Krafts - Sheet1

Question 1

cells that transmit impulses

Answer 1

neurons

Question 2

cells that react to injury

Answer 2

Astrocytes

Question 3

cells that produce myelin

Answer 3

Oligodendrocytes

Question 4

cells that phagocytose intruders

Answer 4

Microglia

Question 5

cells that line ventricles

Answer 5

Ependymal cells

Question 6

“red neurons”

Answer 6

acute injury, dying neurons

Question 7

Cowdry bodies

Answer 7

associated with herpes

Question 8

Negri bodies

Answer 8

associated with rabies

Question 9

Gliosis

Answer 9

proliferation of astrocytes in reaction to injury

Question 10

Neuronal Storage Diseases

Answer 10

Autosomal recessive enzyme deficiency (most), Result: accumulation of enzyme substrate (sphingolipids, mucopolysaccharides or mucolipids) within neuronal lysosomes Leads to a loss of cognitive function, maybe also seizures. Neuronal ceroid lipofuscinoses, Tay-Sachs disease Clinical senerio; child looks normal, then starts missing developmental milestones….

Question 11

leukodystrophies

Answer 11

refers to a group of disorders characterized by degeneration of the white matter in the brain.[1] The leukodystrophies are caused by imperfect growth or development of the myelin sheath, the fatty covering that acts as an insulator around nerve fibers.

Question 12

Tay-Sachs

Answer 12

Deficiency of hexosaminidase A Accumulation of ganglioside in all tissues (nervous system shows the most symptoms) Autosomal recessive; much more common in Ashkenazi Jews Usually begins in early infancy Developmental delay, then paralysis and loss of neurologic function “Cherry-red” spot in retina virtually diagnostic Death within several years

Question 13

Neuronal Ceroid Lipofuscinoses

Answer 13

Deficiency of enzymes involved in protein modification/degradation Lipofuscin accumulates within neurons, leading to neuronal dysfunction Blindness, mental and motor deterioration, seizures Onset ranges from childhood to adulthood

Question 14

Leukodystrophies

Answer 14

Group of disorders characterized by myelin abnormalities Most are autosomal recessive Involve lysosomal or peroxisomal enzymes Deterioration of motor skills, spasticity, hypotonia, ataxia Often appear in children who appear normal, but start missing their developmental milestones

Question 15

Krabbe Disease

Answer 15

Deficiency of galactosylceramidase; Galactocerebroside accumulates, gets converted to galactosylsphingosine (toxic to oligodendrocytes) Loss of myelin and oligodendrocytes in CNS and peripheral nerves “Globoid cells” (fat macrophages/microglial cells) in brain Onset around 3-6 months Rapidly progressive muscle stiffness, weakness

Question 16

B1 (Thiamine) Deficiency

Answer 16

(Needed to make ATP…?) Usually associated with chronic alcoholism; Wernicke encephalopathy Korsakoff syndrome

Question 17

Wernicke encephalopathy

Answer 17

confusion, ophthalmoplegia, ataxia hemorrhage and necrosis in *mammillary bodies*, walls of third and fourth ventricles acute, reversible Vit B1 deficiency (thiamine)

Question 18

Korsakoff syndrome

Answer 18

memory disturbances, confabulation cystic spaces, hemosiderin-laden macrophages in *mammillary bodies*, ventricle walls *thalamic lesions too prolonged, mostly irreversible Vit B1 deficiency (thiamine)

Question 19

B12 Deficiency

Answer 19

Anemia reversible with B12 administration Subacute combined degeneration of spinal cord lower extremity numbness, ataxia, weakness reversible until paraplegia occurs swelling of myelin layers, vacuolization ascending and descending tracts involved

Question 20

Hypoglycemia

Answer 20

Abnormally low Blood Sugar; Most vulnerable: large pyramidal neurons of cortex (“pseudolaminar necrosis”) Also vulnerable: hippocampus and cerebellum

Question 21

Hyperglycemia

Answer 21

Abnormally High Blood Sugar; Most commonly seen in diabetes mellitus Can be associated with either ketoacidosis or hyperosmolar coma Dehydration, confusion, stupor, coma

Question 22

Carbon Monoxide Poisoning

Answer 22

Injury is due to hypoxia Particularly vulnerable areas: cortex (layers III and V) hippocampus Purkinje cells May see demyelination of white matter tracts

Question 23

methanol poisoning

Answer 23

Preferentially affects retina Degeneration of ganglion cells May cause blindness

Question 24

Ethanol and CNS

Answer 24

Acute effects are reversible; chronic effects are not Preferentially affects cerebellum Truncal ataxia, unsteady gait, nystagmus Cerebellar atrophy, loss of granule cells, loss of Purkinje cells, Bergmann gliosis

