34 Cerebrovascular Disease

Question 1

An arteriopathy producing deep blue-red skin lesions of livedo reticularis and livedo racemosa in association with multiple ischemic strokes. Not all with high titers of Antiphospholipid antibodies

Answer 1

Sneddon syndrome (p 872)

Question 2

Definitive therapy for APAS

Answer 2

Warfarin (p872)

Question 3

These are serious diseases of the small blood vessels combined with microangiopathic hemolytic anemia characterized by widespread occlusions of arterioles and capilliaries involving practically all organs of the body

Answer 3

TTP, Moschcowitz syndrome (p873)

Question 4

Rarer familiar form, deficiency of ADAMTS13

Answer 4

The Upshaw-Shulman syndrome (p837)

Question 5

Sporadic TTP is caused by an acquired circulating iGg inhibitor of the Von Willebrand factor cleaving protease

Answer 5

ADAMTS13 : A Disentegrin and Metalloproteinase with thrombospondin type 1 motif, member 13 (p873)

Question 6

Main features of TTP

Answer 6

Fever, anemia, symptoms of renal and hepatic disease (p.873)

Question 7

Neurologic features of TTP

Answer 7

Confusion, delirium, seizures, hemiparesis, nonconvulsive status epilepticus (p873)

Question 8

Overlap among TTP and HUS

Answer 8

Toxemia of preganancy
Hemolytic anemina with elevated Liver Function test and plt count (HELLP)
Hypertensive encephalopathy
PRES

(p.873)

Question 9

A myeloproliferative disorder of unknown cause, characterized by a marked increase in RBC mass and in blood volume and often by increase in WBCs and Platelets

Answer 9

Polycythemia Vera (p.873)

Question 10

The slightly increased incidence of thrombosis in primary polycythemia is attributed to

Answer 10

High blood viscocity
Engorgement of vessels
Reduced rate of blood flow
(p.873)

Question 11

This inherited disease is related to the presence of the abnormal hemoglobin S In the red corpuscles

Answer 11

Sickle cell disease (p.873)

Question 12

This disease js practically limited to persons of central African and certain Mediterranean origins

Answer 12

Sickle cell disease (p.874)

Question 13

Begins in early life and is characterized by “crises” of infection particularly (pneumococcal meningitis), pain in the limbs and abdomen, chronic leg ulcers and infarctions of bones and visceral organs

Answer 13

Sickle cell disease (p.874)

Question 14

To reduce risk of stroke in sickle cell anemia

In stroke prevention trial, risk of first stroke was reduced by 90%

Answer 14

Exchange transfusions

Monitoring if velocities if flow in the MCA by transcranial Doppler examination (p.874)