AMYOTROPHIC LATERAL

SCLEROSIS
Koass Neurologi RSAL Mintoharjo
periode 14 November-17 Desember 2016
Missy Ayuni S 030.08.164
Ria Andini Sutopo030.11.250
Ni Made Fika Dyah I. B 030.12.190
Martha Rianita Odjan 030.09.145
Dela Asrivia 030.10.071
Tiara Aggiana Ayu 030.12.270
Mutia Nur Izzati 030.12.177

Practice Essentials
Amyotrophic lateral sclerosis (ALS) is the
most common degenerative disease of the
motor neuron system. Although ALS is
incurable and fatal, with median survival of 3
years, treatment can extend the length and
meaningful quality of life for patients.

SIGNS AND SYMPTOMS

In 75-80% of patients, symptoms begin with limb
involvement. Initial complaints in patients with lower limb
onset are often as follows:
Tripping, stumbling, or awkwardness when running
Foot drop; patients may report a "slapping" gait
Initial complaints with upper limb onset include the following:
Reduced finger dexterity, cramping, stiffness, and weakness
or wasting of intrinsic hand muscles
Wrist drop interfering with work performance

SIGNS AND SYMPTOMS

With bulbar onset (20-25%), initial complaints are as follows:
Slurred speech, hoarseness, or decreased volume of speech
Aspiration or choking during a meal
Emotional and special cognitive difficulties in some ALS
patients are as follows:
Involuntary laughing or crying
Depression
Impaired executive function
Maladaptive social behavior

SIGNS AND SYMPTOMS (2)

Features of more-advanced disease are as follows:
Muscle atrophy becomes more apparent
Spasticity may compromise gait and manual dexterity
Muscle cramps are common
Rarely, painful joint contractures may result from
immobility
Progression of bulbar disease leads to the
following:
Voice changes: Hypernasality and development
of a strained, strangled vocal quality;
eventually, speech may be lost
Swallowing difficulties, usually starting with

The Image Below Illustrates The 4

Regions of The Body:
The 4 regions or levels of the
body. Bulbar (muscles of the
face, mouth, and throat);
cervical (muscles of the back
of the head and the neck, the
shoulders and upper back,
and the upper extremities);
thoracic (muscles of the
chest and abdomen and the
middle portion of the spinal
muscles);
lumbosacral
(muscles of the lower back,
groin, and lower extremities).

DIAGNOSIS
Definitive diagnosis may not be possible with early ALS.
Confirmation of the disease may require a period of
observation to document its progressive nature and to
exclude alternative diagnoses.
The World Federation of Neurology (WFN) has
developed a diagnostic algorithm that combines the
clinical and, in some cases, electrophysiologic findings.
[1]
The degree of certainty of diagnosis is increased by
the number of body segments that demonstrate upper
motor neuron (UMN) and lower motor neuron (LMN)
signs. UMN signs are mild weakness, spasticity, and
abnormally brisk reflexes; LMN signs are progressive
weakness, wasting, and loss of reflexes and muscle
tone. WFN categories are as follows:

DIAGNOSIS
Clinically probable ALS: UMN and LMN signs in at
least 2 body segments with some UMN signs in a
segment above the LMN signs
Clinically probable, laboratory-supported ALS:
UMN and LMN signs in 1 segment or UMN signs in
1 region coupled with LMN signs by
electromyography (EMG) in at least 2 limbs
Clinically possible ALS: UMN and LMN signs in 1
body segment, UMN signs alone in at least 2
segments, or LMN signs in segments above UMN
signs
Clinically suspected ALS: Pure LMN syndrome with

DIAGNOSIS
Hallmark findings in the electrodiagnosis of ALS
are normal sensory nerve conduction studies and
abnormal motor nerve conduction studies, with
reduced motor compound muscle action
potentials. The needle exam shows changes
characteristic of ongoing denervation and
reinnervation of muscles.
In patients with familial ALS, genetic testing may
be requested after appropriate counseling. The
results of genetic testing may affect not only the
patient, but also family members. Tests for the
SOD1, TARDBP (coding for TDP-43), FUS, ANG,
C9orf72, and FIG4 genes and for the gene
causing Kennedy disease are available

MANAGEMENT
American
Academy
of
Neurology
recommendations for management of patients
with ALS can be summarized as follows [2, 3] :
Riluzole should be offered to all patients with ALS
to slow disease progression
Enteral nutrition via percutaneous endoscopic
gastrostomy (PEG) should be considered to
stabilize body weight in patients with impaired
oral intake; PEG placement probably prolongs
survival to some degree; PEG placement when
forced vital capacity (FVC) is still over 50%
predicted minimizes risk of insertion

MANAGEMENT (2)
Noninvasive ventilation (NIV) should be offered to
treat respiratory insufficiency to prolong survival
and slow the decline of FVC; NIV may be
considered at the earliest sign of nocturnal
hypoventilation or respiratory insufficiency
Mechanical insufflation/exsufflation may be
considered to clear secretions in patients with
reduced peak cough flow, particularly during an
acute lower respiratory infection
Creatine and high-dose vitamin E should not be
used

MANAGEMENT (3)
Invasive
ventilatory
support,
requiring
tracheostomy, may be considered in the following
cases:
Patients who present with respiratory failure and
who are otherwise largely neurologically intact
Patients who want to be kept alive using long-term
invasive ventilatory support as their disease
progresses
Patients in whom secretions cannot be managed
and who therefore cannot benefit from noninvasive
ventilatory support (this occurs very rarely)

MANAGEMENT (4)
The combination of dextromethorphan and quinidine
(Nuedexta)
to
decrease
emotional
lability
(pseudobulbar affect)
Anticholinergics and sympathomimetics for sialorrhea
Mucolytics for thickened secretions
Lorazepam for anxiety
Selective serotonin reuptake inhibitors (SSRIs) for
depression
Nonsteroidal

anti-inflammatory

drugs

(NSAIDs),

THANK YOU