Critical Thinking and Leadership Skills Quiz

SCI and PD Review

• Review on UMNL: SCI and PD
• Presenter: John Matthew D. Zarate, PTRP
• Course: ACEP 2

Spinal Cord Anatomy

• Gross Anatomy: Includes white and gray matter, dorsal root ganglion, sensory neuron soma, ventral root, spinal nerve, central canal
• Bell-Magendie Law: Sensory information travels in the dorsal root ganglion and motor information travels in the ventral root
• Rexed Lamina Classification: Specific laminae and their associated nuclei
• Lamina I (postero-marginal sulcus) - pain, light touch, pressure, temperature
• Lamina II (Substancia Gelatinosa) - pain, light touch, pressure, temperature
• others include Nucleus Propius, Nucleus Intermediomedalis, Dorsal Nucleus of Clark, Sacral Autonomic Nucleus, Anterior horn cell, Central Gray commissure

Blood Supply of the Spinal Cord

• Anterior Spinal Artery: Formed by the union of two vertebral arteries and descends along the anterior median fissure, supplying the anterior 2/3 of the spinal cord
• Posterior Spinal Arteries: Originate from vertebral arteries and descend along the posterior surface of the spinal cord close to the posterior nerve root, supplying the posterior 1/3 of the spinal cord

Ascending Pathways Detailed

• Spinothalamic Tract: Carries pain and temperature sensations
• First-order neuron: Sensory neuron.
• Second-order neuron: Association neuron.
• Third-order neuron: Projecting fiber to sensory region in cerebral cortex.
• Dorsal Column-Medial Lemniscus Pathway: Carries fine touch, vibration, and proprioception sensations
• First-order neuron: Sensory neuron in dorsal root ganglion.
• Second-order neuron: Nucleus gracilis or nucleus cuneatus.
• Third-order neuron: Ventral posterolateral nucleus (VPLN) of thalamus.
• N1 to N3 info about pathways.
• Spinocerebellar Tracts: Carry proprioceptive information to the cerebellum
  • ASCT (Anterior Spinocerebellar) and PSCT (Posterior Spinocerebellar) and Cuneocerebellar tract
• Other ascending pathways include other important tracts
• The posterior and anterior spinothalamic tracts carry sensory information for various sensations

General Senses and Other Senses

• General Sense: Sensory information path involving three neurons: First-order, second-order, and third-order neuron
• Includes pain and temperature sensations transmitted via the Lateral Spinothalamic Tract (STT) and crucially, light touch and pressure sensations via the Anterior Spinothalamic Tract

Spinal Cord Syndromes

• Total Cord Syndrome: Complete transverse lesion interrupting all ascending and descending pathways, affecting bladder and bowel function, and sexual function (erection, ejaculation, orgasm, and decreased vaginal lubrication).
• Brown-Sequard Syndrome: Damage to one side of the spinal cord resulting in ipsilateral loss of proprioception, decreased reflexes, loss of superficial reflexes, and clonus; and contralateral loss of pain, temperature sense.
• Anterior Cord Syndrome: Flexion injuries to the cervical region causing damage to the anterior portion of the spinal cord; resulting motor loss and loss of pain and temperature, but proprioception and kinesthesia are preserved.
• Central Cord Syndrome: Hyperextension injuries to the cervical region primarily affecting the upper limbs compared to the lower ones.
• Posterior Cord Syndrome: Extremely rare, presenting with loss of proprioception, kinesthesia, and two-point discrimination, while sparing pain and light touch sensations.
• Sacral Sparing: Incomplete lesions that may spare function of sacral segments (S4-S5), including external anal sphincter contraction and perianal sensation

Cauda Equina Injuries

• Cauda Equina Injuries: Lower motor neuron lesion occurring below the conus medullaris where regeneration may not occur along the original nerve distribution. Significant distance between the lesion and point of innervation hinders proper function. Factors like glial-collagen scarring and end organ dysfunction might also occur.

Conus Medullaris Injuries

• Conus Medullaris Injuries: Clinically similar to cauda equina syndrome, typically presenting with lower motor neuron deficits (flaccidity).

Clinical Manifestations

• Spinal Shock: A period of areflexia and loss of sensation and motor function below the level of the spinal lesion, lasting from days to weeks. Spinal shock is characterized by the absence of all reflex activity, the loss of the bulbocavernosus reflex, the cremasteric reflex. delayed plantar response (Babinski). and flaccidity.
• Autonomic Dysreflexia: Lesions above T6 lead to hyperreflexia, hypertension, bradycardia. Profuse sweating, headache, and piloerection are common manifestations.
• Postural Hypotension: Orthostatic Hypotension resulting in decreased blood pressure, tachycardia, dizziness, lightheadedness, and faintness.

