lower motor neuron disease (하위운동신경원...
TRANSCRIPT
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Neuromuscular Disease
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Disorders of the Motor Unit
• Motor neuron disease
• Peripheral nerve disorders
• Neuromuscular junction disease
• Muscle disease
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Motor Neuron Disease
• General introduction
– 운동신경원 질환은 신경계의 여러 부위의motor neuron이 손상받는 일련의 질환군으로서 특히 척수의 anterior horn cell이 가장 많이 침범된다. 이 운동신경원 질환은 점차 진행하는 치명적인 질환으로 아직 특별한 치료방법은 없다
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Motor neuron Disease
– 침범부위에 따른 분류1. Motor cortex frontal dementia
2. Corticobulbar tract pseudobulbar palsy
3. Cranial nerve nuclei progressive bulbar palsy
4. Corticospinal tract primary lateral sclerosis(원발성축삭경화증)
5. Anterior horn cell progressive muscular atrophy(PMA)
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Motor neuron Disease
– 특히 corticospinal tract과 anterior horn cell이 혼합되어 퇴행성병변을 보일경우Amyotrophic Lateral Sclerosis(ALS)로 부르며 motor neuron disease와 같은 의미로 쓰인다.
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• Motor cortex frontal dementia
• Corticobulbar tract pseudobulbar palsy
• Cranial nerve nuclei progressive bulbar palsy
• Corticospinal tract primary lateral sclerosis
• Anterior horncell progressive muscular atrophy
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Motor Neuron Disease
• Diseases that can involve Betz cells of the motor cortex, the lower CN motor nuclei, the CST, and/or the anterior horn cells
– Amyotrophic Lateral Sclerosis (ALS)
– Progressive bulbar palsy (PBP)
– Progressive muscular atrophy (PMA), spinal muscular atrophy (SMA)
– Primary lateral sclerosis(PLS)
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LMN disease
• Guillain-Barre Syndrome(GBS)(acute idiopathic post-infectious polyneuropathy)
• Poliomyelitis(acute anterior poliomyelitis)
• Postpolio syndrome
• Progressive muscular atrophy(PMA)
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UMN and LMN disease
• Amyotrophic lateral sclerosis(ALS)(Lou Gehrig`s disease)
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Distinctions b/w Types of MND
UMN LMN
ALS Amyotrophic Lateral Sclerosis yes yes
PLS Primary lateral sclerosis yes no
PMA/SMA Progressive/spinal muscular atrophy no yes
PBP* Progressive bulbar palsy no yes
*limited to bulbar musculature
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Lou Gehrig`s disease
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ALS
• Loss of motor neurons in the cortex, brainstem and spinal cord
• Mix of upper motor neuron and lower motor neuron findings
– Weakness, atrophy, fasciculations
– Slurred speech, difficulty swallowing, shortness of breath
• Can start in any extremity or the bulbar musculature
• Relentlessly progressive
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ALS
• 50 % dead in 3 years, 80% dead in 5 years, 5-10% live more than 10 years
• Death usually from respiratory failure
• Etiology still only theoretical
– Excess glutamate
– Oxidative stress
– Free radicals
– Mitochondrial dysfunction
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ALS
• 5-10% have inherited disease
– Superoxide dismutase (SOD-1) gene defect in about 20% of inherited ALS• Different mutations in the same gene
associated with differences in age of onset, rate of progression
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Treatment in ALS
• Riluzole
– Antiglutamate agent
– Prolonged survival - modest benefits
– Only agent with proven efficacy
• Many other agents tried
– Other antiglutamatergic meds, trophic factors, immunosuppressants, vitamins E & C (antixoidants)
• Supportive care
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Primary Lateral Sclerosis (PLS)
• Pure upper motor neuron disease
• Primary findings are related to spasticity and pathologically brisk reflexes
• Weakness is present - but the spasticity is usually more disabling
• Slowly progressive - in general, less bulbar involvement
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Progressive Bulbar Palsy• “Bulb” refers to the brainstem• Sxs
– Slurring of voice (dysarthria)– Difficulty chewing– Difficulty swallowing (dysphagia)– Shortness of breath - with exertion, later at
rest
• Signs– Weak, fasciculating tongue– Jaw and/or neck weakness– Decreased forced vital capacity– Brisk jaw jerk– Bulbar affect
