Peripheral Nerve Diseases Flashcards
What are the four type of peripheral nerve diseases?
- Inflammatory neuropathies
- Infectious polyneuropathies
- Hereditary neuropathies
- Acquired metabolic/toxic neuropathies
What is one type of inflammatory Neuropathy?
Guillain-Barre Syndrome
What is the pathogenesis/timeline of Guillain-Barre syndrome?
“Acute inflammatory demyelinating polyradiculoneuropathy”
- 2/3 of cases preceded by acute flu-like illness (Campylobacter, CMV, EBV, Mycoplasma, influenza virus)
- T cells and macrophages cause segmental demyelination; antibodies also present [patients get better when antibodies are taken out - plasmaphoresis]
- Remyelination follows
Neuropathy
Problem with nerves
Guillain-Barre Syndrome
- Immune mediated disease
- Body starts attacking virus and then begins attacking myelin
- Life-threatening disease of the PNS
- Flu-like illness, then acute ascending paralysis (starts at feet)
- Immune-mediated demyelination
- Usually resolves with time
Where does Guillain-Barre target and what does it look like on a slide?
- Inflammation around venules, nerves [look dark red/pink on H&E stain]
- Lymphocytes, macrophages, plasma cells
- Widely distributed through PNS
- Most intense in spinal and cranial nerve motor roots and adjacent nerves
What does Guillain-Barre look like in the clinic?
- Symmetric, ascending paralysis
- Rapid-onset weakness, loss of deep tendon reflexes
- Some loss of sensation
- Elevated CSF protein (no lymphocytes though)
- 2-5% mortality (respiratory paralysis, autonomic instability, cardiac arrest, complications of treatment)
- Respiratory therapy may be needed
What type of demyelination happens in Guillain-Barre?
Segmental demyelination - some segments affected, some not
What are the two forms of Infectious polyneuropathies that we studied?
Leprosy & Varicella-Zoster
Describe Leprosy/Hansen Disease:
- Slowly progressive infection of skin and nerves
- Caused by myobacterium leprae
- Transmitted through resp. droplets
- Endemic in poor, tropical countries
- Causes disabling deformities
What are the characteristics of Mycobacterium leprae?
- Acid-fast, obligate intracellular bacterium
- Grows poorly in culture
- Cell wall doesn’t stain with gram stain!
- Hard to kill in normal ways - body forms granulomas to attack and eat the bugs (LIKE TB)
- Bacteria does not like to be too hot
- Only grows at skin temperature
What are the two types of Leprosy?
- Tuberculoid (less severe)
- Lepromatous (more severe)
Describe Tuberculoid Leprosy:
- Less severe, localized
- Dry, scaly skin lesions
- Nerve degeneration (anaesthesia, ulcers, contractures)
- Nice T-cell response to the bug, with nice granuloma formation
What does Tuberculoid leprosy look like?
- Large, red circular lesions on back or other parts
- Granuloma with lighter colored outer rim seen in H&E slides
- Few bugs shown on H&E
- Macrophages form the granuloma
- Nerves may look inflamed with many lymphocytes on the H&E slides
Describe Lepromatous Leprosy:
- More severe, widespread
- Skin, nerves, eye, mouth, testes, hands, feet
- Patient’s immune system doesn’t respond
- Leonine facies <– lion face nodules
- Autoamputation may occur at extremities