Neurology: Case of the Month

Test Yourself — Patient 74

Whipple's disease affecting the CNS;
Acquired immune deficiency syndrome

1. Which of the following ocular signs is pathognomonic for CNS Whipple's disease?
   • [ A ] Superior oblique myokymia
   • [ B ] Oculomasticatory myorhythmia
   • [ C ] Optic apraxia
   • [ D ] Oculofacial synkinesis
2. Which of the following features would suggest a diagnosis of possible CNS Whipple's disease?
   • [ A ] Unexplained dementia with prominent gastrointestinal symptoms
   • [ B ] Unexplained lymphadenopathy and night sweats with rhythmic myoclonic jerking of the face and extremities
   • [ C ] Unexplained fever and arthralgias, with agitated depression
   • [ D ] Any of the above
3. What constitutes appropriate treatment of CNS Whipple's disease?
   • [ A ] Supportive therapy only
   • [ B ] Reversal of the predisposing immunosuppressive state
   • [ C ] Rifampin and ethambutol for three to six months
   • [ D ] A third-generation cephalosporin with good CNS penetration, followed by high-dosetrimethoprim/sulfamethoxazole for 1-2 years
   • [ E ] Glucocorticoids to reduce edema
4. Which of the following diagnostic tests can reliably confirm the diagnosis of Whipple's disease?
   • [ A ] Light microscopic demonstration in duodenal mucosa of macrophages or histiocytes containing PAS-positive material
   • [ B ] PCR of stool specimens for an organism-specific 16S ribosomal RNA
   • [ C ] Culture of the causative organism from CSF
   • [ D ] Characteristic poorly-enhancing, diencephalic lesions on MR imaging
   • [ E ] PCR showing organism-specific DNA in CSF

 

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