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Progressive Muscular Atrophy (PMA)
• Since it’s a lower motor neuron disease only, weakness with decreased reflexes and normal to decreased tone• In general, a more slowly progressive
disease• Bulbar musculature may be spared until
late in disease
The MNDs are a spectrum, and PMA, PLS and PBP can all evolve into ALS
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Spinal Muscular Atrophy (SMA)
• Most common form of inherited MND -autosomal recessive
• Age of onset
– Infancy - Werdnig Hoffman disease
– Adolescence - Kugelberg Welander disease
– Late onset
• Survival motor neuron gene with a modifier gene that effects onset age
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Peripheral Nerve Disorders• Mononeuropathy
– Pattern of weakness and sensory loss conforms to the distribution of a single nerve• Carpal tunnel syndrome• Peroneal palsy at the fibular head
• Mononeuritis multiplex– Multiple nerves affected in a random pattern• Acute onset, frequently painful• Diabetes mellitus, vasculitis
• Polyneuropathy (peripheral neuropathy)– Distal, symmetric
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Polyneuropathies
• Can affect different types of fibers
– Autonomic
– Motor
– Sensory• Large well myelinated
• Small poorly myelinated or unmyelinated
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Symptoms of a Polyneuropathy
• Sensory symptoms
– Start in feet, move proximally
– Hand sxs appear when LE sxs up to knees
– Positive• Pins and needles
• Tingling
• Burning
– Negative• Numbness
• Deadness
• “Like I’m walking with thick socks on”
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Polyneuropathy Symptoms, cont
• Motor
– Weakness first in feet• Tripping
• Turn ankles
– Progress to weakness in hands• Trouble opening jars
• Trouble turning key in lock
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Polyneuropathy: Signs
• Distal sensory loss
– Large fiber
– Small fiber
• Distal weakness and atrophy
• Decreased or absent reflexes
– Ankle jerks lost first
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Stocking glove sensory loss
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Classification of Polyneuropathies
• By types of fibers involved– Pure sensory
– Sensory motor
– Pure motor
– Autonomic
• By pathology– Demyelinating
– Axonal
– Mixed
• By tempo– Acute
– Subacute
– Chronic
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Acute Polyneuropathies
• Guillain Barre Syndrome
• Porphyria
– Neuropathy, psychiatric disorder, unexplained GI complaints
• Toxins
– Glue sniffing (n-hexane)
– Arsenic
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Guillain Barre Syndrome
• Most common cause of rapidly progressive weakness
• Demyelinating neuropathy
• Ascending weakness which may include cranial neuropathies
• Exam reveals symmetric weakness with areflexia and large fiber sensory loss
• Bowel and bladder usually preserved
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Guillain Barre Syndrome, cont
• Respiratory failure can be precipitous
• Other causes of morbidity and mortality– Autonomic instability
– DVT
– Infection
• Immune mediated, may be post infectious
• Treatment– Plasma exchange
– Intravenous immunoglobulin
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Subacute Polyneuropathies• Vasculitis
– Can be isolated to peripheral nerves or part of a more systemic process
• Paraneoplastic
– May be presenting symptom of the cancer
• Chronic inflammatory demyelinating polyneuropathy
– With or without a gammopathy
• Toxins
• Drug
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Chronic Polyneuropathies• Metabolic
– Diabetes mellitus– Chronic renal failure– Chronic liver failure– Thyroid disease
• Nutritional– B12 deficiency
• Infections– HIV– Leprosy
• Inherited
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Evaluation of a Polyneuropathy
• Tempo• Lab work• Nerve conduction
study/electromyography– Distinguishes between axonal and
demyelinating– Helps ascertain severity
• Nerve biopsy– Frequently non-diagnostic– Can establish the dx in certain disorders,
such as vasculitis and amyloidosis
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Neuromuscular Diseases Part 2
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Disorders of the Neuromuscular Junction
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NMJ
• Pre-synaptic
– Lambert Eaton myasthenic syndrome
– Botulism
