Clinical Features of Amyotrophic Lateral Sclerosis and Other Forms of Motor Neuron Disease - UpToDate

27.03.
2024 01:54 Clinical features of amyotrophic lateral sclerosis and other forms of motor neuron disease - UpToDate
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Clinical features of amyotrophic lateral sclerosis and

other forms of motor neuron disease
AUTHORS: Lauren B Elman, MD, Leo McCluskey, MD, MBE, Colin Quinn, MD
SECTION EDITOR: Jeremy M Shefner, MD, PhD
DEPUTY EDITOR: Richard P Goddeau, Jr, DO, FAHA
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Feb 2024.

This topic last updated: Feb 13, 2023.
INTRODUCTION
Amyotrophic lateral sclerosis (ALS), first described by Charcot in the 19th century, is a
relentlessly progressive, presently incurable neurodegenerative disorder that causes muscle
weakness, disability, and eventually death. ALS is also known as Lou Gehrig's disease, after
the famous New York Yankee baseball player who was affected with the disorder [1-3]. Motor
neuron disease (MND) is the preferred term in the United Kingdom, but ALS and MND are
sometimes used interchangeably in the United States.
This topic will review the clinical features of ALS. Other aspects of ALS are discussed
separately.
● (See "Epidemiology and pathogenesis of amyotrophic lateral sclerosis".)

● (See "Diagnosis of amyotrophic lateral sclerosis and other forms of motor neuron
disease".)
● (See "Disease-modifying treatment of amyotrophic lateral sclerosis".)
● (See "Symptom-based management of amyotrophic lateral sclerosis".)
CLINICOPATHOLOGIC FEATURES
The clinical hallmark of ALS is the combination of upper and lower motor neuron signs and
symptoms.
● The upper motor neuron findings of weakness with slowness, hyperreflexia, and
spasticity result from degeneration of frontal lobe motor neurons located in the motor
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27.03.2024 01:54 Clinical features of amyotrophic lateral sclerosis and other forms of motor neuron disease - UpToDate
strip (Brodmann area 4) and their axons traversing the corona radiata, internal capsule,
cerebral peduncles, pontine base, medullary pyramids, and the lateral corticospinal
tracts of the spinal cord. At autopsy, the dorsolateral area of the spinal cord, the region
containing the lateral corticospinal tract, is gliotic and hardened or sclerotic to
palpation.
● The lower motor neuron findings of weakness, atrophy or amyotrophy, and

fasciculations are a direct consequence of degeneration of lower motor neurons in the
brainstem and spinal cord producing muscle denervation.
The neuropathology of ALS is characterized by pathologic inclusions within both upper and
lower motor neurons and glia. Such inclusions also occur in nonmotor frontal and temporal
cortical neurons and in more widespread areas of the brain not typically associated with
classic symptoms of ALS. Inclusions stain positively for ubiquitin; a large subset also stains
positively for TAR DNA-binding protein (TDP-43) and smaller subsets stain for fused in
sarcoma (FUS) protein and optineurin. (See "Epidemiology and pathogenesis of amyotrophic
lateral sclerosis", section on 'Intracellular inclusions'.)
The term "amyotrophic lateral sclerosis" is derived from the combination of the clinical
examination finding of muscle atrophy with the pathologic finding of hardening of the lateral
corticospinal tracts [2-7]. While it was once presumed to be a pure motor disorder, it has
become increasingly apparent that degeneration of other brain regions such as frontal and
temporal cortical neurons may also occur as part of the clinicopathologic spectrum of ALS.
(See 'ALS-plus syndrome' below and 'Cognitive symptoms' below and 'Autonomic symptoms'
below and 'Parkinsonism and supranuclear gaze palsy' below and 'Sensory symptoms'
below.)
SPECTRUM OF MOTOR NEURON DISEASE
ALS is one of multiple degenerative motor neuron diseases that are clinically defined, based
on the involvement of upper and/or lower motor neurons [1,2,8]. ALS is the most common
form of motor neuron disease and includes upper and lower motor neuron pathology.
Progressive muscular atrophy — Progressive muscular atrophy is a progressive lower

