Whipple disease (neurological manifestations)

Neurological manifestations of Whipple disease are rare. Whipple's disease can also appear as a primary neurological disorder in rare cases. It is rarely incidentally found as a differential diagnosis in patients with a progressive neurological deficit.

It has been suggested that neurological involvement occur in all patients, however only 10-20% patients become symptomatic and rest of the cases remain sub clinical or asymptomatic.

Clinical presentation

 Clinical features are non specific and include

  • progressive dementia
  • external ophthalmoplegia
  • myoclonus
  • seizures 
  • ataxia
  • hypothalamic dysfunction - sleep disorders, polydipsia, polyphagia

Radiographic features

The CT scans and MRI of the brain are often normal, but may show cortical or subcortical atrophy, hydrocephalus, focal or intracerebral mass lesions. 


MRI may reveal T2 hyperintense and mildly enhancing lesions in midbrain, mesial temporal lobe, hypothalamus and corticospinal tracts.

No restriction of diffusion is seen.


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Article information

rID: 28028
Synonyms or Alternate Spellings:
  • CNS manifestations of whipple disease
  • Whipple's disease - CNS manifestations

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