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Descripción General de Cuatro Sistemas de Clasificación Funcional Comúnmente Utilizados en La Parálisis Cerebral

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16 views10 pages

Descripción General de Cuatro Sistemas de Clasificación Funcional Comúnmente Utilizados en La Parálisis Cerebral

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© © All Rights Reserved
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children

Review
Overview of Four Functional Classification Systems
Commonly Used in Cerebral Palsy
Andrea Paulson * and Jilda Vargus-Adams
Cincinnati Children’s Hospital Medical Center, Cincinnati, OH 45229, USA; [email protected]
* Correspondence: [email protected]; Tel.: +1-513-636-7480

Academic Editor: David Hall


Received: 5 March 2017; Accepted: 19 April 2017; Published: 24 April 2017

Abstract: Cerebral palsy (CP) is the most common physical disability in childhood. CP comprises
a heterogeneous group of disorders that can result in spasticity, dystonia, muscle contractures,
weakness and coordination difficulty that ultimately affects the ability to control movements.
Traditionally, CP has been classified using a combination of the motor type and the topographical
distribution, as well as subjective severity level. Imprecise terms such as these tell very little about
what a person is able to do functionally and can impair clear communication between providers.
More recently, classification systems have been created employing a simple ordinal grading system of
functional performance. These systems allow a more precise discussion between providers, as well as
better subject stratification for research. The goal of this review is to describe four common functional
classification systems for cerebral palsy: the Gross Motor Function Classification System (GMFCS),
the Manual Ability Classification System (MACS), the Communication Function Classification System
(CFCS), and the Eating and Drinking Ability Classification System (EDACS). These measures are all
standardized, reliable, and complementary to one another.

Keywords: cerebral palsy; classification; severity; function; GMFCS; MACS; CFCS; EDACS

1. Introduction
Cerebral palsy (CP) is the most common physical disability in childhood [1]. CP comprises
a heterogeneous group of disorders that are the result of a non-progressive disruption or injury that
occurred during fetal brain development or within the first two years of life [2]. This disruption
can result in spasticity, dystonia, muscle contractures, weakness and difficulty in coordination
that ultimately affects the ability to control movements [3]. Resultant activity limitations may
affect gross motor movements, fine motor movements, speech and communication, as well as
eating and drinking. Traditionally, CP has been classified using a combination of the motor type
and the topographical distribution. Motor types include terms like spastic, hypotonic, ataxic,
dyskinetic or mixed. The topographic classifications include the limbs that are affected, namely diplegia
(or diparesis), tri-, tetra-, quadri- or hemiplegia. CP severity is frequently described subjectively in
terms of mild, moderate or severe [4,5]. Imprecise terms such as these tell very little about what
a person is able to do functionally. Moreover, when a large range of ability and performance can be
encapsulated by the same term (such as moderate spastic quadriplegia CP), providers struggle to
accurately interpret communications from one another. In research, this can create subject groups that
are very heterogeneous.
Over the last 20 years, clinicians and researchers have created classification systems using
a simple ordinal grading system of functional capacity, allowing a more precise discussion between
providers. These classification systems use a common language to describe function. They do not
describe potential improvements or assess underlying etiology. This paper provides an overview

Children 2017, 4, 30; doi:10.3390/children4040030 www.mdpi.com/journal/children


Children 2017, 4, 30 2 of 10

of four common functional classification systems used in cerebral palsy: the Gross Motor Function
Classification System (GMFCS), the Manual Ability Classification System (MACS), the Communication
Function Classification System (CFCS), and the Eating and Drinking Ability Classification System
(EDACS). These measures are standardized, reliable, and complementary to one another.