• Post-synaptic
– Myasthenia Gravis
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Myasthenia Gravis
• Antibody that alters the acetylcholine receptor
– Binding
– Blocking
– Modulating
• Antibody detected in
– 50% of pts with pure ocular MG
– 90-95% of pts with generalized MG
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Clinical Manifestation of MG• Sxs worsen with exercise, end of day
(Fatigue)• Ocular
– Droopy eyelids (ptosis)– Double vision (diplopia)
• Extremity weakness– Arms > legs
• Bulbar– Dysarthria– Dysphagia
• Respiratory– Shortness of breath
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Approach to treating MG
• Remove any exacerbating factors
– Infections, medication, endocrine disease
• Acetylcholinesterase inhibitors
• Plasma exchange/ intravenous immunoglobulin
• Thymectomy
• Immunosuppressants
– Prednisone
– Imuran (azathioprin)
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Botulism• Presynaptic part of the NMJ• Prevents release of acetylcholine• Food borne
– Infants at particular risk
• Features– Weakness, may be profound– Autonomic system dysfunction– Pupillary involvement
• Dx: – Nerve conduction studies– Stool culture
• Rx: Antitoxin, supportive
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Lambert Eaton Myasthenic Syndrome (LEMS)
• Presynaptic disorder of the NMJ
• Voltage gates calcium channel antibodies impede release of acetylcholine
• Weakness -– more of LE than UE
– bulbar and ocular muscles less often involved
• decreased reflexes - post tetanicpotentiation?
• ANS involvement
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LEMS
• Associated with a cancer in the majority of patients (paraneoplastic)
• Underlying cancer may be previously unrecognized
• Small cell lung cancer the most common
• Rx :
– Underlying cancer
– Guanidine
– 3,4 diamino pyradine
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Myopathies
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Clinical Manifestations of Myopathies
• Proximal muscle weakness
– Waddling gait
– Difficulty climbing stairs
– Trouble lifting arms over head
• Cramps with the metabolic myopathies
• Myalgias with the inflammatory myopathies
• Swallowing and breathing difficulties, when present, are usually late
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Classification of Muscle Disease• Dystrophies
– Duchenne’s Muscular Dystrophy
– Myotonic Dystrophy
• Congenital Myopathies
– Glycogenoses
– Mitochondrial
• Acquired Myopathies
– Polymyositis
– Dermatomyositis
– Inclusion body myositis
– Drug related
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Duchenne’s Muscular Dystrophy
• X-linked recessive
• Absence of dystrophin protein
• Slow to reach motor milestones, sxs by age 5– All walk, may never run
– End up in wheelchair by age 10-12• Steroids may delay time until wheelchair bound
• Muscles replaced by fat may appear hypertrophic
• Frequently mildly mentally retarded
• Life expectancy < 20 years with death related to respiratory failure or cardiomyopathy
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Duchenne Muscular Dystrophy
• Most common muscular dystrophy encountered by anesthesiology
• Incidence 1:3,500 live male births
• Characterized by painless degeneration and atrophy of skeletal muscles
• X-linked disorder
– DMD gene isolated to short arm of the X chromosome at position 21
– Estimated mutation rate is one of the highest for any human disease
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Duchenne Muscular Dystrophy
• DMD gene product: dystrophin– Absent or nonfunctional
in DMD patients
• Associated with muscle cell membranes – In its absence, a
sequence of events occurs that leads to calcium influx into the muscle cells -> cell degeneration and death
• Affects Skeletal, Cardiac, and Smooth muscle
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Progressive disease course
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DMD: Disease Progression
• Under 2 yrs old– Behave like healthy toddlers
• 2-5 yrs old– First outward signs of muscular weakness– Clumsiness, frequent falling, waddling gait,
difficulty climbing stairs– Calf muscles begin to look enlarged
• 6-12 yrs old– Child walks on toes secondary to Achilles tendon
tightening and to compensate for weak quads– Weakening pelvic and shoulder girdles ->
compensatory lordosis
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Gowers’ Maneuver
