Motor neuron diseases is the heterogenous syndrome resulting from dysfunction of upper motor neurons in the precentral gyrus of the frontal lobe and/or lower motor neurons in the ventral horn of the spinal cord. This presentation is focusing on assessment and physiotherapy management in MND

1. PRESENTATION
TITLE
Mirjam Nilsson​​
PHYSIOTHERAPY IN
MOTOR NEURON DISEASE
Dr. Simran A. Mishra
(MPT – IInd year)
Under Guidance of : Dr. Suvarna Ganvir
Department of Neuro-Physiotherapy
DVVPF’s College of Physiotherapy,
Ahmednagar

2. Introduction
3
Classification
4-5
Risk Factors
6
Diagnosis
7
Guidelines
8-9
Assessment and outcome
10-11
Physiotherapy Management
12-32
Recent Advances
33-34
2

3. INTRODUCTION
Motor neuron diseases is the heterogenous
syndrome resulting from dysfunction of
upper motor neurons in the precentral
gyrus of the frontal lobe and/or lower
motor neurons in the ventral horn of the
spinal cord.
3
• The incidence is 2-4/100,000 per annum,
with onset typically in the sixth and
seventh decades.
• The male to female ratio is 2:1
• Male sex, increasing age and hereditary
disposition are the main risk factors
• Life expectancy is typically 2–5 years
after symptom onset
Global, regional, and national burden of motor neuron diseases 1990–2016: a systematic analysis for the Global
Burden of Disease Study 2016 GBD 2016 Motor Neuron Disease Collaborators

4. 4
DARCY UMPHRED’S NEUROLOGICAL REHABILITATION SIXTH EDITION

5. RISK FACTORS FOR ALS
5
Physical Rehabilitation, Susan Sullivan, 7th edition

6. CLINICAL MANIFESTATION
Physical Rehabilitation, Susan Sullivan, 7th edition
• BEHAVIOURAL CHANGES
• PAIN
• FATIGUE
• RESPIRATORY IMPAIRMENT
• TONAL CHANGES
• WEAKNESS
• DYSARTHRIA
• DYSPHAGIA

7. DIAGNOSIS
The Lancet Neurology; 15(11); Al-Chalabi A, Hardiman O, Kiernan MC, Chi.A, Rix-Brooks B, van den Berg LH. Amyotrophic
lateral sclerosis: moving towards a new classification system; 1182–1194;

8. •Recog
nition
&
referral
•Inform-
ation &
support
diagnos
is
•Cogniti
ve
assess
ments
•Prognos
tic
factors
•Organi
sation
of care
•Plannin
g for
end of
life
•Managi
ng
sympto
ms
NICE - GUIDELINES FOR THE ASSESSMENT &
MANAGEMENT OF MOTOR NEURON DISEASES
Guidelines for the management of motor neuron diseases, nice guideline published: 24 february 2016 last updated: 23 july
2019 , www.nice.org.uk/guidance/ng42
• Equipment and adaptations to aid activities of daily living and mobility
• Nutrition and gastrostomy
• Communication
• Respiratory function and respiratory symptoms
• Cough effectiveness
• Non-invasive ventilation

9. GUIDELINES FOR THE PHYSIOTHERAPY MANAGEMENT OF
MOTOR NEURON DISEASES
9
MND
Assessment
& Outcome
measurement
Effective
communicati
on
Goal setting
Treatment
Education
of Patients
& Carers
Advocacy
& Transfer
of Care
• General Recommendations /
Advice
• Maintaining Mobility and Function
• Prevention of complications
• Exercise prescription
• Respiratory Symptom
Management
• Pain Management
• Fatigue Management
• Spasticity management
• Falls prevention
• Provision of assistive devices
• Ergonomic assessment
GUIDELINES FOR THE PHYSIOTHERAPY MANAGEMENT OF MOTOR
NEURON DISEASE (MND) September 2014 1 ST EDITION MND

