Exam 3 Pathophysiology: Neurology and Endocrine

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Examples of neurodegenerative diseases

• Alzheimer’s Disease

• Parkinson’s Disease

• Huntington’s Disease

• Amyotrophic Lateral Sclerosis (ALS)

Neural degeneration

True neuronal death

Neurodegeneration primarily affects the ____ matter.

gray

Shared histological features of neurodegeneration

• Neurofibrillary tangles

• Intracellular or intranuclear inclusions

• Abnormal protein aggregation

Dementia

Chronic, progressive failure of cerebral function

Cerebral functions affected by dementia include

• Orientation

• Memory

• Language

• Judgment

• Decision making

• Behavior

Dementia occurs in ____ people but is not a normal aging process

older

What best describes the onset of dementia

The onset of dementia is insidious, aka slow and gradual progression

Dr. K states the dementia is not a disease, rather a group of symptoms that are caused by an underlying disease. Is he factually correct?

Yes

Pathogenesis of dementia

• Neurodegeneration

• Brain trauma

• Compression of brain tissue

• Genetics

• Chronic inflammation

• Atherosclerosis

• CNS infections

Alzheimer’s Disease

Progressive neurological deterioration that affects memory, thinking, and ability to carry out simple tasks

Which disease is the most common cause of dementia in older people

Alzheimer’s Disease

Key risk factor for Alzheimer’s Disease

Increasing age

Two types of Alzheimer’s Disease

• Late-Onset

• Early-Onset

Late-Onset Alzheimer’s Disease makes up how much percent of Alzheimer’s cases?

90-95%

Late-Onset Alzheimer’s

• Sporadic

• Affects ApoE gene in chromosome 19

Early-Onset Alzheimer’s Disease makes up how much percent of Alzheimer’s cases?

5-10%

Types of Early-Onset Alzheimer’s Disease

• Familial

• Non-familial

Which inheritance pattern causes early-onset familial Alzheimer’s Disease

Autosomal dominant

This inherited condition can increase the risk of developing non-familial early onset Alzheimer’s disease

Down Syndrome

Pathophysiology and Alzheimer’s Disease

Deposition of amyloid plaques (primary) and accumulation of tau proteins

Symptoms of Alzheimer’s Disease

• Amnesia (memory loss)

• Agnesia (difficulty recognizing objects/people)

• Asphasia (speech/language difficulty)

• Anosognosia (loss of self-awareness/inability to recognize illness)

• Apraxia (loss of learned movements)

Symptoms of Alzheimer’s Disease: Amnesia

Memory loss

Symptoms of Alzheimer’s Disease: Agnesia

Difficulty to recognize familiar objects or people

Symptoms of Alzheimer’s Disease: Asphasia

Difficulty with speech and language

Symptoms of Alzheimer’s Disease: Anosognosia

Loss of self-awareness or inability to recognize own illness (physical or mental)

Symptoms of Alzheimer’s Disease: Apraxia

Loss of learned movements

Diagnosis of Alzheimer’s Disease

• Complete historical assessment, ruling out other disorders

• No single definitive test, only confirmatory after death

Parkinson’s Disease

Progressive neurodegenerative disease of the basal ganglia with loss of dopamine producing neurons

Pathogenesis of Parkinson’s disease

Defective alpha-synuclein proteins

Types of Parkinson’s Disease

• Primary

• Secondary

Types of Parkinson’s Disease: Primary

• Idiopathic (unknown origin)

• Increases with age (over 60 years)

• Associated with genetics

Types of Parkinson’s Disease: Secondary

• Environmental causes

• Antipsychotic medications

• Vascular

• Caused by repeated head trauma

• Parkinson-like disorders

Part of the brain affected by Parkinson’s Disease

Substantia nigra

Parkinson’s Terminology: Idiopathic Parkinson’s Disease

Current term for true neurodegenerative Parkinson’s marked by dopamine deficiency in the substantia nigra

Parkinson’s Terminology: Drug-Induced/Secondary Parkinsonism

Reversible type of Parkinson’s caused by drugs, usually antipsychotic drugs

Symptoms of Parkinson’s Disease

• Stone faced

• Resting tremor

• Parkinson’s shuffle

Treatment of Parkinson’s Disease

• Drugs

• Therapy (physical, speech, occupational)

• Monitor respiratory and urinary infections

Huntington’s Disease (chorea)

Progressive, debilitating disease caused by an autosomal dominant inheritance pattern

Which gene is affected by Huntington’s disease

HTT gene on chromosome 4

Huntingtin protein

Protein used for cell signaling and transport, anti-apoptosis, and DNA repair

Huntington’s Disease usually does not manifest until after 40 (True/False)

True

Pathophysiology of Huntington’s Disease

Loss of GABAergic neurons in the caudate and putamen

Huntington’s Disease is a hyper/hypo kinetic disorder

hyperkinetic

Symptoms of Huntington’s Disease

• Mood swings, personality changes

• Restlessness, choreiform movements

• Dementia

People who have this disease are more likely to commit suicide, or succumb to infections and heart disease

Huntington’s Disease

Inheritance pattern of Huntington’s Disease

Autosomal dominant

Amyotrophic Lateral Sclerosis (ALS) or Lou Gehrig’s Disease

Rapidly progressive and fatal neurodegenerative disease of the upper and lower motor neurons

Spastic paralysis

Damage to upper motor neurons

Flaccid paralysis

Damage to lower motor neurons

In this disease, cognition is not worsen to the degree seen in other neurodegenerative diseases, even though it is not universally spared

Amyotrophic lateral sclerosis (ALS)

Pathogenesis of ALS

A mutation of the SOD1 gene damages glutamate channels in astrocytes

• Mostly idiopathic

What type of neuron cells are primarily affected in ALS

Astrocytes

Risk factors for ALS

• Repeated head trauma

• Military veterans

What is the most common cause of death among ALS patients?

