Diseases Flashcards
Seizure Definition
Transient neurologic event of sudden or excessive cortical electrical discharges.
Manifested by disturbance of skeletal muscle function, sensation, autonomic visceral function, behavior or consciousness.
What is a seizure disorder refers to recurrent seizures?
Epilepsy
Seizure causes: _____ injury, ______, metabolic/nutritional disorders, ________ (no know cause)
cerebral
lesions
idiopathic
Pathogenesis of Seizure. Due to alteration in _______ ______ that makes certain neurons abnormally _________ and _________ to changes in their environment (epileptogenic focus)
membrane potential
hyperactive
hypersensitive
Treatment for seizures:
Anticonvulsants: can target Na+ and Ca2+ channels or enhance GABA
What is dementia?
Syndrome associated with many pathologies; characterized by progressive deterioration and continuing decline of memory and other cognitive changes.
Age-related dementia: _______ condition resulting in progressive cognitive decline
Name Types: _______ and ________
irreversible
Alzheimer
Vascular
Dementia - Alzheimer Disease.
Where are degeneration of neurons?
temporal and frontal lobes
brain atrophy
amyloid plaques
neurofibrillary tangles
What are pathologic features of Alzheimer Disease?
Proteinopathies - plaques and tangles
Periplaque inflammatory reaction and neurodegeneration: synapse loss, neuron loss gross cerebral
Neuritic plaque and neurofibrilary tangle.
Where are they located?
Neuritic plaque: forms in extracellular space
Neurofibrillary tangle: intracellular aggregates
What are some pathologic properties of Alzhemier?
Beta amyloid protein (AB peptide)
Neuritic plaque: dense central AB core with inflammatory cells and dystrophic neurities in periphery
What are Tau proteins?
What does an MRI imaging show?
Amyloid accumulation has what biomarker?
Neurodegeneration or neuronal injury has what biomarker?
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
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
Epislon 4 allele of APOE gene (cholesterol metabolism)
Cognitive
What is Vascular Cognitive Impairment?
Syndrome with evidence of ______ ________ or subclinical vascular ____ _______ and cognitive impairment affect at least one ______ domain.
Clinical stroke
Brain injury
cognitive
Vascular Cognitive Impairments:
_____ impairment due to ______ pathologies
cumulative _____ damage more so than large ______ _______
white matter
Cognitive
tissue damage
cerebral infarcts
What are the 4 pathogenesis of vascular cognitive impairment?
1) cerebral amyloid angiography
2) atherosclerosis
3) confluent white matter lesions; small white matter infarcts
4) hyaline substance in vessel wall
What is is the biggest cause of vascular cognitive impairment?
Endothelial dysfunction
What are clinical manifestations of vascular cognitive impairment?
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
What treatment is there for vascular cognitive impairment?
What can we do?
No FDA approved treatment for VCI or vascular dementia
Preventive measure = control risk factors
Delirium:
Hyperkinetic _________ state: autonomic nervous system over activity
_______ state of brain __________
Excited delirium syndrome:
Hypokinetic delirium:
confusional
acute; dysfunction
agitated delirium
hypokinetic confusional state
Delirium:
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
Dementia:
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
Parkinson Disease:
________ neurologic disorder associated with ________ impairment.
Degeneration of what type of neurons? Location?
Progressive; motor
Dopaminergic neurons in the substantia nigra (basal ganglia)
What are signs and symptoms of Parkinson Disease?
Difficulty initiating and controlling movements
Bradykinesia, tremors, rigidity, postural instability coupled with gradual symptom progression and sustained response to therapy
What are clinical Manifestations of Parkinson Disease?
Tremor Masklike face Arms flexed at elbows and wrists Stooped posture Rigidity Hips and knees slightly flexed Short shuffling steps
There are 4 types of Spinal cord and peripheral nerve disease
1) __________ ____________
2) ___________ lateral _________
3) Spinal ________ __________
4) Guillain - _______ Syndrome
1) Multiple Sclerosis
2) Amyotrophic Lateral Sclerosis
3) Cord Injury
4) Barre
Is there a cure for Parkinson Disease?
What drugs can be given?
What surgical intervention?
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
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?
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.
What type of cells are involved in MS?
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
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?
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
What is Amyotrophic Lateral Sclerosis: progressive disease affecting both the _______ and ______ motor neurons
Pathogenesis: ___________
Signs and symptoms of ALS?
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
ALS Pathogenesis:
1) Excitotoxicity caused by what substances? 2 Things
2) Oxidative Stress
3) __________ dysfunction
4) What type of inflammation?
1) Glutamate and calcium influx
3) Mitochondrial
4) Neuroinflammation
Whats are some clinical manifestations of ALS?
How do you diagnose this disease?
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
What happens to the spinal cord in spinal cord injury?
Mechanisms of injury include?
Secondary injury may result from _________, ________, ________ and inflammation.
Cord can be compressed, transected or contused (bruised)
Hyperflexion, Hyperextension, compression
Hemorrhage, swelling, ischemia
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?
immediately; below
Muscles are flaccid; autonomic reflexes
End of spinal shock and reflexes return and flaccidity replaced by spasticity
What type of injury occurs to cause neurogenic shock?
What part of the ANS is affected?
Symptoms of neurogenic shock include?
cervical or upper thoracic injury
SNS; loss of brainstem and higher center control
hypotension, bradycardia, circulatory collapse
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?
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
What is the pathophysiology of autonomic dysreflexia?
Brain detects hypertension by what receptors? Which then leads to what?
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!
Treatment for autonomic dysreflexia include:?
Appropriate stabilization of ________ ______
Neurogenic shock: Intensive care unit to maintain _______ and ______ __________
Spinal vertebrae
May be accomplished surgically with internal fixation or with external fixation and bracing
oxygenation and blood pressure
Guillain Barre Syndrome:
Neuropathic condition characterized by _______ ______ and diminished or absent _________ reflexes.
Inflammatory demyelinating disease of the ________ nervous system or an _________ disorder.
AIDP?
progressive weakness; mytotatic
peripheral; Lower motor neuron (LMN)
Acute inflammatory demyelinating polynueropathy
Guillian Barre Syndrome
What are signs and symptoms? Is the weakness ascending or descending? How does this differ from MS?
Diagnosis?
Treatment?
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