Paraneoplastic limbic encephalitis associated with ovarian teratoma
Editor–I read with great interest the article by Derry and colleagues (Clin Med October 2011 pp 476–8) on autoimmune limbic encephalitis. I would like to highlight an important form of paraneoplastic limbic encephalitis (PLE) which associated with ovarian teratoma.
While PLE has shown preponderance for females in their 60s and above, with small cell lung carcinoma being the most commonly-associated malignancy,1 a relatively new category of PLE associated with ovarian teratoma has been described recently, which was found in young female adults. In a case series, the mean age of reported cases was 25±8 years. They presented with prominent psychiatric symptoms and behavioral disturbances, focal or generalised seizures, refractory involuntary movements and autonomic instability. Central hypoventilation requiring prolonged ventilator support has also been reported in patients with brainstem involvement. Neuroimaging finding is characterised by temporal lobe or brainstem abnormality. Lumbar puncture in patients with PLE typically showed cerebrospinal fluid with lymphocytic pleocytosis.2
Accurate and early diagnosis of PLE can be difficult, as symptoms may precede the tumour diagnosis in up to 60% of patients by a median of three months,3 and the clinical presentation often mimics various forms of infectious and autoimmune disorders. More recently, antoantibody to N-methyl-D-aspartate receptor (NMDAR) of cell membrane antigens found in hippocampus and forebrain has been identified to have resulted in psychocognitive impairment. Presence of anti-NMDAR antibodies in the serum of patients with PLE is strongly associated with an ovarian teratoma, and is concentrated in the nervous tissue of the tumour. Malignancy eradication and immunosuppressive treatment has been shown to reduce morbidity and mortality. The time interval of clinical improvement, however, has been reported diversely from one to four months.2
In summary, PLE can be the first manifestation of ovarian teratoma and should be considered in young females who present with neuropsychiatric symptoms. Early diagnosis with aggressive treatment should be initiated to optimise clinical outcome.
Footnotes
Please submit letters for the editor's consideration within three weeks of receipt of Clinical Medicine. Letters should ideally be limited to 350 words, and sent by email to: clinicalmedicine{at}rcplondon.ac.uk
- © 2012 Royal College of Physicians
References
- ↵
- ↵
- Kataoka H,
- Ueno S
- ↵
- Gultekin SH,
- Rosenfeld MR,
- Voltz R,
- et al.
Article Tools
Citation Manager Formats
Jump to section
Related Articles
- No related articles found.
Cited By...
- No citing articles found.