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151 Cards in this Set

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What are the three types of Peripheral Nerve Disorders?

1) Neuropathies


2) Motor Neuron Disorders


3) Spinal Cord Disorders

A neuropathy can be contrasted to a _______





Myopathy

In a myopathy, what is the cause of muscle weakness?

a defect within the muscle

In a myophathy,


is weakness symmetric or asymmetric?

symetric

In a myophathy,


is weakness proximal or distal?

proximal > distal

In a myopathy,


which is lost first DTR's or muscle strength?

In a myopathy,


DTR's and muscle strength are lost at the same time.

In a myopathy,


how is sensation effected?

In a myopathy, sensation is normal?

In a myopathy,


how is bladder function effected?

In a myopathy, bladder function is normal?

In a myopathy,


what labs are abnormal?

elevated muscle enzymes like


- CPK


- aldolase


- SGPT


- SGOT




are often observed

In a neuropathy,


what is the cause of muscle weakness?

Primary defect within the nerves

In a neuropathy,


is weakness symmetric or asymmetric?

asymmetric

In a neuropathy,


is weakness proximal or distal?

distal > proximal

In a neuropathy,


which is lost first DTR's or muscle strength?

DTR is lost early

In a neuropathy,


how is sensation effected?

Sensation is lost

In a neuropathy,


how is bladder function effected?

autonomic nervous system (e.g. bladder function) is often abnormal

In a neuropathy,


what labs are abnormal?

none lab aberrations are associated with neuropathy

Again: What are the three types of Peripheral Nerve Disorders?

1) Neuropathies


2) Motor Neuron Disorders


3) Spinal Cord Disorders

The three types of Peripheral Nerve Disorders may occur as Upper Motor Neuron Diseases or Lower Motor Neuron Diseases.




How does an Upper Motor Neuron Disease present?

- Spastic paralysis


- Slowness due to decreased control of active movement


- Brisk DTRs


- Babinski sign

The three types of Peripheral Nerve Disorders may occur as Upper Motor Neuron Diseases or Lower Motor Neuron Diseases.




How does an Lower Motor Neuron Disease present?

–Flaccid paralysis


–Fibrillations


–Fasciculations


–Absent or reduced DTRs

How do Fibrillations and Fasciculations compare?

– Fibrillations: invisible rapid and unsynchronized contraction of muscle fibers




– Fasciculations: involuntary contraction of muscles

Which, Fibrillations or Fasciculations can only be detected by EMG?

Fibrillations

Which, Fibrillations or Fasciculations are visible?

Fasciculations

Again: What are the three types of Peripheral Nerve Disorders?

1) Neuropathies


2) Motor Neuron Disorders


3) Spinal Cord Disorders

What are the two types of Neuropathies?

1) Mononeuropathies


2) Polyneuropathies

Are Mono-neuropathies sensory, motor or sensory-motor?

sensory-motor

Are Mono-neuropathies symmetrical or asymmetrical?

asymmetrical

What are the symptoms of Mono-neuropathies?

- flaccid paralysis


- loss of DTRs


- reduced or absent sensation


- hypo-algesia


- analgesia


- Paresthesias (spontaneously occurring sensation) including sensations of pain


- dysesthesias


- Allodynia: conversion of non-painful stimuli (e.g. temperature or touch) into pain


- Hyperalgesia: exaggerated pain in response to mild pain stimuli


- Hyperpathia: exaggerated and prolonged pain from mild pain stimuli

What is hypo-algesia?

hypo-algesia

What is analgesia?

analgesia

What are paresthesias?

Par-esthesias are spontaneously occurring sensation

What is dysesthesias?

dys-esthesias are spontaneously occurring sensations of pain

What is allodynia?

Allodynia is the conversion of non-painful stimuli (e.g. temperature or touch) into pain

What is hyper-algesia?

Hyper-algesia is exaggerated pain in response to mild pain stimuli

What is Hyper-pathia?

Hyperpathia is exaggerated and prolonged pain from mild pain stimuli

What populations are at risk of developing mono-neuropathies?

