Whipple’s disease: management

Evidence-based neurology checklist on whipple’s disease: management: Magnetic resonance imaging (MRI) This shows single or multiple T2 high signal changes There is minimal enhancement with contrast There is no restricted diffusion There are focal brain lesions: cystic or tumoural There is diffuse…

Magnetic resonance imaging (MRI)

  • This shows single or multiple T2 high signal changes
  • There is minimal enhancement with contrast
  • There is no restricted diffusion
  • There are focal brain lesions: cystic or tumoural
  • There is diffuse cerebral atrophy and pachymeningitis
  • Periventricular diffuse leukopathy may be present
  • The MRI may be normal

Magnetic resonance imaging (MRI): lesion locations

Blood tests

Cerebrospinal fluid (CSF) analysis

Upper gastrointestinal biopsy and histology: features

Treatment: induction: for 2 weeks

Treatment: maintenance: for at least 12 months

Treatment: alternative options

References

  1. Black DF, Aksamit AJ, Morris JM. MR imaging of central nervous system Whipple disease: a 15-year review. Am J Neuroradiol 2010; 31:1493-1497.
  2. Panegyres PK, Efis R, Beaman M, Fallon M. Primary Whipple's disease of the brain: characterization of the clinical syndrome and molecular diagnosis. Q J Med 2006; 99:609-623.
  3. Compain C, Sacre K, Puéchal X, et al. Central nervous system involvement in Whipple disease: clinical study of 18 patients and long-term follow-up. Medicine (Baltimore) 2013; 92:324-330. 
  4. Kilani M, Njim L, Nsir AB, Hattab MN. Whipple disease presenting as cystic brain tumor: case report and review of the literature. Turk Neurosurg 2018; 28:495-499. 
  5. Löhr M, Stenzel W, Plum G, Gross WP, Deckert M, Klug N. Whipple disease confined to the central nervous system presenting as a solitary frontal tumor. Case report. J Neurosurg 2004; 101:336-339.
  6. And 3 more. Subscribe to see the full list

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