2. Classification Measures

2.1. Gross Motor Function Classification System (GMFCS)


The GMFCS is the most established and recognized of the functional classification measures in
CP. The GMFCS is a simple, five-level, ordinal grading system created to describe the gross motor
function of an individual with CP. First described in 1997 by Palisano et al. [6], the GMFCS provides
a common language for a practitioner that is meaningful, quick and easy to use. The GMFCS describes
self-initiated movement and use of assistive devices (walkers, crutches, canes, wheelchairs) for mobility
during an individual’s usual activity. Following research to stratify typical motor function for children
with CP, the authors concluded that a five-level classification system worked well to discriminate
clinically meaningful distinctions in motor function. This classification system was initially designed
to be used with children 2–12 years of age [6,7]. The GMFCS was later expanded and revised in 2007
to include ages 12–18, as well as to increase descriptors and differentiations for the levels based on
the child’s age, while taking into account developmental milestones [8]. These more detailed and age
appropriate descriptors allow a more accurate classification. A pictorial representation can be found in
Figure 1, for ages six through 12.

Figure 1. Five Gross Motor Function Classification System (GMFCS) Expanded and Revised levels,
as depicted for children ages 6–12 years. Reproduced with permission [8].
Children 2017, 4, 30 3 of 10

Based on the GMFCS revised and expanded version, an individual classified in GMFCS I is able
to walk without limitations. Individuals less than two years of age are able to crawl on hands and
knees, pull to stand, cruise when holding onto furniture, and are able to attain independent walking
between the age of 18 months and two years. Between two and four years of age, skills include sitting
independently and transitioning between sitting and standing independently. Between age four and
six years, the individual in GMFCS I is able to walk indoors and outdoors independently, climb stairs,
and start to run and jump. Between the ages of six and twelve, additional abilities include walking
up and down curbs, walk community distances, negotiate stairs without railings, and run and jump
(which may include some limitations). Between age 12 to 18, the abilities are the same as age 6 to 12.
A child classified in GMFCS II can walk with limitations. Limitations may include balance or
endurance, use of a hand-held mobility device prior to age 4, use of a railing on stairs, or an inability
to run or jump. GMFCS II functioning may result in the use of wheeled mobility for long distances.
A child before the age of two can sit with upper extremity support, crawl on their stomach, and may
be able to pull-to-stand or cruise with support. Between ages 2 and 4, the child can transition into and
out of sitting without support, can sit without support (but may need to use their upper extremities
for balance), can crawl on hands and knees, cruise with support and walk with a mobility device.
Between the ages of four and six, the individual can transition into and out of standing without
support, walk short flat distances without an assistive device, do stairs with the railing, but is unable
to run or jump. From age 6 to 12, the individual can walk in most terrains but has limitations with
distance or uneven surfaces, may use wheeled mobility for long distances, can do stairs with the
railing, but is able to do minimal or no running and jumping. Between ages 12 and 18, the abilities are
the same as age 6 to 12, but a handheld mobility device may be used for safety.
A child classified in GMFCS III can often walk with a hand-held mobility device indoors, but use
wheeled mobility in the community and for longer distances. GMFCS III level functioning indicates
the ability to sit with little to no external support and to stand to complete transfers. Children in
GMFCS III who are less than 2 years old can roll and occasionally crawl forward when lying on
their stomach, as well as sit with some low back support. Between ages 2 and 4, a child can “W”
sit on the floor with some help getting into the position, crawl on his/her stomach or creep on all
fours, and may pull-to-stand and walk short distances using a handheld mobility device (walker or
gait trainer) with some assistance for maneuvering. From age 4 to 6, a child can sit in a standard
chair but may require extra support to allow full upper extremity function, walk with a handheld
mobility device and do stairs with assistance; typically, wheeled mobility is used for longer distances.
In GMFCS III, children aged 6 to 12 walk with a handheld mobility device indoors and use wheeled
mobility (either manual or powered) for distance, require assistance to move between floors, sitting and
standing, and negotiate stairs with assistance. For ages 12 to 18, the abilities are the same as age 6 to
12, but more variability is demonstrated in primary mobility preferences.
An individual classified in GMFCS IV can sit supported, but self-mobility is limited, often being
transported in a manual wheelchair or using powered mobility. Children before the age of 2 have head
control and can roll, but require truncal support to sit. Between ages 2 and 4, a child in GMFCS IV
can sit with upper extremity support, require assistance to transition into sitting, and may require
adaptive equipment for sitting or standing. At this age, some self-mobility is possible through rolling
or stomach crawling short distances, but reciprocal leg movement is not present. From age 4 to 6,
children require adaptive equipment for trunk control to allow sitting and assistance to move between
positions. Children may walk short distances with a mobility device and with assistance, and use
wheeled mobility for distances, and/or be independent with powered mobility. Children in GMFCS
IV and ages 6 to 12 require adapted seating and assistance with transfers, and utilize wheeled power
mobility independently or manual mobility with assistance in most settings. Many children can have
independent floor mobility with crawling or rolling, or may walk short distances with assistance. For
ages 12 to 18, the abilities are the same as ages 6 to 12.
Children 2017, 4, 30 4 of 10

