Pathology II

Question 1

What is the clinical presentation of inflammatory demyelinating polyradiculopathy?

Answer 1

Symmetric ascending muscle weakness/ paralysis beginning in the lower extremities with facial paralysis in 50% of the cases. Autonomic function may be severely affected (cardiac irregularities, hypertension, hypotension, etc) (p.444)

Question 2

What is the prognosis for inflammatory demyelinating polyradiculopathy?

Answer 2

Almost all patients survive and the majority recover completely after weeks to months (p.444)

Question 3

What lab findings are characteristic of acute inflammatory demyelinating polyradiculopathy?

Answer 3

Increased CSF protein with normal cell count (albuminocytologic dissociation); increased protein may cause papilledema (p.444)

Question 4

What conditions are associated with acute inflammatory demyelinating polyradiculoopathy?

Answer 4

Infections

Question 5

What organisms/ viruses are the most common causes of acute inflammatory demyelinating polyradiculopathy?

Answer 5

Campylobacter jejuni, CMV (p.444)

Question 6

What causes the autoimmune response in acute inflammatory demyelinating polyradiculopathy?

Answer 6

Autoimmune attack of peripheral myelin due to molecular mimicry, inoculations, and stress but no definitive link to pathogens (p.444)

Question 7

How is acute inflammatory demyelinating polyradiculopathy treated?

Answer 7

Respiratory support is critical until recovery. Additional treatment includes plasmapheresis, IV immune globulins (p.444)

Question 8

Name five less common demyelinating/ dysmyelinating diseases.

Answer 8

Progressive multifocal leukoencephalopathy (PML); Acute disseminated (postinfectious) encephalomyelitis; metachromatic leukodystrophy; Charcot-Marie-Tooth disease; Krabbe’s disease (p.445)

Question 9

What is Progressive multifocal leukoencephalopathy (PML)?

Answer 9

Rapidly progressive and usually fatal condition of demyelination of the CNS due to oligodendrocyte destruction (p.445)

Question 10

What conditions are associated with Progressive multifocal leukoencephalopathy (PML)

Answer 10

JC Virus; seen in 2-4% of AIDS patients due to reactivation of latent viral infection (p.445)

Question 11

What is Acute disseminated (postinfectious) encephalomyelitis?

Answer 11

Multifocal perivenular inflammation and demyelination after infection or certain vaccinations (p.445)

Question 12

What vaccinations can contribute to Acute disseminated (postinfectious) encephalomyelitis?

Answer 12

Measles, VZV (p.445)

Question 13

What infections can contribute to Acute disseminated (postinfectious) encephalomyelitis?

Answer 13

Rabies, Smallpox (p.445)

Question 14

What is metachromatic leukodystrophy?

Answer 14

An autosomal recessive lysosomal storage disease (p. 445)

Question 15

What deficiency causes metachromatic leukodystrophy?

Answer 15

Arylsulfatase A deficiency (p. 445)

Question 16

What is the pathophysiology associated with metachromatic leukodystrophy?

Answer 16

Buildup of sulfatides leads to impaired production of myelin sheath (p. 445)

Question 17

What is Charcot Marie Tooth disease?

Answer 17

A hereditary sensory and motor neuropathy related to the defective production of proteins involved in the structure and function of peripheral nerves or the myelin sheath (p. 445)

Question 18

What is Krabbe’s disease?

Answer 18

An autosomal recessive lysosomal storage disease (p. 445)

Question 19

What deficiency causes Krabbe’s disease?

Answer 19

Galactocerebrosidase (p. 445)

Question 20

What is the pathophysiology associated with Krabbe’s disease?

Answer 20

Buildup of galactocerebrosidase destroys myelin sheath (p. 445)

Question 21

What characterizes a seizure?

Answer 21

Synchronized, high frequency neuronal firing (p. 445)

Question 22

What is a partial (focal) seizure?

Answer 22

A seizure affecting one area of the brain. It is often proceeded by an aura and can spread to a generalized seizure (p. 445)

Question 23

Where do focal (partial) seizures most commonly originate?

Answer 23

The medial temporal lobe (p. 445)

Question 24

Name the two types of partial (focal) seizure.

Answer 24

Simple partial, complex partial (p. 445)

Question 25

What distinguishes a simple partial seizure from a complex partial seizure?

Answer 25

In simple partial seizures, consciousness is intact; in complex partial seizures, consciousness is impaired (p. 445)