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Systemic Pathology > Neuro Pathology > Flashcards

Neuro Pathology Flashcards

1
Q

Diseases of Peripheral Nerves

A

Peripheral nerve injury

  • Axonal Neuropathies —problems with axons
  • Demyelinating Neuropathies —problems with myelin covering axons
  • Neuronopathies — problems with neurons
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2
Q

Types of Peripheral Neuropathies

A

Mononeuropathies

  • Polyneuropathies — multiple nerves in a symmetric fashion
  • Mononeuritis multiplex — several nerves in a haphazard fashion
  • Polyradiculoneuropathies — affect nerve roots and peripheral nerves; Symptoms in proximal and distal parts of the body
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3
Q

Neuronal Diseases of the Neuromuscular Junction

A

> Antibody mediated

  • Myasthenia Gravis
  • Lambert-Eaton Myasthenic Syndrome

> Congenital
Caused by toxins

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4
Q

Diseases of Skeletal Muscle

A 
Skeletal Muscle Atrophy
Neurogenic and myopathic changes
Inflammatory myopathies
Toxic myopathies
Inherited diseases of skeletal muscle — muscular dystrophies
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5
Q

Peripheral Nerve Sheath Tumors

A 
Schwannomas 
Neurofibromas
Malignant Peripheral Nerve Sheath Tumors
Neurofibromatosis Type 1 and Type 2
- Type 1 is most common
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6
Q

Neuropathy with Systemic Autoimmune Diseases

A

Rheumatoid arthritis Sjögren’s
SLE
Distal sensory or sensorimotor polyneuropathies

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7
Q

Problems in the CNS come from

A

Malformations
Injury
Trauma —brain trauma, spinal cord injury
Cerebrovascular disease —hypoxia, ischemia, and infarction
Hypertensive cerebrovascular disease — hemorrhage
Infections
Demyelinating Diseases
Neurodegenerative
Genetic, Metabolic
Toxic and Acquired Metabolic Diseases
Tumors

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8
Q

MYASTHENIA GRAVIS

A

Chronic autoimmune disorder causing peripheral neuropathy

  • Circulating antibodies against the nicotinic ACh receptor (specifically the muscle-specific receptor tyrosine kinase) — impair neuromuscular transmission

More common in females

 SYMPTOMS 
Muscle fatigability — not strong enough to support body
- Diplopia
- Dysarthria
- Dysphagia
- Limb weakness
- Shortness of breath
- Ptosis —can affect one or both eyes
- Pale skin to cyanotic 
- SOB 
-sleepiness/dizziness 
-lower BP 
-headache

Diagnostic Tests
- Serum acetylcholine receptor antibody analysis

A drooping eyelid is the most frequent early sign of myasthenia gravis

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9
Q

Botulism

A

Disease of neuromuscular junction caused by TOXIN

Clostridium botulinum — neurotoxin
o Blocks receptors, causing permanent paralysis in the whole body, including the
respiratory system

  • Botox acts by blocking the release of acetylcholine receptors — temporary paralysis
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10
Q

Diseases of Skeletal Muscle/Myopathies

A

Skeletal Muscle atrophy — many disorders

Loss of innervation

Neurogenic and Myopathic changes

Inflammatory

Toxic — recreational drugs, statins, chloroquine, and alcohol

Congenital

Myopathy is very common with use of statins

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11
Q

NEUROFIBROMATOSIS Type 1

NF type 1

A

Common autosomal dominant disorder

  • Peripheral Nerve Sheath Tumor
  • Mutation gene on chromosome 17
  • Associated nonneoplastic tumors
  • Neurofibromas, gliomas, hamartomas
  • Malignant tumors
  • Cutaneous hyperpigmented macules**
  • Brown red spots of the eye ( Lisch Nodules)**
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12
Q

GUILLAIN-BARRE SYNDROME

A

Acute Inflammatory Demyelinating Polyneuropathy
- Inflammation of peripheral nerves
- Demyelination, damage to axons
o Lose myelin, which damages the axons
- Life threatening respiratory paralysis
- Ascending paralysis — loss of pain sensation
- Weakness
- Often preceded by a virus
o 2/3of cases are preceded by an acute influenza like illness
o Cytomegalovirus, Epstein Barr, Mycoplasma

Diagnostic Test: CSF reveals elevated protein count with normal cell count

TXT:

  • Plasmapheresis (plasma exchange)
  • High dosage immunoglobulin therapy
  • Corticosteroids
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13
Q

Infectious Neuropathies

A

Shingles
Leprosy
Diphtheria
Varicella-zoster

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14
Q

Leprosy (Hansen Disease)

A

Mycobacterium leprae

  • Infection
  • Polyneuropathy
  • Large traumatic ulcers
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15
Q

Lyme Disease

A

Late stages

  • Polyradioculoneuropathy
  • Unilateral or bilateral facial nerve palsies
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16
Q

HIV/AIDS

A

Peripheral neuropathy

  • Poorly understood
  • Immune deregulation
  • Mononeuritis multiplex
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17
Q

