Exam II Flashcards
What are the three parts of a motor unit?
Peripheral axon, neuromuscular junction and innervated myocytes
What is the name of the cells that produce myelin in the PNS?
Schwann cells
A peripheral nerve injury that is characterized by direct injury to an axon and degeneration of peripheral segments and results in decreased amplitude and axon density.
Axonal neuropathy
Axonal neuropathy in which myelin loss is a secondary result is called:
Wallerian degeneration
A peripheral nerve injury that is characterized by damage to Schwann cells resulting in random internode demyelination and slow nerve conduction velocity.
Demyelinating neuropathy
Demyelinating neuropathy is also called:
Segmental demyelination
Carpal tunnel syndrome involves entrapment of a single nerve. This pattern of peripheral neuropathy is termed:
Mononeuropathy
“Stocking-and-glove” parasthesia and anasthetia involve diffuse and asymmetric neuronal loss. This pattern of peripheral neuropathy is termed:
Polyneuropathy
Damage that randomly affects portions of individual nerves follows which pattern of peripheral neuropathy?
Polyneuritis multiplex
The disorder that is characterized by acute motor neuron demyelination that results in ascending paralysis is termed:
Guillan-Barre syndrome
GBS is most common in:
Males aged 15-35 and 50-57 years
Which disorder is characterized by symmetric, segmental demyelination/remyelination and can relapse and progress to chronic GBS?
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
What is the most common peripheral neuropathy?
Diabetic neuropathy
Which peripheral neuropathy is considered mixed due to its damage of both axons and myelin?
Diabetic neuropathy
What is the most highly recommended treatment option in diabetic neuropathy?
Daily foot inspection
Which cause of peripheral neuropathy results in altered axonal transport and cytoskeletal damage and is observed in the distal extremities?
Environmental toxins
Which cause of peripheral neuropathy results in damage to peripheral nerves and involves the polyneuritis multiplex?
Systemic vasculitis
Genetic disorders that result in peripheral neuropathy are attributed to mutations in which gene?
PMP22
The genetic disorder that leads to peripheral neuropathy most commonly in young adults and is slowly progressive is termed:
Charcot-Marie-Tooth disease
In myasthenia gravis, autoantibodies block:
Post-synaptic Ach receptors
MG is characterized by:
Thymic hyperplasia and extraocular weakness and becomes worse with e-stimulation
MG is most common in:
Females in the 3rd decade of life and males in the 5th-6th decade of life
Which test inhibits acetylcholinesterase (AchE)?
Tensilon test
In Lambert-Eaton syndrome, autoantibodies inhibit:
Pre-synaptic calcium channels
Which form of NMJ disorder mimics MG and Lambert-Eaton syndrome?
Congenital myasthenic syndrome
Which two bacteria cause infectious NMJ disorders?
C. tetani and C. botulinum
C. _____ increases Ach release, while C. _____ decreases Ach release.
tetani; botulinum
Which muscle fiber is characterized as slow twitch, aerobic, dark and uses fat metabolism?
Type I
Which muscle fiber is characterized as fast twitch, anaerobic, white and uses glycogen metabolism?
Type II
Disuse atrophy and glucocorticoid atrophy primarily involve which fibers?
Type II
The group of most common forms of muscular dystrophy are termed:
Dystrophinopathies
Duchenne muscular dystrophy is characterized by:
Absence of dystrophin
What is the most common dystrophinopathy?
DMD
Becker MD is characterized by:
Dystrophin is present but has variable function
DMD is most common in which population?
Childhood onset (Age 5), wheelchair-bound in their teens and lethal in early adulthood
BMD is most common in which population?
Delayed onset (adolescence), less severe than DMD
What are the features of DMD and BMD?
Pelvic girdle muscles primarily impacted and pseudohypertrophy of calf muscles
Lab values for patients with DMD/BMD include finding increased:
Creatine kinase
How do patients die from DMD/BMD?
Respiratory insufficiency
The type of inflammatory myopathy that is autoimmune and is most common in males aged 45-60 years.
Polymyositis
The most common inflammatory myopathy in children is termed:
Dermatomyositis
The most common myopathy among elderly patients is termed:
Inclusion body myositis
Overproduction of thyroxine, goiter or Graves disease are characteristics of:
Thyrotoxic myopathy
Binge drinking, rhabdomyolysis and a progression into renal failure are characteristics of:
Ethanol myopathy
Drug myopathy is characterized by adverse effects by various agents, the most common being:
Statins
The peripheral nerve sheath tumor that most commonly involves CN8 is called:
Schwannoma (Vestibular schwannoma)
Multiple CNS and cutaneous schwannomas absent vestibular schwannomas is termed:
Schwannomatosis
Neurofibromatosis that exhibits pronounced neurofibromas and mutations on chromosome 17 is characteristic of:
Neurofibromatosis type I
NF1 is also called:
von Recklinghausen disease
Bilateral vestibular schwannomas and the merlin gene that most commonly result in vision and hearing problems are all characteristics of:
NF2
Swelling of the soma and displacement of the Nissl substance are characteristic of:
Reversible neuronal injury
The presence of “red neurons,” eosinophilia and axonal swelling are all characteristic of:
Irreversible neuronal injury
Minimal fibrosis occurs following CNS injury to:
Astrocytes
The presence of glial filaments sprouting is termed:
Gemistocytic astrocyte
Which cells produce myelin in the CNS?
Oligodendrocytes
Which cells are considered CNS phagocytes?
Microglia
What is the function of the choroid plexus?
Produces CSF
The rabies virus produces _____ in the cytoplasm.
Negri bodies
The presence of an “owl’s eye” is indicative of:
Cytomegalovirus
The presence of Lewy bodies is indicative of:
Parkinson’s disease
Neurofibrillary tangles and beta-amyloid plaques are indicative of:
Alzheimer’s
Accumulation of lipids in the cytoplasm or lysosomes is indicative of:
Lipofuscin
Blood-brain-barrier disruption resulting in increased permeability is termed:
Vasogenic cerebral edema
Neuronal/glial membrane injury resulting in increased intracellular fluid retention is termed:
Cytotoxic cerebral edema
What are the three distinct features of cerebral edema?
Flattened gyri, narrowed sulci, and ventricular compression
Increased CSF volume most commonly involves:
Altered flow or resorption
Hydrocephalus that occurs at
Cranial enlargement
Hydrocephalus that occurs at >2 years old results in:
Increased ICP and ventricular enlargement
What is the most common treatment for hydrocephalus?
Shunting