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Flashcards in ALS/CRPS - neuro Deck (12):
1

ALS UMN or LMN?

-ALS at its onset is usually one or the other: all UMN or all LMN degeneration.
-But eventually, all ALS becomes a mix of UMN and LMN pathology/symptoms. In fact, if you are considering a MN disorder that only has UMN OR LMN symptomatology, then it can’t be ALS.
-Mixed UMN and LMN pathology
#1 is ALS
-think ALS in any pt who presents with weakness WITHOUT sensory changes, and their exam shows UMN AND LMN symptoms
-progressive

2

Motor Neuron Disease pathological findings

-Motor Neuron Disease pathological findings
-neuron shrinkage – cell nuclei shrink
-accumulation of pigmented lipid
-accumulation of neurofilaments and other proteins (spheroids)
-eventual neuron death -> denervation = losing the nerve supply to periphery \
-loss of axonal attachment to peripheral muscle -> atrophy = “amyotrophic”
-thinning CSTs -> fibrillary gliosis (firm) come together and lose axons but proliferation occurs and causes it to thicken= “lateral sclerosis”

3

ALS Etiologies

-possibly associated with military service, exposure to pesticides and insecticides, tobacco use
-gene mutations, especially in free radical suppression genes

4

ALS Signs and Sxs

more LMN disease
-onset-symptom usually is asymmetric ascending (toes to ankle to calf and on up) weakness (“stretching in bed before getting up leads to muscle cramps”)
-atrophy and fasiculations - muscle weakness
-if bulbar involvement, onset-symptom usually is dysphagia, difficulty chewing and abnl face+tongue movt’s

more UMN disease
-spasticity
-hyperreflexia
-“muscle stiffness >> muscle weakness”
-Dysarthria – difficulty speaking
-excessive weeping and laughing (loss of UMNs to emotional centers)

5

ALS Dx

-progressive weakness
-simultaneous UMN and LMN symptoms
-“definite” = 3 of 4 sites involved (bulbar, cervical, thoracic, lumbosacral)
-“probable” = 2 of 4
-“possible” = 1 of 4

6

ALS Tx

-there is nothing to arrest the underlying pathology, but, you can prolong survival with riluzole 100mg qday. MOA = reduces neuronal excitotoxicity. Common SEs = nausea, dizziness, wt loss and increased LFTs.
-rule out treatable etiologies
PT referral:
-orthotics (especially foot drop brace -> aids in gait, decreases falls)
-maximize ADLs with adaptive equipment
-ROM and strengthening exercises

Respiratory testing -> Respiratory Therapy and -Pulmonologist referrals
-breathing exercises
-ventilatory-assist options

7

Complex regional pain syndrome (CRPS)

-a disabling neuropathic pain disorder that affects the physical and psychologic aspects of a patient’s life
-controversial, in that there are no universal guidelines, nor any specific tests which rule in or rule out its presence
-often missed by primary care providers
-no cure
-one of the many pain syndromes that are associated with vasomotor (capillary function) and sudomotor (sweat function) changes
-can be sympathetically-mediated pain (improves with a sympathetic nerve block) or non-sympathetically-mediated pain (doesn’t improve post SNB)

8

etiology CRPS

-tissue damage, either with documented nerve damage (CRPS II) or no specific nerve injury (CRPS I). This points toward the need for electrodiagnostics.
-can be just due to sprain/strain
-commonly occurs after surgery, especially toe or foot or ankle surgeries
-also after injections, especially for plantar fasciitis or Morton’s neuroma
-unclear mechanisms, but felt to be due to PNS hypersensitivity and CNS sensitization

9

CRPS signs and symptoms

-pain out of proportion to the extent of tissue damage, or lasting longer than the typical post-surgical healing time
-usually a constant burning crampiness
-hypesthesia (“does a pinch feel like terrible burning?”)
-allodynia (“do nonpainful things give you pain?”)
-hyperpathia (sensation even when stimulus stops- vibrio testing)
-vasomotor changes: skin color, temp, edema and hair or nail changes (one side hot and swollen and red, the other side looks normal; nail changes on one side but not the other; swollen distal extremties in a person who doesn’t normally have peripheral edema)
-sudomotor changes: one side doesn’t sweat like the other (“does one sock get wet and the other doesn’t?”)
-usually unilateral (asymmetric)
-usually distal
-pain may be diffuse or dermatomal
-used to be split into 3 stages, but now more thought of as acute (hot, red, swollen skin) vs. chronic (cold, dusky, shiny skin)

10

CRPS dx

-Budapest criteria
-pain out of proportion to injury extent + at least 1 of 4 (sensory, vasomotor, sudomotor, motor symptoms) + provider must find at least 2 of 4 (above)
-sensitivity = 98% (almost no FalseNegatives)
-specificity = 36% (64% FalsePositives)

11

CRPS Tx

-PT (as early and as aggressive as you can)
-active ROM ASAP, desensitization techniques… -neuropathic pain meds: anticonvulsants > tricyclic antidepressants, neurontin or lyrica ASAP, then cymbalta if no help, consider desipramine, NSAIDs
-consider tramadol short term (maybe for PT only), vasodilators (prazosin or clonidine patch), muscle relaxers (especially tizanidine), DMSO cream or EMLA cream or lidoderm/lidogel
-early referral to pain specialist, physiatrist or neurologist, especially for sympathetic nerve blocks

12

CRPS work-up

-no labs necessary
-XR not terribly helpful
-will show osteopenia, but late in dz
-Triple Phase Bone Scan (TPBS)
-Positive 3rd phase periarticular uptake
-Positive 1st and 2nd phase asymmetric uptake