life such a sitting, reaching and walking (Hadders-Algra & Brogren 2008; Prosser et
al. 2010; Heyrman et al. 2011).
The problems in the adaptation of the degree of muscle contraction might be the reason
that children with CP, more often than typically developing children, show an excess of
antagonistic co-activation during difficult balancing tasks (Van der Heide et al. 2005).
A2. Secondary problems = orthopaedic problems
The abnormal muscle function (increased muscle tone, muscle inactivity) and abnormal
load or non-loading increase the risk to develop secondary orthopedic problems such as
contractures and bony deformities especially during growth.
A deformity can be defined as a structural stand deviation or lever arm dysfunction.
Orthopedic problems occur at all levels including spine, lower limbs and upper limbs.
a. Spinal deformities
Deformities of the spine such as scoliosis, kyphosis and lordosis are associated
with CP. Scoliosis can lead to serious outcomes, while kyphosis and lordosis are
not associated with significant comorbidity. (Renshaw et al. 1995)
In CP, scoliosis is neuromuscular and originating from an upper motor neuron
lesion. Patients with neuromuscular scoliosis may have a variety of scoliotic curve
types depending on the severity of CP involvement. Scoliosis is most common in
children with quadriplegia. Most curves are progressive; the progression of
scoliosis is highest during the adolescent growth spurt, especially when associated
with pelvic obliquity. (Jones et al. 2010)
Scoliosis shows a high prevalence of medical comorbidities in children with CP
like,
respiratory
problems
problems,
gastro-intestinal
disorders,
other
orthopaedic
Figure 5. Images of a 16-year-old girl with CP and moderate mental retardation. The
posteroanterior radiograph (A) and photographs (B, C) show that the patient had a 95,
long, sweeping thoracolumbar curve and severe lordosis. She was malnourished and
experienced intractable vomiting. An upper gastrointestinal series revealed a superior
mesentery artery syndrome related to the scoliosis. A percutaneous jejunostomy tube was
placed to hyperaliment her before the spine surgery. However, prior to surgery the patient
died of bronchoaspiration. (Jones et al. 2010)
Knee
- Flexion contractures: shortening of the hamstrings
- Rotational deformities of the tibia: external rotation (common in
spastic diplegia) (Figure 7)
Figure 7. Spastic diplegia with external rotation of the tibia and hindfoot valgus
(Fabry et al. 2002)
Hips
- Contractures:
- flexion/ adduction
- abduction
- abduction and adduction contracture= wind-swept (Figure 8)
The flexion/adduction contractures are most common.
- Rotational deformities: internal rotation: increased femoral anteversion
(normal
Figure 10. Hip dislocation of the right hip (Pountney et al. 2006)
Elbow flexion
Forearm pronation
Wrist flexion
Finger flexion
- Deformities:
- Joint instability
(Koman et al. 1990; Goldner et al. 1990; Goldner et al. 1988; House et al. 1981)
Figure 11. Thumb-in-palm deformity (House et al. 1981)
B. Activity
At the activity level, limitations will be described for gross motor function, gait and upper
limb function.
GMFCS
II
II
IV
32%
29%
8%
15%
16%
The bar chart below (Figure 13) illustrates the relation between the GMFCS and the
different subtypes of CP
Figure 13. Relation between GMFCS and subtypes of CP (Himmelmann et al. 2006)
The distribution of the children over the 5 GMFCS-levels in fig. 13 differed significantly
between the different CP types (p<0.01).
The main findings for the different subtypes of CP are:
Spastische diplegie: 54% level I-II; with most (38%) at level II (38%) and 31%
at levels IV-V
Rosenbaum et al. 2002 made a prediction of the gross motor developmental patterns in
children with cerebral palsy according to the severity by longitudinal observations of 657
children with CP, using the GMFCS (for severity) and the GMFM (for functionality). They
created motor development curves based on these two parameters. Fig. 14 shows the
predicted average development in gross motor function measured by the GMFM
according to the 5 levels of the GMFCS.
The curved solid lines indicate average performance of the children classified in a
specific GMFCS-level according to their age.
Children in GMFCS-level 1 reached the highest scores on the GMFM, while overall
children in level 5 scored low on the GMFM.
Figure 14 suggests a trend for faster progression to the limit as severity of impairment
increases.
Figure 14. The predicted average development by the Gross Motor Function Classification
System Levels (Rosenbaum et al. 2002)
predict
A: Item 21: assesses whether a child can lift and maintain his/her head in a vertical
position with trunk support by a therapist while sitting
B: Item 24: assesses whether when in a sitting position on a mat, a child can
maintain sitting unsupported by his/ her arms for 3 seconds
C: Item 69: measures a childs ability to walk forward 10 steps unsupported
D: Item 87: assesses the task of walking down 4 steps alternating feet with arms
free
B2. Gait
Wren et al. studied gait abnormalities in 492 children (age 3-24 years old) with CP. The
most common gait abnormalities were stiff knee in swing (80%), crouch (69%),
excessive hip flexion (65%), intoeing gait (64%) and equinus (61%). The prevalence is
illustrated for the total group and for the different topographies in figure 15.
