an aquired autoimmune disorder associated with deficiency of acetylcholine receptors at the NeuroMuscular junction
In Myesthenia gravis, __ take up space on the acetylcholine receptors
myesthenia gravis may also be due to increased
amount of acetylcholinesterase
Signs and symptoms of Myesthenia gravis
weakness and fatigue of muscles, Usually begins with eyelids (ptosis), symptoms fluctuate through day though improve with rest
Medical treatment of Myesthenia Gravis:
Drugs to deactivate acetylchoninesterase, High dose steroids, gamma globulin, other immunomoderlators, thymectomy, plasmapheresis
Physical therapy and Myesthenia Gravis
Rarely needed. Most pts live normal lives. If referred to PT activities should be light, short in duration and few reps
Common symptom to all muscular dystrophies
The term muscular dystrophy refers to
a group of steady progressive, degenerative myopathies with genetic determination
Duchenne's Muscular Dystrophy is
the most common form of MD, affects boys only (asymptomatic mother to male child), boy usually dies by age 20
Gower's sign is __ and is associated with __
child who must "crawl" up his legs to rise to standing (due to hip weakness), in Duchenne's MD
Symptoms of Duchenne's usually appear before age
3. May be significant by age 7
Hypertrophy of calf muscles occurs in
Intellectual/emotional disturbances with Duchenne's?
Children with Duchenne's often end up
Obese and in a wheelchair
Impairments with Duchenne's Muscular Dystrophy
weakness, decreased AROM and PROM, loss of ambulation, decreased pulmonary function, lumbar lordosis (b/c of imbalanced hip muscles), scoliosis
Myotonic muscular dystrophy is
an autosomal dominant disorder, first noticed in adolescence and characterized by myotonia and muscle weakness
a delay in muscle relaxation time
as weakness increases, myotonia
Characteristic appearance of Myotonic muscular dystrophy
long thin face with temporal and masseter muscle wasting, frontal balding, weakness and wasting of SCM
Myotonic muscular dystrophy patients present with weakness and wasting...
distally (foot drop,difficulty opening jars)
Fascioscapulohumoral MD is
autosomal dominant disorder involving face, shoulder girdle muscles (asymmetrical scapular winging) progressing into pelvic girdle. Patients wear corset-like back support and are active throughout adulthood.
Limb girdle MD affects
proximal shoulder girdle and proximal pelvic girdle and LE muscles.
Limb girdle MD can begin
any time, but more severe when starts in childhood
with all forms of Muscular Dystrophy, it is important to exercise but...
not to the point of fatigue
Limb girdle MD is not fatal but...
if the heart or respiratory muscles become involved, this could lead to death
Myesthenia Gravis affects what part of the body?
Begins with eyelids,proceeds to throat, then trunk then limb
Spinal muscular atrophy affects only children, true or false?
What is Gower's sign?
Crawling up legs in order to come ot standing
The common complaint of patients with neuromuscular diseases