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DMD: Disease Progression
• 8-14 yrs old– Lose ability to walk– Decrease in caloric requirements -> even normal
diet leads to obesity– 95% develop scoliosis
• Adult phase– Scoliosis + weakened respiratory muscles,
inactivity, obesity -> compromised lung expansion and function
– Vital capacity decreased approximately 50%– Weak cough -> vulnerable to pneumonia
• Late 20’s– 90% die of respiratory complications, 10% cardiac
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Myotonic Dystrophy• Most common of the dystrophies• Autosomal dominant• Age of onset varies• Myotonia -
– Failure of relaxation of the muscle following contraction
• Neuromuscular features– Distal weakness– Ptosis– Facial weakness – Tongue weakness - dysarthria and dysphagia– Involvement of respiratory muscles
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Myotonic Dystrophy Involvement outside the NM
system
• Heart
– Conduction block
• Decreased fertility, undescended testicles
• Diabetes mellitus
• Mild MR
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Idiopathic Inflammatory Myopathies
• Polymyositis (PM)
• Inclusion body myositis (IBM)
• Dermatomyositis (DM)
• Together - incidence ~ 1:100,000
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The typical patient
• Sent with
– Fatigue
– Weakness
– Muscle aches and pains
– Elevated CPK
• May also get the pt with the dx of PM who failed steroids
– May or may not have had a muscle bx
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Clinical Features of Polymyositis
• May occur at any age but rare under 18
• Subacute onset of proximal > distal weakness including neck flexor weakness– Rare to see it isolated to proximal muscles• Dystrophy
• Muscle pain and tenderness– Seen in 50%
• Facial weakness uncommon
• Respiratory involvement– Mostly in pts with severe, unresponsive disease
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Polymyositis
• Slightly increased risk of cancer
– Bladder, lung, lymphoma
• Biopsy of muscle confirms diagnosis
• Treatment with immunosuppression
– Prednisone
– Methotrexate
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Dermatomyositis• Affects children (Ages 5-15) as well as adults• Females more affected than males• Subacute onset of proximal > distal weakness• Facial weakness in severe cases• Dysphagia in 1/3• Fulminant cases
– Rhabdo– ARF
• Skin changes - present in 60%, frequently first– Skin changes without muscle sxs– Muscle disease without skin changes
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Skin Changes in DM
• Heliotropic rash of eyelids
• Erythematous rash on malar regions, neck, shoulders, or extensor surfaces of arms/legs
• Gottron’s papules– Red, raised, scaly lesion over extensor
surfaces of PIP and DIP
• Nail changes– Thickend cracked nail beds
– Dilated capillary loops
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Associated Conditions with DM
• Malignancy
– Increased, particularly in adults over 40
– Risk greatest within 5 yrs of dx of DM
– Most common cancers:• Ovarian
• Lung
• Pancreatic
• Colorectal
• Joint disease
– Arthritis
– Arthralgias
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Associated Condtions, cont
• GI tract
– Dysphagia
– Delayed gastric emptying
– Intestinal ulceration/perforation
• Lungs
– Interstitial lung disease
• Heart - more common in DM than PM
– Conduction problems
– Cardiopmyopathy
• Overlap CTD
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Etiology and Treatment of DM
• Also Immune mediated
• Identical to PM except
– IVIG is effective in DM but not PM
• Prednisone is still considered first line of choice in many, but consider IVIG as first agent
– if pt very young
– has medical reasons to avoid steroids
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Inclusion Body Myositis (IBM)
• Now thought to be the most common idiopathic inflammatory myopathy in adults
• Prevalence ~5 per million
• Age onset - over 50
• 3 males: 1 female
• Majority sporadic
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IBM - clinical features
• Indolent onset – Sxs up to 10 yrs before medical care
sought
• Typical pattern of weakness - majority but not all pts– Asymmetric– Wrist and finger flexors– Quadriceps
• In contrast to PM– Dysphagia (40-60%) and facial weakness
(30%) more common
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IBM
• Etiology unclear
• “aging” of muscle?
