Clinical Program for
Cerebrovascular Disorders
Mount Sinai Medical Center
A 72 Year Old Man with Multiple Infarctions of
Uncertain Etiology
Clinical Case Presentation
Clara Raquel Epstein, MD
Fellow
A 72 Year Old Man with Multiple
Infarctions of Uncertain Etiology
Clinical Case Presentation
A 72 Year Old Man with Multiple
Infarctions of Uncertain Etiology
Clinical Case Presentation
A 72 year old right handed male four days post-op
laporascopic removal of a gastric lipoma, developed
dysarthria, acalculia and motor aphasia. The patient’s
past medical history was significant for migraine with
visual aura, gout, hypertension, GERD, Type II DM,
hypothyroidism, and hypogonadism.
The patient was s/p left orchiectomy and LND with total
nodal xrt in 1953, and one course of chemotherapy
(6mp/azaserine) 4 years post surgery, s/p malignant
melanoma excision 5 years previous, and s/p Bilroth I
for a gastric lipoma (3/8/00).
A 72 Year Old Man with Multiple
Infarctions of Uncertain Etiology
Clinical Case Presentation
A left frontal and a right posterior parietal infarct were
diagnosed on DWMRI. There was also MRI evidence of
bilateral subacute cerebellar infarctions. Heparin
therapy was initiated. The following day, a platelet count
of 60,000 was noted.
The patient subsequently developed progressive
thrombocytopenia, reticulocytosis, and anemia with
evidence of schistocytes on peripheral smear. Mildly
diminished renal function was noted. After treatment
with plasmaphoresis the patient’s platelet count returned
to normal and the patient’s neurological deficits resolved
over 10 days. He was discharged to home on Ecotrin.
Three weeks subsequent, the patient’s platelet count again
dropped but responded to several additional courses of
plasmaphoresis with adequate therapeutic response.
A 72 Year Old Man with Multiple
Infarctions of Uncertain Etiology
Clinical Case Presentation
On 4/28/00 he again presented to MSMC with a new right
hemianopsia of three days duration which the patient
initially interpreted as a visual migraine.
Review of systems revealed dyspnea on minimal exertion.
General physical examination was remarkable for
decreased breath sounds at the right lung base.
Neurological examination was significant for a new right
hemianopsia. Language function was normal. The
platelet count was within normal limits.
Sunita Mann, MD
Neuroradiology Fellow
A 72 Year Old Man with Multiple
Infarctions of Uncertain Etiology
Clinical Case Presentation
Diagnostic Tests:
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Multiple Infarctions of Uncertain
Etiology
Hospital Course
The patient’s right homonomous hemianopsia
gradually improved with plasmaphoresis. A
diagnostic thoracentesis was performed. The
cytology indicated findings consistent with
adenocarcinoma. In addition, a CT chest scan
revealed a right upper lobe nodule with
perivascular and pretracheal adenopathy, as
well as evidence of possible liver mets and a left
adrenal nodule.
Thrombotic Thrombocytopenic
Purpura
An acute, potentially fatal disorder characterized by:
• Transient Neurologic Deficits
• Fever
• Severe Thrombocytopenia
• Segmented RBCs on the blood smear (helmet
cells, triangular-shaped or distorted RBCs)
• Evidence of Hemolysis (falling Hb level,
polychromasia on the blood smear, elevated
reticulocyte count, elevated level of plasma LDH)
Thrombotic Thrombocytopenic
Purpura
An acute, potentially fatal disorder characterized by:
• Changing manifestations of ischemic damage to
multiple organs
• The characteristic pathologic lesion involving
vessels in multiple organs is a bland, plateletfibrin thrombi (without the infiltrations of
granulocytes within and around vessel walls
characteristic of vasculitis) localized primarily to
arteriolocapillary junctions.
Thrombotic Thrombocytopenic
Purpura
An acute, potentially fatal disorder characterized by:
• Sx: CNS findings, fluctuating jaundice (w/ elevations of
both direct and indirect bilirubin because of the
combination of hemolysis and hepatocellular damage),
proteinuria, hematuria, and mild elevation of the BUN as
evidence of renal damage, episodes of abdominal pain and
changing heart rythms due to myocardial damage.
• TX: Repeated plasmaphoresis and plasma exchange may
be needed to supply enough normal plasma to induce and
maintain a remission.
Antibodies to Von Willebrand FactorCleaving Protease in Acute TTP
• TTP is a potentially fatal disease characterized by
widespread platelet thrombi in the microcirculation. In the
normal circulation, VWF is cleaved by a plasma protease.
• Hypothesis: A deficiency of this protease predisposes
patients with TTP to platelet thrombosis.
• Conclusion: Inhibitory antibodies against VWF-cleaving
protease occur in patients with acute TTP. A deficiency of
this protease is likely to have a critical role in the
pathogenesis of platelet thrombosis in this disease.
NEJM: Transfusion-related
Acute Lung Injury
Mechanism based on considerable evidence suggests:
When complement is activated, C5a promotes aggregation,
margination, and sequestration of neutrophils in the
pulmonary microvasculature.
Rabbit studies as well as observations in patients with the
acute respiratory distress syndrome show that when
complement-activated neutrophils release proteases,
oxygen radicals, and acidic lipids, the pulmonary vascular
endothelium is damaged, with subsequent extravasation of
protein-laden fluid into the interstitium and alveoli.
A 72 Year Old Man with Multiple
Infarctions of Uncertain Etiology