motor neuron disorder. Some experts believe it represents a form of ALS [9,10].
When the disease remains confined to the lower motor neuron, survival may be prolonged
compared with classic ALS. In the largest study, 91 patients initially diagnosed with
progressive muscular atrophy had a longer median survival than 871 patients with ALS (48
versus 36 months) [9]. However, on Kaplan-Meier estimates, the survival curves of
progressive muscular atrophy and ALS crossed at approximately 80 months. Thereafter, the
estimated survival in progressive muscular atrophy was approximately the same as that of
ALS. In an earlier series, 37 patients with progressive muscular atrophy had a median
survival of 56 months [11].
Some individuals who present with isolated lower motor neuron signs never develop clinical
upper motor neuron signs. Many, however, develop upper motor neuron signs later in their
clinical course, at which point the disease is called lower motor neuron-onset ALS. In the
series cited above, upper motor neuron signs developed in 20 of 91 patients (22 percent)
initially diagnosed with progressive muscular atrophy [9]. Typically, upper motor neuron
involvement occurred within two years of symptom onset.
At autopsy, patients with progressive muscular atrophy who never developed clinically
apparent upper motor neuron signs frequently have upper motor neuron pathology,
including corticospinal tract abnormalities and TDP-43 positive inclusions in motor cortex, in
a pattern identical to that of ALS [6,12].
Primary lateral sclerosis — Primary lateral sclerosis is a progressive isolated upper motor
neuron neurodegenerative disorder [13]. Cases of pure upper motor neuron disease make
up a small percentage of ALS/motor neuron disease [14,15]. New diagnostic criteria for
definite PLS require isolated upper motor neuron signs for greater than four years [16].
Compared with ALS, primarily lateral sclerosis is characterized by slower progression, lack of
weight loss, and absence of lower motor neuron findings on examination or
electromyography in the first four years after symptom onset [16-20]. Symptoms usually
begin in the lower extremities and may be symmetric, with impaired gait and spasticity and
hyperreflexia on examination. Corticobulbar symptoms (eg, dysarthria, pseudobulbar affect)
typically develop later in the course. Many patients also have bladder instability and urinary
retention. The early phase of disease has significant clinical overlap with hereditary spastic
paraparesis. (See "Diagnosis of amyotrophic lateral sclerosis and other forms of motor
neuron disease", section on 'Hereditary spastic paraplegia'.)
Patients who develop delayed lower motor neuron findings are typically referred to as
having upper motor neuron-onset ALS. Reports of pathology in clinically defined primary
lateral sclerosis are limited, but disease pathologically isolated to the upper motor neuron
has been described [21-24].
Pure primary lateral sclerosis and upper motor neuron-dominant ALS appear to have a more
benign prognosis than typical ALS [13,19,20,25]. Survival tends to be longer and disease
progression slower in patients classified as primary lateral sclerosis compared with ALS
controls [17]. Survival for patients with upper motor neuron-dominant ALS is intermediate
between that of primary lateral sclerosis and classic ALS.
Progressive bulbar palsy — Progressive bulbar palsy is a progressive upper and lower
motor neuron disorder of cranial muscles. Symptoms remain isolated to the bulbar segment
in progressive bulbar palsy. However, patients initially diagnosed with progressive bulbar
palsy may commonly develop upper and lower motor neuron signs and symptoms involving
other body regions and are then classified as having bulbar-onset ALS [26]. There have been
no reports of specific pathology in progressive bulbar palsy [1-3,27].
Flail arm syndrome — The flail arm syndrome (also called brachial amyotrophic diplegia) is
characterized by progressive lower motor neuron weakness and wasting that predominantly
affects the proximal arm [28-30]. It usually begins proximally and spreads distally to the
point where arm and hand function is severely impaired. It is often asymmetric. Patients
presenting with the flail arm variant of ALS have a longer survival time and a slower rate of
progression both to the spread of signs and symptoms in other body segments and to
development of respiratory muscle weakness [31,32].
Flail leg syndrome — The flail leg syndrome (also called the pseudo-polyneuritic variant of
ALS/motor neuron disease) is characterized by progressive lower motor neuron weakness
and wasting with onset in the distal leg [31,33]. Patients presenting with the flail leg
syndrome have a lower rate of comorbid dementia, a longer survival time, and a slower rate
of progression to involvement of other body segments and of the development of
respiratory muscle weakness [31,32].
ALS-plus syndrome — Classically defined, ALS is considered a degenerative disorder of the