- diabetics


- certain workers


- people remaining in disjointed positions for prolonged periods

Name the six Major Mono-neuropathies

1) median


2) ulnar


3) radial


4) femoral


5) sciatic


6) fibular

What is the median-nerve-associated:


1) Motor paresis


2) DTR lost


3) pain/sensory loss


4) examples

The median-nerve-associated


1) Motor paresis of thumb abduction with thenar atrophy


2) DTR lost is none


3) pain/sensory loss is pain in thumb, second, third fingers and lateral ½ of 4th finger


4) example is Carpal Tunnel

What causes carpal tunnel syndrome?

median nerve injury due to


- repetitive movements


- inflammation (e.g. RA)


- fluid retention (e.g. pregnancy)

What are the findings in people with Carpal Tunnel Syndrom



- motor paresis of thumb abduction


- thenar atrophy


- pain/sensory loss is pain in thumb, second, third fingers and lateral ½ of 4th finger


- worse at night


- awakens people



How do we test for Carpal Tunnel Syndrome?

Tinnel sign by percussing wrist

What is the treatment for Carpal Tunnel syndrome?

– Rest


– Splint


– Diuretics


– Anti-inflammatory


– Steroid injections


– Surgery

What is the ulnar-nerve-associated:


1) Motor paresis


2) DTR lost


3) pain/sensory loss


4) examples

What is the ulnar-nerve-associated:


1) Motor paresis is of


- finger adduction


- thumb adduction


- 5th finger adduction and extension


- 4th finger adduction and extension


2) DTR lost is none


3) pain/sensory loss in


- fifth finger


- medial ½ of 4th finger


4) examples are


- Claw hands (**look up position**)



***Look up benediction sign***

***Look up benediction sign***

What is the radial-nerve-associated:


1) Motor paresis


2) DTR lost


3) pain/sensory loss


4) examples

What is the radial-nerve-associated:


1) Motor paresis of wrist, thumb and finger extensors (i.e. wrist drop)


2) DTR lost is Brachioradialis


3) pain/sensory loss in dorsum of the hand


4) examples are- Saturday night palsy

What is the femoral-nerve-associated:


1) Motor paresis


2) DTR lost


3) pain/sensory loss


4) examples

What is the femoral-nerve-associated:


1) Motor paresis of knee extensors


2) DTR lost is quadriceps


3) pain/sensory loss in anterior, thigh, medial calf


4) there are no nick-names for femoral nerve neuropathy

What is the sciatic-nerve-associated:


1) Motor paresis


2) DTR lost


3) pain/sensory loss


4) examples

What is the sciatic-nerve-associated:


1) Motor paresis of ankle dorsiflexors and plantar flexors (flail ankle)


2) DTR lost is Achilles


3) pain/sensory loss in buttock, lateral calf and most of the foot


4) example is sciatica from herniated disc

What is the peroneal-nerve-associated:


1) Motor paresis


2) DTR lost


3) pain/sensory loss


4) examples

What is the peroneal-nerve-associated:


1) Motor paresis of ankle dorsiflexors and evertors (foot drop)


2) DTR lost is None


3) pain/sensory loss in dorsum of foot and lateral calf


4) example is foot drop that occurs after prolonged leg crossing

What is another name for peroneal nerve?

peroneal nerve = fibular nerve

Again: What are the three types of Peripheral Nerve Disorders?

1) Neuropathies


2) Motor Neuron Disorders


3) Spinal Cord Disorders

What are the two types of Neuropathies?

1) Mononeuropathies


2) Polyneuropathies

Are Poly-neuropathies sensory, motor or sensory-motor?

- sensory


- motor


- or sensory-motor

Are Poly-neuropathies symmetrical or asymmetrical?

symmetrical (generalized; involving all peripheral nerves)

What are the symptoms of Poly-neuropathies?

It depends on whether poly-neuropathy is sensory, motor, or sensory-motor

What are the symptoms of sensory Poly-neuropathies?

numbness and paresthesias in fingers and hands (stocking gloves hypalgesia

What are the symptoms of motor Poly-neuropathies?

distal limbs weakness, atrophy and flaccidity

What are the two types of pathophysiologies that can cause polyneuropathy?

