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Pasquale Striano, MD; Fabio Tortora, MD; Amelia Evoli, MD; Giovannella Palmieri, MD; Andrea Elefante, MD; Federico Zara, PhD; Philip E. Tarr, MD; Salvatore Striano, MD

Arch Neurol. 2007;64(2):277-279.

Background  Good syndrome (GS) refers to a constellation of thymoma and adult-onset immunodeficiency characterized by low or absent B cells, hypogammaglobulinemia, and variable defects in cell-mediated immunity with an inverted CD4/CD8⁺ T-cell ratio. Patients may develop severe or chronic infections as a result of this immunodeficiency.

Objective  To describe a patient with GS who developed cytomegalovirus (CMV) encephalitis and showed a periodic electroencephalographic pattern and myoclonus.

Design  Case report.

Setting  Outpatient neurology clinic at a university medical center.

Patient  A 64-year-old man who developed periodic myoclonus involving the right half of his body and the left arm.

Results  Five years previously, the patient had undergone resection of a pathologically confirmed epithelial thymoma. Quantitative CMV polymerase chain reaction of the serum and cerebrospinal fluid showed strongly positive results (1:10 000), allowing the diagnosis of CMV encephalitis.

Conclusions  To the best of our knowledge, myoclonus and periodic electroencephalographic pattern have not previously been reported in CMV encephalitis. Opportunistic CMV infection should be considered early in the evaluation of patients with GS or a history of thymoma who develop unusual neurological symptoms.

Author Affiliations: Departments of Neurological Science (Drs P. Striano, Tortora, Elefante, and S. Striano) and Molecular and Clinical Oncology and Endocrinology (Dr Palmieri), Federico II University, Naples, Italy; Muscular and Neurodegenerative Disease Unit, Institute G. Gaslini, Genoa, Italy (Drs P. Striano and Zara); Department of Neuroscience, Catholic University, Rome, Italy (Dr Evoli); and Infectious Diseases Service, University Hospital, Lausanne, Switzerland (Dr Tarr).

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