Cerebral Palsy and Progressive Supranuclear Palsy Quiz

Progressive Supranuclear Palsy (PSP)

• Deterioration of cells in the brainstem, cerebral cortex, cerebellum and basal ganglia causes coordination and movement issues.
• Impairs voluntary eye movements, particularly looking up or down.
• Slowed movements, muscle rigidity occur.
• Difficulty controlling gait and balance.
• It progresses but isn't directly life-threatening but can lead to complications such as pneumonia due to swallowing difficulties.
• Choking, pneumonia, head injury, and fracture due to falls are common complications.

Guillain-Barre Syndrome (GBS)

• Disorder affecting the peripheral and cranial nerve axons.
• It's a demyelinating polyneuropathy, where the body's immune system attacks its own nerves.
• The immune system attacks the nerves in the peripheral nervous system, which connects the brain to the body and transmits signals to muscles.
• Damage to these nerves prevents muscles from responding to brain signals.
• First symptom is usually tingling in toes, feet, and legs, spreading upward to arms and fingers.
• Affects both children and adults, but usually adults. Peak incidence in children is between 4 and 10 years.

Cerebral Palsy (CP)

• A group of non-progressive conditions characterized by abnormal motor control and posture.
• Caused by brain insults or injuries during prenatal, perinatal, or infant development.
• A group of permanent, but not unchanging, disorders of movement and/or posture and of motor function.
• It is due to a non-progressive interference, lesion, or abnormality of the developing brain.
• Describes a group of permanent disorders of the development of movement and posture, causing activity limitation.
• Attributed to non-progressive disturbances that occurred in the developing fetal or infant brain.
• Motor disorders are often accompanied by disturbances of sensation, perception, cognition, communication, and behavior, by epilepsy, and by secondary musculoskeletal problems.

Causes of Cerebral Palsy

• Etiology is complex and a number of factors can damage the central nervous system (CNS) at an early stage of its development.
• Risk factors are categorized into pre-conception, prenatal, perinatal, and neonatal/infant periods.

Pre-conception Risk Factors

• Mother's systemic diseases (e.g., high blood pressure, diabetes).
• Drug and stimulant use.
• Malnutrition.
• Poisoning.
• Infections.
• Immune system disorders preceding pregnancy.
• Physical and chemical factors.
• Infertility.
• Abortions.
• Socioeconomic status.

Prenatal Risk Factors

• Vaginal bleeding.
• Placental abnormalities.
• Multiple pregnancy.
• Systemic disease during pregnancy.
• Abnormal fetal pulse.
• Intrauterine infections.
• Intrauterine suppression of development.
• Intrauterine hypoxia.
• Premature rupture of membranes.
• Assisted reproductive technology (in vitro fertilization).

Perinatal Risk Factors

• Premature birth.
• Vacuum assisted delivery.
• Forceps delivery.
• Delivery after due date.
• Prolonged labor.
• Asphyxia.
• Aspiration syndrome.
• CNS insults (hydrocephaly, microcephaly).

Neonatal and Infant Period Risk Factors

• Respiratory distress syndrome.
• Artificial respiratory support.
• Infections (meningitis).
• Hyperbilirubinemia.
• Hypoglycemia.
• Hypothyroxianemia.
• Intracranial hemorrhages.
• Neonatal convulsions.

Associated Problems in Cerebral Palsy

• Children with CP generally have several medical conditions associated with the disorder.
• Primary conditions are a direct result of the brain lesion or malformation.
• Associated etiological factors can be classified according to the timing of insult as prenatal (most common), perinatal, and postnatal.

Neuroanatomic (Neuropathologic) Classification of Cerebral Palsy

• Correlates specific radiologic findings (brain structural alterations) with types of CP.
• Relies on neuroimaging studies such as magnetic resonance imaging (MRI) and computed tomography scan (CT scan).
• Abnormal radiological findings and diagnoses are categorized into five categories: malformations, gray matter damage, white matter damage, ventriculomegaly, atrophy or CSF space abnormalities, and miscellaneous findings.

Therapeutic Classification of Cerebral Palsy

• Based on treatment needs.
• Four types: Non-treatment, Modest treatment, Need for a CP treatment team, and Pervasive support groups.

Functional Capacity Classification of Cerebral Palsy

• Functionally, CP is classified into levels of severity based on functional (motor) abilities and/or limitation of activity.
• Class I: No limitation of capacity practically.
• Class II: Mild to moderate limitation.
• Class III: Moderate to severe limitation.
• Class IV: No capacity.

