د.اثل اطفال خامس
ENCEPHALOPATHIESStatic Progressive
Cerebral Palsy
Cerebral palsy (CP) is a diagnostic term used to describe a group of permanent disorders of movement and posture causing activity limitation, that are attributed to nonprogressive disturbances in the in the developing fetal or infant brain.
The motor disorders are often accompanied by disturbances of sensation, perception, cognition, communication, and behavior as well as by epilepsy and secondary musculoskeletal problems.
CP considered a static encephalopathy, but some of the neurologic features of CP, such as movement disorders and orthopedic complications, including scoliosis and hip dislocation, can change or progress over time.
Classification
Topographic subclassification of spastic CP
Types of spastic CP:
Etiological Classification and Risk Factors for CP:
Etiological classification divides CP into :congenital CP
acquired CP
unknown etiology ≃ 50%
Risk Factors of CP can classified according to time of its effect into :
1. PrenatalPrematurity
LBW / IUGR
Intrauterine exposure to maternal infection (chorioamnionitis, inflammation of placental membranes, umbilical cord inflammation, foul-smelling amniotic fluid, maternal sepsis, temperature>38°C during labor, urinary tract infection) was associated with a significant increase in the risk of CP in normal birth weight infants.
Maternal medical problems
Sever toxemia and eclampsia
Bleeding in the 3rd trimester
Drug abuse, drug therapy and toxic exposure
Trauma
Multiple pregnancies
Placental insufficiency
Congenital malformations
2. Perinatal
Prolonged and difficult labor
Premature rupture of membrane
Presentation anomalies
Vaginal bleeding at the time of admission for labor
Bradycardia
Hypoxic ischemic encephalopathy
3. Postnatal
CNS infection: encephalitis and meningitis.Hypoxia.
Seizure.
Coagulopathies.
Neonatal hyperbilirubinaemia (kernicterus)
Head trauma
Clinical Manifestations
and Associated Problems:Neurological:
Muscle weakness.Abnormal muscle tone.
Balance problems.
Pathological reflexes.
Loss of sensation
Musculoskeletal:
Contracture.
Deformities
Associated problems:
Intellectual impairment
Epilepsy
Visual problems
Hearing loss
Speech and communication problems
Feeding difficulty_ FTT
Respiratory problems
Bladder dysfunction
Constipation
Dental defect
Psychiatric problems
Sleeping difficulties
Early Features that make you thinks of CP:
Infants who weigh less than 1500 grams.Infants who have these behavioral symptoms :
Excessive sleeping
Irritability
Weak cry
Poor sucking
May be “floppy”
May be stiff and arched “opisthotonos”
Infants who have abnormalities with Deep Tendon Reflexes :
Increased or decreased tone
Asymmetrical reflexes because one side of the body is affected
Too brisk of a response
Infants who have persistent primitive reflexes.
Infants who have disparities between motor development and cognitive development (motor skills delayed, but cognitive and language skills are on track)
.
Infancy to two years
Hands often remain clenched in fists
Spastic limb atrophies and non- affected limb becomes dominant.
Delays in walking (walking on toes).
Investigations
Imaging studies enable the physician to define the type and location of the brain lesion and to differentiate progressive neurological syndromes.
Radiology
Cranial ultrasonographyCerebral Computerized Tomography (CT)
Cranial magnetic resonance imaging (MRI)
Electroencephalography (EEG)
Management:
A multidisciplinary team approach aiming at maximizing function in patient with CP, these includePhysical therapy
Occupational therapy
Speech therapy
Counseling and Behavioral therapy
5. Drugs:
Oral Medications: are usually 1st line treatments to relax stiff, contracted or over active muscle, such as:
Baclofen
Dantrolene
Diazepam
Neuromuscular Blocking agents:
Botulinum Toxin A: it is a paralyzing agent derived from Clostridium botulinum. It cause muscle relaxation by chemically blocking acetylcholine release at the neuromuscular junction and cause a chemical denervation for 3-6 months.
Local anesthetics
Intrathecal Baclofen: it is one of the most potent antispastic drugs. Studies have shown it reduce spasticity, pain and improve sleep.
6. Surgical management
Orthopedic surgery.
Selective dorsal rhizotomy.
7. Orthotic & assistive devices.
8. Treatment for other condition associated with CP as epilepsy, incontinence, osteopenia, pain, dystonia --- etc.
Selected dorsal rhizotomy
Neurodegenerative DisordersWhen children present with stagnation of development or frank loss of previously acquired skills. These entities are referred to as neurodegenerative disorders.
Degenerative diseases may affect gray matter (neuronal degenerative disorders), white matter (leukodystrophies), both or specific, focal regions of the brain.
Many white and gray matter degenerative illnesses result from enzymatic disorders within subcellular organelles, including lysosomes, mitochondria, and peroxisomes.
Gray matter degeneration (neuronal degeneration)
It is characterized early by dementia and seizures. This group of gray matter disorders, which cause slowly progressive loss of neuronal function, is separated into disorders with and disorders without visceromegaly (hepatosplenomegaly).Most are autosomal recessive traits except for Hunter syndrome (sex-linked recessive), Rett syndrome (sex-linked dominant), and the mitochondrial encephalopathies (nuclear or mitochondrial DNA defects).