​Kembara Xtra - Medicine- Cerebral Palsy

​Kembara Xtra - Medicine- Cerebral Palsy 
Introduction 

A collection of clinical conditions known as cerebral palsy (CP) are defined by motor and postural dysfunction as a result of long-lasting, nonprogressive abnormalities in the developing brain. A motor disability that limits activity is required for this diagnosis. The type of mobility dysfunction and the functional severity of CP are used to categorize it.

Epidemiology (Incidence and Prevalence) Incidence: 1.5 to 3.0 per 1,000 live births overall; incidence rises as gestational age (GA) at birth falls:
The incidence rises as birth weight decreases: - 146/1,000 for GA of 22 to 27 weeks; - 62/1,000 for GA of 28 to 31 weeks; - 7/1,000 for GA of 32 to 36 weeks; - 1/1,000 for GA of 37+ weeks.


Pathophysiology and Etiology 

 Multifactorial; CP is caused by lesions or static injuries to the developing brain that can happen before birth, during pregnancy, or after birth.
Free radicals, cytokines, and the inflammatory response are probably contributing elements.
The most frequent kind of cerebral palsy (CP) is spastic, and it usually results from either periventricular leukomalacia or germinal matrix hemorrhage.
Genetics There have been studies linking CP to polymorphisms in the thrombophilic, cytokine, and apolipoprotein E genes.

Risk factors include breech birth, preterm delivery, low birth weight, periventricular leukomalacia, perinatal hypoxia/asphyxia, intrauterine growth retardation (IUGR), multiple gestations, in utero stroke, clinical and histologic chorioamnionitis, antepartum bleeding, maternal factors (cognitive impairment, seizure disorders, hyperthyroidism), and abnormal fetal position (e.g., breech
Postnatal: increasing hydrocephalus, sepsis, meningitis, encephalitis, traumatic brain injury or stroke,

Prevention
Effective preventive measures include magnesium sulfate, newborn hypothermia, and prenatal corticosteroids.
Using magnesium sulfate to treat women during preterm delivery is neuroprotective for the fetus and may lower the chance of CP. Unknown is the effect on a term fetus.

 Therapeutic hypothermia is beneficial for term babies who endure intrapartum hypoxia.

Accompanying Conditions 

Seizure disorder, behavioral issues, hearing and vision difficulties, and intellectual and speech and language disabilities.

Decreased linear growth and weight abnormalities (underweight and overweight) Osteopenia Bowel and bladder incontinence Orthopedic: contractures, hip subluxation/dislocation, scoliosis (60%) GI conditions: constipation (59%), vomiting (22%), gastroesophageal reflux Poor dentition, excessive drooling GI conditions: gastroesophageal reflux

Guidelines for an early and precise diagnosis are as follows:
- International recommendations for CP early diagnosis before the age of 12 months: Neuroimaging, the Prechtl General Movements Assessment (GMA) before five months, and the longitudinal use of the Hammersmith Infant Neurologic Examination (HINE) (7) between three and twelve months are all tools for detection.


A clinical diagnosis that takes into account the following factors: - Delayed motor milestones; - Abnormal tone; - Abnormal neurologic exam suggesting a cerebral etiology for motor dysfunction; - Absence of regression (not losing function); - Absence of underlying disorders or other explanation for etiology
Although the pathologic lesion is unchanging, the infant's growth and development may cause changes in the clinical appearance.

Introducing History 

Discover the risk factors that are prenatal, perinatal, and postnatal.
the timing of motor milestones; poor nutrition; frequent vomiting; agitation; abnormal spontaneous general movements; and neurobehavioral symptoms.

 Asymmetries in movement, such as an early predilection for the hand
CP does not cause motor abilities to regress.


clinical assessment 
Spasticity causes increased tone, reflexes, and clonus; Dyskinesia causes abnormal movements; Hypotonia causes decreased tone; Ataxia causes abnormal balance and coordination; Tone can be increased or decreased; Trunk and head control are frequently poor but can be advanced due to high tone; Reduced strength and motor control; Persistence of primitive reflexes; Asymmetry of movement or reflexes; Decreased joint range of motion and contractures; Brisk deep tendon reflexes; Clon CP is categorized as follows:
- Spasticity Unilateral: hemiplegic Bilateral: quadriplegic (UE LE involvement) or diplegic (lower extremity [LE] > upper extremity [UE] involvement)
- Dystonia: increased rigidity and decreased mobility
Ataxia is a loss of ordered muscular coordination. Choreoathetosis is an irregular spasmodic involuntary movement of the limbs or face muscles. UE and fine motor skills can be evaluated using the Manual Ability Classification System (MACS).