1) damage to myelin (demyelinating neuropathies)


2) damage to axons (axonopathies)

What is the etiology of demyelinating neuropathies?

inflammatory illnesses

What is the etiology of axonopathies?

toxins


or


nutritional changes


or


metabolic etiologies

How many poly-neuropathies are there?

six

Name the six poly-neuropathies:

1) Guillan-Barre


2) Diabetic Neuropathy


3) Heavy metal neuropathy


4) nutritional neuropathy


5) infectious neuropathy


6) metabolic neuropathy

What is the pathophysiology of Guillan Barre Syndrome?

demyelinating polyneuropathy

What causes Guillan Barre Syndrome?

Guillan Barre is triggered by an acute infection



What infections trigger Guillan Barre?

- Campylobacter jejuni


- preceding GI infection


- preceding respiratory infection


- HIV


- Lyme Disease


- hepatitis


- CMV Symptom

Describe the progression of Guillan Barre:

- Symmetrical numbness/paresthesias of fingers and toes


- flaccid paralysis (with absent knee/ankle DTRs) in lower limbs ascends


- lower CNs are affected


- weakness of facial muscles


- difficulty speaking,


- difficulty maintaining open airway


- quadriplegia


- respiratory paralysis


- areflexia


- locked-in syndrome (in severe cases)

What are the metabolic features associated with Guillan Barre?

- hypoxia (is common)


- hyponatremia (is common)


- May be associated with SIADH

What is the mental status of patients with Guillan Barre?

normal

What is the prognosis of patient's with Guillan Barre syndrome?

- 80% have complete recovery within a few months to a year


- 20% have mild to severe residual symptoms


- 5-10% recover with severe disability


- 5% have 1 or more relapses (chronic relapsing variety)

How long after the onset of Guillan Barre does recovery begin?

4 weeks

How do we diagnose Guillan Barre?

Lumbar Puncture


CSF has elevated protein without cellular increase (albumin-cytologic dissociation)

What is the key word for the CSF finding in patients with Guillan Barre?

albumin-cytologic dissociation = elevated protein without cellular increase

What is the treatment of Guillan Barre?

- Plasmapheresis


- IV immunoglobulins


- Supportive care

How does Diabetic Neuropathy present?

- loss of sensation in stocking glove distribution


- loss of DTRs in ankles then knees


- impaired sensation in fingertips


- sudden pain (often at night, preventing sleep)

What is the treatment of Diabetic Neuropathy?

- Opioids


- Antiepileptics (gabapentin, pregabalin)


- TCAs


- Duloxetine


- Cream containing capsaicin (depletes substance P)

What heavy metals can cause poly-neuropathy?

- lead


- arsenic


- mercury


- thallium

What are the two main categories of symptoms in patient's with lead poisoning?

1) CNS symptoms


2) motor polyneuropathy

What population is more likely to present with CNS symptoms when they have lead poisoning?

children

What population is more likely to present with motor poly-neuropathy symptoms when they have lead poisoning?

adults

What CNS symptoms do children with lead poisoning present with?

- learning disabilities


- inattention


- poor school performance


- seizures (in severe cases)


- mental retardation (in severe cases)

What motor poly-neuropathy symptoms do children with lead poisoning present with?

foot drop and wrist drop


(symmetrical)

What are the symptoms of Arsenic poisoning?

- Guillain-Barre like syndrome


- mee lines on fingernails


- hyperpigmentation


- hyperkeratosis

What are the symptoms of Mercury poisoning?

- Neuropathy


- cognitive impairment


- ataxia


- visual field changes


- Gums have dark line below teeth

What are the symptoms of Thallium poisoning?

- neuropathy


- alopecia

Which metal poisoning is also associated with pica?

lead poisoning

What nutritional deficiencies that can cause poly-neuropathies?

