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Multiple Sclerosis
NDT Ch. 19 MS
| Question | Answer |
|---|---|
| Definition of MS | Chronic inflammatory, demyelinating disease of CNS |
| S/sx of MS | Paralysis; Cardinal symptoms: Intention tremor, scanning speech, & nystagmus |
| Charcot's Triad | Intention Tremor, Scanning Speech, Nystagmus |
| Incidence of MS | Young adults 20-40; Typically 15-50, peaks at 30; Women > Men 2:1; Mostly white populations |
| Etiology of MS | Unknown |
| Autoimmune Disease Theory | Autoimmune disease induced by a viral/other infectious agent (Herpes, Chlamydial Pneumonia, Increased immunoglobulin & oligoclonal bands in CSF in 65-95%) |
| Viral infection has been shown to precipitate ____% of relapses | 33% |
| Genetic Factor in MS | 15% have (+) family hx; Inherit genetic susceptibility to immune system dysfxn; Major histocompatibility complex proteins on chromosome 6 have been linked to antibody production & MS |
| Pathogenesis | Immune response triggers production of T-lymphocytes, macrophages & immunoglobulins (antibodies); In turn, antigen is activated, producing autoimmune cytotoxic effects w/in CNS |
| Pathogenesis continued... | BBB fails & myelin-sensitized T-lymphocyte cells enter & attack myelin sheath that surrounds nerve |
| Myelin | Insulator; Speeds conduction along nerves b/t nodes of Ranvier; Conserves energy b/c depolarization occurs ONLY at nodes |
| Demyelination | Disruption of myelin sheath; Slowing neural transmission; Causes nerves to fatigue rapidly; With severe disruption, causes conduction block with disruption of fxn |
| Pathogenesis continued... | Local inflammation, edema & infiltrates surround acute lesion & can cause mass effect, further interfering with conductivity |
| Oligodendrocytes | Produce myelin; Early stage, survive initial insult & can re-myelinate; Incomplete process; Eventually involved w/ dx progression & can't repair myelin |
| Which type of MS is associated exclusively with dx of oligodendrocytes? | Primary-Progressive |
| Demyelinated areas fill with what & undergo what process? | Fibrous astrocytes; Gliosis (proliferation of neuroglial tissue w/in CNS & results in glial scars/plaques) |
| Demyelination: Mild & Severe forms | Mild: 10-20% loss; Severe: up to 80% loss |
| Lesions in advanced cases | Acute & Chronic of varying size scattered throughout CNS; Primarily affect white matter early; Lesions of gray matter in more advanced dx |
| Areas of Predilection in Advanced Cases | Optic Nn; Periventricular white matter; SC (CST, posterior white columns) & cerebellar peduncles |
| Benign MS | Best possible; Mild; <20% of cases; Pt is functional in all neuro systems 15 yrs after onset |
| Malignant MS (Marburg's Variant) | Worst possible; Relatively rare; Rapid onset & continual progression leading to significant disability/death within relatively short time after onset |
| Relapsing-Remitting (RRMS) | Most common ~70%; Clearly defined relapses (acute worsening of neuro fxn) followed by remissions; 80% progress to 2ndary progressive; Relapses w/ full recovery OR some remaining neuro s/sx & residual deficit w/ recovery; Between relapses-no dx progression |
| Secondary Progressive (SPMS) | Initial relapsing-remitting course followed by progression with or without occasional relapses, minor remissions and plateaus |
| Primary-Progressive (PPMS) | Rare; 10% of cases; Continuous disease progression from onset, w/o relapses; Some do have occasional plateaus/temporary minor improvements; Typically later onset >40; Permanent neuro disability results from incomplete remissions, dx progression, or both |
| Early MS Initial Sx Categories- most common? | Corticospinal |
| Early MS Initial Sx Categories- CN 3-12 at the root, nuclear or bulbar level? | Brainstem |
| Early MS Initial Sx Categories- None listed | Cerebellar |
| Early MS Initial Sx Categories- Optic N. & Cerebral Hemis | Cerebral |
| What is classified as a relapse? | New & recurrent MS symptoms that last at least 24 hours & are unrelated to another etiology |