10. 20XX Presentation title 10
ASSESSMENT & OUTCOME MEASUREMENT
Subjectiv
e

11. Presentation title
11
ASSESSMENT & OUTCOME MEASUREMENT
Objectiv
e

12. Ashworth, N.L., Satkunam, L.E., Deforge, D. (2011) Treatment in amyotrophic lateral sclerosis / motor neuron disease (Review).
The Cochrane Collaboration. http://www.thecochranelibrary.com.
12
Goals should involve the patient and carer, be focused at the
level of activity / participation, be timed with short term and
long term aims and occur as part of multidisciplinary team
planning.
GOAL SETTING
• Physiotherapy treatment should be functional, goal orientated and preformed at
intensities to avoid fatigue. The goal in the early stages of MND is to
maintain, and optimise, mobility and function.
• In the middle stage of MND the goal is to continue to maintain functional
mobility, manage pain and to carefully monitor and address any symptoms
of respiratory compromise.
• Late phase goals are designed to maximise quality of life for the patient.

13. Early stage :
• Behavioral changes
• Emotional lability (not related to
dementia).
• Speech and swallowing problems
• Weight loss and nutritional
deficiencies
• Information and support needs
13

14. PAIN MANAGEMENT Source of
Pain
Physical therapy for individuals with MND current insights, Vanina Dal Bello-Haas, Hamilton, Degenerative Neurological and
Neuromuscular Disease , e 2018:8 45–54

15. Chiò A, Mora G, Lauria G. Pain in amyotrophic lateral sclerosis. Lancet Neurology. 2018;16(2):144–157. 15

16. FATIGUE MANAGEMENT
" (Gibbons 2013a), defined it as "reversible motor weakness and whole-body tiredness that
was predominantly brought on by muscular exertion and was partially relieved by rest.
Gibbons C, Pagnini F, Friede T, Young CA. Treatment of fatigue in amyotrophic lateral sclerosis/motor neuron disease. Cochrane
Database of Systematic Reviews 2018, Issue 1. Art. No.: CD011005. DOI: 10.1002/14651858.CD011005.pub2.
Gibbons C, et al, 2018
To assess the effects of pharmacological and non-pharmacological interventions
for fatigue in ALS/MND.
All studies assessed fatigue using the Fatigue Severity Scale (FSS).
breathing exercises, exercises with weights, or magnetic brain stimulation on
fatigue in people with ALS/MND, as the evidence was significant but low
quality.

17. FATIGUE MANAGEMENT

18. Assessment and management of respiratory
function are critically important.
• FVC is the measure most commonly used
in patients with ALS, and is a predictor of
survival (Stambler et al., 1998).
• Maximum inspiratory pressure (MIP) and
sniff nasal inspiratory force (SNIF) are
used. (Morgan et al., 2005)
RESPIRATORY MANAGEMENT
Mandler RN, etal., 2010, Approximately 90.8 % of patients were considered to have died
peacefully. Of all deaths, 73.8% were attributed to respiratory causes.

19. • The treatment of choice for initial respiratory support is
NIPPV, using a bilevel intermittent positive pressure
device triggered by the patient’s respiratory efforts
• It has been shown to have many benefits in patients with
ALS when used for at least 4 conse. hours in each 24-
hour period.
Low-flow oxygen is not
recommended for patients
with ALS because it
increases arterial pCO2 and
diaphragm dysfunction in
patients with
neuromuscular disease
Rehabilitation of motor neuron disease, ZACHARY SIMMONS, Handbook of Clinical Neurology, Vol. 110 (3rd series) Neurological Rehabilitation
19
Aboussouan et al.,
2001)
(Gay and
Edmonds, 1995)
RESPIRATORY MANAGEMENT
Cough augmentation
Lung volume recruitment training
Airway clearance techniques
breath stacking, assisted
breath stacking, and
manually-assisted cough.
(Vanina Dal Bello-
Haas., 2018)