• Do not respond to immunosuppression
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Evaluation of the Patient with Suspected Muscle Disease
• Lab– Muscle enzymes (CPK, aldolase)– Erythrocyte sedimentation rate (ESR or sed
rate) if suspect inflammatory disease– Genetic test• Duchenne’s• Myotonic dystrophy
• EMG/NCS• Muscle biopsy
• May provide a definitive diagnosis
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Extremity CN Reflexes WeaknessSensation
ALS Random yes Increased Normal
Polyneuro- Distal> rare Decreased Lost distally >
pathy Proximal distally proximally
LEMS LE > UE rare Decreased or Normal
Prox>distal absent
MG UE>LE yes Normal or dec Normal
+/-prox>distal
Myopathy Prox>distal occ Normal or dec Normal
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Guillian-Barre syndrome
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Guillian-Barre syndrome• Etiology
– Unknown– May be
– Viral infection– Metabolic defect– Defect of immune system
• Pathology– Stage I : lymphocytes migrate to myeline sheath
and axon– Stage II : macropage appear, segmental
demyelination begins, axon not yet affected– Stage III : multifocal myeline sheath and axonal
damage• Occurs central chromatolysis of cell body• Begins to develop denervation atrophy
– Stage IV : extensive axonal destruction• Some nerve cell bodies irreversibly damage
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Guillian-Barre syndrome• Laboratory
– Elevate CSF protein
– Number of cells : constant
• Clinical features– First symptoms• Onset : acute
• May have fever
• Paraeathesia of feet and hands
• Weakness of distal extremity
– Symmetric weakness
– Paralysis from distal to proximal
– Involved spinal nerve and cranial nerve(facial(30%), respiratory)
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Guillian-Barre syndrome– Sensory change• Anethesia
• Paraethesia
• Hyperethesia
– Weakness is maximal : 3 weeks after onset
– Areflexia
– Restrictive pulmonary insufficiency(tracheoctomy 30%)
– Autonomic abnormalities• Tachcardia
• Bradycardia
• Postural hypotension
– Muscle atrophy
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Guillian-Barre syndrome• Outcomes
– Mortality : 10% of all case
– Of those respiratory failure• 20% : severely disabled for life
• 10% : moderately disabled for life
– Mild case : excellent recovery within 6 months or 1 year
– Recurrent : 5 %
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Guillian-Barre syndrome• Evaluation
– Dx– Past history
• Onset, duration, progression of disease• Previous medical history
– Physical examination• Sensory test• ROM• MMT
– Neurological examination• Sensory dematome level• Cranial nerve• DTR
– Functional ability• Gross motor• A.D.L• Bed mobility• Transfer activity• Gait
– Skin and soft tissue• Bed sore• Edema• Atrophy
– Respiratory and cardiac status
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Guillian-Barre syndrome• Precaution during evaluation and treatment
– Avoid fatigue : overexertion and patient fatigue during testing may cause or increase the demyelination process
– Support bony prominences to prevent tactile pressure if tenderness or pain is present
– Avoid possible joint dislocation and subluxation during ROM secondary to supportive tissue weakness
– Avoid overstretching weakened muscles
– Close guarding during transfer and exercise
– Beware of autonomic dysreflexia and orthostatic hypotention
– Note symptoms of possible deep vein thrombosis
– Reevaluate neurological status, muscle strength, ROM weekly
– Progressively increase treatment time and intensity
– Careful fracture
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Guillian-Barre syndrome
• Treatment– Acute phase• Bed rest and pain relief
• Proper bed position
– Prevent bed sore and contracture
• Maintain full passive ROM of all extremities
• Breathing ex.
– Stabilization phase• Continue acute phase
• Active-assistive ROM Ex.
• Gentle stretching Ex.
