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Transcript
Bell’s Palsy, Muscular
Dystrophies -Erb’s Palsy
Victor Politi, M.D., FACP
Medical Director, SVCMC, School of Allied
Health Professions, Physician Assistant Program
Facial Nerve
• 7th cranial nerve supplies all the muscles
concerned with facial expression
• Small sensory component (the nervus
intermedius of Wrisberg); conveys taste
sensation from the anterior 2/3 of the tongue
• Motor nucleus of 7th nerve lies anterior and
lateral to the abducens nucleus
Facial nerve
• A complete interruption of the facial nerve
at the stylomastoid forearm paralyzes all
muscles of facial expression
–
–
–
–
corner of mouth droops
crease and skin folds effaced
forehead un-furrowed
eyelids will not close
Facial Nerve
• Food collects between the teeth and lips,
and saliva may dribble from the corner of
the mouth
• The patient typically c/o a heaviness or
numbness in the face
Facial palsy is usually unilateral
and may be due to trauma,
surgical intervention, tumor,
stroke or infection of the 7th
cranial nerve
The Most Common Form of
Facial Paralysis is idiopathic -Bell’s Palsy
Bell’s Palsy is a form with acute
onset and unknown cause,
possibly viral infection
Edema may play a part leading
to compression of nerve fibers,
with resulting acute unilateral
paralysis of facial muscles
Bell’s palsy afflicts approximately
40,000 Americans each year
It disproportionately attacks
pregnant women and people who
have diabetes, influenza, a cold
or some other upper respiratory
ailment
The common cold sore virus,
herpes simplex, and other
herpes viruses are the likely
cause of many cases of Bell’s
palsy
In addition to one-sided facial
paralysis with possible inability
to close the eye, symptoms of
Bell’s palsy may include pain,
tearing, drooling, hypersensitivity
to sound in the affected ear, and
impairment of taste
The corner of the mouth droop,
and there may be difficulty in
speech, and eating
The symptoms usually
disappear spontaneously but
residual facial immobility and
lip drooping may persist
Bell’s Palsy
• In general, the prognosis for Bell’s Palsy is
very good
• With or without treatment most patients
begin to get significantly better within 2
weeks
Bell’s Palsy
• For some, however, the symptoms may last
longer
• In a few cases, the symptoms may never
completely dissapear
Treatment
• Studies have shown that steroids are
probably the most effective treatment
• Acyclovir combined with prednisone is
possibly effective in improving facial
function
• Other treatments are usually aimed at
protection of the eye from drying during
sleep
Treatment
• Analgesic for pain relief
• Corticosteroid drug to help reduce
inflammation
• Massage of weakened muscles
• Splint to prevent drooping of lower part of
face
Muscular dystrophy refers to a
group of disorders that have
little in common except for their
name and the fact that they are
inherited
Each type of muscular
dystrophy has unique
phenotypic and genetic features
Progressive Muscular
Dystrophies
• Congenital
• Distal
• Scapuloperoneal
Progressive Muscular
Dystrophies
• Duchenne Muscular Dystrophy
(pseudohypertrophic)
• Becker’s (benign pseudohypertrophic)
• Myotonic
• Facioscapulohumeral
• Limb-Girdle (may include several
disorders)
• Oculopharyngeal
Duchenne Muscular Dystrophy
•
•
•
•
•
•
•
•
X-linked recessive
Affects males almost exclusively
Onset by age 5
Progressive weakness of girdle muscles
Inability to walk after age 12
Kyphoscoliosis
Respiratory failure - 2nd-3rd decade
Other organ system involvementcardiomyopathy/ mental impairment
Becker’s Muscular Dystrophy
• Less severe form of x-linked recessive dystrophy
• presentation similar to Duchenne’s except time
course slower
• onset early to late childhood (not usually recognized
until age 5)
• walking continues beyond age 15 - sometimes into
4th decade
• Calf muscle enlargement - prominent
• Death from complications similar to Duchenne’s
may occur after age 40
Facioscapulohumeral Muscular
Dystrophy
•
•
•
•
•
Autosomal dominant disorder
Slowly progressive disorder
Affects males and females equally
Extremely variable in severity
Most common in 3rd or 4th decade, but may
start at any age
• Cases starting earlier in life - worse
prognosis
Facioscapulohumeral Muscular
Dystrophy
• Some patients may remain asymptomatic
throughout life
• Weakness of facial, shoulder girdle,
proximal arm muscles and foot dorsiflexion
weakness
• Other organ system involvement - HTN
Oculopharyngeal Muscular
Dystrophy
• Autosomal dominant (French-Canadian or
Hispanic background)
• Onset in 5th to 6th decade of life
• Slowly progressive weakness of extraocular,
eyelid, face, and pharyngeal muscles
• Cricopharyngeal achalasia
• Other organ system involvementcardiomyopathy
Scapuloperoneal Dystrophy
• Autosomal dominant
• Onset 3rd to 5th decade of life
• Progressive shoulder girdle and foot
dorsiflex or weakness
• Other organ system involvement cardiomyopathy
Limb-Girdle dystrophy
• Autosomal recessive inheritance
• Onset early childhood to adult
• slowly progressive weakness of shoulder
and hip girdle muscles
• Other organ system involvement cardiomyopathy
Limb-Girdle Dystrophy
• Proximal muscle weakness may begin in
either the legs or the arms but usually
progresses to all extremities
• Weakness may begin before age 5 or as late
as the 3rd decade of life
Diagnosis
• Serum CK is elevated
• EMG pattern that of myopathy
• Muscle biopsy shows active myopathy but
nonspecific
Physical therapy and orthopedic
procedures may help to
counteract deformities or
contractures
Brachial Plexus Injuries
• A brachial plexus injury (Erb’s palsy) is a
nerve injury
• The nerves that are damaged control
muscles in the shoulder, arm, or hand and
any or all of these muscles may be
paralyzed
Brachial Plexus Injury
• The brachial plexus is a network of nerves,
conducting signals from the spine to the arm
and the hand
• Injury can occur at any time, most brachial
plexus injuries happen during birth
– about 1-2 babies in 1,000 suffer brachial plexus
injury at birth, about 1 in 10 of these need
treatment
– treatment can consist of exercise, therapy,
surgery or any combination thereof
Brachial Plexus Injuries
• Four types of nerve injury
– Avulsion – Rupture– Neuroma– Praxis-
the nerve is torn from the spine
nerve torn, but not where it
attaches to the spine
scar tissue forms around injury
nerve damaged but not torn
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