20. Exercise Program
• Maintain joint range of movement
• Prevent contractures
• Reduce stiffness and discomfort
• Optimize function and quality of life.
20XX 20
NICE
GUIDELINES., 2019
The evidence related to the benefits and risks of
exercise in PALS is limited. However, the view
that exercise, when prescribed appropriately, may
be physiologically and psychologically beneficial
for PALS, especially when implemented in the
earlier stages of the disease, is being increasing
adopted.
PAGANONI ET AL, &
PLOWMAN ET AL.
Physical therapy for individuals with amyotrophic lateral sclerosis: current insights, Vanina Dal Bello-Haas, Degenerative
Neurological and Neuromuscular Disease

21. 21
Evidence supporting physiotherapy in MND:Review of exercise studies
Guidelines for the physiotherapy management of motor neuron disease (mnd) september 2014 1 st edition mnd

22. 22
Evidence supporting physiotherapy in MND:Review of exercise studies
Guidelines for the physiotherapy management of motor neuron disease (mnd) september 2014 1 st edition mnd
guideline development group

23. Short-Term Effects
• In the first month of
rehabilitation, even
improvements in the
ALSFRS-R score
can be found.
Medium-Term
Effects
• Within 3 months,
significant differences
begin to appear
between the groups
treated with therapeutic
exercise and those
treated with passive
conventional treatment
Long-Term Effects
• Patients treated with
therapeutic exercise
benefited most at a
functional level compared
to those who continued
with a more sedentary
lifestyle.
23
Laura Ortega-Hombrados, to analyze the potential short, medium & long-term effects of a therapeutic
physical exercise (TFE) on the functionality of amyotrophic lateral sclerosis (ALS) patients., (2021)
Ortega-Hombrados, L.; Molina-Torres, G.; Galán-Mercant, A.; Sánchez-Guerrero, E.; González-Sánchez, M.; Ruiz-Muñoz, M. Systematic Review
of Therapeutic Physical Exercise in Patients with Amyotrophic Lateral Sclerosis over Time. Int. J. Environ. Res. Public Health 2021, 18, 1074.
• ROM and stretching exercises are typically accepted modes of exercise for PALS.
• Resistance exercises of unaffected muscles (and possibly affected muscles with strength of at
least grade 3 or above) using a low-to-moderate load and intensity.
• Aerobic activities, such as swimming, walking, and stationary cycling (mode dependent on
safety of PALS), at sub-maximal levels (eg, between 50% and 65% of heart rate reserve) may
be safe and effective in achieving therapeutic goals.
• Aerobic and resistance exercises are more appropriate for PALS in the early or early-middle
stage of ALS and those with more slowly progressive disease, and should be prescribed as soon
as possible after diagnosis.

24. 24
FALLS PREVENTION
Reported rate of falls to be 46% in an
ALS clinic. Falls in PALS have serious
consequences, resulting in increased
morbidity and mortality.
Gil et al found that 1.7% of ambulatory
PALS experienced fall-related deaths.
Dal Bello-Haas V., et al 2010
In 2014 study found that 37% of
ambulatory individuals with relatively
normal clinical balance and mobility test
findings had decreased ability to use
vestibular input and required increased
reliance on visual input for postural
orientation to sustain equilibrium.
Sanjak M, Hirsch MA, Bravver EK, Bockenek WL,
Norton HJ, Brooks BR. Vestibular deficits leading to
disequilibrium and falls in ambulatory amyotrophic
lateral sclerosis. A
Sanjak M, Hirsch MA
Dal Bello-Haas V, Andrews-Hinders D, et al. Development,
analysis, refinement and utility of an amyotrophic lateral
sclerosis database. Amyotroph Lateral Scler Other Motor
Neuron Disord. 2001;2(1):39–46.

