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LMNL VS.

UMNL
DAYNA RYAN, PT, DPT
WINTER 2012

GENERAL TERMINOLOGY
PNS: motor units and associated sensory connections
Includes cranial nerves
CNS: brain and spinal cord
Sign = an objective finding revealed upon physical
examination
Symptom = (subjective) functional components of a disease
perceived by a patient and expressed in a patients history

Peripheral nerve conditions

Bells Palsy
Guillain-Barre
Charcot-Marie-Tooth Disease
Polio and post-polio syndrome
Diabetic polyneuropathy
Alcoholic polyneuropathy
Hansens Disease (leprosy)

Mixed peripheral nerve / spinal cord


conditions
Myotrophic lateral sclerosis (ALS)
Tabes dorsalis

LOWER MOTOR NEURON


LESIONS
Most common signs/symptoms: pain and weakness
Muscle weakness
Paralysis (plegia) = total or severe loss of voluntary
contraction
Paresis = mild or partial loss of voluntary contraction
characteristic of LMNLs
Atrophy common feature of many neuromuscular disorders
-if you dont use it u lose it.
Hypertrophy
Compensatory activation of synergistic muscles to support
weakened muscles may result in compensatory hypertrophy)
Causes muscle imbalance, and can lead to pain.

MUSCLE HYPERACTIVITY
Fasciculations = rapid, fine, painless contraction of groups
of muscle fibers
Visible but not strong enough to move limbs
Commonly seen in anterior horn cell disorders=motor cells
(e.g. ALS)
Cramps = painful muscle spasms
When violent, referred to as convulsions
Clonic: alternating contraction and relaxation
Tonic: sustained contraction=charley horses
Also associated with anterior horn cell disorders

TETANY
= involuntary muscle twitching, spams, or cramps occurring
as a result of a hyperexcitable peripheral nerve
**can also occur in CNS disorders
Fibrillation: small, asynchronous contractions occurring
in a single skeletal muscle fiber
Too small to see (need an EMG to detect)
Present following denervation and with some myopathies
Myotonia: delayed capacity for relaxation in skeletal
muscle (tone disorder)
Contracture: tightness or restricted ROM across a joint
(common for Parkinson's, due to basal ganglia)

LMNL CHARACTERISTICS
Muscle Fatigability (myasthenia)
Decreased muscle endurance because cannot recruit
because of lack of innervation
Hypotonia: decreased muscle tone
Increased risk of subluxation (shoulders, scoliosis)
Abnormal Sensation
Often an early presenting sign of PNS dysfunction
Paresthesias = tingling or pins and needles sensations
Decreased or absent DTRs
Due to disrupted reflex arc (motor or sensory path)
Myopathies exhibit this because muscle cannot respond to
the nerve impulse

SENSORY ABNORMALITIES
THAT ACCOMPANY LMNLS
Anesthesia: Loss of feeling or sensation,
especially the loss of pain.
Hypesthesia: Decreased sensitivity to
sensory stimulation; also called hypoesthesia.
Paresthesia: Abnormal sensation such as
burning, pricking, tickling, or tingling,
especially occurring spontaneously.
Hyperesthesia: Exaggerated unpleasant
sensitivity to touch or other non-noxious
sensory stimuli.

SENSORY ABNORMALITIES
THAT ACCOMPANY LMNLS
Hypalgia: Diminished sensitivity to painful
stimuli:; also called hypoalgia.
Hyperalgia: Excessive sensitivity to painful
stimuli.
Causalgia: sensation of persistent, severe
burning of the skin, often accompanied by
hypersensitivity to touch and temperature
and trophic skin changes

*EXAM*: UPPER MOTOR NEURON


LESIONS
Spasticity = velocity-dependent increased muscle tone accompanied
by hyperactive DTRs
Any damage to the corticospinal tract causes spasticity (crosses at
medulla pyramids)
Predominantly in anti-gravity muscles (UE flexors, LE extensors)
Caused by lack of inhibition to control excitability of alpha motor neurons
Rigidity = increased resistance to passive stretch that is velocity
independent and uniform throughout ROM
-much harder to stretch than spasticity
Occurs in basal ganglia and extrapyramidal disorders
Cogwheel: rhythmic, interrupted resistance (Parkinsons=only really
sever)
Decorticate: usually from lesions in the thalamus, cerebral white matter,
or internal capsule
Decerebrate: from lesions in midbrain or diencephalon

