Neuro Pathology Pt. 3 Flashcards
A degenerative disease affecting both UMNs and LMNs with degeneration of the anterior horn cells & descending corticobulbar & corticospinal tracts
Amyotrophic Lateral Sclerosis (ALS)/ Lou Gehrig’s disease
Signs and symptoms of ALS/Lou Gehrig’s disease
- Progressive disease often leading to death in 2-5 yrs with highly variable symptoms
- Muscular weakness that spreads over time: early onset of limbs then whole body; atrophy, cramping, muscle fasciculations, or twitching (LMN signs)
- Spasticity, hyperreflexia (UMN signs)
- Sensation is preserved; sparing of bowel/bladder function; normal cognition
- Dysarthria, dysphagia, dysphonia 2ndy to pseudobulbar palsy/bulbar palsy
- Respiratory impairments
Describe the 6 stages of ALS/Lou Gehrig’s disease
- I: mild focal weakness, asymmetrical distribution; sx of hand cramping & fasciculations
- II: moderate weakness in groups of muscles, some wasting; modified independence with ADs
- III: severe weakness of specific muscles, increasing fatigue, mild-mod functional limitations, ambulatory
- IV: severe weakness & wasting of LEs, mild weakness of UEs; mod A & ADs required; wheelchair user
- V: progressive weakness with deterioration of mobility & endurance, increased fatigue, mod-severe weakness of whole limbs/trunk, spasticity, hypperrefelxia, loss of head control, max A
- VI: bedridden, dependent ADLs, FMS; progressive respiratory distress
Purpose of Riluzole in treatment of ALS
- Glutamate antagonist
- May slow progression, prolong survival especially with bulbar onset disease
- Could extend survival by 2-3 months
Weakness or paralysis of the muscles innervated by the motor nuclei of the lower brainstem; affects the muscles of the face, tongue, larynx, & pharynx
- Bulbar palsy
Bilateral dysfunction of corticobulbar innervation of brainstem nuclei; a central or UMN lesion analogous to corticospinal lesions disrupting function of anterior horn cells
- Pseudobulbar palsy
Signs and symptoms of pseudobulbar palsy
- Produces similar symptoms as bulbar palsy
- Examine for hyperactive reflexes: increased jaw jerk and snout reflex (tapping on lips produces pouting of lips)
Signs and symptoms of trigeminal neuralgia (Tic Douloureux)
- Brief paroxysms of neurogenic pain (stabbing and/or shooting pain) reoccurring frequently
- Occurs along distribution of trigeminal nerve; restricted to one side of face
- Autonomic instability: exacerbated by stress, cold; relieved by relaxation
- Light touch to face, lips, or gums will cause pain
Define Bell’s palsy
- CN VII lesion resulting in unilateral facial paralysis of both the upper and lower parts of one side of the face
- Acute inflammatory process of unknown cause resulting in compression of the nerve within the temporal bone
Signs and symptoms of Bell’s palsy
- Muscles of facial expression on one side are weakened or paralyzed
- Loss of control of salivation or lacrimation
- Acute onset commonly preceded by a day or two of pain behind the ear
- Sensation is normal
What are the 3 W’s for examining facial nerve
- CN VII
- Whistle (puff out cheeks)
- Wink
- Wrinkle (forehead)
What are the 3 peripheral nerve injury classifications
- Neurapraxia: nerve injury causing a transient & focal chemical/structural loss of function
- Axonotmesis: focal damage to the axon/myelin & varying degrees of peripheral nerve connective tissue; results in Wallerian degeneration within disrupted axons
- Neurotmesis: severance of axon/myelin & all connective tissue structures to include epineurium; complete loss of nerve function & Wallerian degeneration with no connective tissue path
What 3 neuroplasticity properties relate to the healing of a peripheral nerve injury
- Remyelination: a portion of the lost myelin can be replaced to restore neural conduction
- Axonal regeneration: axons that regenerate often do not remyelinate to their pre injury level
- Collateral sprouting: intact axons can pick up dennervated terminal targets
