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Diseases and manifestations of the brain.

Susan Ward

on 29 July 2014

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Transcript of Patho-neurology

Lou Gehrigs Disease=
degenerative, progressive, fatal
muscles weakness and wasting
motor neurons atrophy
corticalspinal tract of spinal cord sclerosis
Most common motor neuron disesase
5000 new cases annually
90-95% occur randomly, others with some familial component
Occurs mostly in 4th -6th decade
Death occurs in 2-5 years d.t. respiratory failure
Men more often then women until after menopause- then equalizes
issues with swallowing, communication and airway protection, respirations
the body attempts to reinnervate and assist the affected muscles but when more than half are affected- re-innervation fails and weakness causes major decompensation.!
progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord
Neurological Pathophysiology
Lou Gerhigs Disease
Trauma, Bleeds
CVA (stroke)
Dementia and Alzheimers
Parkinsons disease
Multiple Sclerosis
Pre quiz
A /myo /trophic Lateral Sclerosis
NO muscle nutrition along the side degeneration and scarring
The injury may be visible- but the impact is huge!
Neurologic Injury
LOC- level of consciousness (LOC)
Pattern of breathing- what part of brain?
Size and reactivity of pupils..what cranial nerves
Eye position and reflex response....what cranial nerves
Skeletal muscle motor response....
Symptoms vary based on:
Coup- Countrecoup
Type of injury-
Focal or diffuse in pattern
Stephen Hawkings
Highly functioning ALS patient
Born in 1942
Suspcious weakness in 1953
Diagnosed in his early 20's
1974 unable to feed himself
1985 tracheostomy for resp failure
2011 Still contributing to scientific evolution- utilizes a speech synthesizer to convert his eye movements to words/sentences/ papers/ books
Spinal Cord Injury
Cognitive Disorders
Dementia- progressive
Delirium- acute
Parkinsons Disease
Motor system disorder
Multiple Sclerosis
demyelination of the axon sheath
What famous scientist has a neurodegenerative disease?
What famous actor has a disease of the neurotransmitter producing cells?
Neuroplasticity refers to:
a. the hardness of the brain after injury
b. the pliability of the brain after injury
c. the ability of the brain to regenerate neuronal connections
d. the ability of scientists to perform plastic surgery to aid brain healing
T or F
Parkinsons disease and multiple sclerosis are both problems that arise in the brain.

**before we start with the pathophysiology, lets review the anatomy of the skull
Alzheimers Disease
irreversible cognitive dysfunction
1. Development of amyloid plaques
(cc) image by nuonsolarteam on Flickr
2. Development of neurofibrillary tangles
fragments of a protein called beta-amyloid peptide mixed with a collection of additional proteins
abnormal collections of a protein called tau
proteins tangle and clump preventing neuronal commmunications
Gradual degeneration leads to
behavior and personality changes,
Decline in cognitive abilities such as
decision-making and
language skills, and
problems recognizing family and friends
Further decline leads to:
Severe loss of mental function with worsening breakdown of the connections between certain neurons in the brain causing neuronal death
90% occurs after age 65 ~Late Onset Alzheimers
For Early onset Alzheimers: genetic predisposition by a mutation in one of three different inherited genes
Treatment may slow but does not prevent deterioration- usually started in the early or mid stages of the disease.These drugs help individuals carry out the activities of daily living by maintaining thinking, memory, or speaking skills. They can also help with some of the behavioral and personality changes associated with AD.
medications-tacrine- Cognex, donepizil- Aricept, revastigmine-Excelon, galantamine- Razadyne,
3. Neuronal degeneration
Dr. Alois Alzheimer. In 1906, Dr. Alzheimer noticed changes in the brain tissue of a woman who had died of an unusual mental illness. Her symptoms included memory loss, language problems, and unpredictable behavior.
Abrupt vs. gradual onset
Typcially a phsyiologic cause
Typically reversible
1. Acute onset of mental status changes or a fluctuating course- more at daytime or when dark
2. Inattention