Indirect Impairments and Complications

• Pressure Sores: Commonly occur in sacrum and ischial tuberosities due to prolonged immobility or pressure; Best treatment is prevention through turning.
• Deep Vein Thrombosis (DVT): Ankle pumps and range of motion exercises are crucial to prevention.
• Contractures, selective stretching, osteoporosis (Wolff's law). Higher levels of spinal cord injuries usually contribute to a higher risk of pulmonary complications.

ASIA Classification

• Complete Injury: No sensory or motor function in the lowest sacral segments (S4 and S5); determined by anal sensation and voluntary external anal sphincter contraction.
• Incomplete Injury: Motor and/or sensory function below the neurological level (including sensory and/or motor function of S4 & S5), is present; motor and sensory levels are graded 3/5 or better for complete motor function.
• Motor Level: The lowest key muscle group graded 3/5 or higher; cephalad levels graded normal (5/5) strength.
• Sensory Level: The most caudal segment with normal sensory function (pinprick and light touch).
• Neurological level of injury (NLI) - The most caudal level at which both sensory and motor functions are intact.
• Zone of Partial Preservation (ZPP) - Used for complete injuries, referring to dermatomes and myotomes below the NLI that are partially innervated; used to determine the extent of the dysfunction affecting the spinal cord.
• Deep Anal Pressure (DAP) - Insertion of the examiner's finger and gentle pressure to the anorectal wall.
• Voluntary anal contraction (VAC) - Tested on the basis of reproducible voluntary contractions around the examiner's finger.

Functional Outcomes (based on spinal cord level)

• Outcome charts displaying likely functional outcomes relative to the level of the spinal injury. Likely assistance tools, or functional outcomes vary based on the level of the injury. Data include Walker, Swing to 4-Point, KAFO, Loftstrand, and AFO

Parkinson's Disease (PD)

• Idiopathic Parkinsonism: A chronic, progressive nervous system disorder with cardinal features including tremor, rigidity, bradykinesia, and postural instability. Usually seen in later (and some cases early) stages of life (70 - 79 years of age).
• Risk factors: Age, toxic exposures (cyanide), heavy metals (e.g., manganese, iron, copper, lead, aluminum, zinc), infection exposures (certain viruses), carbon monoxide, and methanol.
• Neuropathologic features: Mild atrophy of frontal cortex and ventricular dilation. Loss of darkly pigmented area in the substantia nigra pars compacta. Depletion of 70-80% of dopamine before clinical signs. Substance nigra are exposed to high levels of oxidative stress.
• Hallmarks: The presence of abnormal cytoplasmic deposits in neuronal cell bodies within the brain, which are immunoreactiv. a-synuclein, and are known as Lewy bodies.
• Classification of idiopathic PD: Late-onset (most common), Early-onset, Young-onset, and Juvenile.
• Classifications of secondary PD: Postinfectious, toxic, and drug-induced.
• Metabolic causes - calcium metabolism disorders, hypothyroidism, hyperparathyroidism, hypoparathyroidism, and Wilson's disease.
• Parkinson-Plus Syndromes - neurodegenerative diseases affecting substantia nigra, and doesn't respond to levodopa. Include symptoms, onset, and other characteristics of neurological damage.
• Dementia Pugilistica: A form of dementia resulting from repeated concussions or head traumas. Has progressively declining cognitive ability.

Clinical Manifestations of PD

• Cardical Features: Tremor (resting), Rigidity, Bradykinesia (Akinesia), and Postural instability, and other characteristics, including gait, balance and other related motor tasks.
• Bradykinesia: Describes slowness of movement, and slow thinking
• Micrographia: Abnormally small handwriting style that is difficult to read.
• Freezing episodes (motor blocks): Short lived, and often overcome with attentional, and other behavioural tricks using external cues.
• Tremor: Resting tremor is most common, and is often suppressed with activity or sleep.
• Postural tremor: Movement exhibited when muscles are used to maintain posture against gravity.
• Action tremor: Often observed in advanced stages of disease and is associated with movement against gravity.
• Simian posture: a posture that is characterized by having the body slumped forward over, in conjunction with protracted shoulders and flexed hips and knees. Often involves affected postural reflexes that increase individuals' tendency to fall backward or to the side.
• Shadow pillow posture: Demonstrates postural presentation when the patient is in supine position.
• Striatal hand and foot characteristics: Demonstrates presence of hand and foot postures.
• Gait: Different gaits are associated with PD including a shuffling gait (progressive decrease in speed, stride, decrease in BOS and arm swing), festinating gait (progressive increase in speed, shortening of stride, and multiple steps to catch up with centre of gravity for balance), and freezing gait (hesitation or difficulty initiating movement).
• Sensory features: Paresthesia and pain (numbness, tingling, aching, cold, and burning sensations), Postural stress syndrome.
• Other symptoms: impaired swallowing, dysphagial swallowing, choking, aspiration pneumonia, impaired nutrition with significant weight loss, and Excessive drooling (sialorrhea)
• Speech disorder: Hypokinetic dysarthria - characterized by decreased voice volume, monotone/monopitch speech, imprecise or distorted articulation, and uncontrolled speech rate. Often having a hoarse, breatht and harsh tones during speaking. Advanced cases exhibit speech in whispers or entirely mutism.
• Cognitive Dysfunction: Hallucinations, delusions, and psychosis, Bradyphrenia (slowed thinking), visuospatial deficits (vertical perception, topographic orientation, body scheme, and spatial relationships), and PD dementia which occurs in 20% to 40% of patients.
• Abnormal reflexes: Palmomental reflex, Myerson's sign, and glabellar tap

