Diseases

Question 1

Seizure Definition

Answer 1

Transient neurologic event of sudden or excessive cortical electrical discharges.

Manifested by disturbance of skeletal muscle function, sensation, autonomic visceral function, behavior or consciousness.

Question 2

What is a seizure disorder refers to recurrent seizures?

Answer 2

Epilepsy

Question 3

Seizure causes: _____ injury, ______, metabolic/nutritional disorders, ________ (no know cause)

Answer 3

cerebral
lesions
idiopathic

Question 4

Pathogenesis of Seizure. Due to alteration in _______ ______ that makes certain neurons abnormally _________ and _________ to changes in their environment (epileptogenic focus)

Answer 4

membrane potential
hyperactive
hypersensitive

Question 5

Treatment for seizures:

Answer 5

Anticonvulsants: can target Na+ and Ca2+ channels or enhance GABA

Question 6

What is dementia?

Answer 6

Syndrome associated with many pathologies; characterized by progressive deterioration and continuing decline of memory and other cognitive changes.

Question 7

Age-related dementia: _______ condition resulting in progressive cognitive decline
Name Types: _______ and ________

Answer 7

irreversible
Alzheimer
Vascular

Question 8

Dementia - Alzheimer Disease.

Where are degeneration of neurons?

Answer 8

temporal and frontal lobes
brain atrophy
amyloid plaques
neurofibrillary tangles

Question 9

What are pathologic features of Alzheimer Disease?

Answer 9

Proteinopathies - plaques and tangles

Periplaque inflammatory reaction and neurodegeneration: synapse loss, neuron loss gross cerebral

Question 10

Neuritic plaque and neurofibrilary tangle.

Where are they located?

Answer 10

Neuritic plaque: forms in extracellular space

Neurofibrillary tangle: intracellular aggregates

Question 11

What are some pathologic properties of Alzhemier?

Answer 11

Beta amyloid protein (AB peptide)

Neuritic plaque: dense central AB core with inflammatory cells and dystrophic neurities in periphery

Question 12

What are Tau proteins?

What does an MRI imaging show?

Amyloid accumulation has what biomarker?

Neurodegeneration or neuronal injury has what biomarker?

Answer 12

Dissociates, assumes paired helical filament configuration and forms insoluble tangles

FDG PET –> glucose metabolism (synaptic activity); cerebral atrophy (hippocampus) which reflects microscopic neurodegeneration (loss of synpases)

Amyloid: Decrease CSF AB 42

Neurodegeneration: Increase CSF tau and decrease FDG PET

Question 13

What is a major risk factor for Alzheimer?

_______ reserve: different mechanisms including numbers of neurons and synapses, sensitivity of neurons and glia to pathologic process

Answer 13

Epislon 4 allele of APOE gene (cholesterol metabolism)

Cognitive

Question 14

What is Vascular Cognitive Impairment?

Syndrome with evidence of ______ ________ or subclinical vascular ____ _______ and cognitive impairment affect at least one ______ domain.

Answer 14

Clinical stroke
Brain injury
cognitive

Question 15

Vascular Cognitive Impairments:

_____ impairment due to ______ pathologies
cumulative _____ damage more so than large ______ _______
white matter

Answer 15

Cognitive
tissue damage
cerebral infarcts

Question 16

What are the 4 pathogenesis of vascular cognitive impairment?

Answer 16

1) cerebral amyloid angiography
2) atherosclerosis
3) confluent white matter lesions; small white matter infarcts
4) hyaline substance in vessel wall

Question 17

What is is the biggest cause of vascular cognitive impairment?

Answer 17

Endothelial dysfunction

Question 18

What are clinical manifestations of vascular cognitive impairment?

Answer 18

Early: memory loss - especially short term memory, long term memory may be perserved; thinking ability declines, decreasing ability to function at work and in social settings; anxiety, agitation

Question 19

What treatment is there for vascular cognitive impairment?

What can we do?

Answer 19

No FDA approved treatment for VCI or vascular dementia

Preventive measure = control risk factors

Question 20

Delirium:

Hyperkinetic _________ state: autonomic nervous system over activity

_______ state of brain __________

Excited delirium syndrome:

Hypokinetic delirium:

Answer 20

confusional

acute; dysfunction

agitated delirium

hypokinetic confusional state

Question 21

Delirium:

Answer 21

Is an acute state of brain dysfunction
onset is usually abrupt
ANS is over active
Common in critical care units, post surgically or during withdrawal from CNS depressants

Question 22

Dementia:

Answer 22

Is the progressive failure of many cerebral function
Onset is usually gradual
Progressive dementia produce nerve cell degeneration and brain atrophy
Age is the greatest risk factor

Question 23

Parkinson Disease:

________ neurologic disorder associated with ________ impairment.

Degeneration of what type of neurons? Location?

Answer 23

Progressive; motor

Dopaminergic neurons in the substantia nigra (basal ganglia)

Question 24

What are signs and symptoms of Parkinson Disease?

Answer 24

Difficulty initiating and controlling movements
Bradykinesia, tremors, rigidity, postural instability coupled with gradual symptom progression and sustained response to therapy

Question 25

What are clinical Manifestations of Parkinson Disease?