What are the 4 types of peripheral nerve injuries
- Mononeuropathy: involvement of a single nerve
- Mononeuropathy multiplex: involvement of 2 or more nerves without a clear pattern of polyneuropathy
- Radiculopathy: involvement of nerve root(s)
- Plexopathy: involvement of brachial or lumbosacral plexus
Signs and symptoms to look for in polyneuropathy patients indicating autonomic dysfunction
- Vasodilation and loss of vasomotor tone
- Dryness, warm skin, edema, orthostatic hypotension
Common risk factors for polyneuropathy
- Diabetes
- Renal failure
- Alcohol abuse
- Systemic autoimmune disease
- Autoimmune disease-nerve
- Nutritional imbalances
- Hereditary
- Infections
- Certain cancers
- Medications
- Toxins
- Idiopathic: ~25% of patients
Acute inflammatory demyelinating polyradiculoneuropathy presenting with rapid nonsymmetrical loss of myelin in both nerve roots & peripheral nerves
- Guillain-Barré syndrome (GBS)
Signs and symptoms of GBS
- Acute demyelination of both cranial and peripheral nerves (LMN disease)
- Sensory loss, paresthesias, pain
- Motor paresis or paralysis: relative symmetrical distribution of weakness; may produce full tetraplegia with respiratory failure
- Dysarthria, dysphagia, diplopia, & facial weakness may develop in severe cases
- Progression of days to weeks with slow recovery of ~6 months to 2 yrs
Medical management for GBS
- Plasmapheresis: therapy to remove antibodies causing clinical sx
- IVIG (intravenous immunoglobulin): immunosuppression therapy
- Analgesics: relief of pain
Slow progressive muscle weakness occurring in individuals with a confirmed history of acute polio; follows a stable period (usually 15 yrs or more) of functioning
- Postpolio Syndrome (PPS)
Signs and symptoms of postpolio syndrome (PPS)
- Gradual onset of new muscle weakness or fatigue with or without muscle atrophy or muscle/joint pain
- New symptoms >1 yr
- Abnormal fatigue: doesn’t recover easily with usual rest periods
- Myalgia, cramping, joint pain with repetitive injury, hypersensitivities
- Slow progression
- Environmental cold intolerance
- Sleep disturbances
- Decreased functional mobility, aerobic capacity, labile exercise BPs
Medical management for postpolio syndrome (PPS)
- Antidepressants: amitriptyline (Elavil), fluoxetine (Prozac)
- neurotransmitter inhibitors: decreases fatigue & sleep disorders (serotonin, norepinepherine)
Important PT considerations for postpolio syndrome
- Use whole body movements for aerobic training to avoid overworking involved muscles
- Provide discontinuous non fatiguing exercises with increased rest breaks due to fatigue
- Foster weight control/reduction programs
- Avoid muscle training for patients with severe paresis
A postsynaptic neuromuscular junction (NMJ) disorder characterized by progressive muscular weakness and fatiguability on exertion; autoimmune antibody mediated attack on acetylcholine receptors at NMJ
- Myasthenia Gravis
Signs and symptoms of myasthenia gravis
- Partial ptosis (drooping of upper eyelid) often 1st sign: positive ice pack test
- Progressive dysarthria or nasal speech
- Difficulties chewing or swallowing
- Difficult with facial expressions
- Proximal muscles more involved than distal
- Normal sensation
- Rapid fatigue with repeated muscle use
- Normal tone at rest
- Normal reflexes
- Reduced voluntary movement with reps
- Typically seen in females 20-30 yrs & men/women 60-80 yrs
What are the 4 types of myasthenia gravis
- Ocular: confined to extraocular muscles
- Mild/severe generalized: usually involves bulbar & proximal limb girdle muscles; may progress from mild to severe typically within 18 months
- Crisis: myasthenia gravis with respiratory failure; treat as medical emergency
Define ice pack test
- Positive test is decreased ptosis after a 2 minute application of an ice pack to the affected eyelid
Binds presynaptically to the high affinity recognition sites on the cholinergic nerve terminals thus decreasing the release of acetylcholine
