Disease Motor-Sensory Unit Classification Region Muscle Pathophysiology Inheritanc e

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  • 8/14/2019 Disease Motor-Sensory Unit Classification Region Muscle Pathophysiology Inheritanc e

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    Disease Motor-SensoryUnit

    ClassificationRegion

    Pathophysiology Inheritance Mode

    Symptoms Treatment

    Duchennes

    MuscularDystroph

    y

    Muscle -Abn. Dystrophin gene (anchorsactin to membrane/ stabilizessarcolemma)- High MM-CK

    -Hypercontracted fibers-Necrotic Fibers

    X-linkedInheritance

    -Muscle Weaknessprogressively severe- weak pelvic musclesLordosis/scoliosis

    -contractures(staticmuscle shortening)Growers maneuver-push off ground slowly

    No cureOnset 2-3yoDeath 20-30yoresp/cardio failure

    PT for musclesCorticosteroids-slow muscledeterioration

    Myasthenia Gravis

    NeuromuscularJunction

    1. Autoimmune(common)Antibodies to AchRcrosslink/ degradation ofreceptor/ reducesprobablilty Ach will bindto AchR/ Decreasejunctional folds/ larger

    synaptic cleft/ decreaseAchR/ rapid turnoverAchR

    2. Congenital (Rare)Abn. Ach release/AchR

    AutoimmuneorCongenital

    -Muscle weakness-Fatigue due to Achdepletion-weak cranial muscles,eyelids, eyemuscles,ptosis- weakness,

    oropharyngeal muscles,speech difficulty, etc.- worse at end ofday/exercise-Ach depletion recoveryovernight-associated w/otherautoimmune

    Thymus Glandremoved (tumor)-producedantibodies to AchR

    1)Acetylcholinesterase inhibitors

    2)Immunosupressive therapies -cotricosteroids3)Plasmaphoresis

    Amyotrophic

    LateralSclerosis

    Motor Neuron1. LMN2. UMN

    Disease of Corticospinal tractscarry axons from UMN(premotor)fromcortex/brainstem Spinal Cord

    LMN-loss Motorneurons/brainstemUMN- loss Corticospinal tract/Betz cell

    Pallor (paleness) inCorticospinal tracts in spinalcord/ Ventral cortical spinaltract

    Sporadically LMN- fasciculation, losstendon reflexes, weakwasted and twitchingmuscles

    UMN- Spasticity(increasedmuscle tone) , overactivetendon reflexes, ABNBabinski sign (No curlingtoes)

    SPARES OCULAR M/VOLUNTARY control ofBLADDER sphincters

    Dysarthria- slow speechDysphagia- difficulty

    50% Die w/in 3years of onset

    90% Die w/in6years

    No CureRilzule: blockglutamate releaseAntioxidants

    Neurotrophic/grothfactors

    PT

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    swallowingRespiratoryweakness/failure

    Emotional Incontinence-Pseudobulbar Effect

    Gullian

    BarreSyndrome

    Peripheral NerveNeuropathy (axon,

    myelin/sensory/motor/ largediameter/ smalldiameter)

    Acute inflammatorydemyelinating polyneropathy

    (Viral infection) attack myelinsheath around peripheralnerves

    Tingling of hands/feet(Paresthesia)

    Insensitivity to heat/coldDifficulty getting out ofchair , weakness inbreathing muscles, eyes,face, paralysis of legs

    Loss of stretch Reflex

    Normal Babinski

    Reversible/Permanent

    PlasmaphoresisRecovery weeks-months

    Myopathic- abnormalities in skeletal muscleNeuropathic- Abnormalities in peripheral nerves; more pronounced distally than proximally

    Neurogenic- Dennervation/rennervation of muscle; disruptes innervations of muscle


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