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42 Cards in this Set
- Front
- Back
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Describe the general pathway of an UMN in the CNS |
-Originate in the motor cortex (Betz Cells) (Before ant horn cell) -Axons descend vis corticobulbar and corticospinal tracts -Synapse with LMN in brainstem and SC |
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Describe the general pathway of a LMN in the PNS |
-LMN from corticobulbad and Corticospinal tracts run with afferent fibers in peripheral nerves (After ant horn cell) -Synpase with muscle fibers at NMJ |
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Neurogenic vs Myopathic Dz (specific) |
Neuro: ALS, Guillain Barre Myopathic: Duchenne's MD, Poliomyelitis |
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Define Motor Neuro Diseases (MND) |
A group of Progressive neurological disorders that destroy cells that control essential muscle activity Causes gradual muscle weakening, atrophy, and fasciculations and eventually the inability to control voluntary movement |
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What is the Etiology of MND? |
May be inherited or sporadic --Sporadic: Environment, toxic, viral, genetic factors Occurs in all age groups -Children at birth or when the learn to walk -Adults, symptoms appear after 40 More common in Men |
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Common MNDs |
ALS Primary LS Guillain Barre Progressive bulbar palsy Pseudobulbar palsy Progressive muscular atrophy Muscular dystrophy Post Polio |
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Symptoms of MND |
Primary -Weakness -Dec Endurance -Fatigue (central/peripheral) Secondary -Impaired ROM -Pain -Muscle Spasms |
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What is the difference between central and peripheral fatigue? |
C: Inability to recruit motor units (Neurogenic) P: Impaired force generation (Myopathy) |
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Pathophysiology of ALS |
Massive loss of Anterior horn cells of SC and Cranial nerve nuclei in lower brain stem --Muscle atrophy and weakness Demyelination and gliosis of corticospinal tracts and corticobulbar tracts caused by degeneration of Betz Cells (Lateral Sclerosis) |
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Why are most cases of ALS sporadic? |
90% Possibly due to DNA mutations --Complex protein folding disorders --5-10% have DNA mutations associated with frontal lobe dementias |
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Differential Diagnosis of ALS |
Diagnosis of exclusion - no single laboratory test available Genetic Testing Biochemical markers in blood and CSF can rule out other diseases EMG and NCV can ID widespread LMN dz Neuroimaging can rule out other diseases |
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ALS diagnostic criteria per WFN (world federation of neurology) |
1) Signs of LMN degeneration by clinical, electrophysiological, or neuro exam 2) Signs of UMN degeneration by clinical exam 3) Progressive spread of signs within a region or to other regions together with the absence of ---Electophys evidence of other dz that may explain LMN/UMN denegeration --Neuroimaging evidence of other dz that may explain the observed clinical and electrophys signs |
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Abnormal Diagnostic findings 'indicating' ALS |
•Weakness,atrophy, fatigue •Fibrillationsand fasciculations •Unstablemotor units •Increasedduration/amplitudes •Low-amplitudepolyphasicmotor unit potentials •Musclebiopsy – denervation atrophy •Muscleenzymes – elevated creatine phosphokinase •NormalCSF •Nochanges myelogram |
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Review Slide 17 |
Differential Dx of ALS |
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Sensory deficits and ALS |
Sensory deficits have been exclusionary, but some evidence of progressive loss of sensation has been found |
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Cognitive deficits and ALS |
-Generally exclusionary -More common with bulbar onset than limb onset -Some present with Frontotemporal Dementia (FTD) --Lower executive functions, word finding, phrase length --Deficits in action knowledge as opposed to object knowledge |
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Review Chart 19 |
Flow chart to determine ALS based on LMN, UMN, and #of regions |
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Diagnosing ALS early |
1) LE onset > UE onset > Bulbar onset Bulbar onset may be more aggressive --tongue weakness, facial and palatal weakness, swallowing difficulties, dysarthria, dysphagia 2) Fasciculations 3) Fatigue 4) Weakness 5) Pyramidal Tract dysfunction - mix of UMN/LMN signs |
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Describe weakness in early diagnosis |
Pts usually dont notice until functional loss (80% of neuron loss) Often distal to proximal; flexors weaker than extensors Asymmetrical weakness with sparring of fibers even in severely atrophied muscles |
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Based on onset, what are the initial ALS symptoms? |
LE Onset - tripping, stumbling, awkwardness when walking or running UE Onset - Difficulty buttoning clothes, picking up small objects, turning keys Bulbar Onset - Speech problems, slurring, hoarseness, decreased volume |
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How does ALS progress? |
Spreads in a contiguous (next to/sequential) manner. SxS spread locally within a region |
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Prognosis of ALS |
-Relentlessly progressive -Respiratory failure and inability to eat in final stages -Death usually related to pneumonia -Death usually 2-5 years |
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Exercise and ALS |
