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66 Cards in this Set

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Any and all disease of muscle or any type of damage to muscle?
Myopathy
What fails in muscle disease?
Phosphate (energy transfer) Fails
What is the immediate source of energy for muscle contraction?
Hydrolysis of ATP
How is muscle ATP replenished?
Anaerobic Glycolysis
Oxidative Phosphorylation
What enzyme moves phosphate from creatine phosphate to ADP is present in muscle?
Creatine Kinase
What is the biochemical marker leaked out of the sarcolemma for Myonecrosis (death of muscle fibers)?
Serum Creatine Kinase
What are the two most common reactions to injury for muscle?
Atrophy & Myonecrosis
What disease affecting the lower motor neuron can cause myofiber atrophy?
Denervation Atrophy
What is the most common motor neuron diseases of denervation atrophy in Adults?
Amyotrophic Lateral Sclerosis
What is the most common motor neuron diseases of denervation atrophy in Children?
Spinal Muscular Atrophy
Disuse of muscle causes atrophy of what type of fibers?
Type 2 fibers
What causes denervation atrophy?
Peripheral neuropathies or motor neuron disease
What disease is characterized by neuronal muscle atrophy and hypereflexia due to loss of lower motor neurons in the anterior horns of the spinal cord and upper motor neurons that project in corticospinal tracts, respectively?
Amyotrophic Lateral Sclerosis (ALS, Lou Gehrig Disease)
What disease has early symptoms of assymmetric weakness of the hands, manifested as dropping objects and difficulty in performing fine motor tasks, and cramping and spasticity of the arms and legs?
ALS
In ALS what are the involuntary contractions of individual motor units referred to as?
Fasciculations
In ALS what is the pattern of rapid early degeneration of the lower brainstem cranial motor nuclei referred to as?
Bulbar ALS*

Usually affects the tongue early on. Also, have trouble chewing food, bite their tongue often, etc.
What is the most common form of spinal muscular atrophy has its onset at birth or within the first 4 months of life and usually leads to death within the first 3 years of life?
Werdnig-Hoffmann Disease (a SMA)
In Spinal Muscular Atrophy which form is found in later childhood (after 2 years of age) and often survive into adulthood, SMA2 or SMA3?
SMA3

(SMA2 Presents between 3-15 months of age and may live >4 years)
What is an Inflammation of muscle, especially a voluntary muscle, characterized by pain tenderness, and sometimes spasms in the affected area?
Myositis
What is Abnormally long muscular contractions; slow relaxation of a muscle after a contraction tonicity.
Myotonia
What is Muscle tone the elastic tension of living muscles, arteries, etc. that facilitate response to stimuli?
Tonus
What is a general term for abnormal tonicity of muscle, which may be characterized by lack of tone or prolonged, repetitive muscle contractions causing twisting or jerking movements of the body or body part?
Dystonia
What is a myotonia (long muscle contraction) of the extremities only?
Acromyotonia (causes spastic deformity of the hand or foot)

{acro means tip or end of something}
What is a mild, rare, congenital form of myotonia (long muscle contraction) characterized by muscle stiffness?
Thompson's Disease (Myotonia Congenita)
What is a clonic spasm of a muscle or muscle group cramp, muscle spasm, spasm - a painful and involuntary muscular contraction?
Myoclonus
What type of cramp (myotonus) is:
Common
Intermittent w/variable severity during exercise
Myoglobinuria usually Absent
Rhabdomyolysis usually Absent
Weak familial tendency
Exercise-Associated Muscle Cramps
What type of cramp (myoclonus) is:
Rare
Recurrent and severe during exercise
Myoglobinuria Common (sign of degeneration of muscle fibers)
Rhabdomyolysis usually present (lysis of skeletal muscle)
Often electrically silent (rigor)
Strong Familial Tendency
Cramps Due to Metabolic Disorders
What are a group of familial diseases characterized clinically by myotonia, relapsing episodes of hypotonic paralysis (induced by exercise, cold, or hi carb meal)?
Ion Channel Myopathies
What type of Ion Channel Myopathy is a:
* Ca++ channel defect triggered by halogenated inhalation anesthesia and succinylcholine
* Body Temp goes up during anesthesia
* Due to problems with Ryanodine receptor
* Leads to Ca++ influx into muscles
* Muscles degrade due to hypermetabolism
* Would never know there was a problem if they never went under anesthesia
Malignant Hyperthermia
What is the Key problem that triggers Malignant Hyperthermia?
Ryanodine Receptor Problems
What are Genetically Transmitted diseases characterized by degeneration and loss of myofibers and clinically by progressive weakness and elevated Creatine Kinase?
Muscular Dystrophies
What is the most common muscular dystrophy in Children?
Duchenne Muscular Dystrophy
What are the most common dystrophies in adults?
Myotonic Dystrophy
Limb Girdle Dystrophy
What are the proteins located Just Inside the sarcolemma that bind myofibers to matrix and stabilize the sarcolemma during contraction and relaxation?
Dystrophin
What are the proteins embedded in the sarcolemma that bind myofibers to matrix and stabilize the sarcolemma during contraction and relaxation?
Sarcoglycans
What are the proteins located in the Basement Membrane Outside the sarcolemma that bind myofibers to matrix and stabilize the sarcolemma during contraction and relaxation?
Alpha-Dystroglycan, merosin
What happens in muscular dystrophy if the fibroproteins that bind myofibers to matrix are broken down?
Stress Fractures of the Sarcolemma occur during contraction. Influx of Ca++ activate proteolytic enzymes leading to Autodigestion of sarcoplasm (myonecrosis)
**What muscular dystrophy is caused by defects in the dystrophin proteins located just inside the sarcolemma?
Duchenne Muscular Dystrophy
What muscular dystrophy is caused by defects in the sarcoglycans proteins embedded in the sarcolemma?
Limb Girdle Dystrophy
What muscular dystrophy is caused by defects in the basement membrane proteins (2laminin)?
Congenital Muscular Dystrophy
Phenotypic classification is being replaced by genetic-molecular classification, so what would Duchenne MD & Limb Girdle MD have as nomenclature?
Dystrophinopathy
Sarcoglycanopathy
What are the symptoms of Muscular Dystrophy?
See Picture
What cause severe, sometimes fatal, hypotonia and weakness at birth and are rare diseases?
Congenital Myopathies
What is the best-characterized Congenital Myopathy?
**Nemaline ("rod body") Myopathy

