Terapia Ocupacional na Esclerose Lateral Amiotrofica (ELA)

Introdução:

Amyotrophic lateral sclerosis (ALS) is a relentlessly progressive paralyzing disease. Most pa-
tients with ALS die within 2 to 5 years of onset. The mainstay of ALS management is sympto -
matic treatment and palliative care.

Amyotrophic lateral sclerosis (ALS) is a term used to cover the spectrum of neurodegenerative
syndromes characterised by progressive degeneration of motor neurones. However, it is also the
term used in modern clinical practice to indicate the commonest form of the disease, Classical
(Charcot's) ALS. Other syndromes related to this spectrum of disorders include, Progressive bul-
bar palsy (PBP), Progressive muscular atrophy (PMA), Primary lateral sclerosis (PLS), Flail arm
syndrome (Vulpian-Bernhardt syndrome), Flail leg syndrome (Pseudopolyneuritic form) and
ALS with multi-system involvement (e.g., ALS-Dementia). Lord Russell Brain proposed the
term Motor neurone disease (MND) to incorporate these conditions into a single spectrum of dis-
orders 

Amyotrophy" refers to the atrophy of muscle fibres, which are denervated as their corresponding
anterior horn cells degenerate, leading to weakness of affected muscles and visible fascicula-
tions. "Lateral sclerosis" refers to hardening of the anterior and lateral corticospinal tracts as mo-
tor neurons in these areas degenerate and are replaced by gliosis

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterised by progressive

muscular paralysis reflecting degeneration of motor neurones in the primary motor cortex, corti-
cospinal tracts, brainstem and spinal cord.

Approximately two thirds of patients with typical ALS have a spinal form of the disease (limb
onset) and present with symptoms related to focal muscle weakness and wasting, where the
symptoms may start either distally or proximally in the upper and lower limbs. Gradually, spas-
ticity may develop in the weakened atrophic limbs, affecting manual dexterity and gait. Patients
with bulbar onset ALS usually present with dysarthria and dysphagia for solid or liquids, and
limbs symptoms can develop almost simultaneously with bulbar symptoms, and in the vast ma-
jority of cases will occur within 1–2 years. Paralysis is progressive and leads to death due to res-
piratory failure within 2–3 years for bulbar onset cases and 3–5 years for limb onset ALS cases

The weakness is usually of insidious onset, and patients may notice that symptoms are exacer-
bated by cold weather. Although it is usually asymmetrical at onset, the other limbs develop
weakness and wasting sooner or later, and most patients go on to develop bulbar symptoms and
eventually respiratory symptoms (although not necessarily in that sequence). Gradually, spastic-
ity may develop in the weakened atrophic limbs, affecting manual dexterity and gait. During late
stages of the disease patients may develop 'flexor spasms', which are involuntary spasms occur-
ring due to excess activation of the flexor arc in a spastic limb. Occasionally encountered symp-
toms include new bladder dysfunction (such as urgency of micturition), sensory symptoms, cog-
nitive symptoms and multi-system involvement (e.g. dementia, parkinsonism).

Patients with bulbar onset ALS usually present with dysarthria of speech, which may initially
only be apparent after ingestion of small amount of alcohol. Rarely, patients may present with
dysphagia for solid or liquids before noticing speech disturbances. Limbs symptoms can develop
almost simultaneously with bulbar symptoms and in the vast majority of cases will occur within
1–2 years. Almost all patients with bulbar symptoms develop sialorrhoea (excessive drooling)
due to difficulty swallowing saliva and mild UMN type bilateral facial weakness which affects
the lower part of the face. 'Pseudobulbar' symptoms such as emotional lability and excessive
yawning are seen in a significant number of cases.

The management of ALS is supportive, palliative, and multidisciplinary. Non-invasive ventila-

tion prolongs survival and improves quality of life. Riluzole is the only drug that has been shown
to extend survival

Dor, caibra e Espasmos:

Dor pode acontecer devido a varias fatores mas uma causa de dor em pacientes com
ASL são as caibras (involuntarias e repentinas contrações musculares que são
desencadeadas por qualquer esforço de grupos musculares das pernas, braços,
abdomem, costas, mandíbula ou garganta). Esses espasmos podem variar em termos de
severidade e freqüência.
Espasmos podem ser aliviados com exercícios de alongamento e boa hidratação.

Espasticidade - Espasticidade pode ser común na ELA e pode causar mais dificuldade
de mobilidade e coordenação. Alongamentos também ajudam com o manejo da
espasticidade.
Causa Tratamento
Espasmos/caibras/rigidez Alongamentos e exercícios de ADM
Imobilidade/Contraturas Ejercicios de ADM, ortostatismo,
equipamentos e cuidado com
pressao
Edema Massagem, posicionamento, elevaçao
Problemas com sono Elevaçao da cabeça/tronco a 30 graus,
Repsocionamento durante a noite
Fadiga Estratégias de conservação de energia,
exercícios moderados e de curta duração,
uso de adaptações pra evitar exertion,
adequaçao postural que facilite ventilação

O papel do TO:
- educaçao com relação ao prognóstico
- Educaçao ao cuidador em como facilitar mobilidade, observar por sintomas de
fadiga e estratégias em como auxiliar o paciente
- Educaçao com relação a estratégias de conservação de energia
- Orteses e adaptações que facilitem função e prevenção de contraturas
- Recommendaçoes e manutenção de cadeira de rodas e adaptações na mesma
- Educaçao sobre end of life care
O trabalho em equipe: é importante monitorar progressão dos sintomas em geral, e
discutir com outros colegas da equipe sobre novos sintomas e progressão da doença
observados ou relatados para TO. Especialmente importante é observação de sinais de
deterioração respiratória e dysphagia.