- Botulinum Toxin
- Results in hypotonia and/or flaccid muscles allowing therapist to work on improving flexibility in contracted muscles
Signs and symptoms of an acquired myopathy
- Muscle cramps & pain with exertional fatigue
- Weakness that progresses in a proximal to distal direction
- Pts reports difficulty with overhead activity, getting in/out of chair, and stairs
- Normal sensation
- Elevated creatine phosphokinase (CPK), aldolase, lactate dehydrogenase (LDH), & liver function enzymes
Inflammation of the membranes of the brain or spinal cord; typically caused by an infection
- Meningitis
Symptoms of meningitis
- HA
- Fever
- Stiff neck
- Irritability
- Mental confusion
- Sensitivity to light
- Increased HR and rR
- Sleepiness
- Sluggishness
- Positive Kernig’s sign: pain when extending head with hips/knees flexed to 90º
Inflammation of the brain often due to an infection
- Encephalitis
Difference between primary and secondary encephalitis
- Primary: caused by virus that infects the brain or also by mosquito/tick borne/rabies viruses
- Secondary: caused by a faulty immune system reaction resulting from an infection in another area of the body; immune system attacks healthy brain
Symptoms of mild versus severe encephalitis
- Mild: flu-like sx or no sx; treatment includes bed rest, fluids, & anti-inflammatory drugs
- Severe: confusion, agitation or hallucinations, seizures, muscle weakness or paralysis, loss of sensation, & loss of consciousness
Specific COVID-19 neurological symptoms includes
- Loss of smell
- Impaired taste
- Fatigue
- Muscle weakness
- Numbness or tingling in the hands & feet
- Dizziness
- Confusion
- Delirium
Inflammation of one section of the spinal cord; myelin is damaged with interruption of signals that the spinal cord sends to the body
- Transverse Myelitis
Symptoms of transverse myelitis
- Develops gradually over hours to days
- Usually affects both sides of the body below the level of spinal lesion
- Sharp shooting pains down arms or legs
- Abnormal sensations including N/T, coldness, or burning
- Weakness in arms or legs that may progress to paralysis
- Stiffness, tightness, or painful muscle spasms & spasticity
- Exhaustive fatigue that results in decreased activity levels & lifestyle changes
- Bladder/bowel problems, sexual dysfunction depending on level of lesion
Typical prognosis of transverse myelitis
- Most patients achieve at least a partial recovery
- Most recovery occurs in the first 3mo & may continue up to 2 yrs
- ~1/3 of patients are left with permanent disability after the attack
Rare polio-like condition that affects the motor neurons in the grey matter of the spinal cord causing the muscles & reflexes to become weak; occurs mainly in children
- Acute flaccid myelitis (AFM)
Symptoms of AFM (acute flaccid myelitis)
- Sudden onset of arm or leg weakness
- Loss of muscle tone & reflexes
- Difficulty moving the eyes or eyelid drooping
- Facial droop or weakness
- Difficulty with swallowing or speech
- Pain in neck, back, or limb may be early symptom
- Weakness most often in proximal muscles; can experience paralysis of one or all limbs
Prognosis of acute flaccid myelitis
- Most patients regain some strength over time
- Many do not recover full function
- Most affected muscle may be the least likely to recover
Symptoms of myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS)
- Severe or prolonged fatigue & drop in activity level lasting ≥6mo
- Post-extertional malaise (PEM) after physical or mental activity; fatigue not relieved by rest
- Myalgia (muscle pain)
- Cognitive impairments
- Difficulty sleeping: sleep that isn’t refreshing
- Sore throat that is frequent or recurring; tender lymph nodes
- HAs of a new type, pattern, or severity
- Multiple joint pain (anthralgias) without swelling or redness
- Orthostatic intolerance
- Deconditioning, anxiety, & depression are common
