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 movementsDopamine necessary for smooth

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 PhysicianDementia 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 FoundationNational 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 neuropathyneuropathy

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.nationalm ssociety.org/favicon.icohttp://www.nationalm ssociety.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