25. WHO., Fall prevention and management should take a systems
approach, which strives to create safer people & safer environments
• Safer people interventions aim to strengthen awareness, knowledge and skills, and access so
that individuals, organizations and communities can make safer choices when it comes to
preventing falls.
• Safer environment interventions aim to eliminate fall hazards in the home, the community or in
the workplace – for example, providing soft-fall surfaces that can reduce the risk injury; stair
guards to prevent falling down stairs; or scaffolding for construction workers that includes
guard rails and toe boards, planked platforms, and safe access points.
• Interventions to address risk factors include improving physical mobility issues, awareness of
medication use, community infrastructure and housing, public awareness,
25
Step safely: strategies for preventing and managing falls across the life-course, World Health Organization

26. Progressive cervical extensor muscle weakness - cause head to fall
Mild to moderate cervical weakness Soft collar
Moderate to severe cervical weakness Semirigid/rigid
collar
Combined cervical and upper thoracic
weakness - benefit Cervical-thoracic
orthosis, or a Sternal Occipital Mandibular
Immobilizer.
ORTHOTIC MANAGEMENT
Silvia Pancania et. al
Head –up collar - cervical orthosis designed to be
adaptable to a patient's needs using adjustable
removable supports.
Heterogeneity - level of impairment and residual
function - need for personalized intervention.
Varying the number and type of supports applied-
level of support offered during the progression of
the disease - can be changed
S. Pancani et al. Efficacy of the Head Up collar in facilitating functional head movements in patients with Amyotrophic Lateral Sclerosis
Clinical Biomechanics 57 (2018) 114–120

27. 27
Salony majmudar rehabilitation in amyotrophic lateral sclerosis: why it matters muscle nerve. 2014 july ; 50(1): 4–13.
Doi:10.1002/mus.24202.
Weakness of the UEs greatly affects the patient’s ability to carry out ADL. Patients with a painful shoulder
due to subluxation may benefit from a sling, Splinting of the wrist or hand may be indicated to prevent
contractures or to improve the patient’s function, such as the ability to grasp.

28. 28
PROVISION OF AIDS & ASSISTIVE DEVICES
NICE : Provide equipment and adaptations that meet the person's needs without delay, so that people can
participate in activities of daily living and maintain their quality of life as much as possible.
ZACHARY SIMMONS, et al.,2015
assistive devices may be of value in
reducing energy expenditure while
improving function and Quality of Life.
Handbook of Clinical Neurology, Vol. 11 (3rd series) Neurological Rehabilitation M.P. Barnes # 2013 Elsevier

29. 29
Salony majmudar rehabilitation in amyotrophic lateral sclerosis: why it matters muscle nerve. 2014 july ; 50(1): 4–13.
Doi:10.1002/mus.24202.

30. 30
SPEECH & SWALOWING MANAGEMENT
Collaboration with SLP &
Nutritionist
Managing patients Head control,
trunk balance & position in sitting.
For eating & Swallowing –
Strategies like chin tucks – used.
Rehabilitation of motor neuron disease, ZACHARY
SIMMONS, Handbook of Clinical Neurology, Vol. 110
(3rd series) Neurological Rehabilitation

31. • https://www.mndassociation.org/, it provides wide support and information for people
with MND, on all levels.
• It provides knowledge related to disease, available in 17 different languages.
• Also has guide for caregiver
• Useful organizations & websites
• Free and low cost apps
• Emergency contact
• Research Information sheets
31
ADVOCACY

32. RECENT ADVANCES
Physical training in ALS: A pilot, randomized, controlled study Eur J Transl Myol 28 (1): 132-140,
Effects of combined endurance and resistance training in Amyotrophic
Lateral Sclerosis: A pilot, randomized, controlled study
Muscle strengthening and
cardiovascular exercises
can help maintain function
and not adversely affect
the progression of disease
in patients with ALS.
38 ALS patients were assigned
randomly to two groups: one group
underwent a specific exercise
programme (ALS-EP) based on a
moderate aerobic workout and
isometric contractions, and the
second group followed a standard
neuromotor rehabilitation
treatment.
32

