6.MND and management of Motor neuron disease.ppt

education

1. Amyotrophic Lateral Sclerosis
Dr.Sayeed Uddin Helal

2. Motor Neuron Disease
Terminology
Lower motor neuron Upper motor neuron
 
 
Progressive
Muscular
Atrophy
Amyotrophic
Lateral
Sclerosis
Primary
Lateral
Sclerosis

3. Amyotrophic Lateral Sclerosis
Pathology
Degeneration and death of motor nerves
•Upper Motor Neuron
–within brain/spinal cord
•Lower Motor Neurons
–leaves brain (stem)/spinal cord
Relatively spared
•Eye movements and bowel/bladder function

4. Amyotrophic Lateral Sclerosis
Epidemiology
Etiology – unknown
Average age of onset mid-50’s
Mode of transmission
•Sporadic – 90-95%
•Familial – 5-10% (autosomal
dominant)

5. Amyotrophic Lateral Sclerosis
Epidemiology
Male : Female – 3:2
U.S. Prevalence: 30,000
Incidence 1-2.5 / 100,000
Isolated areas of increased incidence
•Kii peninsula of Japan
•Chamorro natives of Guam

6. Amyotrophic Lateral Sclerosis
Clinical Presentation
Lower motor neuron signs
•Weakness, muscle wasting, hyporeflexia,
muscle cramps, fasciculations
Upper motor neuron signs
•Spasticity, hyperreflexia, weakness

7. Amyotrophic Lateral Sclerosis
Clinical Presentation
Asymmetric Weakness – most common
Onset single limb or bulbar
Local spread then regional spread
•Bulbar, cervical, thoracic, lumbosacral
Fasciculations

8. Amyotrophic Lateral Sclerosis
Diagnosis
Prominent upper and lower neuron signs
with a progressive course without
significant sensory or sphincter
abnormalities
Laboratory investigation to search for a
more treatable condition

9. Amyotrophic Lateral Sclerosis
Clinical Signs and Symptoms
Weakness
Hyporeflexia
Pain and cramps
Fasciculations
Wasting
Spasticity
Hyperreflexia
Babinski’s sign
Emotional Lability

10. Amyotrophic Lateral Sclerosis
Atypical Features
Dementia - < 5 %
Sensory loss – atypical
25% complain of paresthesias
Oculomotor dysfunction
Bowel or bladder dysfunction

11. Amyotrophic Lateral Sclerosis

Diagnosis
Two experienced Neurologists
Laboratory Studies
No study to prove or disprove
Look for an alternate diagnosis

12. Amyotrophic Lateral Sclerosis
Laboratory Studies
Nerve conduction studies
•assess for demyelinating vs. axonal
involvement
Electromyography
•confirm ALS
•myopathy

13. Amyotrophic Lateral Sclerosis
Laboratory Studies
MRI cervical spine
•Cervical Spondylosis with cord compression
•Herniated disc
•Syrinx

14. Amyotrophic Lateral Sclerosis
Laboratory Studies
ESR – inflammatory/malignancy
SPEP – monoclonal gammopathy
TSH – hyperthyroidism
B
12
– combined systems degeneration
Calcium/PTH - hyperparathyroidism

15. Amyotrophic Lateral Sclerosis
Prognosis
Variable – difficult to predict in an
individual patient
50% live 3-4 or more years
20% live 5 or more years
10% live 10 or more years
Occasional patients live 20 years

16. Amyotrophic Lateral Sclerosis
Treatment
Rilutek
2 large clinical trials
•Bulbar onset
•Entire population
Endpoint
•Death
•Ventilator dependence

17. Amyotrophic Lateral Sclerosis
Treatment
Bulbar onset
•Prolonged survival
•Improved muscle strength
Entire population
•Prolonged survival
•No effect on decline in muscle strength
Prolonged survival an average of 2-3
months

18. Amyotrophic Lateral Sclerosis
Rilutek 50 mg po bid
Hepatotoxicity
•Serum transaminase levels
•Check every month x 3
•Then every 3 months x 3 for the first year
Adverse effects
•Neutropenia
•Nausea/vomiting

19. Amyotrophic Lateral Sclerosis
Rilutek 50 mg po bid
Reasons for not taking the drug
•Expense
•Minimal benefit
•Unwillingness to take a medication that
would prolong life

20. Amyotrophic Lateral Sclerosis
Management
weakness
fatigue
nutrition
dysphagia
feeding tube
dysarthria
communication
spasticity
cramps
pain
depression
anxiety
breathing
end-of -life

21. Amyotrophic Lateral Sclerosis
Multidisciplinary Approach to Care
Neurologist
Clinical/research nurse
Dietician
Speech/swallowing
therapist
Family/caregivers
Psychologists
Physical therapist
Occupational therapist
Social worker
GI physician
Support organizations
Homehealth/hospice
Pulmonologist