Dementia in a patient with Thymoma and hypogammaglobulinaemia (Good's syndrome)

Abstract

Good's syndrome is extremely rare and refers to an acquired B and T cell immunodeficiency in thymoma patients. The authors of this article present a case report of a 75-year-old, caucasian male patient previously subjected to examinations for secondary dementia and recurrent infections, which revealed paraneoplastic syndrome arose from thymoma. He underwent thymectomy, while his immunodeficiency syndrome sustained with frequent opportunistic infections, constantly requiring intravenous immunoglobulin treatment.

Good Syndrome, a Rare Cause of Refractory Chronic Diarrhea and Recurrent Pneumonia in a Chinese Patient after Thymectomy

“…For some patients, symptoms improved (12), for some symptoms became aggravated (14), and for the others there was no change (15,16). Our patient's symptoms became worse after the surgery.…”

Good’s syndrome: Is thymectomy the solution? Case report and literature review

“…The incidence of Good’s syndrome is estimated as 6%–11% of patients with thymoma. 4 Thymoma itself is rare with an incidence of around 0.15 cases per 100,000 in the USA. 5 A systematic review in 2010 identified 152 patients with Good’s syndrome from all available literature.…”

Thymoma with immunodeficiency with multiple recurrent oral herpetic infections

“…Although thymomas are the most frequently encountered primary neoplasm in the anterior mediastinum in adult patients, they are actually rare malignant neoplasms 1 . Immunodeficiency syndrome associated with a thymoma was first reported by Good and colleagues in 1954 and was commonly referred to as Good's syndrome 2 .…”

“…This syndrome is a rare acquired disease of combined T- and B-cell immunodeficiency accompanying a thymoma and has an incidence rate of approximately 6–11% in thymoma cases 3 . The affected patients are most commonly between 40 and 70 years of age and have a thymoma, fewer or no B cells in the peripheral blood, hypogammaglobulinemia, inversion of the CD4-to-CD8 ratio, and defects in cell-mediated immunity 1, 4. Patients with this primary immunodeficiency are at increased risk of developing severe opportunistic infections including herpes simplex virus (HSV)-related infections 2 …”