TY - JOUR
T1 - Periodic myoclonus due to cytomegalovirus encephalitis in a patient with Good syndrome
AU - Striano, Pasquale
AU - Tortora, Fabio
AU - Evoli, Amelia
AU - Palmieri, Giovannella
AU - Elefante, Andrea
AU - Zara, Federico
AU - Tarr, Philip E.
AU - Striano, Salvatore
PY - 2007/2
Y1 - 2007/2
N2 - 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.
AB - 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.
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U2 - 10.1001/archneur.64.2.277
DO - 10.1001/archneur.64.2.277
M3 - Article
C2 - 17296846
AN - SCOPUS:33846941935
VL - 64
SP - 277
EP - 279
JO - Archives of Neurology
JF - Archives of Neurology
SN - 0003-9942
IS - 2
ER -