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Classification of Cerebral Palsy Subtypes

11:38 EDT 23rd May 2013 | BioPortfolio

Summary

This study, conducted at the National Institutes of Health and the Children's National Medical Center, will evaluate how well different physicians agree in how they classify cerebral palsy subtypes based on patient examination. Cerebral palsy is divided into several subtypes, according to the primary underlying muscle abnormality and its distribution and severity. Classification of these subtypes is important for conducting rehabilitation research studies on patients with the same type of abnormality. However, doctors do not always classify cerebral palsy types in the same way. This study will examine methods for improving agreement among doctors in their classification of cerebral palsy subtypes.

Children between 6 and 18 years of age with cerebral palsy who can voluntarily move their arms and legs may be eligible for this study. Participants will be examined by at least three doctors or therapists. The examinations take 30-60 minutes. For the examination, a doctor or therapist will do the following:

- Observe the patient at rest

- Gently move the patient's arms and legs and then have the patient move his or her arms and legs

- Check the patient's reflexes

- Observe the patient walking, if the patient is able to walk

Patients will be asked to remain in the clinic for up to 3 hours while researchers discuss the examination and may be asked to repeat part of the study examination.

Description

The movement and posture abnormalities that are characteristic of cerebral palsy are associated with underlying abnormalities of muscle tone, including dystonia, spasticity, and rigidity. The subtypes of cerebral palsy are defined according to the predominant muscle tone abnormality, its distribution and severity. There is widespread agreement that CP subtypes should be divided into the following groups. Spastic subtypes (hemiplegia: unilateral asymmetric spasticity; diplegia: bilateral symmetric spasticity, lower limbs more affected than upper limbs; quadriplegia: bilateral symmetric spasticity, upper and lower limbs affected equally), dystonic or athetotic type; and other miscellaneous types: hypotonic, mixed types, etc. These descriptive definitions are not useful when forming homogeneous cohorts of CP subtypes in rehabilitation research studies. Even when a preceding training session establishes precise criteria for each subtype based on these definitions and uses a "four limb" approach (defining the dominant muscle tone abnormality and its severity in each limb) the interobserver agreement in the classification of CP subtypes remains poor. This may be because there are no widely accepted definitions for the most common muscle tone abnormalities in cerebral palsy (spasticity, rigidity, dystonia) and examination methods vary widely. Recently, an interdisciplinary group of clinicians published a consensus document in which they set forth clear definitions for these disorders of muscle tone and recommendations on how to examine for them. Since the CP subtypes are defined according to the dominant muscle tone abnormality, we hypothesize that it may be possible to achieve acceptable interobserver agreement in the classification of the CP subtypes if these definitions and standard examination techniques are incorporated into the preceding training session with the other accepted elements. To test this hypothesis we will train clinicians from three different areas of expertise: child neurology, physiatry, physical therapy to classify subtypes of CP using accepted methods (establishing precise criteria for the subtypes and using a four limb approach) and will also train them to identify muscle tone abnormalities using the definitions and standard examination techniques in the consensus document. Following this training, we will ask the same clinicians to classify subtypes of cerebral palsy in a group of CP children. We will then measure interobserver agreement for the classification of CP using the kappa statistic. Achieving acceptable interobserver agreement in the classification of CP subtypes may facilitate the formation of homogeneous cohorts in research studies.

Study Design

N/A

Conditions

Cerebral Palsy

Location

National Institute of Neurological Disorders and Stroke (NINDS)
Bethesda
Maryland
United States
20892

Status

Completed

Source

National Institutes of Health Clinical Center (CC)

Results (where available)

View Results

Links

Medical and Biotech [MESH] Definitions

Cerebral Palsy

A heterogeneous group of nonprogressive motor disorders caused by chronic brain injuries that originate in the prenatal period, perinatal period, or first few years of life. The four major subtypes are spastic, athetoid, ataxic, and mixed cerebral palsy, with spastic forms being the most common. The motor disorder may range from difficulties with fine motor control to severe spasticity (see MUSCLE SPASTICITY) in all limbs. Spastic diplegia (Little disease) is the most common subtype, and is characterized by spasticity that is more prominent in the legs than in the arms. Pathologically, this condition may be associated with LEUKOMALACIA, PERIVENTRICULAR. (From Dev Med Child Neurol 1998 Aug;40(8):520-7)

Leukomalacia, Periventricular

Degeneration of white matter adjacent to the CEREBRAL VENTRICLES following cerebral hypoxia or BRAIN ISCHEMIA in neonates. The condition primarily affects white matter in the perfusion zone between superficial and deep branches of the MIDDLE CEREBRAL ARTERY. Clinical manifestations include VISION DISORDERS; CEREBRAL PALSY; PARAPLEGIA; SEIZURES; and cognitive disorders. (From Adams et al., Principles of Neurology, 6th ed, p1021; Joynt, Clinical Neurology, 1997, Ch4, pp30-1)

Cadasil

A familial, cerebral arteriopathy mapped to chromosome 19q12, and characterized by the presence of granular deposits in small CEREBRAL ARTERIES producing ischemic STROKE; PSEUDOBULBAR PALSY; and multiple subcortical infarcts (CEREBRAL INFARCTION). CADASIL is an acronym for Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy. CADASIL differs from BINSWANGER DISEASE by the presence of MIGRAINE WITH AURA and usually by the lack of history of arterial HYPERTENSION. (From Bradley et al, Neurology in Clinical Practice, 2000, p1146)

Diffuse Cerebral Sclerosis Of Schilder

A rare central nervous system demyelinating condition affecting children and young adults. Pathologic findings include a large, sharply defined, asymmetric focus of myelin destruction that may involve an entire lobe or cerebral hemisphere. The clinical course tends to be progressive and includes dementia, cortical blindness, cortical deafness, spastic hemiplegia, and pseudobulbar palsy. Concentric sclerosis of Balo is differentiated from diffuse cerebral sclerosis of Schilder by the pathologic finding of alternating bands of destruction and preservation of myelin in concentric rings. Alpers' Syndrome refers to a heterogeneous group of diseases that feature progressive cerebral deterioration and liver disease. (From Adams et al., Principles of Neurology, 6th ed, p914; Dev Neurosci 1991;13(4-5):267-73)

Dementia, Multi-infarct

Loss of higher cortical functions with retained awareness due to multiple cortical or subcortical CEREBRAL INFARCTION. Memory, judgment, attention span, and impulse control are often impaired, and may be accompanied by PSEUDOBULBAR PALSY; HEMIPARESIS; reflex abnormalities, and other signs of localized neurologic dysfunction. (From Adams et al., Principles of Neurology, 6th ed, p1060)

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