Oculo-facio-skeletal myorhythmia as a cerebral complication of systemic Whipple's disease.
A fifth case of oculomasticatory myorhythmia associated with cerebral Whipple's disease is reported. This peculiar abnormal movement has never been described in association with cerebral dysfunction other than Whipple's disease. The present case exhibited rhythmic convergence of the eyes and synchronous (1-2 Hz) contractions of the masticatory muscles and of the proximal and distal skeletal muscles. These abnormal movements occurred 13 years after the beginning of the disease. They were persistent and unchanged until the death of the patient 3 months later. No treatment was effective to suppress the involuntary movements (clonazepam, baclofen, antibiotics). Associated neurological signs included global supranuclear ophthalmoplegia, facial weakness, bilateral ptosis, absent gag reflex, and intellectual deterioration.
Hausser-Hauw
C
C
Department of Neurology, Hôpital Saint-Antoine, Paris, France.
Roullet
E
E
Robert
R
R
Marteau
R
R
D001921:Brain / Q000503:physiopathology*; D005147:Facial Bones / Q000473:pathology; D005149:Facial Expression; D005152:Facial Muscles / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008410:Masticatory Muscles / Q000503:physiopathology; D008875:Middle Aged; D009069:Movement Disorders / Q000209:etiology*; D009132:Muscles / Q000503:physiopathology*; D009886:Ophthalmoplegia / Q000503:physiopathology*; D008061:Whipple Disease / Q000503:physiopathology*