| What could be associated with a relapse? | Viral/bacterial infections (cold, flu, URI, sinusitis); Diseases of major organ systems (Hepatitis, Pancreatitis, Asthma attack); Modest link b/t stress & acute attacks; Major life stress & minor stresses affect immunity & already compromised CNS |
| What is a pseudoexacerbation? | Temporary worsening of MS symptoms, usually within 24 hours |
| What is Uthoff's Symptom? | Adverse rxn to heat; Anything that raises body temp can cause psueoattack |
| Uthoff's Symptom- External Heat Stressors? | Sun exposure, Hot muggy environment, Hot Bath |
| Uthoff's Symptom- Internal Elevations? | Fever, Prolonged Exercise |
| Uthoff's Symptom- gradual or immediate? How long does it last? | Immediate & dramatic- reduced fxn & increased fatigue; Most resolve w/in 24 hours of cooling &/or end of fever |
| Early Symptoms of MS | Minor visual disturbances (double vision); Paresthesias progressing to numbness; Weakness; Fatiguability |
| Advanced Stages of MS | Multiple s/sx with varying involvement; Onset develop rapidly over course of minutes/hours OR insidious over period of weeks to months (less common) |
| Sensory Issues | Usually paresthesia; Anesthesia rare; Focal deficits can produce limited areas of diminished ssn; Altered ssns more common (inc. paresthsias [pins/needles] or numbness of face, body, extremities) |
| Proprioception & Vibration Sense | Position sense is disturbed; LE impairment of vibratory sense |
| Pain | Dysesthesias, Hyperpathia, Trigeminal Neuralgia, L'Hermitte's Sign, Chronic Neuropathic Pn |
| What % experience pain? | 80%, clinically significant pain 55%, almost 1/2 experience chronic pain |
| What type of pain is experienced? | Sharp, Shooting, Electric, Burning |
| Trigeminal Neuralgia (Tic Douloreux) | Demyelinated ssy division of CN 5 innervating face, cheek, jaw; Eating, shaving, touching face can trigger |
| L'Hermitte's Sign | Common symptom of posterior column damage in SC; Neck flexion produces electric shock ssn down spine into LEs |
| Paroxysmal Limb Pain | Abnormal burning/aching (dysesthesias) that can affect any pt of body but more common in LEs; MOST COMMON type of pain in MS; Worse at night & post-exercise; Can be aggravated by increased temp |
| Hyperpathia | Hypersensitivity to minor ssy stimuli (light touch/pressure can cause severe pain rxn) |
| Headaches | More frequent in MS than general population; Migraine/Tension-type |
| Chronic Neuropathic Pain | From demyelinating lesions in ST tracts or in ssy roots; More common in pts w/ minor disability; Burning pain similar to pain from disc herniation |
| Musculoskeletal Pain | Associated with mm & ligament strain developed from mechanical stress, abnormal postures & immobility; Results of weak muscles, powerful spasticity & tonic spasms |
| What 2 common things can worsen pain s/sx? | Anxiety & Fear |
| What visual impairments may an MS patient have? | Optic Neuritis; Scotoma; Light Reflexes; Nystagmus; Eye Problems |
| What % of patients have visual impairments? | 80% |
| What is the cause of altered visual acuity? | Optic N. involvement |
| Is blindness common? | No |
| Optic Neuritis | Common; Ice pick pn behind eye w/ blurring/graying of vision or blindness in 1 eye; Rarely affected both eyes; Self-limiting |
| What is a scotoma? | Dark spot in center of visual field that improves within 4-12 weeks |
| Light Reflexes- Marcus Gunn pupil | Shine bright light into healthy eye-> Reflex contraction in both eyes (consensual light reflex), then shining light into affected eye -> Paradoxical widening/dilation of both pupils |
| Nystagmus | From cblr/central vestibular pathway lesions; Involves involuntary cyclical mvmts of eye that develop when pt looks to side/vertically or when pt moves head |
| Internuclear Ophthalmoplegia (INO) | Incomplete eye adduction (lateral gaze palsy) on affected side & nystagmus of opposite abducting eye with gaze to 1 side |
| What causes INO? | Demyelination of pontine MLF |
| Problems with conjugate gaze & control of eye movements caused by? | BS lesions affecting CN 3, 4, 6 or MLF |