Question 25

Guillain-Barré Syndrome

Answer 25

Life-threatening disease of the PNS Flu-like illness, then acute ascending paralysis Immune-mediated demyelination Usually resolves with time “Acute inflammatory demyelinating polyradiculoneuropathy” 2/3 of cases preceded by acute flu-like illness (Campylobacter, CMV, EBV, Mycoplasma, influenza virus) T cells and macrophages cause segmental demyelination; antibodies also present Remyelination follows

Question 26

Leprosy (Hansen Disease)

Answer 26

Slowly progressive infection of skin and nerves Caused by Mycobacterium leprae Transmitted through respiratory droplet Endemic in poor tropical countries Causes disabling deformities

Question 27

Mycobacterium leprae

Answer 27

Acid-fast, obligate intracellular bacterium Grows poorly in culture Cell wall doesn’t stain with gram stain! (wax coating) Hard to kill in normal ways – body forms granulomas to attack and eat the bugs

Question 28

Two Forms of Leprosy

Answer 28

1) Tuberculoid leprosy (not so bad) Less severe, localized Dry, scaly skin lesions Nerve degeneration (anaesthesia, ulcers, contractures) Nice T-cell response to the bug, with nice granuloma formation 2) Lepromatous leprosy (pretty bad) More severe, widespread Skin, nerves, eye, mouth, testes, hands, feet Patient’s immune system doesn’t respond

Question 29

What is the characteristic finding in an infection with herpes zoster virus (shingles)?

Answer 29

Multinucleated giant cell

Question 30

Hereditary Motor and Sensory Neuropathy Type 1

Answer 30

Also called Charcot-Marie-Tooth disease, demyelinating type Repetitive demyelination and remyelination Muscle loss, loss of sensation but pain intact Childhood or early adulthood High arches, hammer toes, muscle atrophy Normal life span

Question 31

Peripheral Neuropathy in Diabetes

Answer 31

Most common manifestation: symmetric sensory and motor neuropathy involving distal nerves Decreased pain sensation in distal extremities Some patients also have autonomic neuropathy and/or asymmetric neuropathy

Question 32

paraneoplastic effect

Answer 32

a disease or symptom that is the consequence of the presence of cancer in the body, but is not due to the local presence of cancer cells.[1] These phenomena are mediated by humoral factors (by hormones or cytokines) excreted by tumor cells or by an immune response against the tumor.

Question 33

Parenchymal injuries

Answer 33

Concussion Direct parenchymal injury Diffuse axonal injury

Question 34

Traumatic vascular injury

Answer 34

Epidural hematoma Subdural hematoma Subarachnoid hemorrhage

Question 35

Linear skull fracture

Answer 35

Most common Straight crack Usually not serious

Question 36

Depressed skull fracture

Answer 36

Bone displaced inward Comminuted (in pieces) Can damage brain

Question 37

Diastatic skull fracture

Answer 37

Across a suture Suture widens Usually in children

Question 38

Basal skull fracture

Answer 38

More force Distant hematomas… distant from site of impact (usually behind the ear) CSF drainage (nose, ear, eyes) usually the result of tremendous force.

Question 39

Concussion

Answer 39

Definition: Altered consciousness from head injury due to change in momentum of head (head hits rigid surface) Mechanism unknown Symptoms: amnesia, confusion, headache, visual disturbances, nausea, vomiting, dizziness Grading: Grade 1: no loss of consciousness, lasts 15 minutes Grade 3: LOC

Question 40

Chronic Traumatic Encephalopathy

Answer 40

Progressive degenerative disease of the brain Athletes/others with repetitive brain trauma Behavioral/personality symptoms: poor judgment, memory loss confusion aggression apathy depression Then, progressive dementia Histologically looks like Alzheimer disease

Question 41

Direct Parenchymal Injury

Answer 41

Laceration (tearing of tissue) Contusion (bruising) Blows can result in: Coup injury (contusion at point of contact) Contrecoup injury (contusion on opposite side)

Question 42

Coup vs. contrecoup injury

Answer 42

coup brain injury on side of head that hits, contrecoup injury on opposite side

Question 43

Cerebral Edema: vasogenic vs. cytotoxic

Answer 43

Vasogenic Cause: increased vascular permeability Fluid shifts into intercellular spaces in brain Localized or generalized Cytotoxic Cause: cell membrane injury Increased intracellular fluid Typically seen in hypoxia or with metabolic damage

Question 44

Hydrocephalus

Answer 44

Accumulation of too much CSF in ventricals; Five kinds: Communicating Non-communicating Ex vacuo Increased CSF production Normal pressure

Question 45

Treatment of Hydrocephalus

Answer 45

Goal: reduce fluid volume and pressure Get fluid out Surgery to remove tumor or blockage Shunt to redirect CSF drainage Decrease CSF production Acetazolamide (carbonic anhydrase inhibitor) Furosemide (Na, K 2 Cl pump inhibitor)