Neurological Disorders

Question 1

What is dementia?

Answer 1

Dementia is the loss of ordered neural function which is seen in several unrelated disorders.

Question 2

True or False. Dementia is a disease?

Answer 2

False.

Question 3

What is the most common form of dementia?

Answer 3

Alzheimer’s disease

Question 4

At what age do incidence rates of Alzheimer’s beging to increase?

Answer 4

Age 65 and greater.

Question 5

What is DAT?

Answer 5

Disease of the Alzheimers Type.

Question 6

What percentage of all dementia is Alzheimer’s?

Answer 6

64%

Question 7

What are the two main forms (etiological) of Alzhemiers?

Answer 7

Idoipathic or Sporadic Form which accounts for 90% of all cases. Familial or Genetic form, which accounts for 10% of all cases.

Question 8

What gene is affected in idiopathic sproradic Alzhemiers? What is the function of the gene?

Answer 8

Apolipoprotein E gene (APOE). This gene is involved in the formation of lipoproteins.

Question 9

There are 3 genes involved in familial Alzheimers? What are they? Do any of the genes have relavance in other diseases?

Answer 9

The three genes involved in Alzhemiers are: APP - Amyloid Precursor Protein gene, PS1 - Presenilin Gene, and PS2. The Amyloid Precursor Gene (APP) is also a hallmark gene marked in those whom suffer from diabetes. The APP gene has an effect on the pathway of insulin. Individuals that have diabetes have an increased risk of getting Alzhemiers.

Question 10

What chromosomes are involved in Alzheimers? Do any of the chromosomes have relavance in other diseases?

Answer 10

Chromosome involved in Alzhemiers are: 1, 12, 14, 19, 21. Chromosome 21 is also involved in Down’s Syndrome. There is a definiate link between Alzehimers and Down’s.

Question 11

What is sulci?

Answer 11

Sulci are the dips in the brain.

Question 12

What are gyri?

Answer 12

Gyri are the ridges in the brain.

Question 13

What is the pathophysioloy of Alzhemiers?

Answer 13

Atrophy of the cerebral cortex which is characterized by prominent sulci and slender gyri. The amygdala and hippocampus are affected which result in the congnitive losses. Sensory cortex of the brain is unaffected. Alzheimers is also characterized by Lesions found in the brain. The lesions are neuritic plaques (deposits of amyloid plaques) and nerofibrillay tangles (fibrous proteins in cytoplasm). Those affected with Alzhemers also have decreased levels of acetocholin (ACh).

Question 14

Manafestations of Alzhemiers can be broken into 3 categories. What are they? How long do they take to manifest? Can they be reversed?

Answer 14

Manifestations have an insidious onset. They progress in stages over approximately 10 years. The 3 categories of manifestations are: Mild AD (2-4 yrs), Moderate AD (2-10 yrs), and Severe AD (2 yrs).

Question 15

What are the manifestations of Mild AD?

Answer 15

Begin to develop memory problems, usually detected by family member and friends. Careless work habits, but are able to do routine tasks. Familiar routine management is essential.

Question 16

What are the manifestations of Moderate AD?

Answer 16

Moderate AD manifestations occur between 2 and 10 years. They include: decline in cognitions, confusion, language problems (speech, interpretation, reception), some motor disturbance, indifference, problems with ADL’s.

Question 17

What are the manifestations of Severe AD?

Answer 17

Severe mental impairment, minimal voluntary movement, no self care (grooming, bathing, feeding), incontinence (bowel, urine), rigid and flexor posturing

Question 18

What is paraphasia?

Answer 18

Using words in the wrong context. The speaker might use “foot” instead of “shoe”. It is an inabiity to communicate. Paraphasia is a common manifestation during moderate alzhemiers disease.

Question 19

How is Alzheimers disease diagnosed?

Answer 19

There is no definitive test for AD. To properly diagnose the clinical presentation are used in conjunction with excluding other possible causes. Patients will likely undergo many test to rule out other diseases: EEG (electroencphalogram), CT Scan, MRI, and labs would all be completed.

Question 20

What is the treatment for AD?

Answer 20

There is no cure. Patients will undergo symptomatic management and behavioral and environmental manipulations. Pharmacological treaments.

Question 21

What are several drugs which AD patients will take? What do they do?

Answer 21

Memantine: Glutamate Receptor Blockers - Glutmate is a stiumlatory neurotransmitter which increases neural function, which if accumulated, it become toxic. This seems to stem the progression of the disease. Aricept: ACh-esterase Inhibitors - These drugs inhibit an enzyme that breaks up ACh. Acetocholin is greatly diminished in those suffering from AD, the idea is to prevent this decrease. Risperidone: Low Dose antipsychotics Effexor: Anti-depressants Other drugs to control symptoms.

Question 22

What is Multiple Sclerosis?

Answer 22

MS is an autoimmunity disease which targets the myelin in the Central Nervous System.

Question 23

Who does MS typically affect, and what is the usual onset?

