Neurology · Pediatric Neurology
The facts most likely to be tested
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Cerebral palsy is a non-progressive disorder of motor function and posture caused by an insult to the developing fetal or infant brain.
The most common risk factor for cerebral palsy is prematurity and low birth weight.
Spastic diplegia is the most common form of cerebral palsy, frequently associated with periventricular leukomalacia in premature infants.
Clinical presentation often includes hypertonia, hyperreflexia, clonus, and persistent primitive reflexes such as the Moro reflex or asymmetric tonic neck reflex.
Diagnosis is primarily clinical based on a history of delayed motor milestones and abnormal neurological examination findings.
Brain MRI is the neuroimaging modality of choice to identify structural abnormalities like porencephalic cysts or cortical atrophy.
Management is multidisciplinary and focuses on physical therapy, occupational therapy, and baclofen or botulinum toxin for the treatment of spasticity.
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A 14-month-old male is brought to the clinic by his mother due to concerns about delayed motor development. The child was born at 28 weeks gestation and spent 6 weeks in the NICU. On physical examination, the child has increased muscle tone in the lower extremities, hyperreflexia at the knees, and bilateral ankle clonus. He is unable to stand independently and exhibits a scissoring gait when supported. The Moro reflex is still present.
What is the most likely diagnosis?
Cerebral palsy
The patient's history of prematurity combined with the clinical findings of spasticity, hyperreflexia, and persistent primitive reflexes is classic for cerebral palsy, specifically the spastic diplegic subtype.
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High yield triage
Etiology / Epidemiology
Non-progressive motor impairment due to prenatal/perinatal brain injury. Major risk factors include prematurity and low birth weight.
Clinical Manifestations
Characterized by spasticity, hyperreflexia, and clonus. Look for delayed motor milestones and scissoring gait.
Diagnosis
Diagnosis is clinical. MRI of the brain is the gold standard to identify structural abnormalities.
Treatment
Management is multidisciplinary. Baclofen is first-line for spasticity. Avoid benzodiazepines for long-term use.
Prognosis
Most patients reach adulthood. Intellectual disability and seizures are the most common comorbidities.
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Epidemiology & Etiology
Cerebral palsy (CP) is the most common cause of childhood physical disability. Etiology is multifactorial, primarily involving hypoxic-ischemic encephalopathy, intrauterine infection, or congenital malformations. Prematurity (<32 weeks) remains the single most significant risk factor.
Pertinent Anatomy
Injury typically involves the pyramidal tracts (spastic CP) or the extrapyramidal system (dyskinetic CP). Damage to the periventricular white matter is classic in preterm infants, leading to periventricular leukomalacia.
Pathophysiology
The condition results from a static encephalopathy occurring during fetal or infant brain development. The injury disrupts motor pathways, leading to abnormal muscle tone and impaired motor control. Because the lesion is non-progressive, the clinical deficit remains stable, though the presentation may evolve as the child grows.
Clinical Manifestations
Presentation includes spasticity, hyperreflexia, and clonus. Infants often exhibit primitive reflex persistence and hypotonia before developing spasticity. Red flags include asymmetric movement or failure to meet developmental milestones by 6 months.
Diagnosis
Diagnosis is clinical based on history and physical exam. MRI of the brain is the gold standard to rule out metabolic or structural mimics. Genetic testing may be indicated if the etiology is unclear.
Treatment
Treatment focuses on maximizing function via physical and occupational therapy. Baclofen (oral or intrathecal) is the first-line pharmacologic agent for severe spasticity. Botulinum toxin injections are used for focal spasticity. Avoid long-term benzodiazepines due to sedation and cognitive impairment.
Prognosis
Life expectancy is generally normal, though dependent on the severity of motor and cognitive impairment. Seizures occur in 30-50% of patients and require anticonvulsant therapy. Regular monitoring for scoliosis and hip subluxation is mandatory.
Differential Diagnosis
Metabolic disorders: progressive course rather than static
Spinal cord injury: sensory deficits present
Muscular dystrophy: Gowers sign and elevated CK
Hereditary spastic paraplegia: family history and progressive weakness
Brain tumors: progressive neurological decline