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Degenerative Diseases of the CNS
Ch 31 Test 4
80
Pathology
Graduate
11/29/2011

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Term
Can affect gray matter, white matter, or both.
Definition
Degenerative diseases of CNS
Term
Usually affects one area or system of the CNS more than any other area or system: for example, Huntington's affects the neostriatum of the brain
Definition
Degenerative Diseases of CNS
Term
What movement disorder manifestations occur in the gray matter?
Definition
Functional loss and decreased fractionation of the movement, dementia can be present
Term
What movement disorder manifestations occur in the basal ganglia?
Definition
Involuntary movements
Term
What movement disorder manifestations occur in the Cerebellum and Brain Stem?
Definition
Disruption of smooth coordination of muscles
Term
What movement disorder manifestations occur in the white matter?
Definition
Affecting movement by affecting muscle tone. Increase in the excitation of the muscles resulting in spasticity. Decrease in neuronal input causing weakness of the muscles
Term
Sensory changes affect what 2 things?
Definition
Movement and safety
Term
What are diagnoses based on with degenerative diseases of the CNS?
Definition
Based on the neurological changes associated with the known disease process and its progression; in general, deterioration of body function controlled by the brain and the spinal cord. For example, Parkinson's dz affects basal ganglia
Term
Name 5 types of treatment for degenerative diseases of the CNS
Definition
-No cure
-Treat symptoms
-Prevent secondary complications
-Maintain MS and cardiovascular fitness
-Aid in mobility and function to prolong the independency of ADL
Term
What is another term for Amyotrophic Lateral Sclerosis?
Definition
Lou Gehrig's Dz
Term
A devastating disorder of the anterior horn cells of the spinal cord and the motor cranial nuclei that leads to progressive muscle weakness and atrophy
Definition
ALS
Term
A progressive disorder in which the cause is unkown but may be genetic. Affects upper motor neurons (cerebral cortex) and lower motor neurons (anterior horn cells of the spinal cord)
Definition
ALS
Term
What are the clinical manifestations of ALS?
Definition
Asymmetrical weakness; positive Babinski, difficulty with swallowing and chewing, spasticity, muscle fasiculations. Up to 50% of individual with ALS have impaired cognitive functions. EMG shows signs of diffuse denervation with generally preserved nerve conduction velocities.
Term
What is the treatment for ALS?
Definition
No cure
Symptomatic Therapy
Term
What are some good PT treatments for ALS
Definition
Maintain function as much as possible
Slow stretching for spasticity
Gait analysis
Assistive devices/braces
Term
A progressive neurodegenerative disorder characterized by the gradual onset of dementia. One of the most common diseases in the aging population, and it ranks as the 4th most common cause of death.
Definition
Alzheimer's
Term
What is the most common cause of dementia?
Definition
Alzheimer's
Term
An acquired cognitive and behavioral impairment of sufficient severity to markedly interfere with social and occupational functioning.
Definition
Alzheimer's
Term
Affects approximately 5 million ppl in the US and more than 30 million ppl worldwide. In the US, more than 14% of individuals over 65 have this and the prevalence increases to at least 40% in ppl over 80
Definition
Alzheimer's
Term
Pathophysiology and etiology of this are unknown. It has been inconsitently associated with traumatic head injury, low educational achievement, depression, advanced parental age at birth, smoking, downs syndrome in a first degree relative.
Definition
Alzheimers
Term
In some observational studies, the use of estrogen-replacement therapy in postmenopausal women and the regular use of anti-inflammatory agents in both men and women have been associated with lowered risks of this.
Definition
Alzheimers
Term
What are some of the pathological hallmarks of Alzheimer's?
Definition
Bete amyloid (senile) plaques, neurofibrillary tangles, and reactive gliosis at the microscopic level; and cerebrocortical atrophy, which predominantly involves the association regions and, particularly, the medial aspect of the temporal lobe.
Term
How can alzheimer's be diagnosed?
Definition
Clinical, neuropsychologic, and neuroimaging assessments. Routine structural neuroimaging evaluation is based on nonspecific features such as atrophy, a late feature in the progression of the dz. Therefore, developing new approaches for early and specific recognition at the early stages is of crucial importance.
Term
Progressive dementia of unknown cause that comes from cerebral cell death and atrophy as well as the development of insoluble, fibrous amyloid (senile plaques)
Definition
Alzheimers
Term
Manifests as disorders of higher brain function; loss of cognition, memory, and language. There is no cure so just treat the symptoms.
Definition
Alzheimer's
Term
-A high percentage of pts are susceptible to falls
-Encourage group therapy
-Exercises should be short and simple and done in the same order each day
Definition
Alzheimer's
Term
A syndrome of involuntary sustained or spasmodic muscle contractions involving co-contraction of both the agonist and the antagonist. The movements are usually slow and sustained. They often occur in a repetitive and patterned manner, but they can be unpredictable and fluctuate. The resulting frequent abnormal posturing and twisting can be painful and functionally disabling.
