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

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%)