upper and lower motor neurons and does not include symptoms or signs outside of the
voluntary motor system. However, some patients have the clinical features of ALS along with
features of other disorders such as frontotemporal dementia (FTD), autonomic insufficiency,
parkinsonism, supranuclear gaze paresis, and/or sensory loss. Such patients are considered
to have ALS-plus syndrome. Of these, ALS-FTD is the most common.
CLINICAL SYMPTOMS AND SIGNS
The loss of motor neurons results in the primary clinical symptoms and signs of ALS. These
may produce impairment affecting limb ( table 1), bulbar ( table 2), axial ( table 3), and
respiratory ( table 4) function.
Differences in site and segment (cranial, cervical, thoracic, or lumbosacral) of onset, pattern
and speed of spread, and the degree of upper and/or lower motor neuron dysfunction
produce a disorder that is remarkably variable between individuals.
Initial presentation — The initial clinical manifestation of ALS may occur in any body
segment (bulbar, cervical, thoracic, or lumbosacral) and may manifest as upper motor
neuron (see 'Upper motor neuron symptoms' below) or lower motor neuron (see 'Lower
motor neuron symptoms' below) symptoms or signs. Asymmetric limb weakness is the most
common presentation of ALS (80 percent). Upper-extremity onset is most often heralded by
hand weakness but may begin in the shoulder girdle muscles. The "split-hand syndrome"
describes a frequent pattern of weakness and atrophy in ALS that predominantly involves
the median- and ulnar-innervated lateral (thenar) hand intrinsic muscles with relative sparing
of the medial (hypothenar) muscles [34-36]. Lower-extremity onset of ALS most often begins
with weakness of foot dorsiflexion (foot drop), while proximal pelvic girdle onset is less
common.
Twenty percent of patients will have onset in the bulbar segment, which most often presents
with either dysarthria or dysphagia.
Less common patterns of ALS onset include respiratory muscle weakness (1 to 3 percent)
[37], generalized weakness in the limbs and bulbar muscles (1 to 9 percent), axial onset with
head drop or truncal extension weakness, and weight loss with muscle atrophy,
fasciculations, and cramps [1].
Upper motor neuron symptoms — Loss of upper motor neurons results in slowness of
movement, incoordination, and stiffness with relatively little overt weakness. Arm or hand
upper motor neuron symptoms include poor dexterity with resulting difficulty performing
activities of daily living. Leg upper motor neuron symptoms manifest as a spastic gait with
poor balance and may include spontaneous leg flexor spasms and ankle clonus ( table 1).
Dysarthria and dysphagia are the most common bulbar upper motor neuron symptoms
( table 2). Upper motor neuron or spastic dysarthria produces a characteristically strained
vocal quality with slow speech. Upper motor neuron dysphagia results from slow and
discoordinated contraction of the swallowing muscles, which may lead to coughing and
choking.
Another frequent bulbar upper motor neuron symptom is the syndrome of the
pseudobulbar affect [38,39]. This is manifested as inappropriate laughing, crying, or
yawning. This may occur as an early manifestation of ALS or may develop during the disease
course. The observed affect is often mood incongruent or may be triggered by a minor
emotional stimulus. Patients also report difficulty with cessation of the laughing or crying
once it has begun. (See "Symptom-based management of amyotrophic lateral sclerosis",
section on 'Pseudobulbar affect'.)
Upper motor neuron bulbar dysfunction may also result in laryngospasm. This is a short-
lived (usually <30 seconds) reflex closure of the larynx that most often occurs in response to
aspiration of food particles or liquids, including saliva. The patient typically describes a
squeezing feeling in the throat accompanied by impaired inspiration and difficulty speaking;
there may be audible stridor.
Additional manifestations of upper motor neuron bulbar dysfunction may include increased
masseter tone and difficulty opening the mouth. When severe, this is referred to as trismus.
At times there may be involuntary jaw clenching with biting of the sides of the tongue and
cheeks.
Axial upper motor neuron dysfunction may contribute to stiffness and imbalance ( table 3).
Lower motor neuron symptoms — Loss of lower motor neurons results in weakness,
usually accompanied by atrophy ( movie 1) and fasciculations ( movie 2 and movie 3).
Muscle cramps are also common [40].
Hand weakness causes difficulty manipulating small objects (buttons, zippers, coins) and
using writing instruments ( table 1). Proximal arm weakness results in difficulty elevating
the arm to the level of the mouth or above the head. This can produce difficulty with bathing,
dressing, grooming, and eating. Foot and ankle weakness results in tripping, a slapping gait,
and falling. Proximal leg weakness results in difficulty arising from chairs, climbing stairs,
and getting off the floor. Balance may also be adversely affected.
Dysarthria and dysphagia can also result from lower motor neuron damage ( table 2).
Dysarthria may result from weakness of the tongue, lips, or palate. The speech is usually
slurred and may have a nasal quality. Hoarseness may be caused by associated vocal cord
weakness. Dysphagia results from tongue weakness with disruption of the oral phase of
swallowing or from pharyngeal constrictor weakness with disruption of the pharyngeal
phase of swallowing or both. Tongue weakness may lead to pocketing of food between the
cheeks and gums. Pharyngeal weakness often manifests as coughing and choking on food,
liquids, or secretions such as saliva or mucus. Aspiration may result.
Lower motor neuron weakness of the upper face may produce incomplete eye closure
( table 2). Lower facial weakness is more common and may result in poor lip seal that may
contribute to drooling or sialorrhea, particularly in patients with associated swallowing
difficulty. Lower motor neuron weakness of the masseter can cause difficulty chewing; when
severe, it may produce an inability to close the mouth. Lower motor neuron weakness of the
pterygoids may produce difficulty opening the mouth and moving the jaw from side to side.
Severe masseter and pterygoid weakness may contribute to disarticulation of the
temporomandibular joint.
Lower motor neuron weakness affecting the trunk and spine may produce difficulty holding
up the head and difficulty maintaining an erect posture as well as abdominal protuberance
( table 3).
Lower motor neuron weakness of the diaphragm produces progressive dyspnea with
decreasing amounts of effort culminating in dyspnea at rest and with talking along with
reduced vocal volume ( table 4). Diaphragmatic weakness may also result in orthopnea
and sleep disordered breathing.
Extraocular motor neurons residing in the nuclei of the oculomotor (CN III), trochlear (CN IV),
and abducens (CN VI) nerves are spared until very late in the disease course. Patients who
choose long-term mechanical ventilation have a longer clinical course that can include
progressive difficulty with ocular motility. This may culminate in the locked-in state, a clinical
condition characterized by inability to move any voluntary muscle. Such patients may be alert
and awake but completely unable to communicate.
Cognitive symptoms — There is a well-established link between ALS and frontotemporal

