CNS Pathologies Flashcards
Huntingtons Disease most often associated woth
choreic movement = brief, purposeless, involuntary, and random
Huntingtons
Atrophy of the
basal ganglia “BG” (specifically the caudate and putamen nuclei)
Huntingtons
Early Stage
Eye and gaze abnormalities
Clumsiness, and subtle chorea, athetosis, and dystonic posturing
Chorea is increased by
Mental concentration
Emotional stimuli
Performance of complex motor tasks and walking
Huntingtons
Clinical Manifestations
Early Stage
Psychiatric manifestations: depression, anxiety, obsessive-compulsive disorder, and psychosis
Gait abnormalities
Cognitive impairments: decreased attention, memory disorders and impaired executive functions
Dysarthria
Abnormalities of swallowing (dysphagia)
Can cause choking and asphyxia
Huntingtons
Later Stages Clinical Manifestations
Cachexia
Wasting of muscle with weight loss, despite an adequate diet
Sleep disorders
Urinary incontinence
Sever dementia
Patients may be bed-bound, cannot speak, usually die due to respiratory infections
Special Implications Huntingtons
Gait and safety (important)
Auditory stimulus to imrpove gait (metronome)
Huntington at earlier stage (more likely to experience fall)
ALS
Degeneration and scarring of the motor neurons in the
lateral aspect of the spinal cord, brainstem, and cerebral cortex
ALS
Peripheral Nerve changes result in
muscle fiber atrophy or amyotrophy
ALS Classic Assessment Findings
Muscle weakness causes profound limitation of movement
Muscle atrophy
ALS Incidence
Men > Women
Chronic exposure to heavy metals
White or non hispanic
50 or older
ALS
LMN cell death and early denervation
Asymmetrical weakness, usually of the distal aspect of one limb
Cramping with volitional movement
Progressive wasting and atrophy of muscles
ALS
____ more impacted than _____
DF, PF
Bulbar signs:
neuron that innervate ms. controlling articulation, chewing, and swallowing are affected
E.g., inability to hold the eye closed, hoarse voice, drooling, and dysarthria
ALS Clinical Manifestations
Muscle weakness and atrophy
Fasciculations, or spontaneous twitching of muscle fibers
Extensor muscles become weaker than flexor muscles, especially in the hands
Babinski’s and Hoffmann’s signs and abnormal reflexes
Respiratory failure
ALS Diagnosis
Clinical features of weakness, atrophy, and fatigue
EMG: shows fibrillations and fasciculations
Unstable motor units (in rapidly progressing ALS)
Low-amplitude polyphasic motor unit potentials
Muscle biopsy: shows denervation atrophy
Muscle enzymes, such as creatine phosphokinase, elevated
CSF normal
No changes on myelogram
ALS Treatment
Unknown – Riluzole and Radicava can be used
Non-invasive ventilation
Stem cell therapies – promising
Survival is longer for individuals with bulbar symptoms
Up to 80% die at home
Emphasis on maintaining the highest level of function throughout the course of the disease
Educating and supporting the client and family for the rapid decline in degenerative disease processes
ALS Special Implications
Verabl fluency can be affedcted
Regular exercise can help to help fatigue
May complain of pain, start early
Slow continuous stretching help with cramping
Change in gait can be significant (falls common)
DF lost before PF
Hamstring strength important in walking
Engage in helpful device to assist ADL (OT will help too)
A lot will have sexual frustration and boredom
Respiratory changes expected and will lead to death
Aspiration is common (swallowing impacted)
Pseudobulbar affected (random period of laugh and crying)
Alzheimers affected brain areas
cerebral cortex, hippocampus, and amygdala
Neurofibrillary tangles
Senile plaques – interfere with signaling
Atrophy – as the neuron dies the brain will atrophied and shrinks
Plaques and tangles are a normal consequence of the aging process, but their numbers __________ in persons with AD
dramatically increase
Alzheimers
Early Symptoms
Visuospatial deficits
Psychological changes
Mood changes
Subtle personality changes
Indifference, egocentricity, impulsivity, and irritability
Often the first symptom is loss of the ability to learn new information
Alzheimers
Word finding difficulty is ______
Inability to remember ________
Diminished _________
first
names (anomia)
comprehension (later)
Alzheimers
The loss of ability to solve mathematical problems and handle money is typical in the
early stages of AD
Alzheimers Clinical Manifestations
Loss of smell and taste sensitivity leading to a decrease in appetite
Incontinence
Delusions, agitation, and even violence
Sundowning syndrome
Disorders of sleep, eating, and sexual behavior