| Diplopia | Double vision when mm of eye not well-coordinated |
| Are visual disturbances permanent? | No, they frequently remit & seldom are the primary cause of disability |
| What should be monitored if pt has visual disturbances? | Effects on balance and movement |
| Motor Impairments | S/sx UMN; Spasticity in LE > UE (leads to indirect impairments: pn, contractures, abnormal posturing, skin problems) |
| Spasticity | Fluctuates daily, increases with extremes of temp & humidity, infections, tight clothes & certain anti-depressants; Doesn't abate during spontaneous remissions |
| Weakness- CST Lesions | UMN s/sx; Paresis, spasticity, brisk reflexes, involuntary flexor/extensor spasms, clonus, Babinski's, exaggerated cutaneous reflexes, Loss of precise autonomic contron |
| Weakness- Movements | Slow, stiff, weak; results of loss or orderly recruitment & reduced firing rate modulation of motornns |
| Weakness | Reduced strength, power & endurance along with impaired synergistic relationships are evident |
| Weakness in pts with cblr lesions | Asthenia or generalized mmw eakness along with ataxia |
| Weakness secondary to inactivity | Range from mild paresis to total paralysis |
| Fatigue | Subjective lack of physical &/or mental energy perceived by the individual/caregiver to interfere with usual & desired activities |
| Fatigue Occurrence | Daily event in 75-95%; 50-60% report as most troubling symptom; Interferes with physical fining, overall role performance, & perceived health status; Comes on abruptly, w/o warning & resembles an overwhelming flu-like exhaustion |
| Is disease severity related to fatigue severity? | NO |
| Fatigue results from what? | Failure of central activation & excitation-contraction coupling |
| Fatigue Precipitation Factors | Physical exertion, Exposure to heat & humidity; Depression; Sleep disorders; Low self-esteem; Mood disorders; Medical conditions; Secondary complications (respiratory impairment, infection) |
| Side Effects of Meds can also impact fatigue | Analgesics, Anti-convulsants, Anti-depressants, Anti-histamines, Anti-HTN agents, Anti-inflammatories |
| What is a strong psychosocial predictor? | Environmental Mastery (sense of control) |
| How common is spasticity? | Very: ~80%; Range from mild to severe depending on progression of disease; UE & LE |
| Clinical Indications of Spasticity | Impaired voluntary control of mvmt (abnormal co-contraction); Increased DTRs, clonus, flexion or extension synergy patterns; Decreased ROM |
| Spasticity results in what? | Increased fatigue; Impaired functional mobility; Impaired ADLs; Can cause pain, disabling contractures, abnormal posturing & problems w/ skin |
| Fluctuation of Spasticity | Daily & exacerbated w/ fatigue, stress, extremes of temp & humidity, infections, tight clothes (noxious stimuli) & certain anti-depressant (serotonin-reuptake inhibitors); Does not abate during spontaneous remissions |
| Spasticity in Advanced MS | Quite disabling, Difficult to Manage |
| Balance & Coordination | Demyelinating lesions in cblm & cblr tracts common producing cblr s/sx |
| Clinical S/sx Balance & Coordination- Ataxia | Uncoordinated mvmts, progressive of trunk & LEs; Dysmetria, Dyssynergia, Dysdiadochokinesia |
| Clinical S/sx Balance & Coordination- Tremor | Postural; Intention (cblm can't dampen motor mvmts); Vary fine to gross; Severe tremors impose significant limitations infxnal activities; Exacerbated by stress, excitement & anxiety |
| Clinical S/sx Balance & Coordination- Sensory Ataxia | Severe numbness of feet -> Difficulty standing/walking |
| Clinical S/sx Balance & Coordination- Dizziness from lesions affecting cblm/central vestibular pathways | Disequilibrium (difficulties with balance); Vertigo; Nausea; Worse w/ head/eye mvmt; Paroxysmal attack brought on by period of hyperventilation |
| Ambulation & Mobility | Difficulty walking; 1/2 of pts with RRMS require some form of assistance within 15 yrs |
| What does gait look like? | Staggering, uneven steps, poor foot placement, uncoordinated limb mvmts, frequent LOB |
