Whipple disease (neurological manifestations)

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Neurological manifestations of Whipple disease are rare. Whipple disease may appear as a primary neurological disorder in rare cases. It is rarely found as a cause of progressive neurological deterioration in patients.

It has been suggested that neurological involvement will eventually occur in all patients with Whipple disease. However, only 10-20% of Whipple disease patients develop neurological symptoms whilst the rest of the cases remain asymptomatic.

Clinical presentation

Clinical features are non-specific and include:

progressive dementia
external ophthalmoplegia
myoclonus
seizures
ataxia
hypothalamic dysfunction (e.g. 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

T2: hyperintense and mildly enhancing lesions in midbrain, mesial temporal lobe, hypothalamus and corticospinal tracts
DWI: lesions do not demonstrate restricted diffusion