Multiple Diagnoses 
Muscular dystrophy, benign congenital hypotonia, brachial plexus injury, familial spastic paraplegia, dopa-responsive dystonia, transient toewalking, metabolic illnesses (like glutaric aciduria type 1), mitochondrial disorders, and genetic abnormalities (like Rett syndrome) are just a few examples of conditions that could be present.

Diagnostic tests and laboratory results 
Although laboratory testing is not necessary to make a diagnosis, it might aid in ruling out alternative causes.
Testing for metabolic and genetic abnormalities should be taken into consideration if neuroimaging cannot identify a specific etiology or if the clinical presentation has unusual characteristics. In order to detect syndromes, certain brain abnormalities may require genetic or metabolic testing.
Children with cerebral infarction detected on neuroimaging due to hemiplegic CP should be given consideration for diagnostic testing for coagulopathies.

Initial examinations (lab, imaging)
Neuroimaging is not necessary, however it is advised for kids with CP whose etiology has not been determined.

CT should be replaced with MRI.

 80–90% of patients have one or more of the following abnormalities: periventricular leukomalacia, ventricular enlargement, intraventricular or other intracranial bleeding, brain malformation, cerebral infarction, or other abnormalities of the CSF space.

Diagnostic Techniques/Other Screening for comorbid conditions, such as feeding/swallowing issues, seizures, vision/hearing issues, speech and language abnormalities, and developmental delay/intellectual impairment.
Only if there is a history of possible seizures should electroencephalograms (EEGs) be taken.

Treatment 
Treatments that reduce spasticity to prevent painful contractures, manage comorbid disorders, and improve functionality and quality of life are centered on symptom control.

Early intervention for children ages 0 to 3 is a general treatment.
Several treatments improve function:
- Physical therapy to strengthen the muscles of the body, regulate movement, and avoid contractures.
- Occupational therapy to improve daily life activities that are functional
- Speech therapy to improve nutrition and verbal and nonverbal communication. - Equipment that maximizes activity participation:
Spinal bracing (body jacket) and orthotic splinting (ankle-foot orthosis) may delay the progression of scoliosis.
- Augmentative communication for nonverbal people using images, switches, or computers
- Wheelchairs, crutches, walkers, gait trainers, and standers for weight bearing


First Line of Medicine

Diazepam, a GABAA agonist that enables CNS inhibition at the spinal and supraspinal levels to diminish spasticity, is used as a temporary remedy for generalized spasticity; however, there is inadequate data on its effects on motor function.
– Negative consequences include ataxia and sleepiness
Pediatric dosage (children under 12 years old and under 15 kg): 0.5 to 1.0 mg HS; 8.5 to 15.0 kg: 1 to 2 mg HS; youngsters between the ages of 5 and 16 and under 15 kg: 1.25 mg TID
Botulinum toxin type A is injected directly into targeted muscles to treat localized spasticity; there is insufficient data on how it affects motor function. It has a higher functional benefit when combined with occupational therapy, and it lasts for 12 to 16 weeks after injection.

Next Line
A GABAB agonist called baclofen makes it easier for mono- and polysynaptic reflexes to be presynaptically inhibited.
Sedation and sleepiness are side effects.
- Symptoms of abrupt withdrawal include hyperthermia, hallucinations, convulsions, spasticity, and seizures.
Adults: Start with a dose of 5 mg TID and raise it every 3 days to a maintenance dose of 20 mg TID on average, with a daily maximum of 80 mg.
- Initial dose for children (>2 years): 10 to 15 mg/day. Increase to the highest effective dose of 40 milligrams per day. 60 mg/day maximum for children under 8; 60 mg/day maximum for children over 8
The continuous intrathecal method of baclofen (the baclofen pump) allows for a higher maximal response with a lesser dosage to alleviate spasticity.
- Adverse consequences include infection, catheter dysfunction, and CSF leakage - May improve gait in ambulatory adults but does not appear to benefit nonambulatory patients

Furthermore Treated 
Ophthalmology, neurology, orthopedics, physiatry, and physical, occupational, and speech therapists are all included in the multidisciplinary care.

Surgical Techniques 

 Dorsal root rhizotomy preferentially removes L1–S2 dorsal rootlets. Lower limb spasticity is reduced when combined with PT, although there are side effects. Regarding long-term results, there is insufficient evidence.
Surgery for gastrostomy, tendon lengthening, scoliosis management, joint subluxation, and dislocations

Suppportive Therapy 

Aquatherapy enhances gross motor function in patients with a range of motor severities, while therapeutic horseback riding and hippotherapy help patients with balance and postural control.
PERIODICAL CARE PROGNOSIS
Reduced lifespan is closely related to the severity of intellectual disability and functional impairment.