| What causes speech & swallowing dysfunction? | Muscle weakness, spasticity, tremor, ataxia; 40% |
| Speech Dysarthria | Slur/poorly articulated speech with low volume, unnatural emphasis & slow rate |
| Dysphonia | Changes in quality- harshness, hoarseness, breathiness or hypernasal sounds |
| Dysphagia | Poor coordinatoin of tongue & oral mm, difficulty swallowing |
| Signs of Dysphagia | Trouble chewing & maintaining lip seal, inability to swallow & spitting or coughing during/after meals; Can have poor nutritional intake & dehydration |
| Aspiration Pneumonia- What is it? S/sx? | Serious complicatoin that can develops if food/liquid inhaled into trachea; Wet voice quality with gurgling or sounds of congestion & fever |
| Cognitive Impairments | Common (~50%); 10% severe enough to affect ADLs; Related to the specific distribution of the lesions rather than to overall severity, its course or disability status |
| Changes in cognitive function | Impaired attention & concentration (esp. alternating & divided attn); Slowed info processing; Impaired recent memory; Impaired executive fxns; Focal frontal lobe lesions can produce cognitive inflexibility |
| Significant Mental deterioration (Global Dementia) | Rare but may be seen in rapidly progressing disease (malignant MS) or in pts with significant cerebral lesions; Level of cognitive fxn major factors in determining QOL, social fxning, employment, fxn in ADLs |
| How common is depression? | @ Least 50% |
| Symptoms of Depressoin | Feeling of hopelessness/despair; Diminished interest/pleasure in activities; Change in appetite & significant wt changes; Insomnia/Hypersomnia; Feelings of lethargy/worthlessness, fatigue, loss of energy; Decreased concentration; Thoughts of death/suicide |
| Depression | Can be direct result of MS lesions, as a side effect of drugs or as a psych rxn to stresses of dx; Anxiety, denial, anger, aggression or dependency can also occur; Learned helplessness & low self-efficacy also common |
| How common are affective changes? | ~10% |
| Pseudobulbar Affect | Sudden loss of emotional control on multiple occasions that is typically unrelated to external circumstances, depression or underlying mood; Assoc w/ more progressive disability & greater intellectual impairment |
| Euphoria | Exaggerated feeling of well-being, sense of optimism incongruent w/ pt's incapacitating disability; Found primarily in pts w/ advanced dx |
| Affective Changes | Bipolar Disorders can occur; Symptoms have been linked to diffuse, bilateral cerebral involvement (esp pre-frontal cortex & corticobulbar tract involved in emotional control) |
| CV Dysautonomia | ANS involvement; Problems w/ cardioacceleration/reduction in BP response during exercise; May have attenuated or absent sweating responses also |
| How common is bladder dysfunction? | ~80% |
| Bladder Dysfunction | Loss in volitional & synergistic control of micturition reflex; Produced by demyelinating lesions affecting the lateral & posterior spinal tracts unmasking the sacral reflex arc |
| Types of Bladder Dysfunction- Small, Spastic | Failure to store |
| Types of Bladder Dysfunction- Flaccid, Big | Failure to Empty |
| Types of Bladder Dysfunction- Dyssynergic | Problem with coordination b/t bladder contraction & sphincter relaxation |
| S/sx Bladder Dysfunction | Urinary urgency & frequency; Hesitancy in starting urination; Nocturia; Dribbling; Incontinence; Severity associated with severity of other neuro s/sx (particularly pyramidal tract involvement) |
| Bladder Dysfunction | Emptying dysfunction with large residual urine volume increases risk of recurrent UTI & kidnsey damage from frequent UTIs |
| Bowel Dysfunction | Constipation most common; From lesions affecting control of gastrocolic reflex; Presence of spasticity of pelvic flor mm & frequent consequence of inactivity, lack of fluid intake, poor diet & bowel habits, depression, med SE's |
| Sexual Dysfunction | Common (91% men, 72% women) |
| Sex Dysfxn- Women | Changes in sensation; Vaginal Dryness; Trouble reaching orgasms; Loss of libido |