33. Exercise protocol –set by ACSM
 Submaximal isometric
contraction to strengthen the
weak muscles with a score of 3,
4- or 4 - Medical Research
Council scale
 3 reps – for each bilateral
segment
 Each rep- separated by 30 sec
rest.
 Rubber bands with a sequential
 Aerobic exercise - sub-maximal
intensity of 65% of maximum
heart rate adjusted for age, with
a duration of 15-20 minutes .
 Weakness pattern and level of
disability of each patient - cycle
ergometer, ergometry arm-leg
and/or treadmill.
Daily for 5 weeks
Functional Improvement – muscle power,oxygen consumption & fatigue - se
33

34. REFRENCES
34
• Global, regional, and national burden of motor neuron diseases 1990–2016: a systematic
analysis for the Global Burden of Disease Study 2016 GBD 2016 Motor Neuron Disease
Collaborators.
• DARCY UMPHRED’S NEUROLOGICAL REHABILITATION SIXTH EDITION
• Physical Rehabilitation, Susan Sullivan, 7th edition
• The Lancet Neurology; 15(11); Al-Chalabi A, Hardiman O, Kiernan MC, Chi.A, Rix-Brooks B,
van den Berg LH. Amyotrophic lateral sclerosis: moving towards a new classification system;
1182–1194;
• Guidelines for the management of motor neuron diseases, nice guideline published: 24
february 2016 last updated: 23 july 2019 , www.nice.org.uk/guidance/ng42
• GUIDELINES FOR THE PHYSIOTHERAPY MANAGEMENT OF MOTOR NEURON
DISEASE (MND) September 2014 1 ST EDITION MND GUIDELINE DEVELOPMENT
GROUP
• Ashworth, N.L., Satkunam, L.E., Deforge, D. (2011) Treatment in amyotrophic lateral
sclerosis / motor neuron disease (Review). The Cochrane Collaboration.
http://www.thecochranelibrary.com.
• Handbook of Clinical Neurology, Vol. 11 (3rd series) Neurological Rehabilitation M.P. Barnes
2013 Elsevier

35. 35
• Physical therapy for individuals with MND current insights, Vanina Dal Bello-Haas, Hamilton,
Degenerative Neurological and Neuromuscular Disease , e 2018:8 45–54.
• Chiò A, Mora G, Lauria G. Pain in amyotrophic lateral sclerosis. Lancet Neurology. 2018;16(2):144–
157.
• Gibbons C, Pagnini F, Friede T, Young CA. Treatment of fatigue in amyotrophic lateral sclerosis/motor
neuron disease. Cochrane Database of Systematic Reviews 2018, Issue 1. Art. No.: CD011005. DOI:
10.1002/14651858.CD011005.pub2.
• Rehabilitation of motor neuron disease, ZACHARY SIMMONS, Handbook of Clinical Neurology, Vol.
110 (3rd series) Neurological Rehabilitation.
• Guidelines for the physiotherapy management of motor neuron disease (mnd) september 2014 1 st
edition mnd guideline development group
• Ortega-Hombrados, L.; Molina-Torres, G.; Galán-Mercant, A.; Sánchez-Guerrero, E.; González-
Sánchez, M.; Ruiz-Muñoz, M. Systematic Review of Therapeutic Physical Exercise in Patients with
Amyotrophic Lateral Sclerosis over Time. Int. J. Environ. Res. Public Health 2021, 18, 1074.
• S. Pancani et al. Efficacy of the Head Up collar in facilitating functional head movements in patients
with Amyotrophic Lateral Sclerosis Clinical Biomechanics 57 (2018) 114–120.
• Salony majmudar rehabilitation in amyotrophic lateral sclerosis: why it matters muscle nerve. 2014 july
; 50(1): 4–13. Doi:10.1002/mus.24202.

36. THANK YOU
36