behavioral and executive dysfunction that may precede or follow the onset of upper and/or
lower motor neuron dysfunction [41-46]. The pattern of cognitive impairment includes
problems with executive function, language, grammar, and verbal fluency with relative
sparing of memory and visuospatial function. Common behavioral changes include apathy,
loss of empathy, changes in eating behaviors, disinhibition, and perseveration.
While most patients with ALS do not have overt dementia, some degree of cognitive and
behavioral dysfunction is present in approximately one-third to one-half of patients and
becomes increasingly common with advancing disease [44,47,48]. In a cross-sectional study
of 161 patients with ALS, the rate of ALS-specific cognitive impairment as measured by the
Edinburgh Cognitive and Behavioural ALS Screen (ECAS) ranged from 18 percent among
patients with King's clinical stage 1 and 2 disease to 39 percent among those with stage 4
disease (nutritional or respiratory failure) [47]. Behavioral impairment was present in 18 and
27 percent of patients with stage 1 and 2 disease, respectively, and 65 percent of those with
stage 4 disease. Bulbar dysfunction is an independent predictor of cognitive and behavioral
problems [47,48]. (See 'Clinical patterns of progression' below.)
Approximately 15 percent of patients with ALS meet criteria for frontotemporal dementia
(FTD) [41]. Retrospective data suggest that ALS with FTD may be associated with shorter
survival than ALS with normal executive and behavioral function [49]. ALS with FTD may be
familial or sporadic. (See "Familial amyotrophic lateral sclerosis", section on 'C9ORF72 gene'.)
Autonomic symptoms — Autonomic symptoms may occur in ALS as the disease progresses,
although they are typically mild [50]. Constipation occurs frequently and is likely
multifactorial. Delayed colonic motility has been demonstrated. Dysphagia for thin liquids
related to pharyngeal muscle weakness may lead to dehydration that can exacerbate
constipation. Symptoms of early satiety and bloating consistent with delayed gastric
emptying also occur as the disease progresses [51-53]. Urinary urgency without incontinence
is common, while incontinence is uncommon.
Some patients complain of excessive sweating, but whether a disorder of sweating occurs in
association with ALS is controversial [54,55]. Small studies have demonstrated abnormal
sudomotor function, including one study that showed hyperhidrosis early in ALS, with
decline in later-stage disease [50,56,57].
Parkinsonism and supranuclear gaze palsy — Extrapyramidal symptoms and signs of