Diagnosis of myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS)
- Must have 2 major criteria: persistent or relapsing fatigue & reduced physical activity for at least 6 months AND 4 or more of the eight symptoms
- More common in women aged 20-30 yrs
Viral syndrome characterized by acquired and severe depression of cell-mediated immunity
- AIDS (acquired immunodeficiency syndrome)
Symptoms of AIDS
- wide ranging but can exhibit CNS or PNS deficits
- AIDS dementia complex: sx range from confusion & memory loss to disorientation
- Motor deficits: ataxia, weakness, tremor, loss of fine motor coordination
- Peripheral neuropathy: hypersensitivity, pain, sensory loss
Define pain
- The sensory and emotional experience associated with actual or potential tissue damage
Describe the pathway for fast, localized pain
- Transmitted over thinly myelinated A delta fibers
- Functions for localization, discrimination of pain
Describe the pathway for slow pain
- Transmitted over small diameter, unmyelinated C fibers
- Stimulates the spinolimbic tract which is associated with the emotional component of pain
Describe gate control theory for pain
- Transmission of sensation at spinal cord level is controlled by balance between large fibers (A alpha/beta) and small fibers (A delta, C)
- Temporal summation of large myelinated fibers may block activity of small fibers & pain transmission (counterirritant theory)
Describe the endogenous opiates theory for pain
- Endogenous opiates produced throughout CNS can depress pain transmission at various sites through mechanisms of presynaptic inhibition
Define acute pain
- Pain provoked by noxious stimulation & associated with an underlying pathology
Define chronic pain
- Pain that persists beyond the usual course of healing; symptoms that persist for >6mo
What are the 3 types of pain
- Nociceptive: response to an immediate noxious stimulus signaling impending tissue damage
- Neuropathic: damage or disease of the somatosensory nervous system
- Nociplastic: associated with dysfunction of central pain processing (central sensitization); sustained hyperalgesia and/or allodynia (pain due to stimulus that does not usually provoke pain)
Common pain outcome measures
- Patient reported outcomes measurement information system (PROMIS)
- Fear avoidance beliefs questionnaire (FABQ)
- Tampa scale of kinesiophobia
- Chronic pain acceptance questionnaire
Difference between CRPS (chronic regional pain syndrome) type 1 and type 2
- Type 1(reflex sympathetic dystrophy): presents with intense pain throughout the limb but does not involve specific damage to the peripheral nervous system
- Type 2 (causalgia): involves specific damage to the peripheral nervous system typically resulting in both overt motor & sensory neuropathic signs/symptoms
Signs and symptoms of CRPS
- Intense & diffuse pain
- Continuous burning or throbbing pain
- Hyperalgesia & allodynia
- Decreased movement of the affected area
- Cold sensitivity
- Edema in the painful area
- Changes in skin temperature, color, & texture
- Hyperhidrosis (excessive sweating)
- Changes in hair & nail growth
- Atrophy & risk of osteoporosis
Common chronic condition characterized by widespread musculoskeletal pain & fatigue
- Fibromyalgia syndrome (FMS)
Who is more likely to develop FMS (fibromyalgia syndrome)
- Those with RA, systemic lupus erythematosus, or ankylosis spondylitis
Signs and symptoms of fibromyalgia syndrome (FMS)
- Widespread pain described as constant dull ache lasting for at least 3mo
- Multiple trigger points on the head, chest, shoulders, elbows, hips, or knees
- Persistent fatigue both mental & physical
- Sleep disturbances: awaken tired with morning stiffness
- Cognitive difficulties: impaired ability to focus, pay attention, & concentrate on mental tasks
- Atypical patterns of numbness & tingling
- Often coexists with other conditions
- Anxiety & depression are common
- Stress can make symptoms worse
