BLEPHAROSPASM AND VERTICAL OPHTHALMOPARESIS AS PRESENTING SYMPTOMS IN WHIPPLEʼS DISEASE

Abstract

BACKGROUND- Whipple's disease may predominantly manifest with neurologic symptoms, especially myorhythmia and ocular movement disorders. Oculomasticatory myorhythmia and oculofacial-skeletal myorhythmia are well documented pathognomonic signs unique to CNS Whipple's disease. Vertical supranuclear ophthalmoparesis has been reported in many afflicted patients. Blepharospasm has not been reported as a presenting symptom. REVIEW SUMMARY- We report a patient with systemic Whipple's disease who exhibited almost exclusively CNS symptoms. The patient developed both ocular and skeletal myorhythmia without recognizable facial involvement. Later, despite aggressive antibiotic treatment, this patient developed severe aseptic meningitis, although polymerase chain reaction (PCR) for Tropheryma whippelii in the cerebrospinal fluid (CSF) was negative. The movement disorders did not respond to antibiotics or to agents such as carbidopa-levodopa, valproate, carbamazepine, indomethacin, or clonaze-pam. CONCLUSIONS- This case documents the difficulty of early recognition, the spectrum of cardinal neurologic symptoms and complications, and the difficulty in managing of such patients. The role of CSF-PCR for Tropheryma whippelii remains controversial as a means to diagnose CNS Whipple's disease.