Evidence does not support overuse weakness in ALS. -ALS causes a loss of muscle fibers, but those that remain are normal and trainable Strengthening may minimize the atrophy associated with disuse --No heavy eccentric exercises Maintain joint ROM and muscle length to prevent contractures and minimize pain |
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Review Slide 26 |
ALS and exercise training |
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Goals of PT for ALS |
-Maintain functional capacity of innervated muscle fibers -Prevent/minimize effects of disuse atrophy -Prevent limitations in ROM and muscle length -Max aerobic capacity, endurance, and functional level for as long as possible |
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Expected PT prognosis for ALS |
-Pt will become weaker and more functional dependent despite an time of exercise -Specific goals must be modified to reflect these changes -Once exercise becomes too tiring, it is no longer appropriate |
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Review slide 28 |
ALS exercise programming |
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Special Considerations for ALS and exercise |
Practical Goals and realistic expectations Strengthening for muscles >/=3/5 --Low to moderate w/ fq rest Risk of injury is present due to flaccid limbs, joint discomfort, laxity, and stiffness Pulmonary function is dec and supine positions can inc symptoms Clients may be emotionally liable |
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Describe Guillaine Barre |
-Affects nerve roots and peripheral nerves -Usually occurs a few days or weeks after respiratory or viral infection --can be triggered by surgeries and vaccinations -Acute onset with rapid progression over several days |
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Describe Guillain Barre onset progression |
-Initially distal paresthesia with some weakness -Always Symmetrical -Peaks after 2-3 weeks; by week 3, 90% are at their weakest 30% will require ventilation during course Autonomic effects in 50% of patients Low mortality (5%): due to resp failure |
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Variations of Guillan Barre |
Acute motor axonal neuropathy Acute Motor and sensory axonal neuropathy Miller-Fisher Syndrome (CN symptoms, ataxia) Chronic Inflammatory demyelination polyradicuoneuropathy - progressive or RR and weakness |
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Rehabilliation prognosis for Guillian Barre |
Time to max disability -50% in 1 week -70% in 2 weeks -80% in 3 weeks 50% with minor persistent neurological deficits 15% with permanent residual functional deficits 80% return to ambulatory status by 6mo Tib anterior weakness most common persistent deficit |
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Etiology on Guillian Barre |
27% no preceding illness >66% have infectious dz 2 weeks prior to onset Some evidence suggests association with Campylobacter jejuni, mycoplasma penumoniae, cytomegalovirus, epstein barr |
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Epidemiology of Guillain Barre |
No reference test to confirm Dx Males 2:1 to females 1-3/100,000 ppl Incidence inc linearly with age Peak incidence occurs at late adolescence and in the elderly Annual incidence in pts over age 70 inc to 8.6/100,000 |
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Pathophysiology of Guillain Barre |
-Spinal roots and peripheral nerves infiltrated by lymphocytes and macrophages --preceding virus may change the nature of the cells -Macrophages attack myelin sheaths --> slow conduction --> conduction block (Mild cases leave axons intact to be remyelinated within weeks) |
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Initial symptoms of Guillain Barre |
Muscle weakness and tingling sensations usually first appear in the hands and feet and progress upwards --because signals from the extremities must travel the longest which makes them the most vulnerable to interuption |
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Recovery of Guillain barre is dependent on |
Axonal regeneration Recovery can take months or be incomplete In acute axonal neuropathy, myelin is left intact while axon is directly invaded causing permanent muscle weakness |
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Motor presentation of Guillain Barre |
Rapidly evolving, relatively symmetrical ascending weakness or flaccid paralysis Most common conduction block in the peroneal nerve followed by the tibial nerve -Proximal conduction block is evident more often than distal 38% of pts present with severe fatigue Diminished tendon reflexes 20-38% may req ventilator 35-50% develop cranial nerve involvement - mostly facial weakness (oculomotor/oropharyngeal) |
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Autonomic symptoms of Guillain Barre |
ANS symptoms affect 50% - more common in those on a ventilator Dysautotonia (ortho hypo, unstable BP, arrythmias, bowel//bladder) Low CO Fluctuations in BP DVT Urinary Retention |
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Sensory presentation of Guillain Barre |
Distal Hyperesthesias, paresthesisa, numbness Dec Vibratory or position sense Stocking/glove pattern Pain - axial and radicular, joint, myalgias --Muscle aches/stiffness --Worse at night --Symmetrical in large bulk muscles |
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Clinical "pearl" for Dx Guillain Barre |
Back pain no associated with injury Paresthesias Dec Vibratory sense Dec DTR |
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How is Guillain Barre Diagnosed |
Through Clincial presentation --Typically acute onset of symmetrical symptoms --Variable signs and symptoms can be diccifult to diagnose Diminished nerve conduction velocity CSF contains more protein than usual |