O papel do exercício fisico:

Muscle weakness is very common in people with amyotrophic lateral sclerosis (ALS). A
weak muscle can be damaged if overworked, because it is already functioning close to
its maximal limits. As a result of this, exercise programs for people with ALS may be
discouraged. However, if a person with ALS is not active, deconditioning and disuse
weakness occurs, superimposed on the weakness caused by the ALS itself. If the re-
duced level of activity persists, many organ systems can be affected and a person with
ALS can develop further deconditioning, muscle and joint tightness which cause con-
tractures and pain. All these make performing daily activities more difficult. This re-
view found only two randomised studies of exercise in people with ALS. The studies
were too small to determine to what extent exercise for people with ALS is beneficial or
whether exercise is harmful. More research is needed.

When considering the rehabilitation of clients with ALS, there are six recognized
stages of the disease that should be considered. The table below outlines these
stages, along with the appropriate activity level and equipment according to the
client’s level of function(1,2).
 
 
 Stage
Characteristic Clinical Features
Activities to Maintain Motor Function
Equipment
1
Ambulatory; no problems with ADL, mild weakness
Normal activities; active range-of-motion (ROM) exercise
None
2
Ambulatory; moderate weakness in certain muscles
Modification in living; active, assisted ROM exercise
Assistive Devices
3
Ambulatory; severe weakness in certain muscles
Active, assisted, passive ROM exercise; joint pain management
Assistive devices, adaptive devices, home equipment
4
Wheelchair-confined; almost independent, severe leg weakness
Passive ROM exercise, modest exercise in uninvolved muscles
Assistive devices, adaptive devices, wheelchair, home equipment
5
Wheelchair confined; dependent; pronounced leg weakness, severe arm weakness
Passive ROM exercise; pain management
Adaptive devices, home equipment, wheelchair
6
Bedridden; no ADL, maximum assistance required
Passive ROM exercise; pain management; prevention of decubitus ulcers and ve-
nous
thrombosis
Adaptive devices,
Home equipment

Stage Characteristic Clinical Activities to Maintain Equipment

Features Motor Function
1 Ambulatory; no Normal activities; active None
problems with ADL, range-of-motion (ROM)
mild weakness exercise
2 Ambulatory; moderate Modification in living; Assistive
weakness in certain active, assisted ROM Devices
muscles exercise
3 Ambulatory; severe Active, assisted, passive Assistive
weakness in certain ROM exercise; joint pain devices,
muscles management adaptive
devices, home
equipment
4 Wheelchair-confined; Passive ROM exercise, Assistive
almost independent, modest exercise in devices,
severe leg weakness uninvolved muscles adaptive
devices,
wheelchair,
home
equipment
5 Wheelchair confined; Passive ROM exercise; Adaptive
dependent; pronounced pain management devices, home
leg weakness, severe equipment,
arm weakness wheelchair
6 Bedridden; no ADL, Passive ROM exercise; Adaptive de-
maximum assistance pain management; pre- vices,
required vention of decubitus ul- Home equip-
cers and venous ment
thrombosis
The progressive and varied nature of ALS necessitates that occupational therapy
(OT) interventions be client-centred, focusing on ways to adapt to the disability,
modify the environment, and prevent secondary complications (1).
Potential OT goals for ALS clients during early stages of the disease include:
-        Optimizing strength and range of motion at home
-        Decreasing pain and fatigue in neck and extremities with splints and
orthotics
-        Implementing joint protection and pain management strategies

 
During later stages of the disease, potential OT goals for ALS clients can include:
-        Optimizing safety
-        Employing environmental modification
-        Assessing positioning and maintaining skin integrity

Client and Caregiver Education

 

As ALS progresses, loss of functional independence is inevitable. Occupational

therapists play an important role in educating primary caregivers about
proper transfer, dressing, and toileting techniques. Proper lifting and body me-
chanics education is crucial in preserving the health and safety of both the client
and care providers at home. Since excessive fatigue is a common and problematic
symptom of ALS, energy conservation techniques is also helpful education that
occupational therapists can provide to clients and their family (5).  
Assistive Equipment 

•         Raised toilet seat or commode chair“It is vital that we understand

not only our client’s needs now, but anticipated future issues and
have solutions ready when problems arise. It is essential to be re-
sourceful and well informed, especially when prescribing assis-
tive technologies. Some assistive devices may be useful only short
term, while other technologies must be adaptable to all possible
changes in our clients, their environments and occupations” –
Karen Hall, OT Reg.,(Ont). (7)

•
•
•         Bathtub seat or lift

•         Floor-to-ceiling pole

•         Mechanical lifts

Devices for Activities of Daily Living

• Reacher

• Long-handled sponge  

• Utensils with thick handles

• Mugs with thick handles

 • Doorknob turner

• Velcro/elastics to replace buttons or zippers on clothing

• Environmental controls

Mobility Equipment

• Canes and walkers

• Manual wheelchairs

• Power wheelchairs

• Scooters 

Amyotrophic lateral scle-

rosis
 • Lokesh C Wijesekera† andP Nigel Leigh†Email au-
thor
Contributed equally
†

Orphanet Journal of Rare Diseases20094:3

Practice Parameter update: The care of the patient with amyotrophic lateral sclerosis:
Multidisciplinary care, symptom management, and cognitive/behavioral impairment
(an evidence-based review)
R. G. Miller, C. E. Jackson, E. J. Kasarskis, J. D. England, D. Forshew, W. John-
ston, S. Kalra, J. S. Katz, H. Mitsumoto, J. Rosenfeld, C.Shoesmith, M. J. Strong, S.
C. Woolley
Neurology Oct 2009, 73 (15) 1227-1233