Amyotrophic Lateral Sclerosis ALS

Rilutek is expensive

$600-700 for a 30 day supply, but most insurance covers it

Diagnosis of ALS

-History -EMG -MRI and spinal fluid analysis -muscle biopsy -PFTs

impaired sleep treatment

-Nocturnal noninvasive positive-pressure ventilation (NIPPV) -Hospital bed -Mirtazapine -Melatonin

Novel therapies

-ceftriaxone - may possess antiexitotoxic properties (phase III trial) -stem cell therapies -electrical diaphragmatic pacemaker

Fatigue treatment

-personal plan of care -PT/OT to home for modifications -exercise and mobility program -special equipment

___ % live longer than 10 years

10

ALS hereditary

10% FALS (familial ALS)

___ % survive 5 years

20

Around 60% of ALS patient die within

24 hours of deterioration

% of ALS clients that die within ____ months of diagnosis

50 18

Rilutek Dosage:

50 mg po every 12 hours

Palliative care and Hospice

All ALS care is currently palliative

ALS - connection between muscle and

CNS is blocked muscle begins to atrophy

Amyotrophic Lateral Sclerosis ALS also known as

Charcot's disease Lou Gehrig's disease

done to diagnose abnormal electrical activity of involved skeletal muscles

Electromyography (EMG)

Early symptoms of ALS

Fasciculations (twitching) of the face Dysarthria (slurred speech) (80% of pts) Dysphagia (difficulty swallowing) Limb weakness ( 65%)

Who gets ALS

all races Men 40-70 yrs of age (avg 55-65 yrs)

Light-tech device:

alphabet boards, individual picture communication charts, picture symbols, telephone communication systems

treatment for cognitive decline and depression

amitriptyline or SSRI depressants

Muscle biopsy demonstrate small,

angulated atrophic fibers

spasticity treatment

baclofen dantrolen sodium

Sialorrhoea: ( An excessive secretion of saliva)

caused by inability to swallow well, but patients will have increased saliva—50% patients; can lead to aspiration pneumonia

treatment for emotional liability

dextromethorphan hydrobromide quinidine sulphate

common progression -

difficulty walking,cane-walker-wchr lose ability to write, type, feed self difficulty speaking/swallowing -----may need feeding tube---- decrease in lung capacity (<50%=vent) progression can be 6 yrs or many yrs

constipation treatment

docusate - lactulose increased fluid/dietary fiber enema

In familial ALS, the disease is autosomal ______: if a parent has ALS the offspring have______ a chance of inheriting the gene, not all people with ALS gene will develop the disease.

dominant 50%

• Symptoms of ____ may not be evident until about 80% of the motor neurons are lost • Defective articulation • Slow laborious speech • Imprecise consonant production • Marked hypernasality

dysarthria

Rilutek should be taken on an

empty stomach same time each day

ALS causes (unknown) or

excess of a neurotransmitter called glutamate builds up in the synapse causing eventual nerve cell death and subsequent muscle atrophy of the muscle attached to that nerve -or autoimmune problem

MRI of brain and cervical region done to

exclude other conditions

Rilutek common S/E

fatigue somnolence N/D dizzy May cause increased liver enzymes

Cognitive symptoms in ALS are usually due to

frontotemporal impairment and include personality change, irritability, obsessions, poor insight and impairment in language

Depression & anxiety are more common in patients who are at___________________ (about half of caregivers deal with anxiety). However recent study showed severe depression in <20% of patients with ALS as compared to 40-50% of patients with other neurodegenerative motor conditions such as MS, Parkinson's and Huntington's however 50% of patients with ALS struggled with some depression

high age at onset of the disease

Other "mimicking" disorders include:

hyperthyroidism, hypoglycemia, compression of the spinal cord, toxic agents, infections.

Amyotrophic Lateral Sclerosis ALS is an

incurable fatal neuromuscular disease characterized by progressive muscle weakness, resulting in paralysis. It is characterized by rapid deterioration and the selective death of motor neurons in the cerebral cortex, brainstem, and spinal cord

Motor neurons, which control the movement of voluntary muscles, deteriorate and eventually die. When the motor neurons die, the brain can no longer

initiate and control muscle movement. Because muscles no longer receive the messages they need in order to function, they gradually weaken and deteriorate.

ALS patients retain full

intellectual capacity and intelligence However, approximately 15% will develop dementia

Altered communication

light-tech device electronic high-tech device

Urinary urgency can occur especially with those with

lower extremity spasticity

pain for ALS palliative/hospice care

morphine fentanyl

PFTs(Pulmonary function tests): reveal

motor strength deficits such as decreased vital capacity

Early stages of the disease involve surviving motor neurons "sprouting"

new branches (similar to cardiac collateral flow!!) to reinnervate affected muscle fibers in an attempt to preserve muscle strength. However, as the disease progresses the reinnervation fails.

ethical issues involving passing disease

onto children. May not even know you have it til after family is started

Spinal fluid may be done to rule out

other causes

urinary urgency treatment

oxybutynin voiding schedule superpubic catheter

Electronic high-tech devices:

portable amplifiers that increase the patients volume, digital recorders that playback prerecorded words and phrases on command, dedicated voice synthesizers. Speech synthesis software if available for use in laptops. Brain-computer interface communication devices for individuals with advanced ALS .

Pneumovax immunization and yearly influenza immunization help reduce

pulmonary infections

Noisy breathing: atropine helps control

rattily breathing with those who have reduced ability to cough (morphine also given)

most common cause of death ALS

respiratory failure or cardiac arrhythmias due to insufficient oxygenation

impaired sleep maybe from

respiratory insufficiency, difficulty repositioning in bed, pain, anxiety and depression

Riluzole (Rilutek): antiglutamate: 1st drug developed to treat ALS

slows progression of disease by inhibiting release of GLUTAMIC acid in the CNS and protects the neurons

Environmental factors contributing to ALS

smoking exposure to agricultural chemicals and solvents

pain treatment

stretching and ROM exercises support hose NSAIDS and opioids Vitamin E and C (may reduce cramping)

treatment for Sialorrhoea

transdermal scopolamine injections of botulinum toxin

symptoms of ALS

tripping, stumbling, falling lose muscle control/strength n hands/arms difficulty breathing chronic fatigue muscle twitching/cramping

Emotional liability:

uncontrolled laughter or crying, often with minimal provocation and maybe inappropriate to the context of situation

_____ dependent ALS clients may live for many years

ventilator

Respiratory infection (pneumonia) increases as

weakened diaphragm and chest muscles make it difficult to clear lung secretions

Diagnosis typically takes________ process of eliminating other disorders

weeks or months,

________ in about 65% of patients: foot drop, difficulty walking , loss of hand dexterity or shoulder weakness

• Limb weakness