| Sex Dysfxn- Men | Impotence; Decreased sensation; Difficulty/inability to ejaculate; Loss of libido |
| Imaging- MRI | Detects acute & chronic lesions, small & large with high resolution; Scans distinguish new with active inflammation that occurred during preceding 6 wks |
| Imaging- MRI | Lesions seen as "bright spots" |
| What types of scans detect more long-term disease activity? | T2; lesions seen as "black holes"; Darker the lesion = more extensive tissue damage |
| What % of pts with clinically defined MS have well-defined MRI changes? | ~95%; excessive MRI activity = 3+ enhancements on repeat scans separated by at least quarterly intervals; Silent attacks > attacks that cause active s/sx such as paralysis/vision loss by 10:1 |
| Current Neurology Guidelines | MRIs to be performed at pre-defined intervals to document dx progression & response to dx-altering meds |
| CSF | Elevated total immunoglobulin in spinal fluid & the presence of oligoclomal IgG bands; Pts with PPMS have higher immunoglobulin levels in CSF than pts w/ RRMS |
| Evoked Potentials | Up to 90% demo abnormal evoked potentials; Presence of demyelinating lesions on visual, auditory, somatosensory or motor pathways produce slows/abnormal condition |
| Is life expectancy reduced? | Not usually; Only small % die from dx; Only minority are in workforce 10 yrs post-onset; 75% survive 25 yrs after sx onset |
| At how many years will what % require use of AD to walk? | 15; 50% |
| At how many years will what % require WC? | 20; 50% |
| Prognostic Factors- Symptoms | onset with only 1 symptom is one of strongest indicators of favorable prognosis |
| Prognostic Factors- Course of Disease | Benign & RRMS are associated with better prognosis whereas PPMS is generally considered more ominous |
| Prognostic Factors- Age | Young onset more favorable than onset >40 (associated w/ PPMS course & increased disability) |
| Prognostic Factors- Neuro findings at 5 years | One of most important prognostic factors; Significant pyramidal & cblr s/sx w/ involvement at multiple sites associated w/ poorer prognosis & more severe disability |
| Prognostic Factors- MRI Findings | Favorable px factors: Low total lesion burden, Low action lesion formation, Negligible myelin/axon loss |
| For most pts receiving dx-modifying drugs, tx only partially effective in controlling dx | Some show reduced relapses; Reduced severity of attack (acquired neuro deficits); Fewer & smaller damaged areas on MRI; Slower rate of dx progression |
| Frequent relapses/excessive MRI activity may indicate need to switch drug therapy to higher doses & more frequently administered beta-interferons/combination therapies | Agents can't reverse existing deficits; Drugs not indicated for PPMS |
| Common Adverse Effects of Interferon Drugs | Injection-site skin run (sore, rd, pain, bruising, swelling); Flu-like s/sx post-injection (Fever, Chills, Sweating, Mm ache, Fatigue); Injection sites vary to reduce AEs; Depression; Allergic rxn; Liver rxn |
| Common AEs for Copaxone | Similar injection site rxns; Initial flushing rxn immediately after injection (anxiety, chest pain, palpitations, SOB); Does NOT cause flu-like s/sx or depression |
| Problems with adherence using what? | Immunomodulating agens |
| Management of Relapses & Symptoms | Prompt mgmt of flare-ups & symptomatic & supportive rx are ESSENTIAL |
| Corticosteroid Therapy | Rx acute dx relapses, shortening episode duration; Powerful anti-inflammatory & immunosuppressive effects, diminishing swelling within CNS & temporarily repairing BBB; May prevent circulating toxins from entering CNS; Don't modify dx course/recovery |
| Adverse SE's of Corticosteroids | Agitation, nervousness, & insomnia; Increased sweating, appetite & susceptibility to infection; GI distress including heartburn & diarrhea/constipation |
| ACTH (Adrenocorticotropic Hormone) | Long-term immune suppression |
| ACTH SE's | Suppression of blood production by marrow; Bleeding Disorders; Increased risk of infection |
| Management of Spasticity | Mm relaxants; Oral baclofen, tizanidine, dantrolene, diazepam (valium) |