parkinsonism may precede or follow the upper and lower motor neuron symptoms. These
extrapyramidal features may include facial masking, tremor, bradykinesia, and postural
instability [58,59]. In a single-center cohort of 550 patients with ALS, ocular motility
abnormalities were found in the majority of patients, and extrapyramidal abnormalities were
seen in approximately one quarter [60,61].
Sensory symptoms — Sensory symptoms may occur in 20 to 30 percent of patients with ALS
[1,62]. It is not uncommon for patients with ALS, particularly those with distal limb onset of
symptoms, to complain of tingling paresthesia (see 'Pain' below). When queried regarding
sensory loss, these patients typically will deny loss of sensation, and physical examination
does not detect objective sensory loss. At times, however, objective sensory loss may occur
as part of an ALS-plus syndrome and may precede or follow motor symptoms.
Electrophysiologic studies may demonstrate reduction of amplitudes on sensory nerve
conduction and/or slowing of dorsal column conduction on somatosensory evoked potential
testing, even in patients without sensory findings on examination [62-66]. Autopsy may
demonstrate evidence of degeneration within sensory pathways in individuals with and
without sensory loss.
Pain — Nociceptive pain in ALS can arise from a variety of causes that include reduced
mobility, muscle cramps, muscle spasticity, and comorbid conditions [67,68]. Reduced
mobility predisposes to skin breakdown and musculoskeletal pain. Respiratory symptoms
and interventions can lead to pain, with discomfort and skin breakdown from noninvasive
ventilation masks, and irritation from suctioning of secretions and weighing and pulling of
ventilator hoses. In addition, pain with neuropathic features (eg, paresthesia, allodynia,
hyperalgesia) may affect some patients with ALS. Although generally of mild to moderate
intensity, pain in the later stages of ALS can be severe enough to necessitate treatment with
analgesic and sedative medications [67,69,70].
A systematic review published in 2017 found that prevalence of pain in patients with ALS
ranged from 15 to 85 percent [67]. The inconsistency of these findings was attributed to the
heterogeneous methods and relatively small size of the underlying studies.
Clinical patterns of progression — ALS is a relentlessly progressive disorder with a clinical

course that is generally linear, with a relatively constant slope. While the rate of progression
between individuals is variable, the history generally reflects gradual and progressive
worsening over time without intervening remissions or exacerbations.
Symptoms initially spread within the segment of onset and then to other regions in a
relatively predictable pattern [1,23,71,72]. In patients with unilateral arm onset, the most
common (approximately 60 to 70 percent of patients) pattern of spread is to the contralateral
arm, then to the ipsilateral leg, then to the contralateral remaining leg, and then to the
bulbar muscles. In patients with unilateral leg onset, the most common (approximately 60 to
70 percent of patients) pattern of spread is to the contralateral leg, then to the ipsilateral
arm, then to the contralateral arm, and then to bulbar muscles. In patients with bulbar
onset, the most common pattern of spread is to one arm and then to the contralateral arm
[1,23].
Multiple clinical staging systems have been developed to help identify an objective measure
of disease progression. They may also help guide treatment decisions and aid in
prognostication. Commonly used staging systems include the following:
● The King's College ALS staging system classifies disease progression based on the
number of body regions involved as well as the need for swallowing or respiratory
support [73].
● The MiToS system defines the progressive loss of independence in key functional
domains: walking/self-care, swallowing, communicating, and breathing.
Both systems primarily use information that can be gathered from the revised ALS
Functional Rating Scale, the most commonly used measure in ALS clinical trials.
Life-threatening features — The progressive course of ALS may eventually produce one or
both of the life-threatening aspects of the disease: neuromuscular respiratory failure and
dysphagia. Respiratory muscle weakness may be the first manifestation of the disease but
more commonly develops after months or years of progressive limb and/or bulbar muscle
weakness.
Progressive neuromuscular respiratory failure is the most common cause of death in ALS. In
the United States, 5 to 10 percent of patients choose tracheostomy and permanent
ventilation when respiratory compromise becomes severe.
Similarly, progressive dysphagia may be one of the initial manifestations of the disease or
may develop after months or years of progressive limb and/or other bulbar weakness.
Dysphagia poses a risk for aspiration of food, liquids, or secretions with resultant pneumonia
and may also lead to malnutrition and dehydration. These conditions can be minimized in
patients who choose gastrostomy tube insertion and with aggressive management of
secretions. (See "Symptom-based management of amyotrophic lateral sclerosis", section on
'Management of swallowing and nutrition' and "Swallowing disorders and aspiration in
palliative care: Definition, pathophysiology, etiology, and consequences" and "Swallowing
disorders and aspiration in palliative care: Assessment and strategies for management".)
The median survival from the time of symptom onset is three to five years. However,
approximately 10 percent of ALS patients can live 10 years or more. Survival beyond 20 years
is possible but rare and in part depends on treatment decisions made by patients and their
families.
SOCIETY GUIDELINE LINKS
Links to society and government-sponsored guidelines from selected countries and regions
around the world are provided separately. (See "Society guideline links: Motor neuron
disease".)
INFORMATION FOR PATIENTS
UpToDate offers two types of patient education materials, "The Basics" and "Beyond the
Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th
grade reading level, and they answer the four or five key questions a patient might have
about a given condition. These articles are best for patients who want a general overview
and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are
longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th
grade reading level and are best for patients who want in-depth information and are
comfortable with some medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to
print or e-mail these topics to your patients. (You can also locate patient education articles
on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)
● Basics topics (see "Patient education: Amyotrophic lateral sclerosis (ALS) (The Basics)")
SUMMARY
● Characteristic clinical features – The clinical hallmark of amyotrophic lateral sclerosis

