(22) Musculoskeletal Lecture 4: Spina Bifida & Cerebral Palsy

Introduction to Musculoskeletal Disorders

Lecture Focus: Spina Bifida and Cerebral Palsy (CP)

This lecture aims to provide an in-depth understanding of spina bifida and cerebral palsy, including definitions, types, etiology, risk factors, clinical manifestations, nursing management, and treatment strategies for both conditions.

Spina Bifida

• Definition: Spina bifida is a spectrum of congenital neural tube defects characterized by the incomplete closure of the neural tube during embryonic development, resulting in defects in the spine and spinal cord.

• Terminology: The term "spina bifida" translates to "split spine" in Latin, which aptly describes the observable spinal defect.

Types of Spina Bifida

1. Spina Bifida Occulta:

   • This is the most common and least severe form, affecting approximately 10-20% of the population.

   • Often asymptomatic, it may be discovered incidentally through X-rays or scans during evaluations for unrelated issues.

   • Signs may include skin markings such as small dimples, tufts of hair, or pigmented lesions over the defect.

2. Meningocele:

   • In this type, the protective sac of fluid protrudes through the spinal column, but the spinal cord remains intact.

   • Symptoms are typically mild, and neurological function may remain largely unaffected, although the potential exists for complications.

3. Myelomeningocele:

   • This is the most severe form, where both the meninges and spinal cord are involved in the sac.

   • It is associated with significant complications such as infections (meningitis), paralysis, and bladder and bowel dysfunction. Long-term care is often necessary due to these implications.

Clinical Manifestations

• Symptoms vary by the defect's location on the spine and may include:

  • Flaccid paralysis of the lower extremities

  • Lack of bowel and bladder control (incontinence)

  • The majority of individuals may also experience hydrocephalus, a condition characterized by abnormal accumulation of cerebrospinal fluid (CSF) in the brain, necessitating careful management.

Etiology and Risk Factors

• The precise etiology remains unclear, typically involving a complex interplay of genetic, nutritional, and environmental factors.

• Key risk factors include:

  • Maternal nutritional deficiencies, particularly lacking folic acid during pregnancy.

  • Advanced maternal age, along with maternal obesity and diabetes.

  • Medications such as anti-seizure drugs have been associated with increased risk.

Importance of Folic Acid

• Recommended Intake: Women of childbearing age should consume 400-800 mcg of folic acid per day.

• Adequate intake has been shown to reduce the risk of neural tube defects significantly (up to 70%).

Diagnostic Testing

• Prenatal testing can be conducted via ultrasound or measuring elevated maternal alpha-fetoprotein (MSAFP).

• Postnatal diagnosis typically involves physical examination and imaging studies like CT or MRI.

Medical Management

• Surgical intervention is often required for myelomeningocele to mitigate infection risk and promote better outcomes.

• Preoperative Care: The child should be positioned on the abdomen, and the sac should be covered with sterile saline-soaked gauze to prevent drying.

• Postoperative Care: Continued postoperative vigilance is needed, including maintaining abdominal positioning for 10-14 days and monitoring for potential complications like bleeding or infection.

  • Family education is crucial for ongoing care after discharge, encompassing bladder catheterization and prescribed physical exercises.

Prognosis

• The prognosis varies depending on the neurological deficits present at birth.

• Although medical advancements improve care, persistent chronic disabilities and complications are not entirely preventable.

Cerebral Palsy (CP)

• Definition: Cerebral palsy encompasses a range of non-progressive disorders that result in motor dysfunction due to either non-typical brain development or injury, often occurring in utero or perinatally.

• Prevalence: It affects approximately 3.3 per 1,000 live births and is more frequently diagnosed in males compared to females.

Types of Cerebral Palsy

1. Spastic Cerebral Palsy:

   • The most common type, it constitutes around 70% of CP cases and is characterized by increased muscle tone (hypertonia), leading to stiff and tight muscles.

   • This condition results in jerky, awkward movements due to upper motor neuron lesions affecting muscle control.

2. Dyskinetic/Athetoid Cerebral Palsy:

   • It stems from damage to the basal ganglia and is marked by fluctuating muscle tone and uncontrolled movements such as dystonia (involuntary muscle contractions) and chorea (rapid, irregular movements).

3. Ataxic Cerebral Palsy:

   • Resulting from cerebellar damage, this type leads to uncoordinated movements and a shaky, unstable posture affecting balance.

Causes of Cerebral Palsy

• Most cases are believed to develop prenatally due to:

  • Infections during pregnancy, exposure to radiation, or oxygen deprivation.

• Postnatal factors include head trauma and infections.

• Genetic mutations contribute to a small fraction of cases.

Clinical Manifestations

• Individuals may present with symptoms such as:

  • Abnormal muscle tone and poor coordination.

  • Delayed developmental milestones in areas like walking, speaking, and completing tasks requiring fine motor skills.

  • Other indicators may include excessive drooling, difficulty swallowing, and exaggerated reflexes.

  • There's variability in limb involvement which can manifest as quadriplegia (all four limbs), diplegia (two limbs, typically legs), hemiplegia (one side of the body), or monoplegia (one limb).

Medical Management

• While there is no cure for CP, management focuses on enhancing function and independence through various treatments, including:

  • Medications such as Botox for spasticity and baclofen for severe cases.

  • Surgical interventions and incorporating a range of therapies: physical, occupational, and speech therapy aimed at improving communications skills and daily living activities.

Nursing Interventions

• Emphasis on the early diagnosis and initiation of therapy as essential components of effective management.

• Utilizing adaptive equipment (braces, walkers) to assist in daily living activities and promote independence.

• Providing education to parents about therapeutic exercises, feeding strategies, and proper use of mobility aids.

Conclusion

• Key takeaway: A thorough understanding of the definitions, types, clinical manifestations, and treatment protocols for both spina bifida and cerebral palsy is essential.

• Continuous education and awareness of available community resources are crucial for providing support and improving patient care.