RIGIDITY FROM BRAIN INJURY

Fredericks &
Saladin book
Pathophysiol
ogy of the
Motor
Systems

UPPER MOTOR NEURON


LESIONS
Hypotonia: associated with cerebral or spinal shock,
followed by an increase in spasticity
Muscle Weakness/Generalized Fatigue
significant atrophy is rare since reflex activity is still present
Poor Coordination
Loss of fractionation
Movement timing: increased reaction time & increased
movement time (time to build up contraction and complete
movement)
Involuntary movements (tremors, choreas, athetosis, etc)

UPPER MOTOR NEURON


LESIONS
Associated reactions = unintentional movement of one limb
that often occurs during the intentional movement of another
limb
Ataxia = unsteadiness, incoordination, or clumsiness of
movement
Movement is jerky and unprecise
Difficulty regulating force, direction, and velocity of movement
Common in cerebellar lesions
Apraxia = inability to perform a goal-directed motor activity in
the absence of paresis, ataxia, or disturbance of muscle tone
Sensory deficits larger areas compared to PNS lesions

BRAIN STEM

Lesion

Discriminative Touch and


Proprioception

Pain and Temp

Upper midbrain

Loss is all contralateral

Loss is all contralateral

Posteriolateral medulla or
lower pons

Not affected in face


because tracts and nuclei
are superior to medulla.
Not affected in body
because tracts are more
medial.

Pain and temp loss


ipsilateral in face (CNV
uncrossed) and
contralateral in body.

Medial medulla or lower


pons.

Medial lemniscal axons


cross midline in lower
medulla so have
contralateral loss in body.

Spinalthalamic,
Contralateral face affected
from second order neurons
from CNV

Posterolateral upper pons


or midbrain after all tracts
from body have crossed
midline

Contralateral in body.
Proprioception is ipsilateral
in face (only the CNVprop
fibers have not crossed at
this level).

Contralateral in face and


body

BRAINSTEM LESIONS

BRAINSTEM LESIONS

BELLS PALSY
Innervation to
upper face is
bilateral
Innervation to
lower face is
unilateral (from
opposite
hemisphere)
a is Bells Palsy
b is stroke

Person in a and b
requested to
smile and close
eyes

BELLS PALSY:
ETIOLOGY
Unknown in most cases
May be secondary to viral infection causing
swelling in auditory canal or exposure to cold
temperatures
In a small number of cases, secondary to
acoustic neuroma impinging on nerve

BELLS PALSY: SIGNS


& SYMPTOMS
Motor: flaccidity
Mouth droops
Nasolabial fold is
flattened
Eyelid does not close

Sensory:
Decreased taste on
ipsilateral tongue

ANS:
Decreased tearing (dry
eye)

Characteristic Smile

BELLS PALSY:
INCIDENCE AND ONSET
Incidence is 20/100,000 in US each year (affects
20,000-100,000 people in US / year)
Typical onset is overnight
Onset more common between 20-40 y.o.
(increased incidence with increased age)
Increased risk in diabetics and pregnant women
and people with MS

BELLS PALSY:
MEDICAL TREATMENT
High-dose corticosteroids for 5 days followed by a
tapered dose for another 5 days
Antiviral medications, e.g. acyclovir
Because of positive association between HSV and
Bells Palsy
Improves outcomes when paired with corticosteroids

Eye patch, artificial tears (methylcellulose eye


drops every 4 hours)
Gentle massage and gentle heat occasionally used
PT for muscle retraining only if problems persist

DIABETIC
POLYNEUROPATHY
Affects PNS axons primarily (some demyelination)
Etiology: disrupted microcirculation
Onset:
After long duration diabetes
In diabetics who have had diabetes for 25+ years, 50% have
this condition

Occurs in insulin-dependent and non-insulin dependent


diabetes
Some regeneration with control of diabetes

DIABETIC
POLYNEUROPATHY
Characteristics
Large nerve fiber involvement (most common)
painless paresthesias in feet and lower legs
decreased vibration and proprioception sense
decreased DTRs

Small nerve fiber involvement


deep aching pain in legs and burning feeling in feet
decreased touch, pain, and temperature sensations
nocturnal pain and paresthesias

