1. Huntington's disease
1. Definition
1. progressive atrophy of basal ganglia and some parts of cerebral cortex
2. etiology - genetic disorder, autosomal dominant
2. Findings: increased involuntary movements, progressive decline in cognitive, findings usually occur in middle age
1. motor
1. impaired chewing and swallowing
2. chorea
3. dystonicposture
4. gradually becomes bedridden
2. cognitive: less able to organize, plan and sequence behavior
3. mental: personality changes, depression, even psychosis
3. Diagnostics: history and physical exam
4. Management
1. outcomes: postpone dependence
2. supportive care for findings
3. therapies: speech, physical
4. genetic counseling
5. Nursing interventions
1. foster independence in ADL (activities of daily living)
2. reinforce the use of assistive devices for ambulation as needed
3. teach client to:
1. maintain good nutrition
2. get emotional support from support groups, friends
3. seek genetic counseling
   
2. Amyotrophic lateral sclerosis (ALS, Lou Gehrig's disease)
1. Definition
1. progressive atrophy of spinal muscle; bulbar palsy
2. progressive degeneration of the motor neurons of the anterior horn cells of the spinal cord, brainstem, and motor cortex
3. onset in later middle age; more in men than in women
4. clients with ALS usually die within two to six years
5. etiology unknown
2. Findings
1. usually beginning in head and arms, distal portion first
2. mild clumsiness progressing to total incapacity
3. muscle wasting, atrophy, spasticity
4. speech disorders
5. no change in sensation or autonomic system
6. death most often from complications: respiratory failure, urinary or pulmonary infections for stasis
7. mind usually intact
3. Diagnostics: history and physical exam
   
4. Management
1. outcome: keep functional independence as much as possible
2. no cure
3. management of findings
1. muscle relaxants for spasticity
2. therapies:
• speech
• physical
• respiratory
5. Nursing interventions
1. provide for respiratory care and referrals
2. maintain a safe environment focused toward infection prevention
3. prevent complications of immobility
4. postpone dependence
5. maintain nutrition
6. provide emotional support and referrals to support groups
   
3. Dementia
1. Etiology
1. characterized by irreversible, progressive cerebral dysfunction
2. Alzheimer's disease- most common cause of dementia
1. characterized by brain atrophy
2. development of senile plaques and neurofibrillary tangles in the cerebral hemispheres
3. etiology unknown
2. Findings
1. characterized by decreased intellectual functioning
2. Alzheimer's disease has three stages
1. early stage:
• memory loss
• subtle personality changes
• difficulty with abstract thinking
2. middle stage:
• impaired language
• difficulty with motor activity and object recognition
• wandering
• inability to carry out ADLs
• impaired judgment
3. final stage:
• complete loss of language
• loss of bowel and bladder control
3. prognosis - incurable
3. Management - maintenance of functional capacity
4. Nursing interventions
1. meet client's physical needs
2. promote client's independence
3. promote contact with reality
4. establish a routine
5. provide emotional support or caregiver support with appropriate referrals
   
4. Cerebrovascular Accident (CVA, Stroke) (illustration)
5. Definition: decreased blood supply to the brain

                                                      1.            Risk factors

1. hypertension, uncontrolled
2. smoking
3. obesity
4. increased blood cholesterol and triglycerides
5. chronic atrial fibrillation
1. Five classes of stroke: by severity
1. transient ischemic attack (TIA), "angina" of the brain
1. TIA is warning sign of stroke
2. localized ischemic event
3. produces neurological deficits lasting only minutes or hours
4. full functional recovery within 24 to 48 hours
2. reversible ischemic neurological deficit (RIND)
1. similar to TIA
2. findings last between 24 hours and three weeks
3. usual full functional recovery within three to four weeks
3. partial, nonprogressing stroke: some neurological deficit, but stabilized
4. progressing stroke (stroke in evolution)
1. deteriorating neurological status often with grand mal seizure activity
2. with residual neurological deficits
5. completed stroke