(ALS) is the combination of upper motor neuron and lower motor neuron signs and
symptoms. Upper motor neuron findings result from degeneration of frontal lobe
motor neurons and the corticospinal tract. The lower motor neuron findings are a direct
consequence of degeneration of lower motor neurons in the brainstem and spinal cord.
(See 'Clinicopathologic features' above.)
● Spectrum of clinical disorder – ALS is the most common form of motor neuron
disease and includes upper and lower motor neuron pathology. The spectrum of motor
neuron disease also includes other conditions that may be variants of ALS or may
https://www.uptodate.com/contents/clinical-features-of-amyotrophic-lateral-sclerosis-and-other-forms-of-motor-neuron-disease/print?topicRef=5138&source… 10/22
represent different patterns of evolution in ALS. These include (see 'Spectrum of motor
neuron disease' above):
• Progressive muscular atrophy

• Primary lateral sclerosis
• Progressive bulbar palsy
• Flail arm and flail leg syndromes
• ALS-plus syndromes
● Clinical symptoms – The primary clinical symptoms and signs of ALS may produce
impairment affecting limb ( table 1), bulbar ( table 2), axial ( table 3), and
respiratory ( table 4) function. (See 'Clinical symptoms and signs' above.)
• Upper motor neuron symptoms result in slowness of movement, incoordination,

and stiffness typically with relatively little overt weakness. (See 'Upper motor neuron
symptoms' above.)
• Lower motor neuron symptoms cause weakness, usually accompanied by atrophy

( movie 1) and fasciculations ( movie 2 and movie 3). Muscle cramps are also
common. (See 'Lower motor neuron symptoms' above.)
• Cognitive impairment, typically related to frontotemporal executive dysfunction,

may precede or follow the onset of upper motor neuron and/or lower motor neuron
dysfunction in patients with ALS. Frontotemporal dementia (FTD) may be associated
with ALS in 15 to 50 percent of cases. (See 'Cognitive symptoms' above.)
• Autonomic symptoms may occur in ALS as the disease progresses. Constipation

occurs frequently and is likely multifactorial. Dysphagia for thin liquids related to
pharyngeal muscle weakness may lead to dehydration, which can exacerbate
constipation. (See 'Autonomic symptoms' above.)
• Extrapyramidal symptoms and signs of parkinsonism may precede or follow the

upper and lower motor neuron symptoms. These extrapyramidal features may
include facial masking, tremor, bradykinesia, and postural instability. (See
'Parkinsonism and supranuclear gaze palsy' above.)
• Sensory impairment may occur in 20 to 30 percent of patients with ALS, but the
sensory examination is usually normal. Nociceptive pain in ALS can arise from a
variety of causes that include reduced mobility, muscle cramps, muscle spasticity,
and comorbid conditions. (See 'Sensory symptoms' above and 'Pain' above.)
● Clinical progression – ALS is a relentlessly progressive disorder with a clinical course

that is generally linear. Symptoms typically spread within the segment of onset and
then to other regions in a relatively predictable pattern. The progressive course of ALS
eventually produces life-threatening neuromuscular respiratory failure and/or
dysphagia. (See 'Clinical patterns of progression' above and 'Life-threatening features'
above.)
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Topic 5136 Version 37.0
GRAPHICS
Limb signs and symptoms associated with amyotrophic lateral sclerosis
Upper motor neuron signs Upper motor neuron symptoms

Spasticity Stiffness, slowness, and incoordination of
movement
Slowed rapid alternating movements
Hand and/or arm
Increased reflexes
Difficulty performing activities of daily
"Preserved" reflexes in weak/atrophic muscles
living
Distal spread of arm reflexes
Difficulty manipulating small objects or
Hoffman sign writing
Crossed adduction Leg and/or foot
Upgoing toe Gait dysfunction
Triple flexion Slow, stiff gait; difficulty turning

Gait disorder Legs "heavy"
Spastic Poor balance and falling
Lower motor neuron signs Spontaneous clonus
Weakness Spontaneous flexor spasms
Intrinsic hand weakness Lower motor neuron symptoms

