Systemic Disease/Pregnancy

Question 1

TTP vs HUS

Answer 1

TTP presents with hemolytic anemia, thrombocytopenia, fever, neuro deficits/sx, renal dysfunction. Due to deficiency of von Willibrand factor cleaving protease. normal D dimer, fibrinogen, clotting time (as opposed to DIC which would be abnormal)

HUS similar but more severe renal dysfunction, seen in kids with preceding diarrhea/abdominal pain (E coli and Shigella with Shiga toxin)

Question 2

sickle cell disease and neurologic sx

Answer 2

ischemic stroke more common than hemorrhagic
can have intracranial stenosis/moya moya syndrome
cranial neuropathies can occur
screen with TCDs –> if increased velocities consider exchange transfusion to prevent stroke
Hb SS more risk than Hb SC

Question 3

celiac disease neuro manifestations

Answer 3

axonal peripheral neuropathy, inflammatory myopathy, seizures, cerebellar involvement (loss of Purkinje cells).
once cerebellar atrophy occurs, diet won’t improve ataxia.

vitamin E deficiency can also cause neuropathy from chronic malabsorption

Question 4

Whipple disease (path, tx)

Answer 4

periodic acid-Schiff-positive macrophage inclusions on bowel biopsy

tx: sulfonamides

Question 5

neuro manifestations of IBD (Crohn’s, UC)

Answer 5

increased risk of stroke due to venous and arterial thrombosis
neuropathies, myopathies, cranial neuropathies (esp CN 7 - seen in Melkersson-Rosenthal syndrome with tongue fissuring and angioedema)
WM abnormalities on MRI
vitamin deficiencies due to malabsorption

Question 6

neuro sx of hypoglycemia

Answer 6

focal neuro deficits (stroke mimic), encephalopathy, seizures