1. Amyotrophic Lateral Sclerosis (ALS)
Jillian La Monte FNP-S

2. What is ALS?
Amyotrophic lateral sclerosis (ALS), often referred to as "Lou Gehrig's Disease,“ it is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord.
ALS is a progressive degenerative disease that affects the upper and lower motor neurons (UMN and LMN). It is currently incurable and eventually leads to death.

3. ALS

4. Pathophysiology
Degeneration of the UMN and LMN with their respective axons and with gliosis replacing lost neurons. Leads to spinal cord atrophy, thin ventral roots and loss of large myelinated fibers in motor nerves.

5. Etiology
Sporadic: cause is unknown, but elevated levels of glutamate have been found in serum and CSF.
Familial ALS: a genetically transmitted degenerative disease.
Guam ALS and Parkinson-dementia complex: possible relationship to ingestion of the cycad nut or to some other environmental toxin.

6. Epidemiology
Estimated prevalence rates range between 2.7 and 7.4 per 100,000.
Predominant age: Uncommon before age 40. Typical age is years of age.
Predominant sex: Male>Female in sporadic ALS:
After age 70: Male=Female

7. Prognosis ALS usually results in death within 5 years.
Patients who predominantly manifest progressive muscular atrophy have a better prognosis.
There are have been reports of spontaneous arrest of the disease.

8. Risk Factors
Heredity: 5 to 10 percent of the people with ALS inherited it (familial ALS). In most people with familial ALS, their children have a chance of developing the disease.
Age: ALS most commonly occurs in people between the ages of 40 and 60.
Sex: Before the age of 65, slightly more men than women develop ALS. This sex difference disappears after age 70.

9. Environmental Factors
Smoking
Lead exposure
Military service
Smoking cigarettes appears to increase a person's risk of ALS to almost twice that of a nonsmoker. The more years spent smoking, the greater the risk. However, quitting smoking can eventually lower the increased risk.
Some evidence suggests that exposure to lead in the workplace may be associated with the development of ALS.
Recent studies indicate that people who have served in the military are at higher risk of ALS. Exactly what about military service may trigger the development of ALS is uncertain, but it may include exposure to certain metals or chemicals, traumatic injuries, viral infections and intense exertion.

10. Clinical Findings Unexplained weight loss
Limb weakness with variable symmetry and distribution
Gait disorder (steppage-waddling)
Slurring of speech
Inability to control affect (inappropriate laughing, crying, yawning)
Focal atrophy of muscle groups (initially in a myotomal distribution)
Fasciculation's (other than calves)
Hyperflexia
Babinski sign, present in 50% of patients
Spasticity
Sialorrhea
Spares cognitive, oculomotor, sensory, and autonomic functions

11. Differential Diagnosis
Multifocal motor neuropathy
Cervical radiculomyelopathy
Cervical spondylosis
Lead intoxication
Spinal muscular atrophy (adult form)
Familial spastic paraparesis
Lyme disease
Spinal multiple sclerosis
Tropical spastic paraparesis
Myasthenia gravis

12. Diagnostic Test
Lab
No simple reliable laboratory test is available that confirms the diagnosis.
Creatinine kinase levels may be elevated.
Elevated levels of glutamate in CSF and serum
Antimonosialoganglioside autoantibodies in low titer commonly found (of unclear significance)
Possibly reduced levels of nerve growth factor.

13. Diagnostics
Electro diagnostic tests including electromyography (EMG) and nerve conduction velocity (NCV)
Blood and urine studies including high resolution serum protein electrophoresis, thyroid and parathyroid hormone levels and 24-hour urine collection for heavy metals
Spinal tap
X-rays, including magnetic resonance imaging (MRI)
Myelogram of cervical spine
Muscle and/or nerve biopsy
Neurological examination
MRI are typically done with the patient because it needs to rule out any other diagnosis.

14. Treatment
Riluzole 50mg PO BID- decreases the release of glutamate and it slows the disease progression.
Riluzole withhold should be considered for patients developing fatigue. Monitor LFTs for hepatic dysfunction and CBC for neutropenia.
The only FDA approved drug for ALS. It produces a slight prolongation in life expectancy.

15. Additional Treatment
Baclofen 5mg PO TID, initially, followed by gradual increase 5mg/d every 4-7 days; not to exceed 80mg/d divided QID.
Tizanidine 4-8mg PO q8h PRN; not to exceed 36mg/d.
These medications will be used to relieve sever spasticity.