Foot drop Weakness and atrophy
Proximal arm and leg weakness Arm and/or hand

Poor heel and/or toe walking Difficulty performing activities of daily
living
Poor rise from chair
Poor squat Difficulty manipulating small objects or

writing
Gait disorder
Leg and/or foot
Steppage
Difficulty arising from chairs or from floor
Waddling
Difficulty climbing stairs
Reduced reflexes
Foot drop
Muscle atrophy and fasciculations
Tripping, falling
Fasciculations
Cramps
Graphic 72454 Version 3.0
Bulbar signs and symptoms associated with amyotrophic lateral sclerosis
Increased jaw reflex Jaw stiffness with difficulty opening the mouth
Jaw spasticity Spontaneous clenching or biting
Facial diparesis (may be Trismus

asymmetric)
Spontaneous jaw clonus
Increased facial reflexes
Dysphagia
Palmomental sign
Tongue incoordination disrupts the oral phase
Poor palatal elevation
Pharyngeal muscle incoordination disrupts the pharyngeal
Slow tongue movement phase
Lower motor neuron signs Dysarthria
Weak masseter and/or Labial, lingual, and/or pharyngeal components

pterygoids Spastic with slow, strained speech
Difficulty maintaining jaw closure Laryngospasm
Facial diparesis (may be Often triggered by secretions (eg, saliva) or food particles
asymmetric)
Rapid onset
Poor palatal elevation
"Squeezing" feeling, inability to speak, strained speech
Tongue weakness
Short-lived, less than 30 seconds
Muscle atrophy and
fasciculations Pseudobulbar affect
Inappropriate laughing, crying, and/or yawning
Affective response >> emotional trigger
Mood incongruent
Sialorrhea (drooling)
Difficulty managing pharyngeal secretions
Lower motor neuron symptoms

Incomplete eye closure
Difficulty opening and/or closing the jaw
Difficulty chewing
Disarticulation of the temporomandibular joint when severe
Poor lip closure and seal
May contribute to sialorrhea when severe
Dysphagia
Tongue weakness disrupts the oral phase
Pharyngeal muscle weakness disrupts the pharyngeal phase
Coughing and choking induced by drinking, eating, or saliva

secretion
Often thin liquids followed by solids and thick liquids
Dysarthria
Labial, lingual, and/or pharyngeal components
Slurred, nasal, and/or hoarse speech
Hoarseness
Axial signs and symptoms associated with amyotrophic lateral sclerosis
Absent abdominal reflexes Stiffness and imbalance
Lower motor neuron signs Lower motor neuron symptoms
Neck extension weakness Neck extensors
Truncal extension weakness; bent spine Difficulty holding up the head
Abdominal protuberance When severe produces head drop
Increased lumbar lordosis Truncal extensors
Difficulty maintaining an erect posture
Lumbar extensors
Increased lumbar lordosis
Abdominal wall muscles
Abdominal protuberance
Cramps
Respiratory signs and symptoms associated with amyotrophic lateral

sclerosis
Lower motor neuron signs Lower motor neuron symptoms
Tachypnea Dyspnea and/or orthopnea
Vocal and speech Low speech volume
Reduced vocal volume Weak cough
Shortened sentences Sleep disordered breathing
Frequent breath pauses Frequent nocturnal awakenings, possibly with note of

dyspnea
Use of accessory respiratory
muscles Excessive daytime sleepiness and/or fatigue
Abdominal paradox Morning headache
Confusion
Hallucinations
Contributor Disclosures
Lauren B Elman, MD Grant/Research/Clinical Trial Support: Abbvie Pharmaceuticals [ALS]; Amylyx
[ALS]; Avidity Biosciences [FSHD]; Biogen [ALS]; Biohaven Pharmaceuticals [ALS]; Clene Nanomedicine
[ALS]; Denali Therapeutics [ALS]; Harmony Biosciences [Myotonic Dystrophy]; MLBio Solutions [LGMD];
Prilenia Therapeutics [ALS]; Ra Pharmaceuticals [ALS]; Seelos Therapeutics [ALS]. Consultant/Advisory
Boards: Apellis Pharmaceuticals [ALS]; Biogen [ALS, SMA]; Edgewise Therapeutics [Duchenne/Becker
Muscular Dystrophy]; PTC Therapeutics [Duchenne muscular dystrophy]; Roche/Genentech [SMA]. All of
the relevant financial relationships listed have been mitigated. Leo McCluskey, MD, MBE No relevant
financial relationship(s) with ineligible companies to disclose. Colin Quinn, MD Consultant/Advisory
Boards: Amicus Therapeutics [Pompe disease]; Amylyx Pharmaceuticals [ALS]; Biogen [Expanded
access programs]; Clene Nanomedicine [ALS]; Sarepta Therapeutics [Muscular dystrophy]. Other
Financial Interest: GLG [Consultation]; Guidepoint [Consultation]. All of the relevant financial
relationships listed have been mitigated. Jeremy M Shefner, MD, PhD Grant/Research/Clinical Trial
Support: AB Sciences [ALS]; Amylyx [ALS]; Biogen [ALS]; Cytokinetics Incorporated [ALS]; Ionis [ALS];
Mitsubishi Tanabe Pharma America [ALS]; PTC [ALS]; QurAlis [ALS]; Sanofi [ALS]; Wave Life Sciences
[ALS]. Consultant/Advisory Boards: AcuraStem [ALS]; Amylyx [ALS]; Annexon [ALS]; Apellis [ALS]; Clene
[ALS]; Cytokinetics [ALS]; Denali [ALS]; Eikonizo [ALS]; GSK [ALS]; Mitsubishi Tanabe Pharma America
[ALS]; Neurosense [ALS]; Novartis [ALS]; Revalesio [ALS]; RRD [ALS]; Swanbio [ALS]; Vertex [DMD]. All of
the relevant financial relationships listed have been mitigated. Richard P Goddeau, Jr, DO, FAHA No
relevant financial relationship(s) with ineligible companies to disclose.
Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these
are addressed by vetting through a multi-level review process, and through requirements for
references to be provided to support the content. Appropriately referenced content is required of all
authors and must conform to UpToDate standards of evidence.
Conflict of interest policy