Dysarthria

• A motor speech disorder affecting the muscles used for speech production.
• Occurs due to damage, paralysis, or weakness of these muscles.
• The affected area of the nervous system determines the type of dysarthria and the severity of speech impact.
• Can be congenital, developmental, or acquired.

Characteristics of Dysarthria

• As a child starts to talk, dysarthria might become noticeable through slurred speech and an abnormal speaking pattern.
• Speech can range from talking too fast or too slow.
• Damage to mouth and facial muscles limits movement of the jaw, tongue, and lips.
• Difficulty with swallowing and feeding, excessive drooling, and saliva buildup can occur.
• CP is the most common cause.

Dysarthria Definition

• A collective name for speech disorders resulting from disturbances in muscular control over the speech mechanism due to damage of the central/peripheral nervous system.
• Reflects problems in oral communication due to paralysis, weakness, or incoordination of the speech musculature.
• A collective name for a group of neurologic speech disorders resulting from abnormalities in the strength, speed, range, steadiness, tone, or accuracy of movements required for speech production.
• Reflects weakness, spasticity, incoordination, involuntary movements, or excessive or reduced or variable muscle tone.

Childhood Dysarthria Classification

• Classified based on several factors including:
  • Age of onset.
  • Etiology.
  • Site of lesion.
  • Underlying neurological conditions.
  • Clinical features.

Age of Onset in Dysarthria

• Childhood dysarthrias are broadly classified as congenital or developmental or acquired.
• Congenital: Present at birth and also called developmental dysarthrias. Examples include those seen with CP and Moebius syndrome.
• Acquired: Result from disease or event (e.g., traumatic brain injury, stroke, tumor) with onset during childhood (0-15 years) but usually following a period of normal speech and language development.

Etiology of Dysarthria

• Includes eight categories. They encompass a wide range of causes including:
  • Cerebral palsy: Most common cause.
  • Stroke: Can occur in children and adults.
  • Brain tumors: Can affect speech muscles.
  • Infections: Meningitis, encephalitis, and other infections can damage the brain.
  • Traumatic brain injury: Accidents can lead to dysarthria.
  • Neurodegenerative disorders: Huntington's disease and amyotrophic lateral sclerosis (ALS) can cause dysarthria in children.
  • Muscular dystrophies: Can affect speech muscles.
  • Myasthenia gravis: An autoimmune disorder affecting nerve transmission at the neuromuscular junction.

Dysarthria Characteristics

• Specific patterns of manifestation:
  • Diffuse lower motor unit paralysis.
  • Rapid or gradual onset.
  • Sensory loss may be less severe than motor weakness.
  • Bilateral paralysis of the facial nerve (VII).
  • Dysphagia with particular involvement of cranial nerves IX and X.
  • Respiratory function may be impaired because of weak breathing muscles.
  • General prognosis is good, with 65% of patients recovering completely; the rest show persistent weakness and atrophy of the feet and leg muscles.
  • More serious conditions may be life-threatening.

Worster-Drought Syndrome (WDS)

• Also called bilateral perisylvian or opercular syndrome or congenital suprabulbar paresis.
• A type of CP affecting the muscles around the mouth and throat (perisylvian region or operculum).
• Caused by inadequate development of the corticobulbar tracts.
• This abnormality appears to occur in the 12 to 16 week period of pregnancy.

Main Symptoms of Worster-Drought Syndrome

• Drooling.
• Excessive salivation.
• Swallowing difficulties.
• Increased reflexes.
• Speech impairment - severe dysarthria.
• Mild spastic diplegia is common.
• Epilepsy.
• Cognitive and behavioral impairment.

Duchenne Muscular Dystrophy (DMD)

• Disorder of muscles.
• Dystrophies are hereditary myopathies characterized by progressive muscle degeneration and weakness.
• The most common and severe type, also called Pseudohypertrophic muscular dystrophy.
• Onset is between 2 and 5 years; affects boys almost exclusively and progresses rapidly.
• Individuals lack the protein dystrophin, essential for muscular contraction, leading to muscle cell destruction and loss of strength.

Test your knowledge on progressive supranuclear palsy (PSP) and cerebral palsy (CP) with this quiz. It covers symptoms, classifications, and characteristics of these neurological conditions. Challenge yourself to match diseases with their primary characteristics and risk factors related to CP.