DIABETIC
POLYNEUROPATHY
Screening/Examination Tests
NCVs
Monofilament screening with 5.07 / 10 gm. filament
Vibration
Complications
Diabetic ulcers (reducing WB helps)
50% of non-traumatic amputation in US are in diabetic patients

ALCOHOLIC
POLYNEUROPATHY
Affects PNS axon and myelin
Characteristics
Distal muscle weakness and atrophy
Sensory involvement greater in LEs than in UEs
Pain and paresthesias in distal legs and soles of
feet (this does not typically happen in the diabetic
polyneuropathy)
Aching in calf muscles
Decreased ankle reflexes and poor sensation in
feet

ALCOHOLIC
POLYNEUROPATHY
Cause
Insidious onset and slow progression
Exacerbations may occur
Sensory signs and symptoms before motor
Mild case = mild aching in calf and feet
Severe case = severe motor and sensory signs
Prognosis
with abstinence and improved nutrition, a slow,
incomplete recovery may occur
considerable discomfort may remain

STOCKING-GLOVE PATTERN OF
SENSORY IMPAIRMENT

CHARCOT-MARIE-TOOTH (CMT)
DISEASE
Also known as:
Hereditary Motor and Sensory Neuropathy Type I (HMSN)
OR
Peroneal Muscular Atrophy
Lesion site = PNS axon and PNS demyelination, anterior
horn cell and dorsal root ganglion
Onset
Hereditary
Late childhood or adolescence for CMT1
Adulthood for CMT2 (less severe with minimal sensory
loss)
Slowly progressive

CHARCOT-MARIE-TOOTH (CMT)
DISEASE
Characteristics
Pes cavus foot deformity (high arch) and hammer
toes
Symmetric weakness and atrophy in intrinsic foot,
peroneal, and anterior tibialis muscles
Weakness and atrophy rarely go above knee or
elbow
Paresthesias are common
Lead to foot drop (see steppage gait pattern)
Normal lifespan most remain ambulatory throughout
their lives

CHARCOT-MARIE-TOOTH
DISEASE
Foot deformity: high arch pes cavus foot deformity with
hammer toes

TABES DORSALIS
Lesion site: dorsal roots & posterior columns of lumbosacral
and lower thoracic S.C.
Etiology: From syphilis
Diagnosis based on:
Neuroimaging is normal
Abnormal tibial sensory NCVs
CSF has elevated protein level
Significant sensory loss!

TABES DORSALIS
Characteristics
Unilateral or bilateral presentation
Diminished proprioception and vibration in legs
Sensory ataxia of gait
Areflexia
Touch, pain, and temperature sensation are intact
Aching / paresthesias (radicular distribution)
May also have:
Autonomic disturbances (bladder atony(incontinence),
papillary abnormalities, impotence)
Later onset of distal muscle weakness and atrophy (if
anterior horn cells become involved)

HANSENS DISEASE
(LEPROSY)
Lesion site: Peripheral
nerves
Etiology: Mycobacterium
leprae infection
Two forms:
Tuberculoid
Primarily superficial nn

Leprous
Weakness - symmetric
Sensory loss
Skin lesions

GUILLAIN-BARRE
SYNDROME (GBS)
Alternate Name: Acute Inflammatory
Demyelinating Polyradiculoneuropathy = AIDP
Many variants of the syndrome exist
Lesion site
PNS myelin (secondary axonal degeneration in
many patients)
Etiology
autoimmune disorder with unknown trigger (may be
secondary to an upper respiratory or GI infection)

GUILLAIN-BARRE SYNDROME
(GBS)
Primary Characteristics
Weakness
typically symmetric, beginning in distal LE muscles and
ascending through body rapidly including facial muscles
facial and palatal weakness is common ( in about 50% of
cases)

Paresthesias (usually transient)


Diminished or absent DTRs
Flaccid muscle tone

GUILLAIN-BARRE SYNDROME
(GBS)
Early symptoms
difficulty with walking
paresthesia in toes (commonly 1st symptom)
muscle tenderness (tender to touch)
About 50% have respiratory involvement
up to 30% of GBS patients require mechanical ventilation
during the acute phase
Dysarthria=slurring of words, dysphagia=difficulty
swallowing, and diplopia=double vision develop in severe
cases