3. Disorganized thinking
4. Altered level of consciousness
= Delirium
THINK Mnemonic to rule out physiologic causes

T Toxic Situations
CHF, shock, dehydration
Deliriogenic meds (Tight Titration)
New organ failure, e.g, liver, kidney
H Hypoxemia; also, consider giving Haloperidol or other antipsychotics?
I Infection/sepsis (nosocomial), Immobilization
N Nonpharmacological interventions
Hearing aids, glasses, reorient, sleep protocols, music, noise control, ambulation
K K+ or Electrolyte problems
Parkinsons Disease
A motor System Disorder
Reduction in the brain cells that produce a chemical called dopamine that controls muscle movement
Genetic component for transmission
Usually occurs after age 50
The four primary symptoms of PD are
tremor, or trembling in hands, arms, legs, jaw, and face;
rigidity, or stiffness of the limbs and trunk;
bradykinesia, or slowness of movement; and
postural instability, or impaired balance and coordination.
Other symptoms may include depression and other emotional changes; difficulty in swallowing, chewing, and speaking; urinary problems or constipation; skin problems; and sleep disruptions. 
No blood tests for diagnosis
No cure
Nerve cells can use levodopa to make dopamine and replenish the brain's dwindling supply when it is accompanied by  carbidopa that allows conversion to of levopdopa to dopamine IN the brain.
Other drugs: bromocriptine, pergolide, pramipexole, and ropinirole, mimic the role of dopamine in the brain, causing the neurons to react as they would to dopamine. 
An antiviral drug, amantadine, also appears to reduce symptoms
Surgical Intervention:
A therapy called deep brain stimulation (DBS) has now been approved by the U.S. Food and Drug Administration.
In DBS, electrodes are implanted into the brain and connected to a small electrical device called a pulse generator that can be externally programmed.
In a pallidotomy, the surgeon destroys a tiny part of the globus pallidus by creating a scar. This reduces the brain activity in that area, which may help relieve movement symptoms such as tremor and stiffness (rigidity).
The surgeon inserts a hollow probe through a small hole drilled in the skull to the target location. An extremely cold substance, liquid nitrogen, is circulated inside the probe. The cold probe destroys the targeted brain tissue.
There the 10 -20000 neurons cells that release this essential neurotransmitter (dopamine) that helps to control movement and coordination.
symptoms dont occur until after 80% of the dopamine producing cells have died
Multiple Sclerosis
Bleeds result from
a direct injury
result of longstanding effects of hypertension
an anuerysm or artery rupturing
Subdural between skull and dura- usually arterial
Epidural between the dura and the pia mater- usually venous
Subarachnoid and intracerebral- between the pia mater and the brain. can be venous or arterial
Two Types:
Ischemic 80% of strokes
clot formation in a vessel- treatment with TPA in less than 3 hours can improve recovery
emboli from another part of the body
narrowing of vessel over time from atherosclerosis
Hemorrhagic 20% of strokes with 50% mortality, high morbidity
Usually in people over 50- but not limited to age range
more in men than women
higher in african americans and hispanic people
greater in people with a family history
ONE of the TOP FIVE causes of death in the elderly
most frequent cause of disability in the elderly
Headache- "most severe", acute
Decreased LOC- from confused, delirious, lethargic, obtunded stuperous, coma
GCS: eye opening, verbal response, motor response
Brainstem functions: cranial nerve assessments
The cranium is a closed vault comprised of 3 major components:
Brain tissue
Cerebrospinal fluid
Any increase of one component will decrease the others- so a bleed....... will compress the brain and cause trauma and inflammation- further compromising blood flow
Location in Cerebral blood flow determines symptom

Anterior cerebral : control logical thought, personality, and voluntary movement, especially of the legs. Results in opposite leg weakness. aka contralateral weakness or paralysis

Middle Cerebral – Causes contralateral motor/sensory loss, frontal lobe and the lateral surface of the temporal and parietal lobes, including the primary motor and sensory areas of the face, throat, hand and arm, and in the dominant hemisphere, the areas for speech.
Left vs. right sided injury
Expect contralateral hemiparesis or paralysis
Left hemisphere
Language-Aphasia (inability to communicate) – expressive, receptive-grammar/vocabulary, literal
Logic-numerical computation (exact calculation, numerical comparison, estimation)
left hemisphere only: direct fact retrieval[
difficulty learning new information
Right hemisphere
Spatial, perceptual abilities
Judgment-Loss of judgment with no awareness,numerical computation (approximate calculation, numerical comparison, estimation)[
Memory-Short term memory loss
Mood-Indifference, impulsive
Languageintonation/accentuation, prosody, pragmatic, contextual
Blood pressure control
Cholesterol control
Platelet inhibitors like ASA, plavix, aggrenox, warfarin, dibigatran- Pradaxa a direct thrombin inhibitor
Spinal Cord Injury
infection in the subarachnoid space of the meninges
usually bacterial, recovery depends on prompt treatment
symptoms: stiff neck, fever, headache, cerebral dysfuntion- agnosia, amnesia, aphasia, and apraxia.
Seizures are episodes of disturbed brain activity that cause changes in attention or behavior. Seizures cause a disruption of the communication system in the brain which occurs with a sudden, abnormal electrical activity in the brain
Seizures may or may not have convulsive or tonic or clonic movements.
Seizures can have many causes, including medicines, high fevers, head injuries and certain diseases.
Aura- some sense of something changing- feeling of de ja vu, emotional response, change in hearing or vision
Followed by tonic- pulling in of movements to body with loss of consciousness
clonic- muscles contracting and relaxing, eyes will roll back
Many examples on you tube if you are curious
Partial or complete transection of the spinal cord
Stinger or bruise or tissue death related to cord ischemia, inflammation, or transection.
NEVER move a fall victim without stabilizing the neck.
Lossess are related to the level of injury on the spine
Injury can result from:
traumatic blow to back or spine
fractures of vertebra
herniation of discs between vertebra
Various levels of recovery with possible continued problems with
loss of voluntary control,
flaccid paralysis,
loss of muscle tone,
muscle atrophy,
decreased or absent reflexes.
People who survive a spinal cord injury will very likely have medical complications such as
chronic pain
bladder and bowel dysfunction,
an increased susceptibility to respiratory and heart problems.
Susan E. Ward RN, MSN, CCRN, CSC