Diphtheria

A

peripheral nerve dysfunction, diphtheria exotoxin

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18
Q

Diabetic Neuropathy

A

o most common cause or peripheral neuropathy

o Metabolic and secondary vascular changes

o Damage and loss of peripheral nerve fibers (neurons and Schwann cells)

o Ischemic damage of the nerves
- Without circulation, the nerves get damaged

o Major effect is on the small unmyelinated nerve fibers that modulate autonomic
function and thermal and pain perceptions

o Impact on wound healing and ulcerations

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19
Q

Uremic neuropathy

A

Renal failure
o Muscle cramps, diminished deep tendon reflexes
o Axonal degeneration
o Regeneration and recovery are common after dialysis
- Patients in stage 3 or 4 may not be ready for dialysis

20
Q

Other Metabolic, Hormonal, and Nutritional Neuropathies

A

Thyroid dysfunction —hypothyroidism

Vit B12 deficiency = pernicious anemia, the only anemia with neurological symptoms
o Damage to long tracts in the spinal cord and peripheral nerves
o Neurological symptoms, peripheral neuropathy, and problems with gait

21
Q

Neuropathies Caused by Physical Forces

A
  • Trauma
  • Lacerations
  • Compression

o Carpal Tunnel Syndrome — median nerve at the level of the wrist

22
Q

Inherited Peripheral Neuropathies

A 
o Genetically diverse disorders
o Charcot- Marie-Tooth disease
o Hereditary Motor Neuropathies
o Hereditary sensory neuropathies
o Familial amyloidosis
23
Q

Shaken Baby Syndrome

A

Brain hitting the back and front of skull

  • Babies are more fragile, and the shaking can create hemorrhage
  • The brain hits against the bone, causing the internal hemorrhage
24
Q

Trauma and Neuronal Damage

A

Location and extension determine prognosis
- Blunt trauma — brain injury at the original point of contact and on the opposite side of
the brain
o Trauma occurs at one point, but affects two points in the brain
- Rapid displacement of the head and brain can tear axons, severe, irreversible neurologic
damage
- Traumatic tearing of blood vessels — epidural or subdural hematoma
- Skull fractures
- Parenchymal Injuries — concussion
- Direct Parenchymal Injury — contusions and lacerations
- Sudden impacts that result in violent posterior or lateral hyperextension of the neck
may avulse the pons from the medulla or the medulla from the cervical cord — instant
death

25
Q

Traumatic Vascular Injury

A

Direct trauma and disruption of the vessel wall, hemorrhage

  • Epidural (arterial)
  • Subdural (venous)
  • Subarachnoid
  • Intraparenchymal
  • Combination
26
Q

Brain Trauma Sequelae

A

Post-traumatic hydrocephalus

Chronic traumatic encephalopathy — dementia that develops after repeated head trauma

Post-traumatic epilepsy

27
Q

Guideline on antibiotic prophylaxis for dental patients at risk for infection

A

For valve replacement, valve repair, history of bacterial endocarditis, unrepaired
cyanotic heart defects or repaired within 6 months, history of heart transplant with
history of valvulopathy
- NOT for hydrocephalus VA shunts!!
o But may still need prophylaxis depending on the immune status

28
Q

Hydrocephalus

A
  • CSF accumulation
  • Widening of brain ventricles
  • Pressure
  • Genetic abnormalities
  • Developmental disorders like spina bifida/neural tube defects
  • Intraventricular hemmorage
  • Meningitis, tumors, traumatic head injury

TXT: surgery-shunt diverts the flow of CSF to another part of the body such as the abdominal cavity (VP), heart (VA), or around the lung where the CSF can drain and be absorbed

29
Q

Ischemic Stroke

A

syndrome, not a disease
- It is caused by a transient or permanent critical reduction in cerebral blood flow due to
arterial occlusion or stenosis

  • Identification of the underlying mechanisms and etiologies is important so that appropriate therapy can be initiated to decrease the risk of recurrent stroke
30
Q

Global Cerebral Ischemia

A
  • Generalized reduction of cerebral perfusion
  • Cardiac arrest, shock, and severe hypotension
  • Severity depends on location and extent
  • Survival varies
31
Q

Focal Cerebral Ischemia

A

Localized area — arterial occlusion or hyporperfusion

  • Size, location, shape of the infarct, and extent of tissue damage
  • Major source of collateral flow is the circle of Willis
32
Q

Occlusive Vascular Disease

A
  • Embolism — from a distant source
    o Atrial fibrillation, MI, valvular diseases
    o Atheromatous plaques — carotid arteries
    o Cardiac surgery
    o Emboli form other material — tumor, fat, air
  • In situ thrombosis
    o Commonly associated with atherosclerosis and plaque rupture
    o Hypertension and Diabetes
  • Inflammatory processes
    o Infectious vasculitis — syphilis and TB, immunosuppression, and opportunistic
    infection — CMV encephalitis
33
Q

STROKE definition

A

clinical designation that applies to Hypoxia, Ischemia, Infarction, Hypertensive Cerebrovascular disease, and Intracranial Hemorrhage