16. Non-pharmacological Treatment
Breathing care
Physical Therapy
Occupational Therapy
Speech Therapy
Nutritional Support
Psychological & Social Support

17. Complications Aspiration Pneumonia Pulmonary Embolism
Nutritional Deficiency
Complications from wheelchair-bound or bedridden states, including decubitus ulcer and skin infections.

18. Follow-up
Initially every 3 months; frequency to be increased as needed for symptomatic therapy.
Patients with a presumed diagnosis of ALS should have neuroimaging and electrodiagnostic studies.

19. Education The Muscular Dystrophy Association The ALS Association
Families of Spinal Muscular Atrophy

20. Referral Neurologist Surgeon Gastroenterologist Pulmonologist
Respiratory Therapist
Multidisciplinary referrals are needed in order to optimize health care delievery, prolong the survival and enhance quality of life
Neurologist needed for early diagnosis
You may need a trach or g-tube placed
Pulmonologist needed for the management of the ventilator.

21. Question #1
What medication is the only FDA approved medication for ALS?
a. Riluzole
b. Baclofen
c. Azithromycin
d. Tizanidine

22. Answer #1
Riluzole; 50mg PO BID is the only FDA-approved drug for ALS. It produces a slight prolongation in life expectancy by decreasing the release of glutamate and it slows the disease progression.

23. Question #2
2. Average survival rates for patients with ALS? a. 1-2 years b. 3-5 years c. 6-7 years d. >10 years

24. Answer # 2
2. B. 3-5 years, is the average survival rate with 10% patients surviving greater than 10 years.

25. Question #3
3. Early symptoms of ALS include all of the following expect? a. Difficultly walking, tripping or difficultly doing your normal daily activities. b. Slurring of speech or trouble swallowing. c. Muscle cramps and twitching in your arms, shoulders and tongue. d. Tachycardia

26. Answer # 3
3. D. tachycardia; The disease frequently begins in your hands, feet or limbs and then spreads to other parts of your body. As the disease advances, your muscles become progressively weaker. This weakness eventually affects chewing, swallowing, speaking and breathing.

27. Question #4
4. Besides medications additional forms or treatment for ALS include; select all that apply. a. Breathing care b. Physical Therapy c. Occupational Therapy d. Speech Therapy e. Nutritional Support f. Psychological & Social Support

28. Answer # 4
4. All are correct forms of therapy. For the patient suffering from ALS.

29. Question #5
5. What is one of the primary ways of diagnosing ALS? a. EMG b. MRI c. X-ray d. Signs & Symptoms

30. Answer #5
5. D. Signs and Symptoms; ALS is primarily diagnosed through signs and symptoms, the physician observes in the patient and a series of tests to rule out other diseases.

31. Question #6
6. At what age does ALS commonly occur? a b c d. 70 or older

32. Answer #6
6. C. At years of age is the most commonly age in which ALS occurs.

33. Question #7
7. Risk factors for a patient with ALS including all of the following expect? a. Smoking b. Family History c. Age >40 d. Exercise

34. Answer #7
7. D. Exercise, the following family history, age of older then 40, smoking, and sex have all been linked to risk factors for developing ALS.

35. Question #8
8. Although there is no definite laboratory study for diagnosis ALS which lab work will show an elevation? a. BUN and creatinine levels b. Creatinine Kinase c. WBC d. TSH levels

36. Answer #8
8. B. Creatinine kinase levels, may be elevated in patients with ALS.

37. Question #9
9. ALS is a progressive degenerative disease that affects the? a. upper neurons b. lower neurons c. upper and lower neurons d. none of the above.

38. Answer #9
9. ALS is progressive disease that affects the upper and lower neurons. This disease is currently incurable and eventually leads to death.

39. Question #10
10. Multidisciplinary care may be needed for patients with ALS, referrals can be made to all of the following expect? a. Neurologist b. Hematologist c. Pulmonologist d. Surgeon

40. Answer #10
10. B. Hematologist, referrals will be made throughout the span of ALS patients, these include neurologist, which will help with an early diagnosis. Gastroenterologist and surgeon for placements of tracheostomy. and/or G-tube. Patient will be referred to a pulmonologist as well for management of tracheostomy.

41. Reference
Domino, F. (2014). The 5-minute clinical consult premium. Philadelphia: Lippincott, Williams & Wilkins.
Mayo Clinic Staff. (2014, April 09). Disease and conditions amyotrophic lateral sclerosis. Retrieved from
National Institute of Neurological Disorders and Stroke. (2015, March 05). Amyotrophic lateral sclerosis (als) fact sheet. Retrieved from
The ALS Association. (2014). About als. Retrieved from