Pre- Quiz
Tip of the iceberg
Assessment of brain injury
inflammation of the brain that leads to cerebral swelling (remember -closed vault of skull does not respond well to changes of the 3 main components)
nonspecific cause
usually viral- affects the very young and old
difficult to treat unless herpes- then acyclovir
Brain Abcess- bacterial, necrotizing infections usually resulting form other sources- like teeth, sinuses, ears, or penetrating wounds.
Dementia and Delirium
Cognitive Neurological Disorders

Onset between ages-20-40, progression varies
Male/female 1:2
May result from previous viral insult to the nervous system, possibly to someone genetically predisposed
Genetic predisposition by a pattern of antigens (histocompatobility complex antigen HLA), DR5, DQ6- created in reponse to some pathogen trigger the attack on the myelin sheath of the axon- causing temporary or permanent damage.
People vary in their responses- some have remissions and others have slow progression with no remission
Michael J. Fox was diagnosed with young-onset Parkinson’s disease in 1991. Upon disclosing his condition in 1998. Only 30 years old.
A neurological exam may show reduced nerve function in one area of the body, or spread over many parts of the body. This may include:
•Abnormal nerve reflexes
•Decreased ability to move a part of the body
•Decreased or abnormal sensation
•Other loss of nervous system functions
An eye examination may show:
Abnormal pupil responses
Changes in the visual fields or eye movements
Decreased visual acuity
Problems with the inside parts of the eye
Rapid eye movements triggered when the eye moves
Tests to diagnose multiple sclerosis include:
Lumbar puncture (spinal tap) for cerebrospinal fluid tests, including CSF oligoclonal banding
MRI scan of the brain and MRI scan of the spine are important to help diagnose and follow MS
Nerve function study (evoked potential test)
The process of reorganization is dynamic and dependent on the nature of injury, substrates (what caused the insult) involved and the duration since the initial insult.
The brain has the ability to reorganize (i.e., neuroplasticity) after disease or injury and this phenomenon can be facilitated through activity-dependent processes including environmental enrichment, forced-use, complex skills training, and exercise.
Most of our understanding of this activity-dependent plasticity is derived from studies of brain injury related to stroke and spinal cord injury.
Please read: The Brain that Changes Itself by Norman Doidge
The brain compensates for damage in effect by reorganizing and forming new connections between intact neurons. In order to reconnect, the neurons need to be stimulated through activity.

Stimulating experience's can actually change both the brain's physical structure (anatomy) and functional organization (physiology).
The brain's ability to reorganize itself by forming new neural connections throughout life (aka learning, making connections between concepts).
The brain's capacity for recovery from injury is far greater than previously thought.
Anterior brain
Posterior brain
Middle Cerebral – Middle of brain, most fatal
Anterior cerebral – Feeds frontal lobes
Posterior Cerebral – occipital and temporal
Basilar Artery–feeds cerebellum and brain stem
Vertebral Artery
Location in Cerebral blood flow determines symptom

Posterior Cerebral – occipital and temporal
Loss of vision and memory
supply the temporal and occipital lobes of both hemispheres
thalamic syndrome, contralateral hemplegia, hemianopsia ,color blindness, failure to see to-and-fro movements, verbal dyslexia, and hallucinations. The most common finding is occipital lobe infarction leading to an opposite visual field defect.

Basilar/vertebral –feeds cerebellum and brain stem
Impaired gait, speech, swallowing, vision
Assessment: CAM-ICU
May be used in other setting than ICU
Evaluate for features of delirium- mental status change with inattention + diorganized thinking or altered LOC/RASS
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