- B1 (thiamine)


- B3 (niacin)


- B12 (cobalamin)

What are the symptoms of B1 deficiency?

sensorimotor neuropathy, weight loss, edema



What is another name for B1 deficiency?

Beriberi

What are the symptoms of B3 deficiency?

dementia, dermatitis, diarrhea

What is another name for B3 deficiency?

Pellagra

What are the symptoms of B12 deficiency?

Reversible dementia (CNS) and neuropathy/sensory loss (PNS)

How does B12 deficiency cause reversible dementia and sensory loss?

demyelination of the central and peripheral nervous systems

What vitamin is associated with alcohol-induced neuropathy?

B1 (thiamine)

What are the infectious causes of poly-neuropathy?

- Herpes zoster


- Erythema migrans


- Lyme Diseas


- Bell’s palsy


- HIV

What are the poly-neuropathy manifestations of Herpes Zoster?

typically affects the root of Cranial Nerve 5

What are the symptoms of Lyme Disease?



- Erythema migrans


- arthritis


- low grade fever


- Bell's Palsy like syndrome


- mild neuropathy --> GB like symptoms



What are the poly-neuropathy manifestations of HIV?

- peripheral neuropathies (distal pain)


- dementia

What are two Metabolic Neuropathies?

1) Acute intermittent porphyria


2) Metachromatic Leukodystrophy

What are the symptoms of Acute intermittent porphyria?

- dramatic attacks of quadriperesis and severe abdominal pain


- Agitation


- delirium


- depression


- psychosis


- Red urine

What is the cause of Metachromatic Leukodystrophy?

genetic

What is the mode of inheritance of Metachromatic Leukodystrophy?

Autosomal recessive

What is the pathophysiology of the poly-neuropathy aspect of Metachromatic Leukodystrophy?

Demyelination of CNS (white matter) and PNS

What is the presentation of Metachromatic Leukodystrophy?

young adult with frontal cognitive impairment (personality/behavior change)




progresses to dementia




progresses to


spasticity (from PNS demyelination)


and


ataxia (from CNS demyelination)

Again: What are the three types of Peripheral Nerve Disorders?

1) Neuropathies


2) Motor Neuron Disorders


3) Spinal Cord Disorders

Name the three Motor Neuron Disorders:

1) Amyotropic Lateral Sclerosis


2) Werdnig-Hoffman disease (in infants) and Kugelberg-Welander (in children)


3) Poliomyelitis

What is the cause of ALS?



- excessive free radicals


- Most cases are sporadic


- 5-10% of cases are genetic





What is the mode of inheritance of genetic type of ALS?

autosomal dominant

Does ALS affect upper or lower motor neurons?

both

What are the symptoms of ALS?

Earliest symptoms: lower motor neurons


- weakness followed by


- twitching in one leg


- fasciculations in one leg


- cramping in one leg


- atrophy in one arm or leg


These lower motor symptoms then spread to other limbs




Upper motor neurons symptoms include


- muscle spasticity (tight stiff muscles)


- exaggerated DTRs


- Babinski sign




Bulbar and pseudobulbar (crying, laughter) palsy leading to difficulty talking and swallowing

List all the things that are normal in patients with ALS:

- mental status


- ocular motility


- sensation


- bladder/bowel functions

What is the prognosis of patient's with ALS?

- death usually occurs within one year


- mental status is usually normal


- fronto-temporal dementia develops in 10%


- associated with high risk of suicide

What is the treatment of ALS?

riluzole

What is the mechanism of action of riluzole?

- NMDA antagonist


- inhibits glutamate neurotransmission

How does riluzole effect the prognosis of ALS?

may slow progression

Does ALS affect upper or lower motor neurons?

Lower motor neuron

What are the motor neuron symptoms of Werdnig-Hoffman disease?

- Progressive flaccid quadriperesis


- Muscle atrophy, fasciculations and loss of DTRs

start on slide 19 (Poliomyelitis section)

***

What is the cause of Poliomyelitis?

Results from poliovirus infection of motor neurons of anterior horn of spine and brainstem

What population is affected by Poliomyelitis?