Definition
Dystonia
Term
A neurologic syndrome with involuntary, sustained muscle contractions; Primarily the cause is idiopathic; Secondarily the cause is by areas of the brain being damaged or scarred
Definition
Dystonia
Term
Results in an alteration in neural activities in the brain/CNS. Manifests as involuntary ms contractions, abnormal posture, and cervical dystonia
Definition
Dystonia
Term
How is dystonia treated?
Definition
Symptomatic therapy; no cure
Term
Should not be treated with modalities such as massage and electrical stimulation
Definition
CNS dystonia
Term
What should the PT focus on with dystonia?
Definition
Treating the movement disorder with exercise, balance,a nd proprioception training and education of the use of adaptive equipment
Term
What is another term for Huntington's Dz?
Definition
Huntington's Chorea
Term
An incurable, adult-onset, autosomal dominant inherited disorder associated with cell loss within a specific subset of neurons in the basal ganglia and cortex
Definition
Huntington's
Term
What are some characteristic features of Huntington's
Definition
Involuntary Movements
Dementia
Personality disturbance
Behavioral changes
Term
What is the pathophysiology of huntington's?
Definition
The most striking neuropathology in huntington's occurs within the neostriatum, in which gross atrophy of the caudate nucleus and putamen is accompanied by selective neuronal loss and astrogliosis. Marked neuronal loss also is seen in deep layers of the cerebral cortex. Other regions, including the globus pallidus, thalamus, subthalamic nucleus, substantia nigra, and cerebellum, show varying degrees of atrophy depending on the pathologic grade
Term
A progressive disease that is inherited and the cause is unknown. The ventricles enlarge as a result of atrophy of the brain at striatum of basal ganglia, specifically the caudate nucleus and putamen; brain volume can decrease as much as 20%
Definition
Huntington's
Term
What is the most common movement disorder seen in huntingtons?
Definition
Chorea
Term
A state of excessive, spontaneous involuntary movements, irregularly timed, randomly distributed, and abrupt rapid tongue movements, finger-to-thumb tapping, rigidity, dysarthria, dysphagia
Definition
Chorea
Term
This disease results in a cognitive decline that progresses to dementia. Includes early onset behavioral changes such as irritability, untidiness, and loss of interest. Slowing of cognition, impairment of intellectual function,a nd memory disturbances are seen later. Behavioral disorders are most commonly seen by affective illness - depression is more prevalent
Definition
Huntington's
Term
How do you treat huntington's?
Definition
Treat the symptoms because there is no cause
Term
What should the PT do with a patient who has huntington's?
Definition
-Educate pt on safety and gait concerns
-Address postural instability
-Pt may lose inability to dress, self-care, and ADLs
Term
A progressive disease that attacks the CNS and affects multiple systems of the body thru attacks on the nervous system.
Definition
Multiple Sclerosis
Term
An immune-mediated inflammatory disease that attacks myelinated axons in the CNS, destroying both the myelin and the axon in variable degrees, resulting in progressive disability over time
Definition
MS
Term
The cause of this is unknown, but it likely involves a combo of genetic susceptibility and an environmental trigger, resulting in a self-sustaining autoimmune disorder that leads to recurrent immune attacks on the CNS
Definition
MS
Term
The cause of this is unknown, but it likely involves a combo of genetic susceptibility and an environmental trigger, resulting in a self-sustaining autoimmune disorder that leads to recurrent immune attacks on the CNS
Definition
MS
Term
A major cause of disbility in young adults. 450,000 ppl are affected in US with 10,000 new cases per year
Definition
MS
Term
Rarely begins before adolescence; rises steadily in incidence from the teens to age 35 and declines thereafter. Has a predilection for women with a ratio of 2.5:1
Definition
MS
Term
Affects individuals of all racces and socioeconomic groups and is seen all over the world. Most common in white women of northern European descent. Extremely rare in Japan and in black Africa
Definition
MS
Term
A chronic illness that manifested in multiple forms and courses, characterized initially by episodes of reversible neurologic deficits, which, in most patients, are followed by progressive neurologic deterioration over time.
Definition
MS
Term
What are the 4 subtypes of MS?
Definition
Relapsing-Remitting
Secondary Progressive
Primary Progressive
Progressive-Relapsing
Term
Characterized by relapses or attacks, which are periods of neurologic dysfuntion lasting days to months and followed by full or partial recovery. By convention, new symptoms must last at least 24 hours and be separated from other symptoms by at least 30 days to qualify as a new attack. The hallmark is that there is a stable course between relapses. This is the most common pattern, seen in about 85% of newly diagnosed individuals.