Children classified in GMFCS V have more severe limitations with head and trunk control and
self-mobility is only possible using a power wheelchair. Children in GMFCS V before the age of 2 do
not have independent head or trunk control and require assistance to roll. Between ages 2 and 4, a child
has no independent movement and requires assistance for transport using manual mobility devices.
Adaptive equipment is required for sitting and standing, but function is still limited. It is possible
to become independent using power mobility with additional adaptations. From age 4 onwards,
the abilities of children in GMFCS V are stable with a need for complete assistance with transfers
emerging after age 6 [8].

2.1.1. Inter-Rater and Test-Retest Reliability


During the creation of the GMFCS, Palisano et al. found the inter-rater reliability to have
a moderate agreement with a kappa (k) of 0.55 in children <2 years of age and excellent agreement
with a kappa of 0.75 in children 2–12 years of age [6]. This strong inter-rater reliability supports the use
of the GMFCS as a classification of gross motor function in children ages 2–12. Therefore, the authors
concluded that classification under the age of 2 years is possible, but should be done with caution.
The expanded and revised GMFCS underwent validation testing using group consensus methods
and was found to be valid with >80% agreement of participants [9]. Additional studies assessing
the GMFCS inter-rater reliability have been completed. Bodkin et al. [10] compared two experienced
pediatric physical therapists when assessing a patient via videotape, and found the inter-rater reliability
was 0.84. The authors also reported that the GMFCS level assigned remained stable over the two years
of the study [10].
If the GMFCS is to be a useful classification system, it needs to be relatively stable over time,
as a child with CP changes with age and development. A retrospective review was completed in
2000, in order to assess the reliability and stability of the GMFCS over time, and found an inter-rater
reliability of 0.93 and a test-retest reliability of 0.79 [11]. The authors also report that the positive
predictive value of the GMFCS at 1–2 years of age, to predict walking by 12 years of age, was 0.74
and the negative predictive value was 0.90 [11]. Palisano et al. also reported excellent stability of
the GMFCS over time with a weighted kappa coefficient of 0.84 for children younger than 6 years
of age, and 0.89 for children older than 6 years [12]. Additionally, McCormick et al. reported the
stability of the GMFCS from age 12 into adulthood, with a weighted kappa of 0.895. The inter-rater
reliability was 0.978. The positive predictive value of the GMFCS at age 12 for a current wheelchair
user to remain a wheelchair user as an adult was high, with a weighted kappa of 0.96, and for a person
walking without mobility aid to remain without a mobility aid, kappa was 0.88 [13]. Morris et al. found
that family-reported GMFCS level was also a reliable method for measuring gross motor function in
children between the ages of 6–12 years [14]. Therefore, not only can medical providers accurately
classify children rapidly and accurately in clinical settings using the GMFCS, but parents and caregivers
are also effective at selecting the GMFCS for children.

2.2. Manual Ability Classification System (MACS)


The Manual Ability Classification System (MACS) was developed by Eliasson et al. in 2006,
specifically to be an upper extremity analogue of the GMFCS. The MACS is also a simple, five-point
ordinal classification system, analogous and complementary to the GMFCS, and was designed for
use in children ages 4–18 years. The MACS is a validated measure in cerebral palsy that can be used
to classify a child’s typical use of both hands and upper limbs; it is not meant to reflect best use or
individual hand function [15]. Figure 2 shows a portion of the MACS scale.
An individual classified as MACS I is able to handle objects easily with the possibility of
some limitations with very small, fragile or heavy objects, or limitations with fine motor control,
but these limitations do not restrict independence in daily activities. MACS II classification indicates
a decreased level of performance when handling objects; the performance may be slower and may be
impacted by asymmetric hand function. An individual may use alternative ways to handle objects,
Children 2017, 4, 30 5 of 10

