Cerebral palsy classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:  Aditya Ganti M.B.B.S. [2]

## Overview

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Cerebral palsy can be classified based on number of limbs involved, physiologically and functionally. Based on number of limbs involved cerebral palsy can be classified into monoplegia, hemiplegia, diplegia, paraplegia and quadriplegia. Physiologically, cerebral palsy can be divided into a spastic type (pyramidal), and an extrapyramidal type. The extrapyramidal types of cerebral palsy include athetoid, choreiform, ataxic, rigid, and hypotonic. The Manual Ability Classification System (MACS) and the Gross Motor Function Classification System (GMFCS) are two most commonly employed systems for functional classification of cerebral palsy. The Manual Ability Classification System (MACS) classifies children with cerebral palsy into five levels. The levels are based on the children’s self-initiated ability to handle objects and their need for assistance or adaptation to perform manual activities in everyday life. The Gross Motor Function Classification System (GMFCS) also classifies children with cerebral palsy into five levels. The levels are based on self-initiated movement abilities, in particular sitting and walking.

## Classification

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### Topographical Distribution

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Based on number of limbs involved cerebral palsy can be classified into 4 subtypes

TYPE  | Involved Limb

|

Monoplegia | One extremity involved, usually lower
Hemiplegia | Both extremities on the same side involved

Usually, upper extremity involved more than lower extremity

Paraplegia | Both lower extremities equally involved
Diplegia | Lower extremities more involved than upper extremities

Fine-motor/sensory abnormalities in upper extremity

Quadriplegia | All extremities involved equally

Normal head/neck control

Double hemiplegia | All extremities involved, upper more than lower

### Physiologic classification

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Physiologically, cerebral palsy can be divided into a spastic type, which affects the corticospinal tracts (pyramidal) , and an extrapyramidal type, which affects the other regions of the developing brain. The extrapyramidal types of cerebral palsy include athetoid, choreiform, ataxic, rigid, and hypotonic.[1]

| Cerebral palsy

|
|
|
|
|
|
|
|

| Pyramidal|

| Extrapyramidal|

| Spastic|

Spastic diplegia|

| Spastic hemiplegia|

| Spastic quadriplegia|

| Athetoid|

| Choreiform|

| Rigid|

| Ataxic|

| Hypotonic|

#### Spastic

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* Spastic cerebral palsy is the most common form of cerebral palsy.[2]
* Spastic cerebral palsy is usually associated with injury to the pyramidal tracts in the immature brain.
* Spasticity is caused due to exaggeration of the normal musclepassive stretch reflex.
* Histologically altered muscle function leads to the deposition of type I collagen in the endomysium of the affected muscle, leading to thickening and fibrosis, the degree of which correlated to the severity of the spasticity.
* Simultaneous co-contraction of normally antagonistic muscle groups leads to fatigue, loss of dexterity and coordination, and balance difficulties.

#### Athetoid

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* Athetoid cerebral palsy is caused by an injury to the extrapyramidal tracts.
* Athetoid cerebral palsy is characterized by dyskinetic, purposeless movements that may be exacerbated by environmental stimulation.
* With the improvements in prevention of Rh incompatibility leading to kernicterus, the incidence of athetoid cerebral palsy is decreasing.
* Dystonia, characterized by an increased overall tone and distorted positioning in response to voluntary movements, or hypotonia also can occur with athetoid cerebral palsy.

#### Choreiform

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* Choreiform cerebral palsy is characterized by continual purposeless movements of the patient's wrists, fingers, toes, and ankles.
* This continuous movement can make bracing and sitting difficult.

#### Rigid

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* Patients with rigid cerebral palsy are the most hypertonic of all cerebral palsy patients.
* Hypertonicity occurs in the absence of hyperreflexia, spasticity, and clonus, which are common in spastic cerebral palsy.
* Patients with rigid cerebral palsy have a “cogwheel” or “lead pipe” muscle stiffness that often requires surgical release.

#### Ataxic

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* Ataxic cerebral palsy is a very rare type.
* Ataxic cerebral palsy is characterized by the disturbance of coordinated movement as a result of an injury to the developing cerebellum.
* It is important to distinguish true ataxia from spasticity because with treatment many children with ataxia are able to improve their gait function without surgery.

