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Table 3 Clinical features* of 74 patients with Whipple's disease (i.e., patients with histological digestive involvement characterised by positive PAS staining) with neurologic manifestations and those with certain (13) and possible (7) T. whipplei chronic encephalitis without digestive lesions

From: Progressive dementia associated with ataxia or obesity in patients with Tropheryma whipplei encephalitis

Neurologic manifestations Whipple's disease with neurologic manifestations Certain T. whipplei encephalitis Possible T. whipplei encephalitis Certain and possible T. whipplei encephalitis
Number of patients 74 13 7 20
Cognitive impairment 53 (72%) 9 (69%) 7 (100%) 16 (80%)
Ataxia 12 (16%) 9 (69%; p < 10 -3 ) 4 (57%; p = 0.02) 13 (65%; p < 10 -3 )
Supranuclear ophthalmoplegia 29 (39%) 5 (38%) 4 (57%) 9 (45%)
Hypothalamic manifestations 24 (32%) 4 (31%) 2 (29%) 6 (30%)
Dysarthria 9 (12%) 4 (31%) 3 (43%) 7 (35%; p = 0.03)
Cerebellar forms 4 (5.5%) 3 (23%; p = 0.06) 3 (43%; p = 0.03) 6 (26%; p = 0.005)
Myorhythmia 10 (13.5%) 4 (31%) 3 (43%) 7 (35%; p = 0.045)
Oculomasticatory myorhythmia 7 (5.5%) 2 (17%) 2 (29%) 4 (20%)
Upper motor neuron disorder 11 (15%) 4 (31%) 3 (43%) 7 (35%)
Decreasing level of consciousness 25 (35%) 2 (15%) 6 (75%; p = 0.01) 8 (40%)
Myoclonus 29 (39%) 3 (23%) 1 (14%) 4 (20%)
Depression 11 (15%) 3 (23%) 1 (14%) 3 (15%)
Personality changes 22 (30%) 2 (15%) 2 (29%) 4 (20%)
Headache 10 (13.5%) 1 (8%) 2 (29%) 3 (15%)
Apathy 15 (20%) 1 (8%) 1 (14%) 2 (10%)
Muscle weakness 14 (19%) 2 (15%) 2 (29%) 4 (20%)
Seizures 13 (18%) 3 (23%) 1 (14%) 4 (20%)
Nystagmus 11 (15%) 3 (23%) 1 (14%) 4 (20%)
Extrapyramidal movement disorder 8 (11%) 2 (17%) 2 (29%) 4 (20%)
  1. Significant p values are noted in bold.
  2. * Neurological signs were defined as follows: "Decreasing level of consciousness" included somnolence, lethargy, and coma. "Cognitive impairment" included abnormalities of orientation, memory, or reasoning. "Hypothalamic manifestations" included polydipsia, hyperphagia, sexual impotence, changes in the sleep-wake cycle and insomnia, but not isolated somnolence. Oculomasticatory myorhythmia was defined as pendular vergence oscillations of the eyes that were synchronous with masticatory myorhythmia. Both were classified as myorhythmia. "Myoclonus", which is nonrhythmic, was distinguished from myorhythmia. "Cerebellar forms" were considered when a patient presented dysarthria and ataxia.