WEAKNESS AND

HYPOTONIA
Prepared by
Dr Hodan Jama MD
Objectivies
To differentate upper motor neuron disease vurses
lower motor neuron disease
Difine hypotonia
Compare and contrass weakness and hypotonia
Weakness is a decreased ability to voluntarily and
actively move muscles.
may be generalized or localized
Hypotonia is a state of low muscle resistance to
movement.
 Hypotonia can be associated with weakness
 but in some cases is present with normal motor
strength.
ETIOLOGY

Upper motor neurons

Lower motor neurons

Upper motor neurons

Upper motor neurons originate in the

cerebral motor cortex

their axons form the corticospinal tract
ending in the spinal cord and control
voluntary motor activity.
lower
motor neurons

The anterior horn cells,

their motor roots,
peripheral motor nerves,
neuromuscular
junctions
Maintenance of normal strength tone coordination
requires integratecommunication throughout this
complex system including
 the cerebral cortex
 cerebellum
 brainstem
thalamus
basal ganglia
spinal cord
CLINICAL MANIFESTATIONS
Upper motor neurons

control, but not total loss of movement because motor

nuclei o the basal ganglia, thalamus, and brainstem
have tracts that produce simple or complex
stereotyped patterns of movement.
The corticospinal tract permits fine motor activity and
is best tested by
 rapid alternating movements of the distal extremities.
 Mild dysfunction produces slowed, stiff motions
More severe dysfunction produces
 stiff
 abnormal involuntary postures (spasticity) consisting
of
 forearm flexion at the elbow
 wrist and adduction close to the chest, with leg extension and
adduction
 Damage to the spinal cord leaves residual simple,
stereotyped reflex movements coordinated by
local spinal reflexes below the level of the lesion.
Destruction of the lower motor
Neuron, the final common pathway producing muscle
activity,
 leads to total absence of movement with hypotonia
Function is best tested by
measuring the strength of individual muscle groups
 in a young child, by observing the ability to perform tasks
requiring particular muscle groups
 walk up or down stairs,
 arise from the ground,

 walk on toes or heels,

 raise the hands above the head,

 squeeze a ball).
DISEASE OF THE UPPER MOTOR NEURON
Tumors, trauma, infections, demyelinating
syndromes, infarction,
metabolic diseases, and degenerative diseases may
injure
the corticospinal tract, producing an upper motor
neuron pattern
of weakness coupled with increased deep tendon
reflexes,
spasticity, and extensor plantar responses (Babinski
sign).
Clinical Manifestations

The distribution of weakness depends on the location

of the
lesion. A tumor in the left parietal region may produce
a right
hemiparesis. A brainstem glioma may produce a
slowly progressive
quadriparesis. A diffuse disorder of myelin synthesis,
such as a leukodystrophy, would produce a progressive
symmetrical
quadriparesis.
Acute spinal cord lesions, such as infarction or
compression, may produce a flaccid, areflexic
paralysis that mimics lower motor neuron disease.
 A child who exhibits an acute or subacute flaccid
paraparesis is most likely to have either an acute cord
syndrome or Guillain-Barré syndrome.

The hallmarks of spinal cord disease are :
sensory level
 motor level
disturbance of bowel and bladder function
 local spinal pain or tenderness
 The acute cord syndrome may be the result of
transverse
Peripheral Neuropathy

There are many peripheral nerve diseases in

childhood, but the most classic presentations are:
 Guillain-Barré syndrome
chronic inflammatory demyelinating polyneuropathy
(CIDP)
hereditary motor sensory neuropathy (HMSN)
 tick paralysis
Guillain-Barré syndrome
Acute inflammatory demyelinating polyradiculoneuropathy is
postinfectious autoimmune peripheral neuropathy that can
occur about 10 days after a respiratory or gastrointestinal
infection

(classically Mycoplasma pneumonia or Campylobacter jejuni).

It occurs in people of all ages and is the most common cause of

acute flaccid paralysis in children.
The characteristic
Areflexia,
 flaccidity,
 symmetrical ascending weakness
 Progression can occur rapidly, in hours, or more
indolently over weeks.

Typically symptoms start with
 numbness or paresthesia in the hands and feet,
then a heavy,
weak feeling in the legs
Weakness ascends to involve the
 arms
 trunk
 bulbar muscles
 tongue, pharynx, larynx
Deep tendon reflexes are absent even when strength is
relatively preserved.
This polyneuropathy can be difficult to distinguish
from an acute spinal cord syndrome.
 Preservation of bowel and bladder function, loss of
arm reflexes, absence of a sensory level, and lack of
spinal tenderness point toward Guillain-Barré
syndrome.
The cerebrospinal fluid in Guillain-Barré syndrome is
sometimes normal early in the illness but classically
shows elevated protein levels without significant
pleocytosis.
 Magnetic resonance imaging (MRI) with gadolinium
may reveal enhancement of the spinal nerve roots.
 Electrophysiology studies (EMG, nerve conduction
velocity [NCV]) are not always required but can
provide corroborative diagnostic evidence and
prognostic indicators.
Children in early stages of the disease should be
admitted to the hospital.
Those with moderate, severe, or rapidly progressive
weakness should be cared for in an intensive care unit.
Pulmonary and cardiac functions are monitored
continuously.
Endotracheal intubation should be performed in
patients with impending respiratory failure or an
inability to clear secretions.
patients are treated initially with intravenous
immunoglobulin (IVIG).
 Plasma exchange and immunosuppressive drugs are
alternatives when IVIG treatment is unsuccessful or in
rapidly progressive disease.
Physical, occupational, and speech therapies are
mainstays of treatment.
The illness usually resolves spontaneously, albeit
slowly; 80% of patients recover normal function
within 1 to 12 months.
Twenty percent of patients are left with mild to
moderate residual weakness.
 Some children will suffer acute relapse or chronic
symptoms.
Duchenne Muscular Dystrophy

