Cerebral Whipple’s disease: clinical and cerebrospinal fluid findings

Abstract

The case of a patient who had a relapse of cerebral Whipple's disease (WD) one year after discontinuation of a two-years' antibiotic treatment is reported. Neither the clinical course nor the results of magnetic resonance imaging (MRI) and routine examination of the cerebrospinal fluid (CSF) allowed the caring physician to predict the relapse. Retrospective analysis of serial specimens of CSF showed that slight CSF leucocytosis and intrathecal synthesis of IgA might have suggested persistence of infection. The decision to stop antibiotic therapy in cerebral WD is difficult, but evaluation of cell counts and of intrathecal synthesis of IgA may help in the decision. Some patients may need to take treatment indefinitely.

Neurological presentation of Whipple's disease after long-term antibiotic treatment: a case report

“…Currently, recommended antibiotic therapies include an initial 2-week course of intravenous cephalosporins (for example, ceftriaxone) or meropenem for 2 weeks followed by 1-year oral trimethoprim-sulphamethoxazole [ 1 ]. As described before, clinical relapses such as in our patient with recurrent CNS manifestation may occur even after long-term oral antibiotic treatment with trimethoprim-sulphamethoxazole if the causative agent was not eradicated timely from the CNS [ 10 ]. Patients treated primarily with tetracycline show a high relapse rate of 35%, especially CNS relapse.…”

The spectrum of central nervous system involvement in Whipple's disease

“…Systemic TW‐specific immunoreactivity and especially IgG and IgM response are low in patients with WD, which paradoxically may even be used to distinguish patients from asymptomatic carriers who express a stronger immune response [36, 37]. Individual case reports, however, have sporadically reported detection of intrathecal IgA or CSF oligoclonal bands [8, 38]. To our knowledge, TW‐specific antibodies have not yet been studied in CSF.…”