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