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Guillian-Barre syndrome
– Recovery phase• Above all• Strengthening Ex.• Functional training
– Bed mobility– Transfer– Come to sit– Balance training– Standing and balance– Gait training
– Discharge• Discharge evaluation• Follow-up plan• Home program
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Poliomyelitis(Acute anterior poliomyelitis)
• Etiology– Poliovirus• Type I(Burnhild)
• Type II(Lansin)
• Type III(Leon)
– Average incubation : 3~30 days, Tidy : 7~14 days
– Transmission : human contact, hand, mouth,ingestion
• Lesion• Anterior horn cell of spinal cord
• Motor nuclei of cranial nerve
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Poliomyelitis• Classification
– Abortive type• No nervous symptoms(Nonparalytic type)• Gastrointestinal symptom : vomiting, nausea, diarrhea
– Meningeal type• Nonparalytic type• Meningitic sign(meningeal irritation)
– Neck stiffness: gentle flexion of the neck is met with boardlike stiffness
– Headache– Rises intracranial pressure– Vomiting– High fever– Convulsion(경련) – Positive of Brudzinski`s sign : forceful flexion of the neck
on the chest causes flexion of both legs and thighs– Positive of Kernig`s sign : patient supine with hip flexed 90
degree. Knee cannot be fully extended
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Poliomyelitis• Spinal type
– Paralytic type
– Lesion of anterior horn cell of spinal nerve
• Bulbar type
– Paralytic
– Lesion of motor nuclei of cranial nerve
• Encephalitic type
– Nonparalytic
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Poliomyelitis• Classification as progress and symptoms
– Acute stage(1~4 weeks or 6 weeks)• General symptoms
– Fever : peak-3 days
– Headache
– Increase pulse rate
– Vomiting
– Diarrhea
– Abdominal distress
• Meningitic sign– Nausea
– Vomiting
– Neck stiffness
– Convulsion
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Poliomyelitis– Paralytic stage• Severe general symptoms
– Coma
– High fever(39.5~40.5 degree)
– Muscle tenderness and pain
• 1~3day after severe symptoms– Drop fever
– Motor paralysis
– Recovery stage(first few month after onset)(1months ~ 2yrs)• Maximum recovery occurs in the first few month
– First 3 month : 50%
– First 6 month : 20%
• Critical period in physical therapy
– Residual stage(잔류기)
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Poliomyelitis
• Complication– Muscle weakness• L/E > U/E
• L/E : tibialis anterior > peroneus > quadriceps > gluteal muscle
• U/E : detoid
– Contracture
– Fatigue
– Respiratory insufficiency
– Decreased functional activity
– atrophy
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Poliomyelitis• Treatment
– Acute stage• Resting• Decrease pain : Sister Kenny hot pack(46.1~48.9 degree 60~62.8
degree))• Prevent deformity : proper positioning, ROM Ex.• Bulbar type : protect life- O2 supply
– Recovery stage• Hubbard tank
– Start : 98 F– After 10 min. : 102 F(below 12 yrs : 100 F)– Agitator : 7~8 min.– Decrease temperature : 98 F
• Range of motion Ex.• Strengthening Ex.• Muscle reeducation Ex.• EST : prevent atrophy• Correct deformity
– Brace : support when standing and gait– Prevent deformity– Prevent to stretching of ligament and muscle– 90 degree ankle posterior stop : foot drop
• Stretching Ex.
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Postpolio syndrome
• Definition : appear neurological, musculoskelectal, psychological problem in later(average 35 yrs after acute onset)
• Most common physical problems– Profound fatigue(예전과는 다른 피로감)– Pain– New weakness– Decrease function– Decrease safety– Decrease quality of life– Muscle fasciculation and cramps– Hypoventilation– Swallowing difficulty– Sleep disturbance
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Postpolio syndrome
• Etiology– Unknown– May be• Reactivation of polio virus• Loss of neuron after age 60
• Management of postpolio syndrome– Alleviate and prevent the cause of pain• Incidence
– 80% in those walking without orthotic assistance– 100% in those using crutch or manual w/c for locomotion
• Characteristic of pain– Appear 1 or 2 days after activity– Increased by physical activity and decreased by rest
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Postpolio syndrome
• Causes– Over loaded
– Long term microtrauma – due to use ligament and bone structure
– Postural abnormality
– Gait deviation
• Management– Decrease energy expenditure and over work
– Orthotic support
– TENS
– Correct body alignment
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Postpolio syndrome– Abnormal fatigue• Incidence : 90%
• Management– Decreasing energy expenditure
– Breaking activities up into parts with frequent rest
– Change of activity
– Daily rest periods or a nap as necessary
– New muscle weakness• Abnormal muscle activity or atrophy occurs
– With previous paresis muscle
– Without previous paresis muscle
• Most common muscle : calf muscle group
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Postpolio syndrome• Management
– Decreasing the work load of the muscle– Weight reduction– Locomotion
» Light weight w/c or motorized vehicle» Modifying environment
– Correction of posture and gait deviation » Mechanical restoration of lumbar curve in seating
system : w/c, automobile» Thoracolumbar corset to support abdominal muscle
paralysis– Orthotic application
» Minimize leg length difference» Restore weight bearing on the weaker leg» Control unstable or painful joint» Minimize gait deviation» Increase shock absorption» Decease energy expenditure
– Modify activity– Orthotic assistance
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Postpolio syndrome
• Environmental cold intolerance– Causes : result from sympathetic neuron
involvement loss of vasocontriction– Management • Clothing• Heating pad or hot water
• Sleep disturbance– Incidence : 50%– Causes : pain, stress, underventilation– Management• Foam mattress• Water bed• Modification sleeping posture
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Postpolio syndrome
• Exercise– Avoid excessive Ex.