Answer 23

MS usually affects women at a two times higher rate than men. Onset is usually between 20-40 years, affecting caucaisen individuals. The disease is more prominent in colder regions.

Question 24

What is the etiology of MS?

Answer 24

The actual etiology of MS is unclear. However, it is a complex trait with familial tendency. If you have a 1st degree relative with MS there is a 15 times greater chance of getting the disease. MS is also thought to have a viral trigger (EBV - Epstein-Barr Virus).

Question 25

What are the most commonly targeted structures in multiple sclerosis?

Answer 25

1. Optic Nerve. 2. Periventricular region (peri=around). 3. Cerebellum. 4. Spinal Column. 5. Brain Stem.

Question 26

Describe the pathophysiology of MS?

Answer 26

Demyelination in the brain and spinal cord from immun and inflammatory damage (plaques) cause coordination problems for the patient. The oligodendrocytes die and become necrotic. Lymphocytes and Macrophages infiltrate in plaques, affecting morot and sensory neurons.

Question 27

What is found with-in the sclerotic plaques which form in MS?

Answer 27

Lymphocytes and Macrophage infiltration.

Question 28

What are the manifestations of MS? Why does the location and extent of the manifestations vary?

Answer 28

There is a lot of varietion in the manifestations because MS can affect many different regions of the brain and spinal column. MS typically exhibits waves of exacerbation and remission, sometimes lasting 3-4 years. As it progresses the manifestations get worse. Patients present with visual impairment, paresthesias and fatigue. Decreased muscle strength, and bladder/bowel dysfunction are common. Sufferers also experience gait and coordination problems.

Question 29

How is MS treated?

Answer 29

There is no cure for multiple sclerosis. Steroids for acute relapse which helps to suppress the autoimmune response. Methotrexate with concurent folic acid. Methotrexate inhibits the job of folic acid, preventing cell division and DNA synthesis. Interferon is given for those with persistent relapses. Symptomatic treatment is also given.

Question 30

How is MS diagnosed?

Answer 30

Multiple Sclerosis is diagnosed through several tests: MRI will be conducted, looking at the brain for sclerotic plaques/patches in the brain. Secondly, collection of CSF looking for elevated levels of Immunoglobulins (IgG) antibodies.

Question 31

What do elevated protein levels tell us, when found in the CSF?

Answer 31

Elevated levels tell us there is a problem with: 1. Autoimmune problem in the CNS. 2. Inflammation in the CNS. 3. Blood brain barrier is compromised.

Question 32

What is Amyotrophic Lateral Sclerosis (ALS)?

Answer 32

Amyotrophic Lateral Sclerosis (ALS) is the most common motor neuron problem. It is a progressive, degernative, disorder. ALS is typically seen in middle aged men (x2 more likely), resulting in death within 2 - 5 years. Death is typically the result of repiratory failure due to motor neuron problems. Amyotrophic Lateral Sclerosis IS NOT caused by an autoimmunity.

Question 33

There are two forms of ALS. What are they? What is the incidence rate of each form? What genes are implicated in ALS?

Answer 33

Sporatic and Familial. Sporatic accounts for 90-95% of all cases. Familial accounts for 5-10% of all cases. The most common gene implicated in both sporatic and familial forms of ALS is the gene SOD1: Superoxide Dismutase Gene. SOD1 is involved with the breaking up of free radicals within the body. Without this gene free radicals proliferate and cause cellular damage in the neurons.

Question 34

What is the pathophysiology of Amyotrophic Lateral Sclerosis?

Answer 34

Amyotrophic Lateral Sclerosis is a degenerative disease of motor neurons in the anterior horn cells in the spinal cord, motor nucleus in the brain stem, and the upper motor neurons in the cerebral cortex. The mechanism is unclear, but it is proposed that excessive free radical or accumulation of the neurotransmitter glutamate could be causing toxicity.

Question 35

What are the two purposed mechanisms of injury in Amyotrophic Lateral Sclerosis?

Answer 35

Free radicals due to abnormal Superoxide Dismutase Es. Accumulation of neurotransmitter Glutamate.

Question 36

What are the manifestations of Amyotrophic Lateral Sclerosis?

Answer 36

Generalized weakness, since the muscles are not being used, they atrophy, the muscles which do work spontaneously twitch. Inability to utilize muscle for swallowing resulting in dysphasia (trouble swallowing), and dysarthria (trouble with speech because tounge is a muscle). ALS is also accompanied with problems of aspiration due to acute imflammation and damage of the respiratory tract. Sensory function and Cognition is not affected.

Question 37

How is Amyotrophic Lateral Sclerosis diagnosed?

Answer 37

ALS is diagnosed through the clinical manifestation of the diesase. The patient will also undergo electromyography which stimulated the nerves. The muscle response to the stimulation is measured.

Question 38

What is the treatment for Amyotrophic Lateral Sclerosis?

Answer 38

There is no cure for ALS. Treatment is largely supportive/comfort. Riluzole is a common drug (neuroprotective drug), it is suspected to work through glutamate toxicity and modulation, but the success rate is poor, only extending life a more more months.