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Neuromuscular Disorders

Neuromuscular Disorders. Brenda P. Johnson, PhD, RN. Parkinson’s. Progressive neurodegenerative disease of basal ganglia Affects 500,000 in U.S. and 4 million worldwide Characterized by: Resting tremors- unilaterally initially Rigidity Bradykinesia. Pathophysiology.

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Neuromuscular Disorders

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  1. Neuromuscular Disorders Brenda P. Johnson, PhD, RN

  2. Parkinson’s • Progressive neurodegenerative disease of basal ganglia • Affects 500,000 in U.S. and 4 million worldwide • Characterized by: • Resting tremors- unilaterally initially • Rigidity • Bradykinesia

  3. Pathophysiology • Lose 9-13% of dopamine-producing neurons/decade of life • Dopamine has inhibitory effect on excitatory cholinergic neurons (cerebellum) • Dopamine necessary for smooth, coordinated movements

  4. Pathophysiology • Degeneration of substantia nigra and dopamine leads to hyperactivity of cholinergic neurons • Lewy bodies – dense aggregates of proteinsDementia with Lewy Bodies: An Emerging Disease - March 1, 2006 -- American Family Physician • Loss of controlled movements

  5. Types of PD • Parkinson’s disease • Parkinsonism • Post-encephalitic syndrome • Side effects of antipsychotic drugs • Toxic reaction to a chemical agent • Outcome of severe carbon monoxide poisoning

  6. Etiology • Multifactorial causesNational Parkinson Foundation • Genetics – mutations on chromosome 6 linked to Lewy body pathology and accumulation of toxic proteins in neurons • Abnormality of gene that protects cells from oxidative stress • Environmental factors – chemicals, toxins/poisons, viruses pesticides and herbicides; oxidative stress

  7. Manifestations • Extrapyramidal symptoms • Frequent falls or tripping • Difficulty walking • Memory loss • Pill-rolling tremor – unilateral and then bilateral • Dementia – 1/3 in late stages • Constipation • Urinary retention • Orthostatic hypotension • Masklike fascies • Depression • Seborrheic dermatitis • Speech changes • micrographia

  8. Diagnosis • H & P 65-75% accurate • Exclude other causes • Wilson disease • Hypothyroidism • MRI

  9. Treatment • Pharmacological • Dopaminergic • Anticholinergic • Augmenting release of dopamine • Surgery • Deep Brain Stimulation • Transplantation

  10. Guillain-Barre’ Syndrome • Acute Inflammatory Demyelinating Polyneuropathy • Damage to myelin sheaths that surround nerves • Most frequently acquired autoimmune neuropathy

  11. Etiology • Usually follows a viral gastrointestinal infection such as Campylobacter jejune or Cytomegalovirus • Thought that viruses share similar antigens with the peripheral nerve tissue and, thus, elicit an immune response • In rare cases, GBS has been linked to immunizations (first cases noted following Swine flu vaccine), surgery, and childbirth

  12. Signs and Symptoms • Rapid onset of: • Weakness – typically beginning with legs and ascending • Parasthesia • Reflex loss • Breathing may be affected in severe cases (20% require ventilator assistance) • Heart in rare cases

  13. Diagnosis • H & P • Lumbar puncture – elevated proteins • EMG – excitability of peripheral nerves • Rate of progression varies from patient to patient

  14. Treatment • 80-90% have spontaneous recovery • Corticosteroids • Immunoglobulin (IVIg) • Plasmaphoresis

  15. Residual effects • 70% full recovery • 20% 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

  16. Multiple Sclerosis • Disorder of neurotransmission resulting from demyelination and destruction of axons of motor, sensory, and autonomic nerves • Multifactorial • Autoimmune component • Activation of cytotoxic T-cells • Macrophages attack myelin • Lesions or plaques occur • Interruption of nerve transmission • Loss or decrease in functioning

  17. Types of MShttp://www.nationalmssociety.org/favicon.ico • Relapsing- remitting • Relapsing-progressive • Chronic progressive

  18. Manifestations • Ataxia • Impaired sensation • Weakness • Numbness • Poor coordination • Tremors • Bowel or bladder problems • Spasticity or muscle stiffness • Slurred speech • Nystagmus • Memory problems • Visual disturbance • paralysis

  19. Diagnosis • History • MRI • Evoked Potential test • In difficult to diagnose patients, LP or MRI scan

  20. Treatment • Beta Interferons ( relapsing-remitting) • Cytotoxic drugs (chronic-progressive) • Steroids (relapsing-progressive) • Symptomatic drugs • Baclofen • Valium

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