Pediatric Neurological and Neuromuscular Conditions



Pediatric Neurological and Neuromuscular Conditions







TERMS




QUICK LOOK AT THE CHAPTER AHEAD



  • Children with neuromuscular and neurological disease require a thorough assessment and monitoring of all systems.


  • Head injuries can be described as mild, moderate and severe and are often assessed using the Glasgow Coma Scale.


  • Hydrocephalus is caused by an obstruction of cerebrospinal fluid or impaired absorption of cerebrospinal fluid.


  • Children with neuromuscular conditions require collaboration of several disciplines such as physical therapy, occupational therapy, speech, and social work in order to maximize their growth and development.


There are two basic assumptions of neurodevelopment in children:



  • Early identification leads to better outcomes.


  • Comprehensive, multidisciplinary, and continuity of therapeutic services are important components for reaching potential development.

Table 42-1 lists the developmental, health, and psychosocial considerations to apply in the following pediatric neurological and neuromuscular conditions.









Table 42-1 Developmental, Health, and Psychosocial Considerations





















































General
Considerations

Assessments and Needs

Health Promotion and Illness
Prevention


Perinatal history impact


vs. acquired or progressive degenerative disorder

Prematurity or low birth weight


Teratogenic exposure


Genetic condition


Anoxia/hypoxia or trauma to CNS


Infection (local or systemic)


tumor/mass; capsulated or invasive cancerous, fatty (lipoma), or arteriovenous malformation (AVM)

Comprehensive patient and family history


Ongoing laboratory, diagnostic imaging, and multidisciplinary assessments


Coordinated primary, specialty, and rehabilitative care


Sensory manifestations

Cranial nerve involvement


Deficits in any of the 5 senses (sight, hearing, smell, taste, touch)


Impairments in speech or language communication

Vision, auditory, and speech and language screenings at developmental milestones and primary care visits


Therapeutic and adaptive equipment intervention


Muscle tone and motor control abnormalities

Muscle tone strength, weakness, paralysis, or dyskinesia of movement (i.e., spasticity, ataxia, choreiform)


Deep tendon, primitive and pathological reflexes (tonic neck, Babinski, etc.)


Range of motion


Coordination


Delays in milestones


Head, trunk, limb control


Postural control and alignment

Low or high muscle tone at risk for developmental delays, deformities, and injury


Postural and limb supports


Positioning and prevention of deformities 24/7


Adapted equipment for ADLs, seating, and sleeping


Degree of personal support and equipment needs may vary depending on the environment (household or community) and level of endurance


Anomalies/other congenital defects

Associated anomalies or comorbid conditions

Comprehensive and coordinated management for promoting health and functional status


Alterations in physical growth and sexual development

Identify adjusted growth charts for diagnoses (Down’s syndrome) if available

Assessments, interventions, and psychosocial supports for growth delays, short stature, precocious puberty


Behavior/emotional


Intellectual/educational


Vocational

At risk for lower self-concept and isolation, inappropriate behaviors


Attention deficits (heightened or lower) disorders


Mood or psychiatric disorder


Sleep disorders


Cognitive delays due to localized or generalized CNS deficits

Psychological and neuropsychological evaluations and interventions


Peer activities: support groups, camps, and recreational opportunities


Nutritional needs

Adequate fluid intake


Timing and frequency


Special dietary needs


Augmented feeding; assistance, parenteral, gastrostomy


Gastroesophageal reflux disease evaluation

Promotion of growth and development


Dietary/nutrition consultation


Supplementary feeding program


Exercise and weight management program


ADLs; toileting and hygiene

Bowel and bladder programs that include behavioral, pharmacological, and dietary components


Personal care assistance needs


Personal care during menses

Habilitative/rehabilitative programs for incontinence


Age-appropriate self-management training in personal care


Reinforcement of privacy and confidentiality


Alteration in sexual health care needs

Alterations in sex organs or development


Risk for precocious or delayed puberty


Incontinence, infertility, and/or impotency issues


Pregnancy risks for fetus or mother

Evaluation of sexual functioning and fertility


Reinforcement and support for risk of sexual abuse


Encourage social supports and circle of friends that can build an environment of trust, empowerment, and freedom of expression


Adaptive equipment; recreational, educational, and vocational

Need for communication aids; orthotics, trunk, or limb braces; ambulation aids or wheelchairs; adaptive seating

Assessments of needs at different developmental, educational, and societal milestones with identification of resources and suppliers


Comfort, safety, and emergency measures

Altered status of consciousness


Breathing difficulties due to positioning, obstruction, and/or chronicity


Skin breakdown


Muscular skeletal trauma or falls


Risk for abuse or neglect


Allergies and toxicities


Poor progressive prognosis, DNR needs

Ongoing assessment and priority of comfort and safety across environments of care


ADLs, activities of daily living; DNR, do not resuscitate.




