Catalog

Central nervous system demyelination due to Whipple’s disease

No MMS code

Link to WHO Definition

Central nervous system demyelination due to Whipple’s disease – http://id.who.int/icd/entity/1911167360

This is caused by the weakly Gram-positive rod-shaped bacillus, Tropheryma whippeli. It typically presents with a malabsorption syndrome but the central nervous system is also affected. The most common features are brainstem syndromes (oculo-masticatory myorhythmia-rhythmic myoclonic movements of the jaw, lower face and eyes). This is said to be pathognomonic of cerebral Whipple's disease. It can also present with dementia and an akinetic-rigid syndrome.