Myasthenia gravis associated with Good’s syndrome: a case report and
review of literature
Background: Good’s syndrome (GS) is a rare secondary immunodeficiency
disease associated with thymoma, which is characterized by chronic
recurrent infection. Due to abnormal immune function, more than half of
GS are complicated with autoimmune diseases, such as myasthenia gravis
(MG) and pure red cell aplastic anemia (PRCA).Case presentation: We
report a case of type III late-onset myasthenia gravis (MG) with
thymoma, which was gradually improved after mechanical ventilation,
Intravenous steroid pulse, intravenous immunoglobulin , and tacrolimus.
After weaning, the patient had a repeated myasthenia gravis crisis
caused by lung infections, urinary tract infections, bacteremia, and
septic shock, resulting in difficulty in weaning. The subsequent
immunological evaluation showed hypogammaglobulinemia, decreased B
lymphocytes , and an inverted proportion of CT4+/CD8+ cells, which
confirmed the diagnosis of GS.Conclusions: GS should be strongly
suspected and immunological examinations performed when recurrent
opportunistic infections occur in patients with MG associated thymoma.
Early identification and intravenous administration of immunoglobulin
can reduce the incidence of future infection and improve the
prognosis.We summarize 16 previously reported cases of MG patients with
GS. The average age of onset of MG was 53 ±18 years old, and the ratio
of male to female was roughly equal. Mostly manifested as systemic
myasthenia gravis (77%), half of the patients had bulbar paralysis,
15% had myasthenia gravis crisis, and only 8% only involved
extraocular muscles. Thymomas of type B and Type A were the most common.
GS symptoms improved in 7 of the 10 patients, suggested thymectomy
played a positive role.