Explanation:
Stiff knee in swing: Decreased arc of knee motion from maximum knee
extension in stance to peak knee flexion in swing, and/ or delay in peak swing
knee flexion to mid- or terminal swing, hindering foot clearance.
Crouch: Knee flexion > 1 SD above the mean for normal in a significant portion
of stance phase.
Intoeing gait: Internal foot progression > 1 SD more than mean for normal.
Equinus (61%): Ankle plantair flexion > 1 SD below the mean for normal
during stance phase, with or without hindfood and/ or forefoot varus or valgus.
Calcaneus
Ankle varus
Ankle valgus
Recurvatum
Scissoring
Out-toeing
Rotational malalignement
Figure 15. Prevalence of gait abnormalities in CP patients referred for gait analysis
(Wren et al. 2005)
In general, the typical gait pattern in children with diplegia is characterised by:
Lumbal lordosis
MACS
II
II
IV
14%
38%
17%
17%
14%
The children with hemiplegia were mainly seen in level II, but also in levels I and III.
Children with diplegia and ataxia were found in levels I to V, whereas children with
dyskinetic CP and tetra- or quadriplegia were apparent in levels IV and V.
In this study, the relationship between the MACS and GMFCS was also investigated
(Figure 17).
Figure 17. Distribution of the children with CP between the levels of the Manual Ability
Classification System (MACS) and the Gross Motor Function Classification System
(GMFCS) (Eliasson et al. 2006)
The correlation between GMFCS and MACS was 0.79 (p< 0.05), with complete
agreement in 49%. A large variation occurred at MACS level II, where 40% of the
children were scored and where the agreement between GMFCS and MACS was just
35%. Differences were also observed in other levels.
Unilateral spastic CP accounts for 38% of children with CP and is the most common type
of CP among children born at term and second to bilateral spastic CP among preterm
infants (Sellier et al. 2010). In children with unilateral CP, the upper limb is usually more
involved than the lower limb and the extent to which they can use their hands is critical
to their overall development. Consequently this target group is most investigated.
Children with unilateral cerebral palsy often present upper limb impairments including
increased muscle tone; impaired sensation; reduced strength and range of motion;
decreased speed (Brown et al. 1987), which results in difficulties in unimanual function
such as controlling grasp. (Eliasson et al. 1995; Sakzewski et al. 2010). Many children
with hemiplegia also present with deficits in bimanual coordination, which is problematic
as most daily activities involve the coordinated use of both hands (Sakzewski et al.
2010). Independence in these tasks is achieved using adaptive strategies to
compensate for poor bimanual skills. (Arner et al. 2008) Adaptive strategies may include
learning to do activities exclusively with the unimpaired hand, despite having reasonable
unimanual capacity of the impaired arm. This is called a developmental non-use.
C. Participation:
Impairments and limitations in activities influence the participation level such as in
school, sports or other social activities in the community.
Vision
Overall visual impairments like strabismus, hemianopsia are presented in 62-71% of
the children with CP. Severe visual impairments are reported in 10-12% of the children
(Reid et al. 2005; Odding et al. 2006; Krgeloh-Mann et al. 2009).
Epilepsy
Epilepsy occurs in up to a third of children with CP. Seizure types vary and many
individuals will have more than one type (Carlsson et al. 2003).
Cognitive impairments
Cognitive impairments are common although the reported incidence varies between
studies because of differing definitions.
The SCPE reports frequencies ranging from 23-44% with any learning disability (IQ<70),
and 30 to 41% with severe disabilities (IQ<50). (SCPE, 2000).
Hearing impairment
Hearing impairment is much less common, occurring in less than 8% of the children with
CP (Reid et al. 2005; Odding et al. 2006; Surman et al. 2006).
Feeding problems
Gastrointestinal as well as feeding problems are frequently present in children with CP.
Sucking and swallowing problems the first 12 months of life are common: 57% and 38%
respectively. In 60% of the children severe feeding problems even precede the diagnosis
of CP (Odding et al. 2006).
by fig. 18. In children with motor function at GMFCS level 1, 79% had no accompanying
impairment, contrasting with 6% of those at GMFCS level 5. In children with GMFCS
level 5, 89% had two or more accompanying impairments.
Figure 18. Proportion of learning disability, epilepsy and severe visual impairments by
GMFCS levels in 353 children with CP. (Himmelmann et al. 2006)