Neurodegenerative Disorders 1 Flashcards
Degenerative diseases can affect what types of matter
Gray, white, or both
Prognosis for degenerative diseases is what
Steady decline in function because there is no cure for many of them
Huntington’s Disease affects what age group?
Most often associated with what kind of movement?
30-40 y/o
Choreic movement
Huntington’s is a progressive _______ disorder
Genetic
Huntington’s disease is abnormalities of ______, ______ _______, and _______
Movement, personality disturbances, and dementia
pathogenesis of huntingtons
Ventricles are enlarged and atrophy of basal ganglia
The normal balance of inhibition and excitation responses in the ___ and ____ that allows for smooth controlled movement is disrupted
BG and thalamus
Early stage clinical manifestations for Huntington’s
eye and gaze abnormalities
Clumsiness, subtle chorea, athetosis, dystonic posturing. Psychiatric manifestations (depression, anxiety, OCD, psychosis), gait abnormalities, cognitive impairments (decreased attention, memory disorders, impaired executive functions), dysarthria, and dysphagia.
Chorea is increased by
mental concentration, emotional stimuli, performance of complex motor tasks and walking
Late stage clinical manifestations for Huntington’s
Cachexia (wasting of muscle with weight loss despite adequate diet), sleep disorders, urinary incontinence, severe dementia, patients may be bed bound, cannot speak
Huntington’s pts usually die due to
Respiratory functions
Atrophy of BG specifically what 2 structures occur in Huntingtons
Caudate
Putamen Nuclei
What is also known is Lou Gehrig’s Disease?
Amyotrophic Lateral Sclerosis (ALS)
What is the most common form of adult onset progressive motor neuron disease
ALS
ALS is degeneration and scarring of the motor neurons in the ______ aspect of the spinal cord, brain stem, and cerebral cortex
Lateral
______ nerve changes result in muscle fiber atrophy or amyotrophy in ALS
Peripheral
ALS affects upper or lower motor neurons?
Both
ALS typically leads to mortality within how many years
5 (although exceptions as seen with Hawking)
LMN cell death and early denervation leads to
Asymmetrical weakness, usually distal aspect of one limb
Cramping with volitional movement
Progressive wasting and atrophy of muscles
what type of decline is. Noted in 50% of individuals with ALS?
Cognitive
Bulbar signs:
neuron that innervates muscle controlling articulation, chewing and swallowing are affected (inability to hold eye closed, hoarse voice, drooling, dysarthria
Clinical manifestations of ALS
Muscle weakness and atrophy
Fasciculation (spontaneous muscle twitching)
Extensor muscles become weaker than flexor muscles esp in hands
Babinski’s and Hoffmann’s signs and abnormal reflexes
Respiratory failure
Normal response to babinski test vs babinski sign
Negative (normal): Toes flex and Plantarflexion
Positive (Abnormal): Toes extend and fan
Hoffmann test and sign
Flicking the nail or tip of middle finger
Positive sign: Involuntary flexion of the thumb and/or index finger.
Negative (normal) Sign: No movement
How is ALS diagnosed
Clinical features of weakness, atrophy, fatigue
EMG: Shows fibrillations and Fasciculations
Unstable motor units (in rapid progressing)
Low amplitude polyphasic motor unit potentials
Muscle biopsy: shows denervation atrophy
Muscle enzymes such as creatine phosphokinase are elevated
CSF normal
No changes on myelogram
Treatment for ALS
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 maintains the highest level of function throughout the course of disease (education and supporting client and family for rapid decline)
