Neurological Disorders

Question 1

Dementia

Answer 1

Not a specific disease – group of
symptoms caused by brain
damage
- Progressive
 Loss of cognitive functioning
(thinking, remembering, reasoning)
 Interferes with daily activities
 Occurs in older people
 Not a normal aging process

Question 2

Dementia Mechanisms

Answer 2

◦Neuron degeneration
◦Atherosclerosis/vascular
◦Brain tissue compression
◦Brain trauma
◦Genetic predisposition

Question 3

Alzheimer
Disease

Answer 3

 Progressive neurological
disorder effecting memory,
thinking skills, the inability to
carry out simplest tasks
 MOST COMMON type of DEMENTIA
 Late onset (95%)
 Sporadic
 Alteration in apolipoprotein E
(chromosome 19)
 Early-onset familial (5%)
 Chromosome 21

Question 4

Alzheimer
Disease Pathophysiology

Answer 4

 Extracellular deposition of β-amyloid
 Senile (neuritic) plaques
 Intracellular accumulation of tau protein
 Neurofibrillary tangles

Question 5

Alzheimer
Disease Signs and symptoms

Answer 5

◦ Extend over 10 to 20 years
◦ Behavioral changes
◦ Irritability, hostility, mood
swings
◦ Forgetfulness ->
progressive memory loss
◦ Lack of concentration
◦ Impaired learning or use of
language
◦ Poor judgment

Question 6

Alzheimer’s
Disease Diagnostics

Answer 6

No definite clinical diagnostic tests
 Confirm – postmortem brain biopsy
 Exclusion of other disorders
 Careful medical and psychological
history
 Treatment
 No specific treatment
 Anticholinesterase drugs
* Temporary improvement
 Team approach needed to support
patients and caregivers
 Occupational, speech, physical,
psychologists

Question 7

Parkinson’s Disease

Answer 7

 Progressive, neurodegenerative disease of the melanin-containing dopaminergic neurons in
the substantia nigra, pars compacta
 Severe degeneration of the basal ganglia

Question 8

Parkinson’s Types

Answer 8

Types
 Primary – (Idiopathic) - majority
 Secondary
 Environmental
(pesticides/herbicides)
 Anti-psychotic medications
 Pseudo-Parkinsonism -
Muhammad Ali

Question 9

Parkinson’s
Disease Treatments

Answer 9

• Dopamine agonist
• Levodopa
• MAO-B inhibitor
• Anticholinergic drugs
• Amantadine
• Team approach
• Speech, physical, exercise
  therapy
• Psychiatric comorbidities
• Dietary

Question 10

Huntington’s Disease

Answer 10

 Progressively debilitating
neurodegenerative inherited
disease
 Autosomal dominant disorder
 Chromosome 4 (4p16.3)
 Huntingtin (HTT) gene
 Signaling, transporting, anti-
apoptosis, DNA repair
 Does not usually manifest until
older ~ 40s
 Progressive atrophy of brain

Question 11

Huntington’s Pathophysiology

Answer 11

Pathophysiology
- Hyperkinetic disorder
- Involves basal ganglia (nuclei) and frontal cortex
- Depletion of gamma-aminobutyric acid (GABA) in the
basal nuclei (caudate nucleus & putamen)
- Levels of acetylcholine in brain appear to be reduced

Question 12

Huntington’s Disease Signs and symptoms

Answer 12

◦ Mood swings, personality
changes
◦ Restlessness, choreiform
(purposeless) movements in
arms and face

Question 13

Huntington’s Diagnostics

Answer 13

DNA analysis

Question 14

Huntington’s Treatment

Answer 14

◦ Currently no specific
treatment
◦ Symptomatic therapy only

Question 15

Huntington’s Mortality

Answer 15

◦ Infection
◦ Heart disease
◦ Suicide

Question 16

Amyotrophic Lateral Sclerosis
(ALS)

Answer 16

Also referred to as Lou Gehrig’s disease
 Rapidly progressive & fatal
neurodegenerative disease of the upper
and lower motor neurons
 Pathophysiology: not clear
 SOD1 gene mutation
 Damage to glutamate uptake channels in
astrocytes
 Military veteran – Gulf War
 Risk - repeated trauma (athletes)
 No indication of inflammation around the
nerves
 Cognition and sensory: UNIMPAIRED

Question 17

Amyotrophic Lateral Sclerosis [2]

Answer 17

 Loss of upper motor neurons
 Spastic paralysis & hyperreflexia
 Damage to lower motor neurons
 Flaccid paralysis and hyporeflexia
 Progressive muscle weakness
 Loss of fine motor coordination
 Stumbling and falls are common.
 Death due to respiratory failure

Question 18

Vascular
Disorders

Answer 18

 Ischemia - interference with
blood supply
- Damage and manifestations
depend on cerebral artery
involved
- Global or local
 Hemorrhage
- Increased intracranial
pressure (ICP) will cause
local ischemia and
generalized symptoms.
 Vascular malformations
- Arteriovenous malformation

Question 19

Cerebrovascular
Disorders

Answer 19

 Transient ischemic attacks
(TIAs)
 Cerebral vascular accident
(CVA [stroke])
+ Thrombotic stroke
- Arterial occlusions caused by
thrombi
+ Embolic stroke
- Fragments of a thrombus that
broken free from a thrombus
 Hemorrhagic stroke

Question 20

Transient
Ischemic
Attacks
(TIAs)

Answer 20

 Transient episode of neurologic dysfunction caused by focal brain, spinal cord, or retinal ischemia without acute infarction
 Neurological dysfunction lasting <1
hour from ischemic event
 Reversible
 Causes
- Atherosclerosis – most common
- Partial occlusion of an artery - small embolus
- Vascular spasm
- Arteritis
- Mass lesions

Question 21

Transient
Ischemic
Attacks S&S

Answer 21

Signs and symptoms
 Difficult to diagnose after the
attack
 Directly related to location of
ischemia
 Intermittent short episodes of
impaired function
* e.g., muscle weakness in
arm or leg
 Visual disturbances
 Numbness and paresthesia
 Transient aphasia or confusion
may develop.

