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
| 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%) |
Significant p values are noted in bold.
* 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.