Cerebral palsy

• CP or cerebral palsy is a chronic/permanent reduced function (symptom description) indicating non-progressive motor difficulties (coordination, muscle strength, balance, tone) caused by injuries (lesions) in the developing brain (damages usually happened during the prenatal period). 
• Cerebral palsy is an umbrella term that includes a group of non-progressive motor disorders? (increased spasticity!)
• Neither peripheral nervous nor muscular diseases are considered CP.
• Due to other neurological additional problems (e.g., epilepsy, language disturbance, visual impairment, hearing loss, etc.) and the physiological development in children, cerebral palsy is not an unchangeable condition.

• W J Little (1862) found that it is hypoxia at birth causing brain damage (Little disease or spastic diplegia). 
• Sir William Osler first introduced the term "cerebral palsy" in 1889.     
• Sigmund Freud has noted that children with CP have other symptoms such as epilepsy, visual impairment, hearing loss, etc.

Freud thought the injury could happen during the development of the brain (in earlier stages than Little thought), i.e., during the fetal period.

• Mother's age > 35 år
• Prematurity
• ABO or Rh-immunisation (jaundice)
• Small for age
• Dysfunktion of the uterus
• Infection during pregnancy
• Asphyxia

Two hundred children in each cohort are diagnosed with cerebral palsy in Sweden (between 2-4 children per 1000 born in Sweden).

The rising number is due to improved neonatal care and more effective documentation of CP cases in the national registry.

In the 50s the dystonic CP was more usual due to kernicterus.

Underlying causes ( medical diagnosis!):

• Intracerebral/intracranial hemorrhage
• Cerebral infarction
• Hypoxia
• Cerebral infections during the fetal period or the first two years of life
• Scarring in the brain (gliosis)
• CNS malformations

Cerebral = related to the brain 
Paresis = weakness
Plegi (paralysis) = total paralysis
Monoparesis = weakness in one extremity
Diparesis= weakness in two extremities (legs)
Tetraparesis = weakness in all four extremities
Unilateral = one sided  or bilateral = dubble sided

Peri: around
Ventricular: large cavities in the brain that contain cerebrospinal fluid
Leuko: white
Malacia: softening or degradation of tissue

CP consists of various motor syndromes:         

• Spastic CP
• Athetoid CP
• Atactic CP
• Mixed CP

Most usual form (50 %)
Spasticity = increased muscle tonus
Pyramidal pathway always affected 
(cortex, corona radiata, capsula interna, spinal cord)

30% of CP
Dys = bad or difficult
Athetosis = involuntary, persistent, and movements like a snake in the extremities
Extrapyramidal system ( motor-modifying system) is affected
Basal ganglia are always damaged 

Tonus change
Caused by trauma, hypoxia, bleeding, kernicterus (severe jaundice in newborns) 

10% of CP
Ataxia is dominant clinical symtom
Ataxia = abnormal coordination
The cerebellum is most affected
Injury at the right side of the cerebellum causes right-sided motor deficit.

Ataxia can affect motor ability in the legs, arms, and even head and neck muscles (atactic speech)

Kombination av olika motoriska svårigheter.

• Decortication (extensive and diffuse damage in the cortex)
• Decerebration (extensive and diffuse damage in subcortical areas and the brainstem)

• Subtypes ( spastic, athetoid,…)
• Lateralisation (unilateral or bilateral)
• Topography (monoplegia, hemiplegia, diplegia, triplegia, tetraplegia)
• Function (GMFCS): Gross motor function classification system, MACS: Manual ability classification system, gait analysis
• Etiology (cause)!
• Additional symptoms/disorders 

Abnormal gait in children with cerebral palsy depends on many different factors:

• Muscle weakness, spasticity, contractures, dyscoordination, sensory abnormalities, and pain
• Gait analysis helps physicians decide before orthopedic surgery or other treatments, for example, injection of botulinum toxin. 
• Clinical observation/assessment + dynamic EMG

• CP is a clinical diagnosis (imaging, EEG, and blood test are needed for overall assessment)
• Neuroradiologicka investigations are needed to map the damage where it locates, when it is happened (MRI, CT-scan, fMRI, PET, or SPECT).

• Developmental delay/derangement
• Learning difficulties
• Speech disorders (due to pseudobulbar palsy)
• Dysphagia (due to pseudobulbar palsy)
• Chronic constipation
• Gastroesophageal reflux
• Visual defect (cerebral visual impairment)
• Hearing loss
• Skeletal deformities
• Epilepsy
• Perceptual disorders
• Behavioral problems 
• Pseudobulbar palsy

• Pharmacologic (Botulinum-toxin, Baclofen)
• Surgical (Rhizotomy, orthopedic operation, intrathecal Baclofen-pump, deep brain stimulation)
• Motor training och aids (physiotherapist, occupational therapist)
• Treatment of additional symptoms: urotherapist, treatment of osteopenia and osteoporosis

• Regular controls by a multidisciplinary team on child and adolescent habilitation
• CPUP (follow-up program for cerebral palsy) was started in 1994. It has been designated as a national quality register since 2005. All county councils and regions in Sweden and parts of Norway participate in CPUP (overall assessment).

It depends on the cause, severity, and additional problems (a child with refractory epilepsy has a worse prognosis than those without epileptic seizures).

GMFCS 
MACS

• Definition of cerebral palsy! (paradigm shift)
• Great need for research in adults with cerebral palsy (CP is not only found in children and adolescents!)
• Development of clinical methods for functional assessment of children and adults with the motor deficit, for example, gait analysis
• Development of advanced movement aids

Neonatal Encephalopathy and Cerebral Palsy, 
Ralph Adamo, Acog, 2003.

Management of the Motor Disorders of Children with Cerebral Palsy, 
David Scrutton, MargareMayston, 2004.

Neurology of the newborn, 
Joseph J, Volpe, 2008.

www.cpup.se 
www.ucp.org ( United cerebral palsy)
www.nchpad.org ( The national center on physical activity and disability)