Question 22

Transient
Ischemic
Attacks Diagnosis & Management

Answer 22

Diagnosis
- Important to rule out other
causes
- MRI (preferred), CT scan
- Carotid doppler
- Angiography
 Management
- Risk stratification
- Initiation of stroke prevention
therapy

Question 23

Cerebrovascular
Accidents (CVAs)

Answer 23

 A CVA (stroke) is an infarction of brain tissue that results from lack of blood.
 Ischemic - Occlusion of a
cerebral blood vessel
 Hemorrhagic - Rupture of
cerebral vessel
 5 minutes of ischemia 
irreversible neuronal damage

Question 24

Cerebrovascular
Accidents Facts

Answer 24

 Leading cause of disability
 Fifth leading cause of death in US
 Common denominator - associated with hypoperfusion
 African-American higher risk

Question 25

Cerebrovascular Disorders Hemorrhagic Stroke

Answer 25

 Third most common cause  Hypertension is primary cause  Bleeding  compressed brain tissue  ischemia, edema, increased ICP and necrosis  Types - Intracerebral hemorrhage - Subarachnoid hemorrhage

Question 26

Cerebrovascular Disorders S&S

Answer 26

 Depend - Location of obstruction - Size of artery involved  Contralateral motor & sensory deficit  Initially flaccid paralysis then spastic paralysis develops weeks later  NIH Stroke Scale (NIHSS) - To assist with rapid diagnosis - Predict size and severity - Predict short- and long-term outcomes

Question 27

Stroke S&S

Answer 27

Abrupt onset of hemiparesis, hemisensory deficits ◦ Monocular or binocular visual loss ◦ Visual field deficits ◦ Diplopia ◦ Nystagmus ◦ Dysarthria ◦ Facial droop ◦ Ataxia ◦ Vertigo (rarely in isolation) ◦ Aphasia ◦ Headaches ◦ Sudden decrease in level of consciousness

Question 28

Stroke Treatment

Answer 28

 Focused on restoration of perfusion, counteracting ischemia, and prevent necrosis  Fibrinolytic (tPA) – 4.5 hours or less  Antiplatelet agents (aspirin)  Mechanical thrombectomy  Blood pressure control  Glucocorticoids  Supportive treatment  Occupational, physical, speech, rehabilitation begins immediately.  Treat underlying problem to prevent recurrences.

Question 29

Stroke Prevention

Answer 29

 Cardiovascular disease: platelet antiaggregant  aspirin  Hyperlipidemia - statins (cholesterol lowering drugs)  Healthier diet  Control hypertension – antihypertensive  Control diabetes type II  STOP smoking  Exercise to decrease weight  Lifestyle modifications/interventions

Question 30

Infection and Inflammation of the CNS

Answer 30

 Meningitis - Bacterial - Viral (aseptic) - Fungal

Question 31

Meningitis Pathophysiology

Answer 31

◦Routes of Inoculation ◦ Hematogenous ◦ Direct contiguous spread ◦Organisms ◦ Neisseria meningiditis ◦ Streptococcus pneumonia

Question 32

Meningitis Treatment

Answer 32

- Rapid diagnosis and treatment essential to prevent morbidity and mortality ◦ Aggressive antimicrobial therapy ◦ Glucocorticoids ◦ Reduction of cerebral inflammation and edema ◦ Vaccines are available

Question 33

Multiple Sclerosis

Answer 33

◦ Autoimmune ◦ Progressive, inflammatory, demyelinating disease of the central nervous system ◦ 1 : 100,000 in US and Europe ◦ Women : men – 2 : 1 ◦ Age: 20-40 years ◦ Relapses/remissions ◦ In time, neural degeneration becomes irreversible. ◦ Function is lost permanently

Question 34

Multiple Sclerosis Pathophysiology

Answer 34

◦ Inflammation: demyelination: axonal degeneration ◦ Target oligodendrocytes

Question 35

Multiple Sclerosis: S&S

Answer 35

◦ Manifestations determined by areas of demyelination ◦ Paresthesia, areas of numbness, burning, tingling ◦ Weakness ◦ Impaired gait ◦ Visual disturbances ◦ Blurred vision ◦ Diplopia (double vision), scotoma (spot in visual field) ◦ Urinary incontinence ◦ Loss of coordination, bladder, bowel and sexual dysfunction ◦ Dysarthria - weakness in the muscles used for speech

Question 36

Multiple Sclerosis Diagnostics

Answer 36

 Complete neurological examination  No specific marker  MRI for diagnosis and monitoring – test of choice  Cerebrospinal fluid analyses  Evoked potential tests

Question 37

Multiple Sclerosis Treatment

Answer 37

 No definitive treatment approved at this time  Several research trials in progress  Therapy includes physical therapy, occupational therapy  Manifestations require individual attention.