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ABSTRACT

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In the acute setting, the fields of Neurology and Internal Medicine are often intertwined, necessitating a working understanding of general principles of Internal Medicine for the practicing neurologist. This chapter explores the neurologic manifestations of selective hematologic disease, direct neurologic involvement of systemic malignancy, and neurologic complications of commonly performed inpatient procedures. Examples of hematologic diseases include red blood cell disorders (eg, sickle cell disease, nutritional anemia, neuroacanthocytosis, and polycythemia vera), bleeding diatheses and platelet disorders (eg, disseminated intravascular coagulation, immune thrombocytopenic purpura, thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, and essential thrombocytosis), and white blood cell disorders (eg, plasma cell disorders such as monoclonal gammopathy of uncertain significance, polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes [POEMS] syndrome, multiple myeloma, and Waldenströ m macroglobulinemia, chronic myelogenous leukemia, and acute leukemia). Discussion of systemic malignancies includes breast cancer, colon cancer, pancreatic cancer, prostate cancer, and Pancoast tumor. Descriptions of commonly encountered inpatient procedures include cardiac catheterization, cesarean section, upper gastrointestinal endoscopy, and arthroplasty.

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NEUROLOGICAL COMPLICATIONS OF HEMATOLOGICAL DISEASE

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Red blood cell disorders and neurological disease

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CASE 51-1

A 26-year-old man with sickle cell disease is admitted for acute vaso-occlusive pain crisis. Over the course of his life, he has received inconsistent care for his known hemoglobin SS disease. On the third day of hospitalization, the patient develops sudden onset of slurred speech and right-sided weakness.

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How do red blood cell disorders manifest neurologically?
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The primary function of red blood cells is the transportation of oxygen via hemoglobin, a protein molecule comprising 2 α- and two β-globin chains. Disruptions in red blood cell production (diminished or accelerated), alterations of red blood cell membrane structure, and abnormalities in hemoglobin all risk directly inhibiting neurologic cellular function via impaired oxygen delivery. The varied underlying pathophysiologic mechanisms of red blood cell abnormalities, both directly and indirectly, pose the risk of contributing to additional neurologic sequela.

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Are basic laboratory tests effective in identifying the presence of hematologic disease?
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Reassuringly, the initial serological assessment for hematologic disease, the complete blood count, provides very helpful information when a red blood cell disorder is suspected. The number, morphology, and hemoglobin content of red blood cells are all reported in the red cell indices. Evaluation of the peripheral blood smear provides further morphologic detail. With the addition of another common blood test, the reticulocyte count, bone marrow activity can also be assessed.

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A clinician’s responsibility in the investigation of suspected red blood cell disease is to use pertinent historical information—including family and social history—along with physical examination findings, to pursue more directed testing.

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What tests are unique to sickle cell disease, as depicted in Case 1?
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The presence of sickle hemoglobin (HbS) is the hallmark of sickle cell disease and can be measured by hemoglobin electrophoresis. In ...

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