Neuromuscular DisordersBrenda P. Johnson, PhD, RN

ParkinsonsProgressive neurodegenerative disease of basal gangliaAffects 500,000 in U.S. and 4 million worldwideCharacterized by:Resting tremors- unilaterally initiallyRigidityBradykinesia

PathophysiologyLose 9-13% of dopamine-producing neurons/decade of lifeDopamine has inhibitory effect on excitatory cholinergic neurons (cerebellum)Dopamine necessary for smooth, coordinated movements

PathophysiologyDegeneration of substantia nigra and dopamine leads to hyperactivity of cholinergic neuronsLewy bodies dense aggregates of proteinsDementia with Lewy Bodies: An Emerging Disease - March 1, 2006 -- American Family PhysicianLoss of controlled movements

Types of PDParkinsons diseaseParkinsonismPost-encephalitic syndromeSide effects of antipsychotic drugsToxic reaction to a chemical agentOutcome of severe carbon monoxide poisoning

EtiologyMultifactorial causesNational Parkinson FoundationGenetics mutations on chromosome 6 linked to Lewy body pathology and accumulation of toxic proteins in neuronsAbnormality of gene that protects cells from oxidative stressEnvironmental factors chemicals, toxins/poisons, viruses pesticides and herbicides; oxidative stress

ManifestationsExtrapyramidal symptomsFrequent falls or trippingDifficulty walkingMemory lossPill-rolling tremor unilateral and then bilateralDementia 1/3 in late stagesConstipationUrinary retentionOrthostatic hypotensionMasklike fasciesDepressionSeborrheic dermatitisSpeech changesmicrographia

DiagnosisH & P 65-75% accurateExclude other causesWilson diseaseHypothyroidismMRI

TreatmentPharmacologicalDopaminergicAnticholinergicAugmenting release of dopamineSurgeryDeep Brain StimulationTransplantation

Guillain-Barre SyndromeAcute Inflammatory Demyelinating PolyneuropathyDamage to myelin sheaths that surround nervesMost frequently acquired autoimmune neuropathy

EtiologyUsually follows a viral gastrointestinal infection such as Campylobacter jejune or CytomegalovirusThought that viruses share similar antigens with the peripheral nerve tissue and, thus, elicit an immune responseIn rare cases, GBS has been linked to immunizations (first cases noted following Swine flu vaccine), surgery, and childbirth

Signs and SymptomsRapid onset of:Weakness typically beginning with legs and ascendingParasthesiaReflex lossBreathing may be affected in severe cases (20% require ventilator assistance)Heart in rare cases

DiagnosisH & PLumbar puncture elevated proteinsEMG excitability of peripheral nervesRate of progression varies from patient to patient

Treatment80-90% have spontaneous recoveryCorticosteroidsImmunoglobulin (IVIg)Plasmaphoresis

Residual effects70% full recovery20% able to walk, but not run (1 year following onset)8% unable to walk unaided (1 year following onset)2% bedridden and ventilator dependent (1 year following onset)May also have some degree of pain, fatigue, and emotional effects

Multiple SclerosisDisorder of neurotransmission resulting from demyelination and destruction of axons of motor, sensory, and autonomic nervesMultifactorialAutoimmune componentActivation of cytotoxic T-cellsMacrophages attack myelinLesions or plaques occur Interruption of nerve transmissionLoss or decrease in functioning

Types of MShttp://www.nationalmssociety.org/favicon.icoRelapsing- remittingRelapsing-progressiveChronic progressive

ManifestationsAtaxiaImpaired sensationWeaknessNumbnessPoor coordinationTremorsBowel or bladder problemsSpasticity or muscle stiffnessSlurred speechNystagmusMemory problemsVisual disturbanceparalysis

DiagnosisHistoryMRIEvoked Potential testIn difficult to diagnose patients, LP or MRI scan

TreatmentBeta Interferons ( relapsing-remitting)Cytotoxic drugs (chronic-progressive)Steroids (relapsing-progressive)Symptomatic drugsBaclofenValium