neurobiology Flashcards
demyelination of axons in PNS is called
Gullian-Barre Syndrome (GBS) (due to prior infection / Inflammatory process )
Main Symptoms of Gullian-Barre Syndrome (GBS)
● Progressive ascending motor weakness;
● Motor and sensory loss affecting the face,
limbs, trunk, and diaphragm;
● Areflexia due to damage of both reflex
arcs of the deep tendon reflex;
● Inflammatory cells (lymphocytes and
macrophages) found within peripheral
nerves.
Disorders Associated With Defective Myelination
Charcot-Marie-Tooth Syndrome
Gullian-Barre Syndrome (GBS)
Multiple Sclerosis (MS)
autoimmune demyelination of axons in CNS
Multiple Sclerosis (MS)
Main symptoms of multiple sclerosis MS
● numbness in one or more limbs;
● tingling or pain in parts of the body;
● partial or complete loss of vision in one eye at a
time and double or blurred vision
● speech disturbances
demyelination in PNS caused by gene mutations
Charcot-Marie-Tooth Syndrome
Charcot-Marie-Tooth Syndrome is due to mutation of what gene ass. with schwann cells
Connexin ( therefore failure of gap junctions)
main symptoms of
Charcot-Marie-Tooth Syndrome
● Demyelination affects both motor and
sensory nerves;
* weakness of the foot and lower leg
muscles is typical;
● patients have a high- stepped gait with
frequent tripping or falls;
* deformities in the foot and digits
Disorders Associated With Defective Ion Channels:
Lambert-Eaton Syndrome
Cystic fibrosis
Cystic fibrosis affects what channels
inherited disease with impaired functions of Cl- ion channels
Organs commonly affected with recurrent infection ass. in cystic fibrosis is
Lungs and airway
CFTR (cystic fibrosis transmembrane conductance
regulator ) gene provides instructions for making
CFTR protein, which is located in the cell membrane
and functions as an ion channel through which Cl- ions and water are transported to maintain the
fluidity of mucus and secretions
Function of the Cystic fibrosis transmembrane conductance regulator gene
Provides instructions for making
CFTR protein, which is located in the cell membrane
and functions as an ion channel through which Cl-
ions and water are transported to maintain the
fluidity of mucus and secretions;
Lambert-Eaton Syndrome affects what channels
loss of voltage-gated Ca2+ channels in the presynaptic terminals
at the NMJ and other synapses;
Lambert-Eaton Syndrome is ass. with
small-cell carcinoma of the lung :derived from neuroendocrine cells expressing voltage-gated Ca+ channels);
Main symptons of lambert-eaton syndrome
dry mouth, constipation, reduced
sweating, orthostatic hypotension, and impotence
reduction in the release of the transmitter (Ach)
at the NMJ causing muscle weakness