but remains independent in daily activities. An individual in MACS III handles objects slowly and
often with limited success, requiring assistance or set-up for activities. Some activities can be completed
independently with appropriate adaptations and set up, but other activities cannot be adequately
performed without assistance. MACS IV functioning indicates a need for continuous support and
assistance or the use of adapted equipment to complete only a part of a daily activity, with an inability
to complete the full activity. Individuals in MACS V do not handle daily objects and may be able to
participate minimally with simple movements or may require total assistance.

Figure 2. Summary of the five Manual Ability Classification System (MACS) levels. Reproduced with
permission [15].

2.2.1. Intra-Class Correlation


During development of the MACS, it underwent reliability testing between professionals, as well
as between a professional and a parent. The MACS has an intra-class correlation coefficient (ICC)
between professionals of 0.97 and between a professional and parent of 0.96, indicating outstanding
reliability [15]. Morris et al. explored parent MACS ratings and also reported the MACS to have high
reliability, with ICC of 0.7–0.9, indicating agreement in classification between families and professionals.
The authors therefore concluded that the MACS is a reliable and valid classification method for manual
ability in children with CP [16].
In 2013, Ohrvall et al. completed a study that assessed the stability of the MACS over time
and found that the ICC was 0.97 and two ratings over a one-year interval had an 82% agreement.
The stability for two ratings performed at a 3–5-year interval had an ICC of 0.96 and an agreement
of 78%. When MACS was followed over four ratings, 70% of children remained at the same rating,
and the score was consistent for younger and older children, indicating that the rating stability is not
influenced by age [17].
Children 2017, 4, 30 6 of 10

2.2.2. Inter-Observer Reliability


In 2009, Plasschaert et al. published a study that assessed the inter-observer reliability of the
MACS when used in children ages 1–5 with cerebral palsy. They found the inter-observer reliability of
the MACS to have a linear weighted kappa (k) of 0.62. When assessing only children <2 years of age,
the k = 0.55, which is a moderate reliability, but when assessing children 2–5 years of age the k = 0.67,
demonstrating good reliability. The authors concluded that classification between 2–5 years of age is
possible, but that classification at age <2 should be done with caution, and recommended an additional
tool be created to better classify these young children [18].

2.3. Mini-MACS
The Mini-MACS was developed by Eliasson et al. in 2016 and is designed to classify children‘s
manual ability for those aged 4 years or younger. The Mini-MACS underwent reliability testing
between professionals, as well as between a professional and a parent. The Mini-MACS has an ICC
between professionals of 0.97, and between a professional and parent of 0.90, indicating good reliability
and validity. The wording of the Mini-MACS is very close to that of the MACS, with descriptions more
relevant for younger ages when compared to the MACS [19].
In Mini-MACS I, children can have slight limitations in handling objects with higher levels of
coordination, and may need some assistance compared to their peers. At Mini-MACS II, children have
more difficulty with everyday objects, require practice, often use an alternative approach, and require
additional assistance compared to peers. Children in Mini-MACS III can handle easy objects
independently for short periods of time but requires additional time and often assistance to support
the object. In comparison, Children in Mini-MACS IV can perform simple actions when handling
an object, for example grasping and releasing an item, but require constant support for handling.
Children in Mini-MACS V may be able to complete simple movements with constant support, for
example pushing a switch or button [19].

