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IMAGINE (a film about ALS) Imagine Living With ALS ALS Informational Movie Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis (ALS)

ALS is a progressive, always fatal neuromuscular disease that begins with muscle weakness and as it progresses, results in total paralysis and the inability to speak and swallow. Sadly, the mind and senses remain intact.

ALS (Amyotrophic Lateral Sclerosis ) Prognosis: Phase Angle a Prognostic Factor for survival Clinical Advances in Adult Stem Cell Therapy - Dr. Jorge Paz Rodriguez (Miami) A.L.S./Lou Gehrig's Disease: A Family's Story Often Awesome The Series: An ALS Love Story - Episode 32


ALS Clinical Syndromes

* Primary lateral sclerosis (or UMN-onset ALS): Only the UMN degenerate, leaving the LMN Intact. The limbs become stiff and spastic without the development of significant weakness or atrophy. Those who have initial pure UMN findings later develop features of classic ALS.

* Progressive muscular atrophy (or LMN-onset ALS): Only the LMN are affected, leading to severe wasting and weakness in the muscles with loss of reflexes.

* Progressive bulbar palsy: The disorder starts in the muscles of speech and swallowing, causing slurred speech and choking. Patients eventually lose all ability to speak and swallow, and most later develop classic ALS.



At the present time, there are about 30,000 people living with this disease in the United States alone. Each day, an additional 15 people are diagnosed. Estimated 5000 people in the United States are diagnosed with the disease each year.

Often Awesome The Series: An ALS Love Story - Episode 33 Often Awesome The Series: An ALS Love Story - Episode 28 Lou Gehrig's Disease (ALS): Spotlight on Stem Cell Research - Introduction Lou Gehrig's Disease (ALS): UCSD Team's Stem Cell Therapy Approach CIRMTV


Mode of Acquisition

· Sporadic ALS: 90% of ALS cases; arises spontaneously when motor neurons degenerate and die prematurely of unknown causes.

· Familial ALS: 5% to 10% of ALS cases; may be inherited as a dominant or recessive disease. In dominantly inherited ALS, such as occurs with the SOD1 mutation, each child of an affected parent has a 50% chance of inheriting the mutation and developing the disease.

Revised El Escorial Research Diagnostic Criteria (World Federation of Neurology)

  • Clinically Definite ALS: UMN signs and LMN signs in three regions
  • Clinically Probable ALS: UMN signs and LMN signs in two regions with at least some UMN signs rostral to (above) LMN signs
  • Clinically Probable ALS—Laboratory-supported: UMN signs in one or more regions and LMN signs defined by EMG in at least two regions
  • Clinically Possible ALS:
  • UMN signs and LMN signs in one region (together) or
  • UMN signs in two or more regions
  • UMN and LMN signs in two regions with no UMN signs rostral to (above) LMN signs

UMN=upper motor neurons (UMN signs: clonus, Babinski sign, absent abdominal skin reflexes, hypertonia, loss of dexterity), LMN=lower motor neurons (LMN signs: atrophy, weakness), regions=bulbar, cervical, thoracic lumbosacral, EMG=electromyography



ALS Untangled

Dr. Hickey believes that ALS and a number of other decease, including primary lateral sclerosis, myelodysplastic syndrome, renal failure, multiple sclerosis, Parkinson's disease, and fibromyalgia, are caused by heavy metal poisoning. As a result, patients who present with these varied conditions receive similar treatments in his center.



Cough- assist ( MIE) mechanical inhale and exhale helps ALS weakened muscles due to degeneration of motor neurons to remove secretions and clear lungs.

* Place tubing on machine and check settings.

* Inhale time - 2;     Exhale - 1.5;         Pause - 2

* Check pressures on machine

A.  Occlude open end

B.   Turn machine on

C.    Check pressures and adjust as needed

*   Inhale -  30;         Exhale  +  40

*   Place on patient's trach and turn on machine

*   Perform 5 cycles 30/40 and stop

*   Allow recovery time ( ask patient if ready);   Repeat times 5

*   Assess for clear lungs. Continue if not clear

*   For secretion clearance: no more than 5 cycles at a time until clear

To deflate cuff ( trach balloon) and capture secretions above the trach

*   Change settings of MIE to: Inhale -  5;  Exhale -  0;  Pause -  5

*   Use 10 ml syringe to deflate cuff, after 2 seconds, inflate the cuff and turn of the machine, remove tubing off patient and suction trach and mouth.

*  Use PMV ( Posse Muir Valve ) as speaking valve after deflating the cuff.


Helpful Links,  ALS:

ALS in the U.S.A. — the Stats and More
An Eye on Clinical Trials
We're Seeing the Light
Worming a Way In to Understand ALS
ALS Drug: Where We Stand With Ceftriaxone…and More
Models of the Stem Cell Kind
New target molecule could advance the cause of healing in ALS
ALS CLASSROOM: ALS and the Brain
New ALS Human Cell Cultures Underway

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