Peripheral Nerve Diseases Flashcards
What are 2 Inflammatory Neuropathies?
- Guillain Barre
2. Chronic Inflammatory Demyelinating Poly(radiculo)neuropathy
Guillain Barre is an acute inflammatory demyelinating polyneuropathy. What may precede muscular symptoms?
Flu-like illness
– resolves before symptoms appear
What occurs early in the disease process with Guillain Barre?
DTRs disappear
What is the main symptom of Guillain Barre patients?
ASCENDING paralysis
= Distal limb weakness –> proximal weakness
What is the main symptom of Guillain Barre patients?
ASCENDING paralysis
= Distal limb weakness –> proximal weakness
What are a few common causes of Guillain Barre?
Campylobacter Jejuni
CMV, EBV, Mycoplasma
Prior vaccination
What 2 things will be seen on histo with Guillain Barre?
- Perivenular and Endoneurial infiltration by inflammatory cells
- Segmental Demyelination
What 2 things will be seen on histo with Guillain Barre?
- Perivenular and Endoneurial infiltration by inflammatory cells
- Segmental Demyelination
What antibodies will be positive with Guillain Barre?
Anti-Myelin Antibodies
What change in CSF will be seen with Guillain Barre?
Increased CSF protein
What are 2 possible treatments for Guillain Barre?
Plasmapheresis
IVIg
Radiculoneuropathy is seen with both peripheral inflammatory neuropathies. What is it?
Demyelination of spinal nerve roots and peripheral nerves
What is the most common acquired inflammatory peripheral neuropathy?
Chronic Inflammatory Demyelinating Poly(radiculo)neuropathy
What are the signs of Chronic Inflammatory Demyelinating Poly(radiculo)neuropathy?
Symmetrical mixed sensorimotor symptoms > 2 MONTHS
Symmetrical mixed sensorimotor symptoms for longer than 2 months?
Chronic Inflammatory Demyelinating Poly(radiculo)neuropathy
What change to what nerve is seen with Chronic Inflammatory Demyelinating Poly(radiculo)neuropathy?
Surval nerve = Onion bulb
What distinguishes Chronic Inflammatory Demyelinating Poly(radiculo)neuropathy from Guillain Barre?
Time and response to steroids!
What are 3 Infectious Polyneuropathies of peripheral nerves?
- Leprosy
- Diphtheria
- Varicella-Zoster
What are the 2 types of Leprosy?
Lepromatous
Tuberculoid
What organism is to blame for Leprosy?
Mycobacterium Leprae
With Lepromatous Leprosy, Mycobacterium Leprae invades what cells and causes what to occur?
Invades Schwann cells
= Loss of BOTH myelinated and Unmyelinated axons
What 2 histological changes will be seen with Lepromatous Leprae?
- Endoneurial fibrosis
2. Thickening of perineural sheaths
Endoneurial fibrosis and thickening of perineural sheaths is seen with?
Lepromatous Leprosy
What are the symptoms of Lepromatous Leprosy?
Symmetric polyneuropathy – loss of pain sensation
Large ulcers
Lepromatous Leprosy preferentially affects where?
COOL extremities
– symmetric polyneuropathy and large ulcers
Tuberculoid Leprosy is a cell mediated immune response. What differentiates it from Lepromatous Leprosy?
LOCALIZED nerve involvement
What can be seen in the dermis of Tuberculoid Leprosy patients and what differentiates it from Lepromatous Leprosy?
Granulomatous nodules in the dermis
– LOCALIZED nerve involvement
What unique loss will present with Diphtheria?
Loss of proprioception and vibratory sensation
What muscle dysfunctions will be prominent with Diphtheria?
Bulbar and respiratory muscle dysfunctions
Bulbar and respiratory muscle dysfunctions with loss of proprioception and vibratory sensation is likely?
Diphtheria
Varicella-Zoster Virus affects _____. And once it is reactivated it causes?
Affects sensory ganglia => Shingles
What dermatomes are often affected with the painful vesicular eruption from Varicella-Zoster Virus?
Thoracic or Trigeminal Nerve Dermatomes
What follows the death of sensory neurons with the Varicella-Zoster virus?
Axonal degeneration of peripheral nerves
What is the most common cause of Peripheral Neuropathy?
Diabetes
What type of polyneuropathy is seen with Diabetes and why?
Ascending distal symmetric sensorimotor polyneuropathy
= Decreased # of axons
Ascending distal symmetric sensorimotor polyneuropathy is seen with Diabetes. What are some symptoms?
Numbness
Decreased pain sensation and balance
Paresthesias
What are diabetic patients at risk for?
Increased foot/ankle fractures and ulcers
==> Amputation
With Diabetes, what will the Endoneurial Arterioles show histologically?
PAS (+)
Thickened, and basement membrane reduplicated
Vascular changes contribute to the damage seen with Diabetes. What histo change will be seen?
Endoneurial Arterioles = PAS (+) and thickened reduplicated basement membranes
Paraneoplastic Neuropathies often precede cancer diagnosis. What is a common cause of a sensorimotor neuronpathy?
Small cell lung cancer (neuroendocrine)
Besides Small cell lung cancer, what other neoplasm can cause paraneoplastic neuropathies?
Monoclonal Gammopathies (B cell neoplasms)
Monoclonal Gammopathies (B cell neoplasms) can cause paraneoplastic neuropathies. What do they secrete that damage the nerves?
Monoclonal Immunoglobulins = Demyelination
What are the symptoms of a paraneoplastic neuropathy from a Monoclonal Gammopathy (B cell neoplasm)?
POEMS
- polyneuropathy
- organomegaly
- endocrinopathy
- monoclonal gammopathy
- skin changes
Traumatic Neuroma
Axons continue to grow despite misalignment
If you see small bundles of axons randomly oriented, it is likely a?
Traumatic Neuroma
Peripheral nerves can also be subjected to physical forces causing compression neuropathies. What are 2 examples?
Carpel Tunnel
Saturday night palsy
What anatomic structures are affected with Carpel Tunnel?
Median Nerve
Transverse carpal ligament
Carpel tunnel is also associated with what endocrine change?
Hypothyroidism
Saturday night palsy affects what nerve?
Radial nerve
What is an inherited peripheral neuropathy?
Charcot-Marie-Tooth
What is an inherited peripheral neuropathy?
Charcot-Marie-Tooth
What are the signs of Charcot-Marie-Tooth?
Distal muscle atrophy
Sensory loss
FOOT DEFORMITIES
Distal muscle atrophy, sensory loss and foot deformities may be?
Charcot-Marie-Tooth
– inherited peripheral neuropathy
What is the more severe Charcot-Marie-Tooth form that can present at childhood?
CMT2 - axonal injury
What is the less severe Charcot-Marie-Tooth that presents in the 2nd decade?
CMT1 - demyelinating neuropathies
Charcot-Marie-Tooth is inherited how?
Autosomal Dominant