2.4. Communication Function Classification System (CFCS)


Thirty one to 88% of individuals with CP are estimated to have a concomitant communication
disorder [20,21]. The CFCS was developed by Hidecker et al. in 2011 and is a valid measure in cerebral
palsy that assesses everyday communication (not optimal communication) [22]. The CFCS is a simple,
five-point ordinal classification system, and was designed to be analogous and complementary
to the GMFCS and MACS. Being an effective communicator requires both sending and receiving
information, and the CFCS accordingly assesses both how information is expressed and how it
is received. The CFCS allows all methods of communication (e.g., vocalizations, manual signs,
eye gaze, pictures, communication boards, speech generating devices) to be included when assessing
an individual’s classification. In this way, the CFCS is inclusive and descriptive for individuals who do
not vocalize to communicate. The CFCS also takes into account familiar and unfamiliar communication
partners [22].
An individual who communicates at a CFCS level I is known as an “effective sender and receiver
with unfamiliar and familiar partners”. The individual is able to communicate at a comfortable pace,
send and receive information with familiar and unfamiliar partners, and any misunderstandings
are easily corrected. An individual who communicates at a CFCS level II remains an effective
communicator, but the pace of communication is slower. An individual at this level is known as
an “effective but slower paced sender and/or receiver with unfamiliar and/or familiar partners”.
The primary difference between a level I and level II communication functioning is the pace of the
communication; however, both are effective at sending and receiving information.
An individual who communicates at a CFCS level III is known as an “effective sender and
receiver with familiar partners”. The primary difference from CFCS I and II is that communication
is effective with familiar partners, but is usually not effective with an unfamiliar partner, due to
Children 2017, 4, 30 7 of 10

decreased intelligibility. At CFCS level IV, an individual is an “inconsistent sender and/or receiver
with familiar partners”. An individual may occasionally communicate with familiar partners, but it
is the inconsistency of this interaction that makes the distinction for level IV compared to level III.
An individual who communicates at a CFCS level V is a “seldom effective sender or receiver even with
familiar partners”. This is in contrast to a level IV where there is inconsistency with communication;
a level V consistently has ineffective communication.

2.4.1. Inter-Rater and Test-Retest Reliability


During development of the CFCS, it underwent evaluation for test-retest reliability, as well as
inter-rater reliability. The test-retest reliability was reassuring at 0.82. The inter-rater reliability was
found to be 0.66 when two professionals were classifying an individual and 0.49 when a professional
and a parent were assigning a classification. Professional inter-rater reliability improved to 0.77
for individuals over the age of three years. Overall, the CFCS had good test-retest reliability and
professional inter-rater reliability, with slightly less parent-professional inter-rater reliability [22].
The CFCS has been translated into Dutch and Farsi and these measures have also demonstrated good
reliability [23,24].

2.5. Eating and Drinking Ability Classification System (EDACS)


In addition to impairments of gross motor function, fine motor function and communication,
individuals with CP can also have impairments in eating and drinking, as a result of difficulty with
motor control. Between 27% and 90% of people with CP are estimated to have some degree of difficulty
with eating or drinking [25].
The EDACS was developed by Sellers et al. in 2014, and is a valid measure to assess eating
and drinking ability for children with CP, ages 3 and older [26]. This classification is a simple
five-point ordinal system, designed to be analogous and complementary to the GMFCS, MACS and
CFCS. The EDACS assesses eating and drinking safety (aspiration and choking) as well as efficiency
(amount of food lost and time taken to eat). The EDACS also adds an additional three-point ordinal
scale that addresses the amount of assistance a person needs: independent; requires assistance;
or dependent for eating and drinking [26].
An individual in EDACS I can independently eat and drink safely and efficiently, no different
from their peers. A cough or gag could be present for very challenging textures. An individual in
EDACS II eats and drinks safely, may have some limitations in terms of food loss, and generally
requires more time to complete a meal than peers. A cough may be present with fast flowing liquid
or large quantities of food. Both EDACS I and II include the ability to eat a wide range of textures,
consistent with their peers. At EDACS III, an individual eats and drinks with some limitation to
safety as well as efficiency. Hard lumps of food may be difficult to manage, or choking may be a risk.
Many individuals in EDACS III primarily eat pureed or mashed foods with occasional soft chewing of
limited textures. A cough may be present with fast flowing liquid or a large bolus of food.
In comparison, an individual in EDACS IV or V cannot swallow food and drink without risk of
aspiration. At EDACS IV, there are significant limitations with safety; however, the risks of aspiration
can be managed and oral feeding is possible. An individual in EDACS IV will eat smooth purees or
well mashed foods only. The coordination of swallowing and breathing may be difficult and there may
be signs of aspiration. An individual in EDACS V is unable to eat or drink safely, and relies on tube
feeding for nutrition; he or she may manage small tastes or flavors. Those in EDACS V are at high risk
for aspiration.
The EDACS also assesses the level of assistance required for feeding, via a simple three-point
classification system (independent; requires assistance; or totally dependent). To obtain the
“independent” classification, an individual must bring food and drink to their mouth without
assistance. If an individual uses adaptive equipment or requires another individual to use an adaptive
equipment when assisting them in bringing food or drink to their mouth, they would be classified as
Children 2017, 4, 30 8 of 10

