Cerebral palsy diagnosis and management: the state of the art

Abstract

C erebral palsy, or static encephalopathy, is defined as a primary abnormality of movement and posture secondary to a nonprogressive lesion of a developing brain. It actually represents a group of disorders rather than a single disease entity. Abnormal motor control and tone in the absence of an underlying progressive disease is the clinical hallmark of cerebral palsy. By definition, all neurodegenerative and metabolic conditions, which may initially mimic the disorder, are excluded from this diagnostic category. In 1862, William James Little, an orthopedic surgeon, provided the first description of “spastic rigidity” related to prematurity and birth complications, referring to the condition as Little’s disease. William Osler later introduced the term “cerebral palsy” in 1888. Subsequently, Sigmund Freud observed that antepartum and postpartum factors might be causally related to cerebral palsy. Since the earliest definition of cerebral palsy, many others have attempted to establish a unified description of the disorder. Controversy and confusion result when we attempt to relate causal factors to the physical and functional characteristics of cerebral palsy. Currently, cerebral palsy has been described as an “umbrella term” to refer to children with a wide range of static cerebral disorders with associated motor impairment. Inclusion and exclusion criteria useful for making the diagnosis of cerebral palsy have been written for the purpose of maintaining disease registries, such as the Western Australian Cerebral Palsy Register. Before the availability of modern laboratory techniques, some partial trisomies, chromosomal deletions, and x-linked hydrocephalus syndrome were included in the diagnosis of cerebral palsy. Although it is recommended that these nonprogressive cerebral abnormalities associated with motor impairments continue to be included in the diagnostic grouping of cerebral palsy, consistency among practitioners is lacking. It is well recognized that very low birth weight infants are at risk for cerebral palsy. Recent attention has focused on the outcomes of very low birth weight infants who survived the neonatal period without severe intraventricular hemorrhage or periventicular leukomalacia. It is estimated that the academic achievement of 30% to 50% of these children falls in the subnormal range. Furthermore, attention-deficit hyperactivity disorder is diagnosed in 20% to 30% and psychiatric disorders in 25% to 30% by adolescence. Perhaps the vulnerable premature brain is harmed during a critical period of development by stressful environmental conditions, including infant-provider interactions, constant noise, and bright lights. The impact of chronic lung disease, recurrent apnea and bradycardia, hyperbilirubinemia, and other physiologic stressors on the developing infant are poorly understood. However, if these at-risk children do not have abnormalities of motor function, they are not included in the diagnostic grouping of cerebral palsy.