They are easily identified, but not well understood how they are related to the dysfunction of the muscle.
Where is the weakness predominantly in DM1?
DM1 (myotonic dystrophy) is weakest in the Distal
Where is the weakness predominantly in DM2?
DM2 (myotonic dystrophy) is weakest in the Proximal
What type of myotonic dystrophy is:
§ is caused by a CGT trinucleotide expansion in the DMPK (dystrophia myotonica protein kinase) gene on chromosome 19q13. In DM1, this gene is expanded over 37 CGT repeats.
§ The more repeats, the more severe the dystrophy and the earlier the onset of symptoms.
* the onset of the disease is earlier with each successive generation (anticipation)
DM1
What type of myotonic dystrophy:
§ is caused by a CCTG expansion of the ZNF9 (Zink Finger protein 9) gene on 3q21.
* Neither mutation affects the coding portion of these proteins and it is not know how these mutations affect muscle and other organs.
DM2
What are the 3 subgroups of Inflammatory Myopathies?
1) Infectious IM
2) Noninfectious IM
3) Systemic inflammatory diseases that involve muscle along with other organs
What type of myopathies are characterized by myonecrosis and mononuclear inflammatory infiltrates and clinically by weakness and soreness of muscles and elevated Creatine Kinase and erythrocytes sedimentation rate?
Inflammatory myopathies
What are the main Non-Infectious Inflammatory Myopathies (autoimmune in origin)?
Polymyositis
Dermatomyositis
(inclusion body myositis)
What inflammatory myopathy:

* affects predominantly adults who present with subacute or chronic proximal weakness (without a rash) and elevated CK.
§ Muscle biopsy shows endomysial mononuclear cells and myonecrosis.
§ is cell-mediated autoimmune in which cytotoxic T-cells and macrophages invade and destroy myofibers.
Polymyositis
What inflammatory myopathy:

§ affects children and adults.
§ It causes a purple (heliotrope) discoloration of the upper eyelids, edema around the eyes and mouth, skin rash on the face and over extensor surfaces of the extremities, muscle pain, weakness and stiffness of muscles.
§ Contractures, subcutaneous calcification, intestinal ulceration, and other extramuscular manifestations are frequent in children
Dermatomyositis
What type of Toxic Myopathies presents as atrophy, myonecrosis, regeneration and interstitial lymphocytosis?
Thyrotoxic
What type of Toxic Myopathies presents as rhabdomyolysis, myoglobulinemia and renal failure?
Binge Drinking
What type of Toxic Myopathies presents as steroids, chloroquine, statins?
Drug-Induced
What are the treatments for Myasthenia Gravis?
AntiCholinesterase drugs, Corticosteroids, and Thymectomy
In Myasthenia Gravis, what is usually the consequential method of death?
Death due to Respiratory Problems
Improvement of strength following administration of edrophonium (Tensilon test) or Neostigmine are diagnostic for?
Myasthenia Gravis
What disease is caused by circulating antibodies (IgG) binding to acetylcholine receptors?
Myasthenia Gravis
What are two benign neoplasms of muscle?
Rhabdomyoma
Leimyoma
What are two malignant neoplasms of muscle?
Rhabdosarcoma
Leiomyosarcoma
What is a very rare benign tumor of skeletal muscle that can be found in the heart?
Rhabdomyoma
What is the most common neoplasm in women due to the uterus?
Leiomyoma (smooth muscle benign neoplasm)

I think Leiomyoma as a hula dancer (linking the female factor)
What is the most common soft tissue sarcoma of children/adolescence?
Rhabdomyosarcoma
What is a rare malignant tumor of smooth muscle resistant to radio/chemotherapy except for cKit+ (CD117) GIST that is responsive to Gleevec?
Leiomyosarcoma