Motor Neuron Disease

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Motor neuron diseases:
 Progressive degeneration and loss of motor neurons in the spinal cord with or without similar lesions in the motor nuclei of the brainstem or the motor cortex
Motor neuron disease = Amyotrophic lateral sclerosis
Spinal muscular atrophy:
 Only lower motor neuron signs

Amyotrophic lateral sclerosis (ALS)
Also known as Lou Gehrig’s disease
Degeneration of UMN and LMN
Accuracy of clinical diagnosis ~ 95%

Clinical Manifestations
Weakness : legs, hands, proximal arms, oropharynx
Often hands are affected first, usually asymmetrically
Muscle cramps (hypersensitivity of denervated muscle)
Weight loss
Respiration is usually affected late
Sensation is not clinically affected
Bladder function is spared 
Combination of overactive reflexes with Hoffmann signs in arms with weak, wasted, and fasciculating muscles is virtually pathognomonic of ALS
Course is relentless and progressive without remissions, relapses, or even stable plateaus
Death from respiratory failure, aspiration pneumonitis, or pulmonary embolism
Mean duration of symptoms is about 4 years (27-43 months) ; 20% live > 5 years

Clinical Classification:
Primary Lateral Sclerosis
 Purely UMN
Pseudobulbar Palsy
 Bilateral involvement of corticobulbar pathways
 Pseudobulbar affect
Progressive bulbar palsy
 Prominent dysarthria and dysphagia (LMN)
Progressive Spinal Muscular atrophy
 LMN in limbs; anterior horn cell degeneration

No effective drug therapy
Riluzole (Rilutek)
 Glutamate inhibitor
 Prolongs life by 3 to 6 months
 No visible effect on function or quality of life
Symptomatic (sialorrhea, dysphagia, tracheostomy)
Emotional support
Immunosuppressive therapy
 Only for lymphoproliferatlve disease, monoclonal gammopathy, conduction block or high titers of antibodies to GM, or MAG

Chest Trauma

Posted by e-Medical PPT

Injuries of chestSimple/Closed Pneumothorax
Opening in lung tissue that leaks air into chest cavity
Blunt trauma is main cause
May be spontaneous
Usually self correcting

Treatment for Simple/Closed Pneumothorax
ABC’s with C-spine control
Airway Assistance as needed
If not contraindicated transport in semi-sitting position
Provide supportive care
Contact Hospital and/or ALS unit as soon as possible

Open Pneumothorax
Opening in chest cavity that allows air to enter pleural cavity
Causes the lung to collapse due to increased pressure in pleural cavity
Can be life threatening and can deteriorate rapidly

Symptoms/Signs of Open Pneumothorax
Sudden sharp pain
Subcutaneous Emphysema
Decreased lung sounds on affected side
Red Bubbles on Exhalation from wound( a.k.a. Sucking chest wound)

Subcutaneous Emphysema
Air collects in subcutaneous fat from pressure of air in pleural cavity
Feels like rice crispies or bubble wrap
Can be seen from neck to groin area

Treatment for Open Pneumothorax
ABC’s with c-spine control as indicated
High Flow oxygen
Listen for decreased breath sounds on affected side
Apply occlusive dressing to wound
Notify Hospital and ALS unit as soon as possible

Tension Pneumothorax
Air builds in pleural space with no where for the air to escape
Results in collapse of lung on affected side that results in pressure on mediastium,the other lung, and great vessels

Needle Decompression
Locate 2-3 Intercostal space midclavicular line
Cleanse area using aseptic technique
Insert catheter ( 14g or larger) at least 3” in length over the top of the 3rd rib( nerve, artery, vein lie along bottom of rib)
Remove Stylette and listen for rush of air
Place Flutter valve over catheter
Reassess for Improvement

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