Clinical Program for Cerebrovascular Disorders

Mount Sinai Medical Center

A 72 Year Old Man with Multiple Infarctions of Uncertain Etiology

Clinical Case PresentationClara 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 PresentationA 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 PresentationA 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: ??

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, platelet-fibrin 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 Factor-Cleaving 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