DEGENERATIVE DISORDERS

Syringomyelia & Syringobulbia

• Syringomyelia - abnormal fluid-filled cavity, called a syrinx, forms in the spinal cord causing pressure on the spine
• Syringobulbia - fluid-filled cavity now reaches the brainstem
• associated with Arnold-Chiara malformations (protrusion of the brainstem inward blocking CSF flow) and spina bifida
• Signs and Symptoms
• sensory deficits/defects
• dysphagia
• nystagmus
• ataxia
• impairment of pain and temperature sensations
• disturbed bladder control
• muscle wasting/weakness
• especially shoulder muscles
• cranial nerve palsies
• ptosis (drooping of eyelids)
• anhidrosis (lack of sweating)
• miosis (pupillary constriction)
• Nursing Diagnoses & Management
• Altered Thermoregulation r/t absence of sweating
• tepid sponge bath
• light clothing
• Risk for Injury r/t loss of pain & temperature sensation
• keep sharp objects away
• avoid floor wax
• check temperature of water before bath
• Activity Intolerance r/t shoulder muscle atrophy
• cluster activities
• ROM exercises
• assist with ADL's
• provide rest periods

Huntington's Disease

• discovered by George Huntington
• area affected: striatum of the basal ganglia
• onset: 30-40 years
• increased amount of glutamine and mutant Huntingtin (mHtt) causes damage to the brain
• results: decreased GABA & acetylcholine; increased dopamine (produces chorea)
• NO CURE
• Signs and Symptoms
• chorea (jerky, random, uncontrollable movements)
• athetosis (writhing movements of hands and feet)
• dementia (loss of cognitive ability for at least 6mos)
• emotional decline
• intellectual decline
• Nursing Diagnoses and Management
• Disturbed Thought Process r/t intellectual decline
• provide intellectual stimuli everyday; e.g. crossword puzzles, sudoku
• frequent reminders of things to do
• promote independence
• Impaired Communication r/t unintelligible words
• provide ample time for patient to talk
• do not hurry conversations
• Risk for Injury r/t uncontrollable movements
• assist in ambulation
• provide assistive devices
• wear sturdy shoes
• avoid slippery floors

Parkinsons's Disease / Paralysis Agitans

• discovered by James Parkinson
• area affected: substantia nigra of the basal ganglia
• onset: 30-60 years
• death of dopamine-producing cells in the brain
• results: decreased dopamine; increased acetylcholine
• NO CURE
• Signs and Symptoms
• PILL-ROLLING TREMORS
• BRADYKINESIA
• RIGIDITY
• POSTURAL INSTABILITY
• festinating gait (small steps in walking; difficulty in starting to walk and difficulty to stop)
• speech and swallowing disturbances
• Treatment
• LEVODOPA-CARBIDOPA (Sinermet) - drug of choice; supplies much needed dopamine
• dopamine agonists (bromocriptine [Parlodel], pergolide) - stimulates live dopaminergic cells to produce dopamine
• anticholinergics (ipratropium) - reduces levels of acetylcholine
• Nursing Diagnoses and Management
• Disturbed Thought Process r/t intellectual decline
• provide intellectual stimuli everyday; e.g. crossword puzzles, sudoku 
• frequent reminders of things to do
• promote independence
  
• Impaired Movement r/t joint rigidity
• Risk for Falls r/t postural instability
• perform ROM exercises
• assist in ambulation
• provide assistive devices
• wear sturdy shoes
• avoid slippery floors
• Risk for Aspiration r/t difficulty in chewing and swallowing
• allow ample time to chew
• eat in an upright position
• soft diet
• Impaired Communication r/t unintelligible words
• provide ample time for patient to talk
• do not hurry conversations

Alzheimer's Disease / Primary Degenerative Dementia

• discovered by Alois Alzhemier
• area affected: neurons of the cerebral cortex
• onset: 65 years and older
• caused by neurofibrillary tangles (aggregates of proteins supporting the microtubules; meaning, clumps of the proteins that help give shape to your cell) and neuritic/amyloid plaques (insoluble fibrous proteins) in the neurons of the brain
• results: decreased acetylcholine
• NO CURE
• Types
• familial - early onset, below 65 years, runs in the family
• sporadic - late onset, above 65 years, doesn't run in the family
• Signs and Symptoms
• AGNOSIA - loss of ability to recognize objects, persons, sounds, shapes, etc.
• AMNESIA - loss of memory
• APHASIA - inability to produce and/or comprehend language
• APRAXIA - loss of ability to carry out purposeful movements
• Stages
• Early / Mild
• increased self-centeredness
• forgetfulness
• difficulty learning
• confusion
• poor judgment
• irritable
• unable to do simple tasks
• Middle / Moderate
• wandering
• agitation
• disorientation
• physical impairment
• delusions
• depression
• impaired decision making
• Late / Severe
• immobility
• inability to recall names
• total dependence
• impaired motor and verbal skills
• bowel and bladder incontinence
• aspiration pneumonia
• Treatment
• acetylcholinesterase inhibitors (galantamine, rivastigmine [Exelon], donepezil) - reduces rate of breaking down acetylcholine
• Nursing Diagnoses and Management
• Disturbed Thought Process r/t intellectual decline
• provide intellectual stimuli everyday; e.g. crossword puzzles, sudoku 
• frequent reminders of things to do
• promote independence
  
• Risk for Injury r/t forgetfulness
• assist in ambulation
• provide assistive devices
• wear sturdy shoes
• avoid slippery floors
• provide identification
• always close doors
• cover electrical sockets, chemical containers, and other dangerous objects

Amyotrophic Lateral Sclerosis / Lou Gehrig's Disease / Charcot's Disease

• area affected: motor neurons of the anterior horn of the spinal cord
• onset: 40-60 years
• excess glutamate leads to cell injury and then cell death
• NO CURE
• Signs and Symptoms
• MUSCLE WASTING & WEAKNESS
• difficulty moving
• dysphagia - difficulty swallowing
• dysarthria - difficulty speaking
• spasticity - muscle spasms
• hyperreflexia - overexaggerated reflexes
• present Babinski's reflex - fanning of toes
• bowel and bladder incontinence
• ultimately, respiratory arrest due to weakening of diaphragm and intercostal muscles
• Treatment
• riluzole - reduces glutamate
  
• Nursing Diagnoses and Management
• Impaired Movement r/t muscle weakness
  
• Risk for Injury r/t difficulty in movement
  
• perform ROM exercises
  
• assist in ambulation
• provide assistive devices
• wear sturdy shoes
• avoid slippery floors
• Risk for Aspiration r/t difficulty in chewing and swallowing
• allow ample time to chew
• eat in an upright position
• soft diet