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27.03.

Official reprint from UpToDate®

Clinical features of amyotrophic lateral sclerosis and

Literature review current through: Feb 2024.

● (See "Epidemiology and pathogenesis of amyotrophic lateral sclerosis".)

● The lower motor neuron findings of weakness, atrophy or amyotrophy, and

SPECTRUM OF MOTOR NEURON DISEASE

Progressive muscular atrophy — Progressive muscular atrophy is a progressive lower

ALS-plus syndrome — Classically defined, ALS is considered a degenerative disorder of the

CLINICAL SYMPTOMS AND SIGNS

Cognitive symptoms — There is a well-established link between ALS and frontotemporal

Parkinsonism and supranuclear gaze palsy — Extrapyramidal symptoms and signs of

Clinical patterns of progression — ALS is a relentlessly progressive disorder with a clinical

SOCIETY GUIDELINE LINKS

INFORMATION FOR PATIENTS

● Characteristic clinical features – The clinical hallmark of amyotrophic lateral sclerosis

• Progressive muscular atrophy

• Upper motor neuron symptoms result in slowness of movement, incoordination,

• Lower motor neuron symptoms cause weakness, usually accompanied by atrophy

• Cognitive impairment, typically related to frontotemporal executive dysfunction,

• Autonomic symptoms may occur in ALS as the disease progresses. Constipation

• Extrapyramidal symptoms and signs of parkinsonism may precede or follow the

● Clinical progression – ALS is a relentlessly progressive disorder with a clinical course

Use of UpToDate is subject to the Terms of Use.

15. Chiò A, Calvo A, Moglia C, et al. Phenotypic heterogeneity of amyotrophic lateral

51. Toepfer M, Folwaczny C, Klauser A, et al. Gastrointestinal dysfunction in amyotrophic

57. Oliveira Santos M, Castro I, Castro J, et al. Assessment of sympathetic sudomotor

65. Pugdahl K, Fuglsang-Frederiksen A, de Carvalho M, et al. Generalised sensory system

66. Amoiridis G, Tsimoulis D, Ameridou I. Clinical, electrophysiologic, and pathologic

Limb signs and symptoms associated with amyotrophic lateral sclerosis

Upper motor neuron signs Upper motor neuron symptoms

Crossed adduction Leg and/or foot

Upgoing toe Gait dysfunction

Triple flexion Slow, stiff gait; difficulty turning

Lower motor neuron signs Spontaneous clonus

Weakness Spontaneous flexor spasms

Intrinsic hand weakness Lower motor neuron symptoms

Proximal arm and leg weakness Arm and/or hand

Poor squat Difficulty manipulating small objects or

Graphic 72454 Version 3.0

Bulbar signs and symptoms associated with amyotrophic lateral sclerosis

Upper motor neuron signs Upper motor neuron symptoms

Jaw spasticity Spontaneous clenching or biting

Facial diparesis (may be Trismus

Lower motor neuron signs Dysarthria

Weak masseter and/or Labial, lingual, and/or pharyngeal components

Inappropriate laughing, crying, and/or yawning

Affective response >> emotional trigger

Difficulty managing pharyngeal secretions

Lower motor neuron symptoms

Difficulty opening and/or closing the jaw

Disarticulation of the temporomandibular joint when severe

Poor lip closure and seal

May contribute to sialorrhea when severe

Tongue weakness disrupts the oral phase

Pharyngeal muscle weakness disrupts the pharyngeal phase

Coughing and choking induced by drinking, eating, or saliva

Often thin liquids followed by solids and thick liquids

Labial, lingual, and/or pharyngeal components

Slurred, nasal, and/or hoarse speech

Graphic 75146 Version 2.0

Axial signs and symptoms associated with amyotrophic lateral sclerosis

Upper motor neuron signs Upper motor neuron symptoms

Absent abdominal reflexes Stiffness and imbalance