Cns6 - Toxic And Deficiency Diseases Of Ns

Front 1

What are the 2 metabolic disorders of amino acid metabolism resulting in mixed gray/white matter dysfunction?

Back 1

-PKU
-MSUD - maple syrup urine disease

Front 2

What enzymes may be deficient in PKU?

Back 2

-Phenylalanine hydroxylase
-Dihydropteridine reductase

Front 3

What is MSUD?

Back 3

An inability to metabolize the ketoacids of the branched chain AAs - leucine/Ile/Val

Front 4

What are 6 CNS disorders caused by toxic substances?

Back 4

-Heavy metal poisoning
-Chronic alcoholism
-Methanol poisoning
-Central pontine myelinolysis
-CO poisoning
-Reye's syndrome

Front 5

How do heavy metals exert their toxic effects?

Back 5

By interfering with intracellular enzymatic processes - esp those related to oxidative reduction

Front 6

What are the 4 main pathological findings in acute heavy metal poisoning?

Back 6

-Focal hemorrhages
-Necrosis
-Demyelination
-Brain edema

Front 7

What is likely the cause of death in acute metallic mercury poisoning?

Back 7

Renal failure (not brain)

Front 8

What is the manifestation seen in chronic lead and arsenic poisoning?

Back 8

Peripheral neuropathy

Front 9

What does chronic lead poisoning in children lead to?

Back 9

Lead encephalopathy

Front 10

Where does chronic mercury poisoning cause degeneration?

Back 10

-Cortex
-Basal ganglia
-cerebellum

Front 11

What are the resulting clinical manifestations of chronic mercury induced degeneration?

Back 11

Mental retardation
Tremor
Ataxia

Front 12

Where was methyl mercury dumped into water as industrial waste resulting in mercury poisoning in people who age shellfish and other fish from the water?

Back 12

Minamata Bay

Front 13

What are the 3 components of chronic alcoholism that contribute to CNS damage?

Back 13

-Direct toxic anesthetic effects of alcohol on neurons
-Hepatic insufficiency
-Malnutrition/Vit B1 deficiency

Front 14

What are 7 cns diseases associated with chronic alcoholism?

Back 14

-Cerebral atrophy
-Metabolic astrocytosis
-Wernicke-Korsakoff's syndrome
-Alcoholic cerebellar atrophy
-Alcoholic polyneuropathy
-Head injuries
-Subdural hematoma

Front 15

What is cerebral atrophy in chronic alcoholism partly related to?

Back 15

Head injuries because they're so drunk

Front 16

What is the hallmark of Metabolic astrocytosis?

Back 16

Proliferation of Alzheimer's type II astrocytes in the cortex and basal ganglia

Front 17

What are Alzheimer's Type II Astrocytes again?

Back 17

Large pale astrocytes with naked nuclei

Front 18

What is the cause of metabolic astrocytosis in chronic alcoholism?

Back 18

Hepatic encephalopathy and coma

Front 19

What are the 4 morphologic hallmarks of Wernicke-Korsakoff syndrome? How does it affect the neurons?

Back 19

-Hemorrhages/necrosis
-Vascular proliferation
-Gliosis
Relatively good preservation of neurons!

Front 20

In what 4 locations are the morphologic changes of Wernicke-Korsakoff seen?

Back 20

-Periventricular gray matter
-Periaqueductal gray matter
-Mammillary bodies
-Medulla around 4th ventricle

Front 21

What are the 6 clinical manifestations of Wernicke-Korsakoff syndrome?

Back 21

-Confusion
-Confabulation
-Hypothermia
-Ophthalmoplegia
-Ataxia
-Coma

Front 22

What specific nucleus is affected by necrosis and hemorrhage in the periaqueductal gray matter?

Back 22

Oculomotor nucleus

Front 23

What does alcoholic cerebellar atrophy consist of?

Back 23

-Atrophy in the anterior and superior cerebellar vermis
-Loss of Purkinje cells

Front 24

What is the clinical manifestation of alcoholic cerebellar atrophy?

Back 24

Ataxia

Front 25

Why do alcoholics get head injuries and subdural hematomas?

Back 25

-Because they're drunk alot
-Because they have cerebellar ataxia

Front 26

What are 3 specific reasons a chronic alcoholic might get a subdural hematoma?

Back 26

1. Frequent head injuries
2. Stretching of bridging veins of cortex/dura
3. Coagulopathy

Front 27

Why do chronic alcoholics have stretching of bridging veins of the cortex/dura?

Back 27

Because their brains are shrinking - cortical atrophy

Front 28

Why do chronic alcoholics have coagulopathy?

Back 28

Because of liver cirrhosis

Front 29

What does methanol poisoning damage? What does it lead to?

Back 29

Retinal ganglion cells - leads to blindness.

Front 30

In what patients is Central pontine myelinolysis seen?

Back 30

-Chronic alcoholics
-Chronically debilitated patients

Front 31

What is Central pontine Myelinolysis?

Back 31

Demyelination and necrosis in the center of the pons.

Front 32

What causes central pontine myelinolysis?

Back 32

Hyponatremia and rapid overcorrections to hypernatremia

Front 33

How does CO poisoning damage the brain?

Back 33

By preventing delivery of the much needed O2 to the brain.

Front 34

Where does CO particularly cause damage? What type of damage does it produce?

Back 34

Basal ganglia/cortex - hemorrhages, necrosis, gliosis, and cavitation.

Front 35

What is a hallmark morphology seen in CO poisoning?

Back 35

Bright cherry red color of blood

Front 36

What will chronic carbon monoxide poisoning result in?

Back 36

Necrosis and cavitation of the globus pallidus

Front 37

What 2 changes is Reye's syndrome composed of?

Back 37

-Encephalopathy
-Fatty degeneration of the viscera (liver/kidney/heart)

Front 38

What is the cause of Subacute combined degeneration of the spinal cord?

Back 38

Vit B12 deficiency

Front 39

What spinal cord tracts are affected by subacute combined degeneration of the spinal cord?

Back 39

-Posterior columns
-Corticospinal tracts

Front 40

What other condition will accompany subacute combined degeneration of the SC due to vit B12 deficiency?

Back 40

Pernicious anemia - megaloblastic madness

Front 41

3 clinical manifestations of demyelination/degeneration of axons in the posterior columns/corticospinal tracts:

Back 41

-Loss of vibration/position sense
-Spasticity
-Ataxic gait

Front 42

What is Wilson's disease?

Back 42

A disorder of copper metabolism caused by a decrease in ceruloplasmin.

Front 43

What name is synonymous with Wilson's disease?

Back 43

Hepatolenticular degeneration

Front 44

What is the inheritance pattern of Wilson's disease?

Back 44

Autosomal recessive

Front 45

What is the result of a deficiency of ceruloplasmin?

Back 45

Increased free copper in the plasma

Front 46

Where does the free copper that is increased in Wilson's disease get deposited?

Back 46

-Basal ganglia - esp Putamen

Front 47

What is the result of copper deposition in the putamen?

Back 47

Necrosis and cavitation

Front 48

How does liver involvement in Wilson's disease contribute to the brain problems?

Back 48

It produces a metabolic acidosis leading to widespread metabolic astrocytosis of the cortex and basal ganglia

Front 49

What are 4 disease manifestations of Wilson's disease?

Back 49

-Parkinson symptoms
-Dystonia
-Wing-beating movement
-Mental retardation