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37 Cards in this Set

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ALS
lou gehrig's disease
progressive neurodegen w/ UMN and LMN loss
which rootlets have more degen?
ant rootlets are grossly smaller than post roots
epidemiology
b/w ages 40-60
20,000 americans w/ ALS
1-3/100,000 worldwide
men more than women
more whites- but not race rel
gene defect in Familial ALS
Cu/Zn SOD1 gene
etiological theories
selective MN damage from a bunch of injuries including:
free rad, excitotoxins, odixative stress, neurofil dysfxn, mito dysfxn, proinflam cytokines
CNS plasma levels in ALS patients
inc glutamate
dec glutamate in cerebral/SC tissues
variable loss of transporter


type of mutation in Cu/Zn SOD1 gene
Glial Glutamate Trasporter Protein in motor cortex and SC

multiple mutations
mutation in Cu/Zn SOD1 causes
in theory


what does it result in?
1. reduced superoxide anion radical detoxification
2. accumulation of superoxide anions
*Toxic Gain of Function
Immune Hypothesis


which Ab?
immune complexes found in Kidneys and S.I.
Abs against MN populations
10% have monoclonal Abs- IgM
Spinal cord has...(immune)
1. Inflammatory cell infiltrates
2. macrophages w/ MHC-II
3. elevated IL-6 levels
Toxic Gain of Fxn affects which receptor?
Androgen Receptor Protein
defects in DNA repair
impaired mito fxn
UMN signs

LMN signs
disruption of CST and CBT
preferentially fast acting CST
in ant horns of SC
prox m more ventral
distal m more dorsal
Common Symptoms
1. general weakness
2. bulbar symptoms- chewing, swallowing
3. Hand atrophy
4. foot drop
5. leg spasticity
6. Fasiculations
7. difficulty w/ fine motor mvmts
where does the weakness appear first?
in the distal limbs

cramping, aching, and twitching seen first
LMN dysfxn
1. weakness
2. atrophy
3. fasiculations
4. Hyporeflexia
5. cramps
UMN dysfxn
1. muscle weakness
2. stiffness-spasticity
3. Hyperreflexes
Bulbar Symptoms
difficulty swallowing and chewing
respiratory weakness
Sialorrhea-excessive drooling
Diagnosing ALS depends heavily on
1. History
2. Physical Examination
General physical exam presentations
1. diaphragmatic and intercostal weakness
2. out of breath
3. cyanotic
4. paradoxical abdominal movements
5. accessory muscles in resp
Neurological exam



Pseudobulbar effect
1. normal mentation
2. Cranial nerve involvement
3. tongue scalloping/atrophy
4. hand atrophy
inappropriate laughing or crying
corticobulbar-limbic system
where does the hand atrophy begin?
at the dorsal interosseus muscle
scalloped loss
if creatine levels are over 1000 then it is....

nerve conduction studies show
NOT ALS


sensory nerve conduction is NORMAL
EMG measurement of denervation should be
widespread
need to see 3 limbs, 3 muscles supplied by dfft nerve roots for conclusive diagnosis
muscle biopsy used to

find..
exclude other disorders that mimic ALS
1. Grouped Atrophy
2. Angulated Fibers- single m cells atrophic in relat to other normal m cells
MRI

Lumbar punctures

resp fxn
cervical and lumbar SC

rarely needed

evaulated
many diseases have similar symptoms to ALS
the next 11 or so
Cervical Spondylosis
multifocal motor neuropathy
hyperthyrodism
HIV
Heavy metal intox
Lymphoma
tandem cervical/lumbar spine les
very similar to ALS. NO bulbar
similiar symptoms
always test for it w/ symptoms
from fillings
excluded thru MRI or lumbar punct
Benign fasiculations


Fasiculations w/o weakness is...
1/3rd of presenting patients come with this out of concern

NOT a cause for concern
Treatment
muscle spasms
aspirations
baclofen or tizanidine
teach safe swallowing, PEG
-want FVC>50%
Excessive Salivation-Sialorrhea
use anticholinergics
sal-tropine/atropine
suction
botox to parotids
increased viscosity of saliva


repiratory difficulties
treat w/ hydration
meat tenderizer

BiPAP(non-invasive), tracheostomy
Pseudobulbar symptoms
SSRIs, amantidine
Riluzole (Rilutek)
glutamate antagonist that prolongs survival for Bulbar ALS

only FDA approved med for ALS
Vitamin Regimens

Cox-2 inhibitors

Creatine
Vit C, E, Beta-carotene

NOT used anymore

some evidence, drink lots of water with it
Recomended Treatment Regimen
1. Riluzole 50mg
2. creatine 5g/day
3. co-enz Q10
4. antioxidant vit (C/E/beta c)
ALS variants
1. ALS w/ dementia
2. AD and AR familial ALS
3. AR juvenile ALS
4. Paraneoplastic MN disease
5. ALS-parkinsonism-dementia complex
Prognosis
w/ respiratory insufficiency they live about 3-4 years post diagnosis