Neuro-Whipple confirmed five years after a presumptive diagnosis of a primitive CNS vasculitis

Abstract

Sirs: We report a case of Whipple’s disease (WD) with an unusual presentation. The patient was a 22year-old woman with no particular medical history. Three months after an isolated self limited episode of gastro-enteritis, a rapid onset in less than 12 hours of a bilateral myelopathy with a sensory level corresponding to T8 was observed. There was an aseptic lymphocytic meningitis on CSF analyses. MRI showed a medullar lesion enhanced by gadolinium and extending from T1 to T8 on T2weighted images (Fig. 1 A). Despite antibiotic and antiviral therapy, the clinical picture evolved toward the installation, four days later, of stroke-like cerebral disturbances (facial central paralysis, left upper limb weakness and upbeat nystagmus) with secondary deterioration leading to swallowing difficulties and respiratory failure; the patient was placed on mechanical ventilation. Brain MRI showed multiple high signal lesions with no mass effect in the pons and the right peduncle, sub-thalamic region, internal capsule and temporal lobe on FLAIR, diffusion weighted images and T2 (Fig. 1 B). Numerous etiologic investigations undertaken were negative: viral, bacterial, parasitic and fungal serologies, PCRs performed on blood and CSF (including T.whippelii), auto-antibodies and tumor markers screening as well as gastro-colic fibroscopies with salivary gland and duodenal biopsies (Periodic Acid Schiff (PAS) staining was negative). The presence of numerous signal abnormalities on MRI suggestive of vasculitis, supported by the finding of vascular irregularities in the vertebro-basilar system on angiography, led to instituting a corticosteroid treatment with consequent clinical, biological LETTER TO THE EDITORS