Acute neurologic deficit caused by cerebrovascular etiology

  • Hemorrhagic stroke or ischemic stroke
  • In ischemic stroke, patients do not exhibit GI symptoms
  • Test — CT scan hemorrhage
  • Vascular rupture with bleeding
  • Bleeding extended in anticoagulated patients
  • Mortality is high
34
Q

risk factor most commonly associated with

lobar hemorrhages is…

A

Cerebral amyloid angiopathy (CAA)

35
Q

The most important effects of hypertension on the brain include:

A

o Lacunar infarcts
o Slit hemorrhages
o Hypertensive encephalopathy
o Massive hypertensive intracerebral hemorrhage

36
Q

Demyelinating Diseases

A

Multiple Sclerosis

Neuromyelitis Optica

Acute Disseminated Encephalomyelitis

Acute Hemorrhagic Encephalomyelitis

37
Q

Acquired v. Critical Role Demyelinating diseases

A

Acquired conditions — preferential damage to myelin with relative preservation of axons

Critical role — myelin nerve conduction
- Widespread and severe neurologic deficits

38
Q

Multiple Sclerosis

A

Autoimmune in which the immune system destroys myelin, resulting in slowing of conduction and exposure of axons which may lead to permanent loss of the axon and subsequent loss of nerve function

Related to genetic predisposition

  • An environmental trigger than causes an immune attack
  • Thus get loss of myelin and nerve damage

Clinical course - relapsing and remitting episodes

Key diagnostic factors

  • Visual disturbance in one eye
  • Peculiar sensory phenomena

Other diagnostic factors

  • Female, age 20-40
  • foot dragging or slapping,
  • leg cramping
  • fatigue
  • urinary frequency
  • bowel dysfunction, -
  • spasticity/increased muscle tone
  • increased deep tendon reflexes
  • imbalance/incoordination-
39
Q

Orofacial Manifestations of MS

A
  • Intermittent facial numbness
  • Facial palsy or spasm
  • Paroxysmal pain syndromes (neuropathic)
    o High-frequency episodes of shock-like or lancinating pain
    o Trigeminal neuralgia (1-5% of patients)
  • Mild dysarthria
  • Lhermitte sign
  • Monocular visual disturbances
40
Q

Neurodegenerative Diseases

A
  • Alzheimer Disease
  • Parkinson Disease
  • Multiple System Atrophy
  • Huntington Disease
  • Spinocerebellar Disease — ataxias
  • Amyotrophic Lateral Sclerosis (ALS)
41
Q

Huntington Disease

A

Autosomal dominant

  • Progressive movement disorders and dementia
  • Degeneration of neurons
  • Hyperkinetic, dystonic movements, all parts of the body (chorea)
  • Progressive and fatal
  • Key diagnostic factors
    o Positive family history of Huntington disease, known expansion of the CAG
    repeat length at the N-terminal end of the huntingtin gene, impaired work or
    school performance, personality change, irritability and impulsivity, chorea,
    twitching or restlessness, loss of coordination, deficit in fine motor coordination,
    slowed rapid (saccadic) eye movement
42
Q

Amyotrophic Lateral Sclerosis (ALS)

A

Progressive disorder
- Loss of upper motor neurons in the cerebral cortex and lower motor neurons in the
spinal cord and brainstem
- Toxin protein accumulation
- Denervation of muscles — weakness
- Slightly affects more men
- Symptoms
o Asymmetric weakness of the hands, difficulty in performing fine motor tasks
o Cramping and spasticity of the arms and legs (extremities)
o After — respiratory muscles  severe pulmonary infections
o Abnormalities in swallowing and phonation

43
Q

Seizure classification

A

Partial — seizure activity starts in one area of the brain
o Simple — retain awareness
o Complex —altered awareness and behavior

  • Generalized —seizure involves whole brain and consciousness is affected
    o Tonic clonic —“grand mal” or convulsions
    o Absence — “petit mal” or staring fit
    o Atonic/tonic — “drop attack”
    o Myoclonic — sudden muscle jerks
44
Q

Neuronal Reaction to Injury

A
  • Acute (e.g. trauma; lack of oxygen or glucose)
  • Chronic (e.g. degeneration; accumulation of products)

Neuronal death occurs either by necrosis (unplanned) or apoptosis (programmed cell death)

45
Q

Acute neuronal injury

A 
(“red neurons”)
- Evident by 12-24 hrs after irreversible injury
- Changes:
• Neuronal body
• Nucleus
• Nucleolus
• Nissl substance
• Cytoplasm
46
Q

Chronic or Subacute neuronal injury

A

Subacute and chronic neuronal injury (“degeneration”)
- Slow(er) process
- Changes:
• Cell loss (initially difficult to detect)
• Often selective functional area
• Cell could be apoptotic
• Reactive gliosis (indicator of cell injury, regardless the etiology)

47
Q

Reactive gliosis

A

process that defends the body, but it’s also a good indicator of cell injury for us to look at

  • Doesn’t occur unless there’s injury
  • Occurs regardless of etiology

Gliosis refers to the glia (astrocytes and oligodendrocytes, but NOT microglia)

  • Microglia are NOT glia! They come from somewhere else!
  • Not schwann cells either because we’re in the CNS

Systemic Pathology (8 decks)

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