GUILLAIN-BARRE SYNDROME
(GBS)
Can occur at any age, but mostly 5th-8th decade
Disease course:
Maximal onset in less than 4 weeks, many in a few days
Static phase (plateau of 2-4 weeks)
Recovery takes months to years
Recurs in 10% of cases

Static Phase
Recovery

Onset

Weeks

GUILLAIN-BARRE SYNDROME
(GBS)
5% mortality rate
At 6 months, 85% are ambulatory
At 1 year, 20% remain significantly handicapped by
weakness
At 2 years, 8% have not achieved full recovery
Poorer prognosis with:

onset at an older age


protracted time before recovery begins
need for artificial respiration
significantly decreased amplitude of evoked motor potential
(a sign of axonal degeneration)

POLIO & POST-POLIO


SYNDROME (PPS)
Also called: PostPolio Muscular
Atrophy (PPMA)
Lesion site:
anterior horn cells
(little CN
involvement)

Etiology
Polio virus initially.
Existing motor neurons reduce
the number of collateral sprouts
resulting in more muscle cell
death.
As motor neurons die with age,
there is no redundancy in the
system for other motor units to
take over, so motor function is
decreased.
NOT due to reactivation of the
polio virus.

POLIO & POST-POLIO


SYNDROME (PPS)
Decades after onset of polio (mean of 25
years post-polio onset)
to of polio survivors are expected to
develop post-polio syndrome (some
sources say up to 2/3 of all polio survivors
will get PPS)
more common in women than men
occurs more frequently in those with more
severe initial polio symptoms

POLIO & POST-POLIO


SYNDROME (PPS)
Signs and Symptoms
Sensation
Not affected
Joint and muscle pain
Intolerance to cold

Motor
Paresis or flaccid
paralysis
Decreased or absent
DTRs=lmn
Decreased endurance for
physical activity

Exercise recommendations:
Never exercise to point of
fatigue (use general body
conditioning and low resistance
exercises)
Monitor vitals before and after
exercise (remember that
respiratory muscles are also
affected)
Caution patient to stop exercise
if pain persists or weakness
increases

POLIO & POST-POLIO


SYNDROME (PPS)
Original signs &
symptoms
Asymmetric paralysis
Leg affected more
than arm
More severe
proximally than
distally
No eye muscles
involved (CN
involvement usually
only temporary)

Post-polio S & S
Decreased
strength in
previously affected
muscles
New muscle
weakness
Increased muscle
atrophy
Myalgia
Joint pain

AMYOTROPHIC LATERAL
SCLEROSIS (ALS)
Also called Lou Gehrigs
Disease
Lesion site: anterior horn
cells, lateral
corticospinal tract, motor
nuclei of brainstem, and
motor area of frontal lobe
(pre-central cortex)
Etiology unknown except
in a few inherited cases

AMYOTROPHIC LATERAL
SCLEROSIS (ALS)
LMN signs
Progressive muscle wasting
Weakness (asymmetric weakness is often presenting
sign)
Fasciculations (especially evident in tongue)
cramps

UMN signs
Spasticity
Hyperreflexia
Positive Babinski

AMYOTROPHIC LATERAL
SCLEROSIS (ALS)
Most common to see:

Weakness (greater in UEs than in LEs)


Atrophy
Fasciculations
Increased DTRs
Positive Babinski

Later may see dysphagia and dysarthria


Onset:
90% of all cases have onset between 40-70 years of age

Course is variable and progressive to all striated


muscles (except extraocular)
Death typically secondary to respiratory muscle
involvement

AMYOTROPHIC LATERAL
SCLEROSIS (ALS)
Prognosis
Death in 2-5 years commonly from respiratory
compromise (mean death is 3-4 years after onset if
patient does not get ventilatory support)
20% survive more than 5 years
those who have ALS before age 50 generally live
longer

AMYOTROPHIC LATERAL
SCLEROSIS (ALS)
Functions typically preserved throughout disease:
Intellect is not affected
Eye movements
Bowel and bladder control

Exercise recommended to:


prevent disuse atrophy
maximize strength in remaining innervated muscle cells

Common problems with swallowing, speech, postural control,


and respiration
Treat pain with modalities such as TENS
Scapulohumeral joints may be affected with increased muscle
weakness . . . Can get glenohumeral joint subluxation