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ALS stands for

Amyotrophic Lateral Sclerosis

A rapidly progressive disorder associated with degeneration of motor nerve cells, replacing them with scar tissue


Typical lifespan post-ALS diagnosis

5 years

Neurons affected by ALS

both upper motor and lower motor neurons including brainstem, spinal cord and corticospinal tract

Median age of onset of ALS


ALS affects men more often than women in what ratio


The most common cause of death associated with ALS

respiratory failure

This percent of ALS cases are familial


One theory of the cause of ALS is

buildup of free radicals in the body

ALS is often diagnosed by

Exclusion, EMG can confirm fasiculations

Signs and symptoms of ALS (9)

weakness, atrophy, fatigue, spasticity, hyperreflexia, dysarthria, dysphagia, emotional lability, fasiculations

Sensory involvement with ALS?


Treatment of ALS is

symptomatic, and includes PT to make patient feel better and family education

PT for ALS patients might include

strengthening, stretching, ROM, transfer training, wheelchair fitting, gait training, assistive devices, AFOs

Spinal muscular atrophy affects

muscles throughout body but predominately proximal muscles. Weakness in legs often greater than in arms

Sensory affected with Spinal muscular atrophy?


Spinal muscular atrophy tends to impact these vital muscles

respiratory muscles

Intellectual activity affected with Spinal Muscular atrophy?


Autosomal recessive genetic disease caused by missing or mutated gene called survival motor neuron

Spinal muscular atrophy

4 types of spinal muscular atrophy

Type I (Werdnig Hoffman Disease), Type II, Type III (Kugelberg-Welander or Juvenile SMA), Type IV: Adult Onset

Type IV, adult Onset SMA typically

begins after age 35, loss of function occures rapidly when ill or gradually

Diagnosing SMA occurs via

a blood test looking for the absence of SMN1 gene (also suggestive history and physical exam)

PT for SMA patients

ROM, Seating systems, Chest PT, Aquatics, Standing frames/wheelchairs, Orthoses, Assistive devices

Scientific name for Guillain Barre

Acute Inflammatory Demyelinating Polyradiculoneuropathy

2/3 of Guillain Barre patients report

a fever with respiratory or GI tract disorder 1-3 weeks prior to onset

Test results for Guillain Barre patients

increased CSF production with normal cell count

Inflammation and demyelination in all nerves due to a cellular immunologic response...

Guillain Barre

Three stages of Guillain Barre

Acute, Plateau, Recovery

Signs and symptoms of Guillain Barre

ANS dysfunction, Sensory loss, Symmetrical muscle weakness distal to proximal (evolves in hours to up to 10 days), decreased DTRs

Acute phase of Guillain Barre ends when

no new signs and symptoms develop

Plateau phase of GB can last

several days to 2 weeks

This percent of Guillain Barre Patients return to normal


PT for Guillain Barre

ROM, strengthening, Splits, gait training

Biggest complain for GB patients


Medical management of Guillain Barre includes

repeated plasmopheresis, steroids, gamma globulin, other immunomodulators, psychological support, proper analgesics

Myesthenia gravis is

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

muscle weakness

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

Duchenne's MD

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


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