Muscular dystrophies are a group of genetic muscle

diseases
characterized by progressive myofiber degeneration
and the gradual replacement of muscle by fibrotic
tissue.
 Duchenne muscular dystrophy is the most common
muscular dystrophy
and one of the most common genetic disorders of
childhood.
 Duchenne muscular dystrophy is an X-linked
disorder (Xp21) affecting approximately 1 in 3500
boys
 Resulting from a gene mutation of dystrophin.
 Becker muscular dystrophy is an allelic disorder
associated with more mild symptoms
 its mutations at least partially preserve the function
of the resulting gene product.
Infant boys are only rarely symptomatic in early
infancy.
 At about 2 to 3 years of age, boys develop
an awkward gait and an inability to run properly.
Some have an antecedent history of
 mild slowness in attaining motor milestones
 poor head control during infancy.
Examination shows
firm calf hypertrophy
 mild to moderate
proximal leg weakness
with a hyperlordotic
waddling gait
 Gower sign
Arm weakness is evident by age 6 years, and most boys
are wheelchair dependent by age 12 years.
 Other manifestations include cardiomyopathy,
scoliosis, respiratory decline, and, in some boys,
cognitive and behavioral dysfunction.
Many boys with Duchenne live into adulthood.
Most die in their 20s or early 30s, usually as a result of
progressive respiratory decline or cardiac dysfunction.
Laboratory and Diagnostic Studies

Serum CK levels are always markedly elevated

 Diagnosis is established by genetic testing for the
dystrophin gene mutation.
Prenatal diagnosis is possible.
 Approximately one third of cases represent new
mutations.
 Occasionally, the diagnosi is not made until a muscle
biopsy shows muscle fiber degeneration and
regeneration accompanied by increased intrafascicular
connective tissue.
Treatment

Steroid therapy is now instituted to slow the pace of

the disease and delay motor disability.
 Supportive care includes physical therapy, bracing,
proper wheelchairs
 Treatment of cardiac dysfunction or pulmonary
infections
A multidisciplinary approach is recommended.
Through improvements inmanagement, patients are
now living significantly longer than in the pre-steroid
era
The most common causes of acute
ataxia
postinfectious acute cerebellar ataxi
 drug intoxications.
 Discrete lesions within the posterior fossa, tumors
 (e.g., medulloblastoma, ependymoma, cerebellar
astrocytoma), multiple sclerosis, strokes, and
hemorrhages may cause ataxia.
 Other causes include benign paroxysmal vertigo,
head trauma, seizures, postictal states, migraine,
paraneoplastic opsoclonus-myoclonus syndrome
associated with neuroblastoma, and inborn errors of
metabolism.
 Congenital disorders also may produce chronic,
nonprogressive ataxia.
Drug intoxication is the most common cause of acute
ataxia
among children. Overdosage with any sedative-hypnotic
agent
can produce acute ataxia and lethargy, but ataxia without
lethargy
usually results from intoxication with ethanol or
anticonvulsant
drugs. It is important to ask about any medications or drugs of
abuse the patient may have access to. Treatment is supportive.
Postinfectious acute cerebellar ataxia may occur 1 to
3 weeks following varicella, infectious mononucleosis, mild
respiratory or gastrointestinal viral illnesses, or other infections.
The pathogenesis is uncertain and may represent either
a direct viral infection of the cerebellum or, more likely, an
autoimmune response precipitated by the viral infection
and directed at the cerebellar white matter. Symptoms begin
abruptly, causing truncal ataxia, staggering, and frequent
falling. Dysmetria of the arms, dysarthria, nystagmus, vomiting,
irritability, and lethargy may be present. Symptoms,
which may be severe enough to prevent standing or sitting,
usually peak within 2 days, then stabilize and resolve over
The usual symptoms of ataxia are a broad-based, unsteady
gait (truncal ataxia) and intention tremor or dysmetria (overor
undershooting of the target due to abnormal distance
perception). An intention tremor worsens as the arm/hand
approaches the target. Classically, these symptoms stem from
disorders of the cerebellar pathways, but peripheral nerve
lesions causing loss of proprioceptive inputs to the cerebellum
(Guillain-Barré syndrome) may present with similar symptoms.
In addition, weakness may intensify or mimic ataxia, so
strength must be assessed, along with coordination.