#### Hypotonic

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* Hypotonic cerebral palsy is characterized by weakness in conjunction with low muscle tone and normal deep tendon reflexes.
* Many children who ultimately develop spastic or ataxic cerebral palsy pass through a hypotonic stage lasting 1 or 2 years before the true nature of their brain injury becomes apparent.
* Persistent hypotonia can lead to difficulties with sitting balance, head positioning, and communication.

### Functional Classification of Cerebral Palsy

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The Manual Ability Classification System (MACS) and the Gross Motor Function Classification System (GMFCS) are two most commonly employed systems for functional classification of cerebral palsy.[3]

#### Manual Ability Classification System

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The Manual Ability Classification System (MACS) classifies children with cerebral palsy into five levels. The levels are based on the children’s self-initiated ability to handle objects and their need for assistance or adaptation to perform manual activities in everyday life.[4]

| Does the child handle most kind of
daily activities independently
( during play and leisure, eating and dressing)

|
|
|
|
|
|
|

| Yes|

| No|

| Does the child handle even more difficult tasks
with fair speed and accuracy and
does not need alternative ways to perform|

| Does the child perform number of mannual tasks
which commonly need to prepared or adapted
and help is needed occasionally|

Yes|

| No|

| Yes|

| No|

Level 1
Handles objects easily and successfully|

| Level 2
Handles most objects with
reduced quality and speed of acheivement|

| Level 3
Handles objects with
difficulty but needs preparation|

| Can the child perform
easy activites with frequent support|

| Yes|

| No

| Level 4
Handles easy activites
with limitations and support|

| Level 5
Cannot handle daily activites
has severely limited abilities to perform even simple actions

#### Gross Motor Function Classification System

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The Gross Motor Function Classification System (GMFCS) also classifies children with cerebral palsy into five levels. The levels are based on self-initiated movement abilities, in particular sitting and walking.[5]

Level  | Description

|

1  |

* Can walk at home, school, outdoors and in the community without any support.
* Can climb stairs without the use of a railing.
* Can perform gross motor skills such as running and jumping, but speed, balance, and coordination are limited.

2  |

* Can walk in most settings and climb stairs holding onto a railing.
* May experience difficulty walking long distances and balancing on uneven terrain, inclines, in crowded areas or confined spaces.
* Can walk with physical assistance, a handheld mobility device or used wheeled mobility over long distances.
* Have only minimal ability to perform gross motor skills such as running and jumping.

3  |

* Can walk using a hand-held mobility device in most indoor settings.
* Can may climb stairs holding onto a railing with supervision or assistance.
* Can use wheeled mobility when traveling long distances and may self-propel for shorter distances.

4  |

* Can use methods of mobility that require physical assistance or powered mobility in most settings.
* Can walk for short distances at home with physical assistance or use powered mobility or a body support walker when positioned.
* At school, outdoors and in the community children are transported in a manual wheelchair or use powered mobility.

5  |

* Children are transported in a manual wheelchair in all settings.
* Children are limited in their ability to maintain antigravity head and trunk postures and control leg and arm movements.

## References

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1. ↑ Agarwal A, Verma I (2012). "Cerebral palsy in children: An overview". J Clin Orthop Trauma. 3 (2): 77–81. doi:10.1016/j.jcot.2012.09.001. PMC 3872805. PMID 26403442.
2. ↑ Shamsoddini A, Amirsalari S, Hollisaz MT, Rahimnia A, Khatibi-Aghda A (2014). "Management of spasticity in children with cerebral palsy". Iran J Pediatr. 24 (4): 345–51. PMC 4339555. PMID 25755853.
3. ↑ Compagnone E, Maniglio J, Camposeo S, Vespino T, Losito L, De Rinaldis M, Gennaro L, Trabacca A (2014). "Functional classifications for cerebral palsy: correlations between the gross motor function classification system (GMFCS), the manual ability classification system (MACS) and the communication function classification system (CFCS)". Res Dev Disabil. 35 (11): 2651–7. doi:10.1016/j.ridd.2014.07.005. PMID 25062096.
4. ↑ Paulson A, Vargus-Adams J (2017). "Overview of Four Functional Classification Systems Commonly Used in Cerebral Palsy". Children (Basel). 4 (4). doi:10.3390/children4040030. PMC 5406689. PMID 28441773.
5. ↑ Carnahan KD, Arner M, Hägglund G (2007). "Association between gross motor function (GMFCS) and manual ability (MACS) in children with cerebral palsy. A population-based study of 359 children". BMC Musculoskelet Disord. 8: 50. doi:10.1186/1471-2474-8-50. PMC 1919364. PMID 17584944.

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