Movement Disorders Flashcards
Criteria for Parkinson’s disease
Brady kinesia
resting tremor
rigidity
Which parts of the brain are involved with movement disorders
Basal ganglia which includes caudate nucleus, putamen, Globus pallidus, subthalamic nucleus, substantial nigra, thalamus
Is movement disorder related to perimeter or extra pyramidal system
It is related to extrapyramidal system
What are the neurotransmitters of the basal ganglia that contribute to movement disorder symptoms
Dopamine inhibitory, GABA inhibitory, acetylcholine excitatory
DAG
Parkinson’s disease is characterized by
Parkinsonism Neuronal loss in the substantial nigra Dopamine depletion in the stratum Responsiveness to dopaminergic treatment Presence of Lewy bodies Front of critical circuitry dysfunction
Neuropathology of Parkinson’s disease
Dopaminergic neuronal loss in the substantial nigra
Dopaminergic deneevation of the stratum
affects ventral lateral cells in the group
Lewy body and lewy neurites in the brainstem
Alpha sin nuclein pathology outside the nigrostriatal system affecting symptoms such as psychiatric, cognitive dysfunction, autonomic deficiencies, sensory disturbance, pain, sleep
Most cases of Parkinson’s disease are
Sporadic and 90% of cases
10% of the cases are genetics
Risk factors for Parkinson’s disease
Each increase, Hispanics, being male, viral encephelitis, drugs with dopamine antagonistic properties, neuroleptics, substances, herbicides, pesticides, heavy metals, iron levels, drinking contaminated water, gut dysfunction
Medication for Parkinson’s disease
Treating early to middle stages include levodopa, dopamine agonist, MAO B inhibitors
Anticholinergic drugs useful for patients under 70 with tenors
Amantadine for younger patients or later when dyskinesia becomes problematic
DBS treating subthalmic nucleus and Globus pallidus
Neuropsych assessment results in Parkinson’s disease
Poor attention, poor processing speed, dysarthria reduced speech output, hypophonia, micrographia, visual spatial constructional, learning and memory can benefit from cue recognition, difficulty coming up with strategies in remembering things, exocrine functions, flexibility, sensory motor functions, emotional functioning including depression and anxiety,
Huntington’s disease
Fetal autosomal dominant Nuro degenerative disorder
CAG trinucleotide repeats on chromosome 4
Earlier onset is inversely associated with CAG repeats
Is Huntington’s single or multi system
Huntington’s disease is characterized as a single focal or multi system degenerative disease
motor symptoms of Huntington’s disease include the following
Chorea Hypokinesia Dystonia Tic Incoordination
Other features include
unintended weight loss
Sleep circadian rhythm disturbances
Autonomic nervous system dysfunction
Neuropathology of Huntington’s disease
Loss of medium spiny cells seen in caudate nucleus and putamen
Indirect basal ganglia thalamiccortical circuitry
Chorea and motor inpersistence only in early in the disease course
Neurodegeneration in cerebral cortex, thalamus, pallidum, brainstem, cerebellum
With disease progress, further degeneration in striatum circuits, frontal and temporal neo cortical regions
At death, 25 %reduction in brain volume
Neuro findings and Huntington’s
Atrophy in caudate and putamen
Box car ventricles
Epidemiology of Huntington
Mean onset 30 to 50,
range from 2 to 85 years
gender no difference
Morbidity of Huntington
Psychiatric problem
depression high suicide rate
Heart failure in 30%
Weight loss
Disease duration is 15 to 20 years
Pneumonia, heart disease, suicide common causes of death
Amount of CAG repeats determine the severity of Huntington’s
Less than 27 of CAG repeats is normal
27 to 35 is intermediate
36 to 40 is reduced
more than 40 is full
Presentation and symptoms of Huntington’s
Involuntary, unwanted movements
Initially they occur in distal extremities
Unstable gait
Unwanted movements spread from distal to proximal
Person look slightly intoxicated
Chorea movements present during waking hours
Hard to swallow and talk
Dysarthria and dysphasia prominent
Everyone develops hypokinesia, akinesia, rigidity eventually
Behavioral problems
Irritability, depression, anxiety, obsessive compulsive, psychosis, apathy, suicide
Cognitive symptoms with executive function impairment
Why do people with Huntington’s disease have weight loss
Weight loss unintended because of chorea in longer CAG repeat
Treatment for Huntington’s
No cure, no way to slow it down, treatment is focused on helping person live comfortably
Cognitive changes in Huntington’s disease can be detected how many years prior to dx
15
Most sensitive changes cognitively in prodromal period for Huntington’s
Psycho motor and attention issues
Juvenile Huntington’s disease what is frequently observed
Seizures, beaver disturbances, learning difficulties, CAG repeats generally greater than 55, having an infected father
Lewy body dementia
Characterized by parkinsonism and cognitive decline
Lewy body dementia include three types of dementia
Parkinson’s disease with dementia
Diffuse Lewy body disease
Lewy body variant of Alzheimer’s disease
What is the second most common form of dementia after Alzheimer’s disease
Lewy body related dementia
 Symptoms of presentation of Lewy body dementia
Fluctuating cognition with variations in alertness and attention
Recurrent visual hallucinations
REM sleep behavior disorder
Features of parkinsonism
Survival time is Lewy body dementia
Eight years