ACQUIRED BRAIN INJURY

An acquired brain injury (ABI) is defined as an insult to the central nervous system (CNS) that impedes function most often due to hypoxia or anoxia (reduced oxygen to the brain due obstructed airway, near drowning, electrocution, etc.), a traumatic event such as blunt trauma to the head or speed-associated injury (motor vehicle or fall), or CNS cell damage from a tumor, mass (arterial venous malformation), or bleed. The encephalopathy event occurs after the age of 2 years or is most often classified as cerebral palsy (below). ABI often impacts development and requires various acute and/or chronic treatments and rehabilitation programs.


Pathophysiology

The ABI event and the impact of decreased blood flow, oxygenation to the tissues of the brain, and resulting inflammatory effects may cause damage to cell tissues and scarring that are localized or diffuse and global. Sequelae may be mild to severe and may include the following associated disorders:




  • Trauma to the scalp or face (laceration) or cranium (skull fracture)


  • Period of loss of consciousness, concussion, and/or comatose state (Table 42-2)


  • Period of increased intracranial pressure


  • Seizure disorder


  • Cognitive, learning, and/or vocational deficits


  • Sensory deficits (vision, hearing, taste/smell)


  • Language and/or communication deficits (aphasia, dysphagia)


  • Behavioral or mental health disorders


  • Sensory-motor-movement disorder (e.g., emiplegia/paresis, ataxia, etc.)


Treatment

There are three phases of treatment:



  • Emergency and critical care: Focus is on preserving brain homeostasis and managing intracranial pressure and any other injuries.


  • Acute rehabilitation (usually the first 2 months after the event): Focus is on preventing complications, orienting the patient, patient/family teaching and support, and safety measures.


  • Long-term rehabilitation and multidisciplinary follow-up.


CEREBRAL PALSY

Cerebral palsy (CP) is an umbrella term for a group of CNS conditions that occur pre- or perinatally or early in life (often classified as CP if diagnosed
before 2 years of age). Conditions may be mild to severe, and further classifications of CP are complex and definitions have varied worldwide. Classifications of CP include severity (mild, moderate, or severe), type of movement (spastic, athetoid, or other dyskinesia), and the parts of the body involved (quadriplegic/paresis, hemiplegic/paresis, or paraplegic/paresis). Dysarthria is the impaired, stammering, or slurred speech pattern and dysphagia is the swallowing difficulty resulting in drooling that may be associated with CP.








Table 42-2 Glasgow Coma Scale to Assess Level of Consciousness


















Eye opening

Spontaneous opening (4), to loud noise (3), to pain (2), no response (1)


Verbal response

Smiles, uses words (5), irritable, confused (4), inappropriate crying or words (3), grunts, incomprehensible sounds (2), none (1)


Motor response

Spontaneous movement (6), withdraws to touch (5), withdraws to pain (4), abnormal flexion (3), abnormal extension (2), none (1)



Pathophysiology

The underlying etiology is not progressive (although sequelae may be due to growth factors and associated conditions) and includes the following:



  • Maternal factors during pregnancy (high parity, thyroid disorder, history of pregnancy loss, teratogen/substance abuse exposure)


  • Congenital viral infections


  • Intraventricular bleed in premature neonates


  • Low birth weight


  • Birth asphyxia


  • Hypoxic episode or ABI during infancy

Neuroimaging (static and dynamic) is critical to identify the extent and location of brain damage and presence or absence of anomalies. Neurodevelopmental evaluations, electrophysiological studies, and multidisciplinary comprehensive assessment are essential during infancy, performed as needed in early childhood, and used for long-term follow-up to rule out genetic or progressive disorders and support treatment programs.

Tags:
Oct 17, 2016 | Posted by in NURSING | Comments Off on Pediatric Neurological and Neuromuscular Conditions

Full access? Get Clinical Tree
Get Clinical Tree app for offline access