Motor Neuron Diseases (MNDs)

• Description: A heterogeneous group of diseases affecting motor neurons leading to progressive loss of muscle strength and function. Amyotrophic Lateral Sclerosis (ALS), Primary Lateral Sclerosis (PLS), and Progressive Muscular Atrophy (PMA) are common forms.
• ALS is commonly known as Lou Gehrig's or Charcot's Disease and is marked by gradual degeneration and loss of motor neurons in spinal cord, brainstem, and brain.
• Classifications: Spraodic (most common), Juvenile, familial includes autosomal recessive and dominant ALS and Gumanian ALS.
• Pathophysiology: Degeneration of motor neurons in cortex, brainstem, anterior horn, and spinal cord.
• Possible underlying causes include: Superoxide Dismutases, Glutamate, Autoimmune reaction, Lack of neurotrophic factors, or other environmental factors including viral infection (exogenous or environmental). TDP-43 protein plays a role.
• Clinical manifestations: Muscle weakness (focal, asymmetric, beginning in LEs, UEs, or bulbar.), atrophy, fasciculations, hyporeflexia or areflexia, hypotonia or flaccidity, muscle cramping, spasticity, and other symptoms related to bulbar dysfunction, respiratory impairment, and cognitive issues.

Diagnosis of MNDs/ALS

• Laboratory studies: EMG, nerve conduction studies (NCV), and muscle/nerve biopsies.
• Neuroimaging studies: Used to evaluate for other diseases that can potentially have overlapping symptoms.

Prognostic Factors for ALS

• Good prognosis: Younger age of onset (<35-40), limb onset, good psychological well-being
• Poor prognosis: Bulbar onset ALS, pulmonary dysfunction, LMN onset, short time period of S/Sx from onset, presence of executive or fronto-temporal dementia.

Management of PD

• Sinemet (Levodopa + Carbidopa): The gold standard for PD treatment, to help Dopamine reach brain. Carbidopa helps prevent dopamine from being broken down before affecting the brain.

Dysinesias

Occur typically at peak L-dopa dose or when the patient is transitioning between "on" and "off" states. Also presented as choreo-athetosis-in quality. Initially appear as facial grimacing, twitching of the lips and tongue protrusion.

Early-Stage Treatment (<5 years)

• Dopamine Agonists
• MAO-B Inhibitors
• Anticholinergics

Other Movement Disorders

• Huntington's disease: Characterized by involuntary movements (chorea) that gradually take on Parkinson-like features later on. Also have dementia and behavioral changes.
• Friedrich's Ataxia: Hereditary and progressive disorder. Has characteristics including limb and gait ataxia, diminished MSRs, joint position sense, and sensory appreciation. Also commonly present are foot and lower limb weaknesses, scoliosis.
• Athetosis: Slow, involuntary, writhing, and twisting movements, often involving the distal upper extremities. Can occur as an isolated movement disorder and is frequently observed with normal intellect.
• Chorea: Rapid, irregular, and jerky movements involving multiple joints. Can be used as a term to describe an individual's abnormal movements, and is associated with Huntington's disease,
• Choreoathetosis: Describes a more combined movement disorder involving chorea and athetosis.
• Dystonia: Sustained involuntary muscle contractions of agonist and antagonist muscles that can cause abnormal posturing and twisting movements. Often includes fluctuating muscle tone that ranges from normal, low to extreme hypertonia.
• Hemiballismus: Large-amplitude, violent, and flailing movements of one side of the body, frequently associated with damage in the subthalamic nucleus (located in the basal ganglia)

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