Affects mostly children

What is the very first symptoms of Poliomyelitis?

acute febrile illness

Does Polio affect Upper Motor Neuron or Lower Motor Neuron?

Lower Motor Neuron

What are the neurological signs of Polio?

Lower Motor Neuron Signs


- asymmetric paresis


- fasciculations


- absent DTRs

In patients with Polio, what is one of the worst neuro symptoms?

throat and chest muscle paralysis necessitating respiratory support

What neuro symptoms are normal in patients with poliomyelitis?

- normal mentation


- normal eye movement


- normal bladder/bowel control

Again: What are the three types of Peripheral Nerve Disorders?

1) Neuropathies


2) Motor Neuron Disorders


3) Spinal Cord Disorders

List the high yield spinal cord disorders

1) Spino-cerebellar ataxia


2) Brown Sequard Syndrome


3) Spinal Cord Transection


4) Disk herniation


5) Subacute combined degeneration


6) Anterior Spinal Artery (ASA) Infarction


7) Syringomyelia

*** See slide 21 for spinal cord anatomy ***

*** See slide 21 for spinal cord anatomy ***

List the spino-cerebellar ataxias:

Friedreich ataxia, ataxia-telangiectasia, olivopontocerebellar atrophy, and Charcot-Marie-Tooth disease

What is the cause of spin-cerebellar ataxias?

Genetic mutations cause loss of myelin and degeneration of spinocerebellar tracts and cerebellum

What is the genetic profile of patients with spina-cerebellar ataxia?

- autosomal recessive


or


- autosomal dominant




- excessive trinucleotide repeats on different chromosomes

What are the symptoms that all types of spin-cerebellar ataxia present with?

- ataxia


- loss of vibration and position sense

What is the cause of Brown-Sequard Syndrome?

lateral hemisection of spinal cord

What are the symptoms of Brown-Sequard Syndrome?

1) cortico-spinal tract signs


2) dorsal column signs


3) spino-thalamic tract signs

What are cortico-spinal tract signs?

ipsilateral paresis, hyperactive DTRs and Babinski sign

What are dorsal column signs?

ipsilateral fine touch position and vibration sensory loss

What are spino-thalamic tract signs?

contralateral pain and temperature loss

What are the signs of transecting the spine at C3 vertebrae and above?

quadriplegia and loss of diaphragm function, necessitating the use of a ventilator for breathing

What are the signs of transecting the spine at C4 through C7?

sensory loss, partial quadriplegia, apnea, bladder/bowel/sexual impairment

What are the signs of transecting the spine at the thoracic vertebrae level?

sensory loss, paraplegia, bladder/bowel/sexual impairment

What are the signs of transecting the spine at the lumbosacral verterbrae level?

sensory loss, lower extremities weakness, bladder/bowel/sexual impairment

Where do the majority of disk herniations occur?

L4 through L5




or




L5 through S1

Weakness of the ankle may be due to injury at what spinal cord level?

S1 or S2

What level of the spinal cord is injured in Cauda Equine syndrome?

L1 through S5

What are some of the causes of Cauda Equina Syndrome?

- trauma


- tumors


- disks


- spinal stenosis

What are the symptoms of Cauda Equine syndrome?

- Pain in legs and low back (saddle-like distribution)


- Lower extremities weakness (sometimes paraplegia)


- Loss of DTRs


- Incontinence


- Sexual dysfunction

How do we diagnose Cauda Equine Syndrome?

CT or MRI

What is the cause of Subacute combined degeneration?

B12 deficiency

Which spinal columns does Subacute combined degeneration affect?

lateral column




and




dorsal column

What are the symptoms of Subacute combined degeneration?

combined upper and lower motor neurons symptoms

Which spinal columns does Anterior Spinal Artery (ASA) Infarction affect?

entire cord except dorsal columns

What are the symptoms of Anterior Spinal Artery (ASA) Infarction?

- quadriplegia


- a-reflexia

Which spinal columns does Syringomyelia affect?

spino-thalamic tract

What are the symptoms of Syringomyelia?

loss of pain and temperature senses