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
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