“requiring assistance”. A classification of “totally dependent” indicates that the individual requires
another person to bring the food or drink to their mouth [26].

2.5.1. Intra-Class Correlation


As the EDACS was developed, it also underwent agreement testing and reliability testing between
speech and language pathologists (SLP), as well as between an SLP and a parent. The EDACS scored
78% on absolute agreement between two SLPs, with an ICC coefficient of 0.93. When a discrepancy
occurred, it was by a single EDACS level with only one exception that was of more than a single
level. The EDACS had an absolute agreement of 58% when classified between an SLP and a parent,
with an ICC of 0.86. Parents either agreed with the SLP or scored their child one classification level
more able than the SLP. Overall, the EDACS had good agreement testing and reliability testing with
slightly less parent-SLP agreement, making this a valid and reliable classification system [26].

3. Discussion
With the development of the four instruments discussed in this review, providers have
a framework for a common language to better describe and communicate about the vastly
heterogeneous functional abilities of individuals with CP. This common language is important for quick
and accurate transmission of information from one care provider to the next or from care provider to
caregiver. Using the GMFCS, MACS, CFCS, and EDACS, descriptions of patients are more accurate
and meaningful than solely traditional topographic descriptions, but can be used in conjunction with
these descriptors as a complementary inclusion. In addition to facilitating communication between
providers, these classification systems also standardize populations for clinical research. A summary
of the five classification levels can be found in Table 1.

Table 1. Five classification levels of the Gross Motor Functional Classification System (GMFCS),
the Manual Ability Classification System (MACS), the Communication, Function Classification System
(CFCS) and the Eating and Drinking Ability Classification System (EDACS).

GMFCS MACS CFCS EDACS


Handles objects easily Effective sender and Eats and drinks safely and
I Walks without limitation
and successfully receiver efficiently
Handles most objects Eats and drinks safely but
Walks with limitations Effective but slow paced
II with reduced with some limitations to
(no mobility aid by 4yo) sender and receiver
speed/quality efficiency
Handles objects
Effective sender and Eats and drinks with some
Walks with hand-held with difficulty,
III receiver with familiar limitations to safely; there may
mobility device help to prepare or
partners also be limitations to efficiency
modify activity
Self-mobility Handles limited Inconsistent sender and
Eats and drinks with
IV with limitations, number of objects in receiver with familiar
significant limitations to safety
may use power adapted setting partners
Seldom effective sender
Transported in manual Does not Unable to eat or drink safely,
V and receiver with
wheelchair handle objects consider feeding tube
familiar partners

Additionally, providers should remember that ongoing development throughout childhood


requires them to note the age of each individual when assigning a functional level. The use of these
various classification systems should be adopted as a standard care for the diagnosis and management
of all children with CP.
Not only do the GMFCS, MACS, CFCS, and EDACS refine and simplify descriptions of patients
with CP, they have done this in a robust fashion. These classifications have been found to be reliable and
stable over time. With modest training, these measures can be assessed rapidly and accurately—and
Children 2017, 4, 30 9 of 10

not just by professionals, but also often by family members. The simplicity, yet richness, of these
classifications have transformed the description and quality of care for children with cerebral palsy.

Acknowledgments: The authors would like to thank the Division of Physical Medicine and Rehabilitation, at the
Cincinnati Children’s Hospital Medical Center, for their support.
Author Contributions: Dr. Paulson and Dr. Vargus-Adams contributed equally to the concept and writing of
this paper.
Conflicts of Interest: The authors declare no conflict of interest.

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