Continuum, Neurologic Manifestations Of Systemic Disease, February 2008, Volume 14, Issue 1

Infective and noninfective endocarditis are rare diseases that have strong predispositions for neurologic complications. Although cerebral embolism is the principal mechanism of neurologic injury in both diseases, they differ greatly in the types of clinical presentation, cardiac valvular and cerebral infarct characteristics, and medical and surgical treatment approaches. Therefore, the evaluation of patients with endocarditis warrants an understanding and appreciation for neurologic symptoms and signs, known risk factors, available neurodiagnostic tools, and potential treatment options. It is hoped that rapid recognition and appropriate early management may help reduce the significant neurologic morbidity and mortality associated with endocarditis.(C) 2008 American Academy of Neurology

Systemic lupus erythematosus (SLE) and the antiphospholipid antibody syndrome (APS) are autoimmune diseases that carry a high risk of thrombosis and other nervous system manifestations. APS is frequently seen in patients with SLE, but can be seen in patients without it. The nervous system is commonly affected in both children and adults with SLE and is also associated with a worse prognosis and more cumulative damage in children and adults. Neuropsychiatric lupus manifestations can occur in the absence of either serologic activity or other systemic disease manifestations. The American College of Rheumatology established case definitions for 19 central and peripheral nervous system syndromes. APS is defined as an episode of arterial or venous thrombosis leading to tissue ischemia or recurrent fetal loss in the presence of antiphospholipid antibodies of moderate to high titer or a lupus anticoagulant that is present on at least two occasions at least 12 weeks apart. APS is classified as primary if it occurs in an individual without and secondary when it occurs with SLE or some other connective tissue disease. This chapter will review the neurologic manifestations of SLE and APS, highlighting their interrelationships, as well as the clinical differences in each when the other is not present. The diagnosis, pathophysiology, and treatment of these manifestations will also be discussed.(C) 2008 American Academy of Neurology

Neurologic complications of coronary artery bypass graft (CABG) surgery include stroke, coma, encephalopathy, neurocognitive decline, and peripheral nerve injuries. The causes of adverse cerebral events after cardiac surgery are multifactorial; however, the use of cardiopulmonary bypass in particular has been implicated. Compared with CABG, more complex cardiac surgeries, such as valve replacement and aortic arch surgery, are associated with higher rates of mortality and morbidity, including cerebral complications. Aortic arch surgery is associated with unique complications related to the use of hypothermic circulatory arrest. Knowledge of the risk factors associated with neurologic complications after cardiac surgery is critical for diagnosis and management.(C) 2008 American Academy of Neurology

Involvement of the nervous system by sarcoidosis is defined by pathologic proof either directly in the nervous system or systemically with appropriate findings consistent with sarcoidosis on neuroimaging and spinal fluid examinations. CNS sarcoidosis can present without systemic manifestations. Chronic meningitis is the most common clinical presentation, but cranial neuropathy, myelopathy, peripheral neuropathy, and myopathy are all possible. MRI imaging and gadolinium enhancement of the CNS is important for diagnosis. Spinal fluid abnormalities can be used as markers of disease activity during therapy. Biopsy of abnormal lymph node, lung, skin, meninges, or brain confirms the diagnosis. Conjunctival biopsy is a reasonable site for blind biopsy in CNS cases. Corticosteroids remain the mainstay of therapy, but the use of tumor necrosis factor [alpha]-antagonists shows significant promise in treatment of refractory CNS sarcoidosis cases.(C) 2008 American Academy of Neurology