Definition
Relapsing-Remitting MS
Term
Characterized by an intial pattern of relapse and remission that changes into a steadily progressive pattern over time in more than 50% of relapsing individuals. There sometimes are continued relapses during this phase. This conversion generally occurs 5 to 10 years after the initial onset of relapsing symptoms
Definition
Secondary Progressive MS
Term
Characterized by a steady decline in neurologic function from the outset with episodes of minimal recovery. The most common clinical presentation of this is myelopathy, a gradual, progressive weakening and wasting of muscles, which is typically seen in persons with onset past the age of 40 years.
Definition
Primary Progressive MS
Term
Characterized by a progressive dz from the onset with clear exacerbations. This is considered the rarest form of MS.
Definition
Progressive-Relapsing MS
Term
Typically a dz of young adults, abnormal autoimmune reaction in the CNS; possible genetic susceptibility to a virus
Definition
MS
Term
A primary demyelinating disorder. The pathologic findings include inflammation, demyelination, and axon loss. It is predominately a T cell mediated inflammatory disorder with overproduction fo proinflammatory cytokines, resulting in demyelination, axon loss, and cell death
Definition
MS
Term
Name 9 clinical manifestations of MS
Definition
Optic Neuritis
Sensory changes
Weakness
Fatigue
Spasticity
Ataxia
Pain
Bowel and Bladder Symptoms
Depression
Term
Typically presents as a unilateral, painful decrease or loss of vision
Definition
Optic neuritis
Term
Most often the initial complaints with MS. This is often a paresthesia or dysesthesia noted in one extremity or in the head and face. Visual blurring, diplopia, weakness, and balance problems also may be early signs
Definition
Sensory changes
Term
The single most common and most disabling symptom of MS; resistive exercises often result in a feeling of increased fatigue and weakness rather than a feeling of increased strength
Definition
Fatigue
Term
Heat, either from increased ambient temperature or from fever often increases ___
Definition
Weakness
Term
Velocity-dependent stiffness about a joint: is an extremely common problem with MS, occurring in 90% of all cases
Definition
Spasticity
Term
How do you treat MS?
Definition
No cure so treat the immune system dysfunction
Term
What can PTs do for MS pts?
Definition
-Encourage them to be active
-Be aware of balance disorders
-Pts complain of weakness and fatigue
-May need assistive devices
Term
A chronic progressive CNS neurodegenerative disease that typically occurs in adults older than 65
Definition
Parkinson's
Term
What is usually the first symptoms of parkinsons?
Definition
Unilateral resting tremor of the hand (pill-rolling tremor)
Term
Common clinical features include cogwheel rigidity, bradykinesia, and postural instability. Rigidity and bradykinesia tend to be more disabling than the tremor
Definition
Parkinson's
Term
The basic pathophysiology is a lack of dopamine-producing cells in the basal ganglia. This disorder is treated both surgically and medically.
Definition
Parkinson's
Term
A disorder of the extrapyramidal system (motor structures in the basal ganglia)
Definition
Parkinson's
Term
May result from degeneration of dopamine-producing cells in the substantia nigra, resulting in decreased levels of dopamine in the striatum. Symptoms usually begin to appear when the dopamine levels drop by at least 50%
Definition
Parkinson's
Term
Associated hyperactivity of cholinergic neurons in the caudate nuclei results in an imbalance in the normal dopamine-to-acetylcholine ratio, which contributes to the symptoms. The exact cause of these changes remains unknown.
Definition
Parkinson's
Term
Progressive disease of the motor component of the CNS; unknown cause but some forms may be caused by toxic agents
Definition
Parkinson's
Term
Occurs when the substantia nigra loses its ability to produce dopamine; increase in activation of motor systems.
Definition
Parkinsons
Term
What are the general manifestations of parkinsons?
Definition
Unintentional tremors, "pill-rolling" tremor, rigidity, bradykinesia, akinesia, "mask" face, impoverished movement gait, flexion of the neck, trunk, hip, and knee posture
Term
What is the typical posture of a parkinsons pt?
Definition
Expressionless face
Drooling
Bent posture
Tremor of hands
Stiff, shuffling gait
Term
Name 8 causes of balance impairment with parkinson's pts.
Definition
1. Loss of postural reflexes
2. Visuospatial deficits
3. Retropulsion
4. Start hesitation
5. Freezing
6. Festinating Gait
7. Orthostatic Hypertension
8. True vertigo
Term
How can parkinson's be treated?
Definition
Drug therapy
Term
What does a PT work on with a parkinson's pt?
Definition
Balance
Gait
Conditioning
Respiration
Term
What is the leading cause of death in parkinson's pts?
Definition
Respiratory complications
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