Carcinomatous meningitis A delusive, but lethal enemy

Abstract

Summary A 58-year old woman with a long-standing history of chronic recurrent headache, depression and anxiety presented with new onset occipital headaches, tinnitus, gait instability and worsening of mood disorder. A first extensive work-up failed to objectify a somatic disorder and a psychiatric origin was suspected. However, 6 weeks later focal neurological deficits including complex visual disturbances, seizures and cognitive decline appeared, initially parallelled by fever. Cerebrospinal fluid (CSF) analysis showed increased opening pressure and mild pleocytosis. After exclusion of infectious and auto-immune meningoencephalitis, carcinomatous meningitis was diagnosed on repeated CSF analysis. Due to a signet ring cell morphology of the malignant cells in the CSF, a gastric origin was suspected despite absence of any primary tumour after another extensive diagnostic work-up. Detection of serum anti-Ma2 auto-antibodies is in line with the hypothesis of an underlying intestinal neuroendocrine tumour, albeit there was no evidence of a paraneoplastic syndrome in this patient. The patient died within 2 months of new onset headaches. Carcinomatous meningitis is a rare condition which can manifest itself by multifocal neurological signs including neuropsychiatric symptoms and complex visual disturbances long before tumour diagnosis. A high index of suspicion with consequent and if necessary repeated CSF cytological analysis is crucial for correct and rapid diagnosis of this potentially fatal condition.