NEURO-Amyotrophic Lateral Sclerosis (ALS)

What is the median survival rate for someone with ALS? Is the progression of ALS painful or painless?

3-5 years Painless

What is the most common form of progressive motor neuron disease? What is the most common acquired motor neuron disease?

ALS

What is ALS?

Amyotrophy- Loss of cortical motor neurons, thinning of corticospinal tracts Lateral sclerosis- Loss of fibers in te lateral columns resulting in fibrillary gliosis

What is the prognosis for patients with ALS? What is the average total time between symptoms and death in ALS?

Average 5 year survival is 25% 224 months

Diagnosis is qualified into these criteria: Definite Probable Possible Explain.

Definite is when 3/4 areas are involved from bulbo, cervical, thoracic, lumbosacral Probable is when 2/4 are involved Possible is when 1/4 is involved

How does the patient present?

Development of cramping with movement in the morning Spontaneous twitching Hand extensor weakness

What other tool for Dx can we use for ALS?

EMG

What features are spared in ALS? (4)

Extraoccular eye movements Mentation Bowel and Bladder function Sensation!!!!!

ALS is a descending disease that starts in the upper motor neurons and descends- meaning what would be initial findings on physical exam? What would be seen with lower motor neuron involvement? In extreme cases, bulbar invovlement may also occur...what tells us this on exam?

Initial findings would include +babinski and hyperreflexia FASCICULATIONS Hyper gag reflex, sometimes respiratory issues

What is flail arm syndrome? What is ALS-plus syndrome What is progressive bulbar palsy?

LMN weakness, distal and asymmetric UMN/LMN with frontotemporal dementia Progressive UMN and LMN cranial muscles

What is peripheral motor neurons affected WITHOUT corticospinal motor involvement called?

Lower motor neuron disorder

What are the epidemiologic risk indings? What are genetic predisposing factors?

Pesticides and smoking Gene problems with SOD1

A patient with ALS will have what findings on exam?

Ptosis Clonis Muscle atrophy

What treatment can be used for moderate extension of survival for ALS patients? What else can be done for these patients?

Riluzole AFO brace, finger extension splits, respiratory support