Respiratory failure

Symptoms of ALS

• Limb cramping/weakness

• Incoordination

• Slurred speech

• Dysphagia (difficulty swallowing foods)

Mean survival of ALS

3-5 years

Cerebrovascular Disease definition

Neurological disorders caused by pathologic processes in blood vessels

Causes of cerebrovascular diseases

• Ischemia

• Hemorrhagic

• Vascular malformation

• Increased intracranial pressure

Transient Ischemic Attack (TIA)

Transient focal neurologic deficit from brain, spinal cord, or retinal ischemia without infarction

Symptoms of transient ischemic attacks are usually

acute and resolves within 1-24 hours

Causes of transient ischemic attacks

• Atherosclerosis

• Vascular spasm

• Arteritis

• Mass lesions

Symptoms of transient ischemic attack

• Muscle weakness in arm/leg

• Vision disturbance

• Numbness

• Confusion

Cerebrovascular Accident (CVA) / Stroke

Infarction of brain tissue from hypoperfusion

What is the leading cause of disability?

Stroke

__% of strokes are recurrent

25%

Which racial group is at a higher risk for strokes

African Americans

Types of Strokes

• Ischemic

• Hemorrhagic

Types of Strokes: Ischemic Stroke

Blood vessel occlusion by a thrombus or an embolus (blockage)

Types of Ischemic Strokes

• Thrombolic: narrowing blood vessels

• Embolis: segment of thrombus that broke off

Risk factor for ischemic strokes

Atherosclerosis

Types of Strokes: Hemorrhagic Stroke

Rupture of a cerebral blood vessel

• Has high morbidity and mortality

Types of Hemorrhagic Strokes

• Intracerebral

• Subarachnoid

Risk factor for hemorrhagic strokes

Hypertension

Modfiable risk factors for stroke

• Hypertension

• Diabetes

• Smoking

• Sedentary lifestyle

Non-modifiable risk factors for stroke

• Age

• Sex

• Family history

• Ethnicity

Symptoms of stroke (BE FAST)

• Balance (loss of)

• Eyes (vision changes)

• Face drooping

• Arm weakness

• Speech difficulty

• Time (call 911 ASAP)

Initially, stroke starts off as _____ paralysis, but changes to _____ paralysis in later weeks

flaccid, spastic

Acute complications of stroke

• Increased ICP/cerebral edema, herniation

• Hemorrhagic transformation of ischemic stroke

• Seizures

• Aspiration pneumonia

Chronic complications of stroke

• Post stroke disability

• Asphasia, dysphagia

• Depression

• Vascular dementia

Meningitis

Inflmmation of the meninges, the membrane that surrounds the brain and spinal cord

Difference between viral and bacterial meningitis

• Viral meningitis is more common but less severe

• Bacteria meningitis is less common but more severe

Types of bacteria that cause bacterial meningitis in newborns

• Group B streptococci

• E. coli

• Listeria monocytogenes

Types of bacteria that cause bacterial meningitis in children and teens (primary cause)

• Streptococcus pneumoniae

• H. influenzae

• N. meningitidis

Routes of Inoculation for meningitis

• Pathogen enters blood stream and crosses blood-brain barrier

• Direct spread: organisms enter CSF via structures or foreign objects

Symptoms of meningitis

• Headache

• Fever

• Stiff neck

• Nausea

• Vomiting

• Lethargy

Levels of glucose, proteins, and type of immune cell found in Bacterial Meningitis

Low glucose, high proteins, neutrophils

Levels of glucose, proteins, and type of immune cell found in Viral meningitis

Normal glucose, mildly high proteins, lymphocytes

Levels of glucose, proteins, and type of immune cell found in Fungal/TB meningitis

Low glucose, high proteins, lymphocytes

Autoimmune diseases that target the nervous system

• Multiple sclerosis

• Myasthenia gravis

Multiple sclerosis (MS)

Chronic, progressive, and immune mediated inflammatory disease with progressive demyelination of CNS neurons

• Occurs in relapses

What type of neurons cells are primarily affected in multiple sclerosis (MS)

Oligodendrocytes

Women are more likely to develop multiple sclerosis than men (True/False)

True

Type age of onset for multiple sclerosis

20-40 years

Pathophysiology of Multiple Sclerosis

Damage of the myelin sheath slows action potential of neurons

Symptoms of Multiple Sclerosis

• Paresthesia

• Numbness

• Weakness

• Poor coordination

• Loss of bowel control

Myasthenia Gravis

Acquired, chronic, autoimmune disease that attacks acetylcholine receptors

Which immunoglobulin bodies attack acetylcholine receptors in myasthenia gravis?

IgG

Diagnostic test for Myasthenia Gravis

Serum AChR test

Progressive muscle weakness is a hallmark for myasthenia gravis (True/False)

True