Neurology

Cerebral Palsy

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A child with cerebral palsy being assessed by a physician https://en.wikipedia.org/wiki/Cerebral_palsy

 

Introduction

Cerebral Palsy is a symptom rather than a specific disease. It is a group of non-progressive but often changing, motor impairment syndromes. Affects movement and postures secondary to lesions or anomalies of the brain arising in the early stages of development.

 

Epidemiology

  1. Incidence of 7:1000 live births. Prevalence of about 3.6/1,000.
  2. >100,000 Americans under the age of 18 years.
  3. Annual cost to society exceeds $5 billion.
  4. Premature born children have higher incidence than full term.
  5. Other neurologic disabilities accompany CP: about 45% have epilepsy. Many have visual-motor or other learning difficulties.
  6. Despite the development of high tech obstetric care, the prevalence had not changed in the past 20 years.

 

Classification and Etiology

  1. In many children, the etiology is unknown, but is believed to be a perinatal insult in the majority of cases.
  2. Some associations are:
    1. Prematurity
    2. Intrauterine growth restriction
    3. Intrauterine infection
    4. Multiparity
    5. Low birth weight
    6. Perinatal asphyxia 
    7. Many patients with CP have an uneventful prenatal course, deliveries, and Apgar score >7.

 

Types

  • Spastic syndromes
    • Spastic Diplegia
      1. Typically in LBW infants born prematurely. The lower extremities are more involved than the upper with increased muscle tone (spasticity), increased DTRs and a + Babinski reflex.
    • Spastic Quadriplegia
      1. Involves all four extremities. These patients are affected most.
      2. Most have severe intellectual disability and have epilepsy, microcephaly, growth failure, and visual defects. 
    • Spastic Hemiplegia
      1. Associated with LBW and severe asphyxial insults as well as vascular insults (within distribution of middle cerebral artery). 
      2. Seizures are common but cognitive function can be normal. Motor impairments and language difficulties can be severe. 
  • Extrapyramidal
    1. Associated with kernicterus. 
    2. Characterized by hypotonia, choreoathetosis, and dystonic movements. 
  • Atonic
    1. Associated with marked hypotonia, brisk reflexes, and severe cognitive delays. 
    2. The presence of brisk reflexes helps to distinguish from other hypotonic disorders

 

Diagnosis

  • Developmental history.
  • Physical examination with careful attention to growth patterns, dysmorphic features and skin stigmata of neurocutaneous disease, and thorough neurologic examination
  • Clinical diagnosis of CP is based on the pattern of abnormalities on neurologic exam. Use of a mnemonic, POSTER.
                
    • Infants with 4 or more of these findings are likely to receive the diagnosis of CP later in childhood. 

P - abnormal Posture: fisting with adducted thumbs, hyperextension and adduction (scissoring) of lower extremities, and hyperextension of trunk (arching).

O - poor Oral-motor coordination: poor sucking-swallow coordination, poor lip closure on the nipple, difficulty handling textured foods, or excessive drooling. Older kids may have trouble with drooling, chewing or articulation.

S - Strabismus: commonly associated with CP.

T - abnormal muscle Tone: increased resistance to passive movement of the extremities and decreased axial tone.

E - delayed integration of primitive reflexes, delayed Evolution of automatic responses: persistent palmar grasp, Moro, asymmetric tonic neck reflexes. Poor equilibrium, delayed protective response.

R - deep tendon Reflexes: brisk, with clonus.

 

Approach to the Child and Family

  1. Because it is difficult to predict later abilities on the basis of early developmental assessment, it is important to allow the family of an infant or young child with delayed development to remain hopeful yet realistic about the child's future abilities.
  2. If the future implication is unknown, it is best to be honest about the uncertainty and be available to the family for support.
  3. The physician can also serve as an advocate to the family and child by coordinating medical, therapeutic, and education services and supporting the family through the process of diagnosis and long-term management.
  4. The general pediatrician may seek referrals to a developmental pediatrician, geneticist, or a child neurologist for further consultation. 

 

Diagnostic Studies

  1. 60-70% of individuals with severe to profound intellectual diability have a definable cause. This may help in guiding the family in prognosis and genetic counseling.
  2. A diagnosis will be valuable to families.
  3. Some studies to consider include: genetic studies, neuroimaging, metabolic studies, ophthalmologic evaluation., auditory studies.
    1. MRI can be used to identify a lesion in the brain in a majority of cases of CP.

 

Treatment

  1. The provision of PT and OT programs is the mainstay of treatment for young children with CP.
  2. Management of seizure, spasticity (medications e.g. baclofen or botulinum toxin), orthopedic impairments, and sensory impairments.
  3. Surgical treatments include selective dorsal rhizotomy and selective encephalotomy, although it is unclear which subgroup of children would most benefit from these procedures.
  4. Family support is essential.
  5. Optimal benefits of early intervention are realized when the physician is an advocate and works collaboratively with the service agencies in all stages of the process, including identification, referral, and monitoring of developmental progress. 

 

Prognosis

  1. The majority of children survive into adulthood, with a 30 year survival rate of about 87%.
  2. Motor impairment is variable among the different subtypes of CP, but studies have helped improve prognostication in this regard.

 

References

  1. Kuban K.C. and Leviton A. Medical Progress: Cerebral Palsy. NEJM 1994
  2. Behrman R.E. Kliegman R.M. Arvin A.M. Nelson Essentials of Pediatrics, 3rd ed. Philadelphia, WB Saunders, 1998
  3. Gartner J.C. and Zitelli B.J. Common & Chronic Symptoms in Pediatrics. Mosby-Year Book, Inc., 1997
  4. Yin R. Reddihough D. Ditchfield M. Collins K. Magnetic resonance imaging findings in cerebral palsy. Journal of Paediatrics and Child Health April 2000
  5. Patrick J.H. Roberts A.P. Cole G.F. Therapeutic choices in the locomotor management of the child with cerebral palsy--more luck than judgement? Arch Dis Child 2001
  6. Kinsman S.L. Predicting gross motor function in cerebral palsy. JAMA 2002
  7. Palmer F.B. Strategies for the early diagnosis of Cerebral Palsy. The Journal of Pediatrics August 2004
  8. Strijbis E.M. Oudman I. Van Essen P. MacLennan A.H. Cerebral palsy and the application of the international criteria for acute intrapartum hypoxia. Obstet Gynecol 2006
  9. Yeargin-Allsopp M. Van Naarden B.K. Doernberg N.S. et al. Prevalence of cerebral palsy in 8-year-old children in three areas of the United States in 2002: a multisite collaboration. Pediatrics 2008
  10. Nelson K.B. Causative Factors in Cerebral Palsy. Clinical Obstetrics and Gynecology 2008