Answer 25

``` Tremor Masklike face Arms flexed at elbows and wrists Stooped posture Rigidity Hips and knees slightly flexed Short shuffling steps ```

Question 26

There are 4 types of Spinal cord and peripheral nerve disease 1) __________ ____________ 2) ___________ lateral _________ 3) Spinal ________ __________ 4) Guillain - _______ Syndrome

Answer 26

1) Multiple Sclerosis 2) Amyotrophic Lateral Sclerosis 3) Cord Injury 4) Barre

Question 27

Is there a cure for Parkinson Disease? What drugs can be given? What surgical intervention?

Answer 27

No cure Can give restoring dopamine levels or activity by administering dopamine precursors, dopamine agonists, monoamine oxidase inhibitors and anticholinergics, antidepressants therapy Ablative surgical procedures may be helpful for motor symptoms

Question 28

Multiple Sclerosis: Inflammatory _________ disease of the CNS that primarily affect what population? Why is it an autoimmune disease? What does it destroy? Where does demyelination occur? What causes this disease?

Answer 28

Demyelinating Young adults Autoimmune results in inflammation and scarring (sclerosis) of myelin sheath covering nerves CNS and often affects the optic and oculomotor nerves and spinal cord Unknown, but immunological abnormalities genetics and environmental factors suspected.

Question 29

What type of cells are involved in MS?

Answer 29

Demyelinate: dendritic cells, monocyte/macrophages Autoreactive T cells: helper T and cytotoxic T lymphocytes, Natural Killer cells ODC: oligodendrocyte and endothelial cells and pericyte and neurons

Question 30

MS is characterized by what? What are some trigger factors for these symptoms? What are some manifestations? Treatments? What kind of medications can be given and when is it given?

Answer 30

Exacerbation and remissions; exacerbated by heat, infection, trauma and stress Double/blurred vision, weakness, poor coordination, and sensory deficits; bowel and bladder control may be lost; memory impairment common No cure; short term steroid therapy may be helpful during acute exacerbations and immune-modifying drugs may slow progression of symptoms

Question 31

What is Amyotrophic Lateral Sclerosis: progressive disease affecting both the _______ and ______ motor neurons Pathogenesis: ___________ Signs and symptoms of ALS?

Answer 31

Upper and lower Unknown; genetic associations and CSF analysis Weakness and wasting of the upper extremities usually occur, followed by impaired speech, swallowing and breathing

Question 32

ALS Pathogenesis: 1) Excitotoxicity caused by what substances? 2 Things 2) Oxidative Stress 3) __________ dysfunction 4) What type of inflammation?

Answer 32

1) Glutamate and calcium influx 3) Mitochondrial 4) Neuroinflammation

Question 33

Whats are some clinical manifestations of ALS? How do you diagnose this disease?

Answer 33

Clinical: weakness, atrophy, cramps, stiffness, irregular twitching of muscles, hyper reflexia in weak, atrophied extremity (highly suggestive) Diagnosis: clinical signs and symptoms, EMG, nerve conduction studies (transcranial magnetic stimulation, UMN), MRI and serum laboratory testing

Question 34

What happens to the spinal cord in spinal cord injury? Mechanisms of injury include? Secondary injury may result from _________, ________, ________ and inflammation.

Answer 34

Cord can be compressed, transected or contused (bruised) Hyperflexion, Hyperextension, compression Hemorrhage, swelling, ischemia

Question 35

Spinal shock occurs __________ and characterized by temporary loss of reflexes ________ the level of injury. What do the muscles look like and what is lost? When do the reflexes return?

Answer 35

immediately; below Muscles are flaccid; autonomic reflexes End of spinal shock and reflexes return and flaccidity replaced by spasticity

Question 36

What type of injury occurs to cause neurogenic shock? What part of the ANS is affected? Symptoms of neurogenic shock include?

Answer 36

cervical or upper thoracic injury SNS; loss of brainstem and higher center control hypotension, bradycardia, circulatory collapse

Question 37

What is autonomic dysreflexia which occurs during spinal cord injury? Its usually what type of complication due to injury of __________? Injury occurs where? What can trigger this reflex or common stimulus? What are some signs and symptoms of this?

Answer 37

Acute (hyper) reflexive response to sympathetic activation below the level of injury. Usually a secondary complication due to injury of ANS. Injury at or above T6 Visceral stimulation (full bladder or bowel) and activation of pain receptors below the injury are common stimuli? Signs: hypertension, headache, bradycardia, flushing above the level of injury, and clammy skin below the skin of injury

Question 38

What is the pathophysiology of autonomic dysreflexia? Brain detects hypertension by what receptors? Which then leads to what?

Answer 38

Stimulus generates sympathetic response --> widespread vasoconstriction most significantly in sphanchic vasculature --> increase peripheral resistance --> shunt blood to general circulation --> increases BP Baroreceptors. It activates PSNS to lower BP --> PSNS over activity + Lack of sympathetic tone --> peripheral VASODILATION!

Question 39

Treatment for autonomic dysreflexia include:? Appropriate stabilization of ________ ______ Neurogenic shock: Intensive care unit to maintain _______ and ______ __________

Answer 39

Spinal vertebrae May be accomplished surgically with internal fixation or with external fixation and bracing oxygenation and blood pressure

Question 40

Guillain Barre Syndrome: Neuropathic condition characterized by _______ ______ and diminished or absent _________ reflexes. Inflammatory demyelinating disease of the ________ nervous system or an _________ disorder. AIDP?

Answer 40

progressive weakness; mytotatic peripheral; Lower motor neuron (LMN) Acute inflammatory demyelinating polynueropathy

Question 41

Guillian Barre Syndrome What are signs and symptoms? Is the weakness ascending or descending? How does this differ from MS? Diagnosis? Treatment?

Answer 41

Muscle weakness that begins in LOWER extremities and spreads to the proximal spinal neurons. Progressive ascending weakness or paralysis and affect respiratory muscles. MS is descending --> weakness starts from top to bottom Diagnosis: history, physical examination and nerve conduction studies and lumbar puncture Treatment: supportive, plasmapheresis, immunoglobulin