• Strengthening• Aerobic• Sport Ex.
– Careful fatigue• Low resistance• Low repetition• Careful monitoring
– Beneficial Ex.• Relaxation Ex.• Passive Ex.• Yoga• Nonresistive underwater Ex.• Easy, noncompetitive recreational swimming
• Psychosocial consideration
Add to the work load
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Progressive muscular atrophy(PMA)
• Motor neuron disease– Poliomyelitis(invoved anterior horn cell)
– Progressive muscular atrophy(involved anterior horn cell)
– Amyotrophic Lateral Sclerosis(involved anterior horn cell and pyramidal tract)
– Spinal muscular atrophy(involved anterior horn cell, cranial nerve nuclei)• Werdnig Hoffman disease
• Kugelberg Welander disease
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Progressive muscular atrophy
• Etiology of PMA– Cause : unknown– Incidence : male > female
• Involved lesion– Degeneration of anterior horn cell
• Clinical symptoms– First symptoms : begin weakness and atrophy of lumbrical, thernar,
hypotherna muscle– Claw hand– Progressively weakness of proximal muscle– Asymmetrical involvement– L/E : progressively weakness from foot muscle to proximal muscles– Severe case : involved respiratory muscles– Decreased or lost of DTR– Appear fasciculation and cramp– Gradually progress 2~20 yrs (average 10 yrs)
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PMD vs PMA
PMD PMA
Onset
First weakness
Involved area
Symmetric
Genetic
Child
Proximal
General
Symmetrical
Genetic
Young or adult
Distal
Local
Asymmetrical
No genetic history
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Progressive muscular atrophy
• Treatment
– Impossible expect to recover by treatment, no effective treatment
– Prevent deformity
– Maintain muscle power
– Maintain functional level
– Maintain respiratory function• Breathing Ex.
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Amyotrophic lateral sclerosis
• Definition– Degenerative disease
– Involved both upper and lower motor nerve
– Involved both cranial nerve and spinal motor nerve
• Etiology and incidence– Cause : unknown
– Incidence : 1.5/100000(U.S)
– Onset : 25~60 yrs
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Amyotrophic lateral sclerosis• Process of disease
– Start of degeneration change at pyramidal(corticospinal, corticobulbar)tract
– Progressively involved anterior horn cell and cranial nuclei
• Clinical symptoms– Early stage• Appear spasticity and weakness at U/E > L/E• Appear Babinski sign
– Later stage• Decreased spasticity and DTR• Decreased or loss of Babinski sign
– No sensory change– Muscle weakness and atrophy– Bulbar palsy• Dyspnea• Dysphagia• Dysphasia• Speech disturbance
– Respiratory problem
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Amyotrophic lateral sclerosis• Process
– Survival : 15~20% : above 5 yrs, die : 1~10 yrs
• Treatment goals– Maintain muscle power
– Maintain ROM
– Maintain respiratory function
– Prevent pulmonary complication
– Maintain functional ability and A.D.L
– Emotional support
• Treatment– Active and assistive EX.
– ROM Ex.
– Functional and A.D.L training
– Chest PT• Coughing
• Postural drainage