| Adverse Effects of Mm Relaxants | Sedation, weakness, fatigue; PT must be alert to these changes & communicate w/ MD to achieve optimal dosing for rehab; Recognize that at time spasticity can enhance fxn, substituting for lack of strength |
| Carbamezapine effective in reducing what? | Paroxysmal (Sudden, sharp onset) spasms |
| Pts who don't adequately respond to conservate rx (intractable spasticity/spasms) may benefit from? | Intrathecal baclofen into CSF of L-spine; Significant reduction in spasticity & spasms in LEs & trunk with less improvement reported in UEs |
| Adverse Effects of Intrathecal Baclofen | Pump failure; Infection; Lead displacement |
| Surgical Interventions indicated more for? | Severe spasticity; Spastic paralysis for many yrs resulting in non-functional limb & serious complications (contractures/skin breakdown) |
| Surgical Techniques | Severing tendons (tendinotomy), Nerves (neurectomy) or Nerve roots (Rhizotomy) |
| Phenol Nerve Blocks | Reduce spasticity (6+ month effective); Botox can temporarily block nerve/mm |
| Extensor Spasticity good for? | Assist standing during stand-pivot xfer |
| Paroxysmal Pain Mgmt | Reponds best to carbamezapine, amitriptyline, Gabapentin |
| Dysesthesia Mgmt | Low doses of amitriptyline, imipramine, desipramine |
| Pain/Discomfort from Spasms/Spasticity Mgmt | OTC/Prescription Anti-Inflammatories |
| Pain & Numbness Mgmt | Brief course of corticosteroids |
| Narcotic Analgesics | Problematic, not typically prescribed |
| Symptomatic Rx of Fatigue | Amantadine HCl: 1st line therapy is anti-viral & dope agonist that has significant impact on lessening fatigue; Modafinil; Premoline (CNS stimulant with undesirable SE of anorexia, irritability, insomnia) |
| Mgmt of Dizziness & Vertigo | Anti-Nausea drugs; Severe cases may require administration of short course corticosteroids |
| Severe Tremor Treatment | Surgery (Thalamotomy) or Deep brain stimulation that involves implanting electrodes into thalamus; DBBS is experimental for MS still |
| Agents approved by FDA for Alzheimer's Rx | Slow cognitive decline; Moderate benefits in memory deficits have been observed |
| Depression Mgmt | Anti-depressants, in which some can also help lessen fatigue |
| How to treat pts with pseudobulbar affect? | Anti-depressant amitriptyline |
| What non-medicinal way can pts do to help cope with stresses? | Professional counseling & participation in support groups; Active lifestyle also helps reduce depression & anxiety |
| Overactive/Spastic Bladder Mgmt | Anticholinergics; Oxybutynin; Imipramine |
| Adverse Effects of Overactive/Spastic Bladder drugs | Dry mouth, Tachycardia; Accommodation disturbances |
| Dietary Recommendations when taking spastic bladder meds | 8 glasses water/day; Limit caffeine/alcohol intake |
| Flaccid Bladder- Alternate Techniques for emptying | Crede Maneuver- manual downward pressure over lower abdomen; Intermittent self-catheterization |
| Flaccid Bladder- Dietary Recommendations | Limit intake of citrus juices; Drink cranberry juice daily |
| Dyssynergic Bladder (combined dysfunction ) Mgmt | Alpha-adrenergic blocking agents; Prazosin; Temsulosin; Anti-spasticity agents |
| In the rare occasion where bladder s/sx can't be controlled with meds/ISC, what do you do? | Continuous cath or surgical urinary diversion |
| What results from retention of urine in bladder & from catheterization procedures? | UTIs; Antibiotics used to treat |
| How to manage constipation? | Dietary changes: increased fluid & fiber, bulk-forming supplements, stool softeners |
| What is not recommended for management of constipation? | Regular/continuous use of stimulant laxatives & enemas |
| Incontinence Management | Dietary: avoid irritants (caffeine, alcohol); Adjust meds used to reduce spasticity (can contribute to problem); Add meds to control bowel spasms |
| Progressive-Relapsing (PRMS) | Progressive disease from onset but without clear acute relapses, +/- some recovery or remission; Common in people who develop disease after 40 |
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