Brueghel Syndrome as a New Manifestation of HIV Encephalopathy

and fluctuating somnolence. The most striking feature involved facial abnormal involuntary movements consisting of a complex, bilateral and constant ly evolving facial dystonia which includ ed, either successively or simultaneously, frowning, blepharospasm, eyelid-opening apraxia, contractions of paranasal muscles and lips reminiscent of rabbit syndrome, and multidirectional movements of the jaw with frequent wide opening of the mouth ( fig. 1 a). These movements led to a dramatic and continuous facial grimacing which worsened when the patient was stimulated and disappeared during sleep. In addition, she exhibited diffuse spas ticity, hyperreflexia, bilateral Babinski sign, urinary incontinence and incomplete paraplegia. Blood tests showed marked pancytopenia and liver dysfunction. HIV staging was C3 with a low CD4 count (CD4 16/mm 3 , normal 600–1,950/mm 3 ) and high viremia (600,000 copies/ml). Brain magnetic resonance imaging (MRI) scan showed, on T 2 weighted and FLAIR images, confluent hyperintensities involving the lenticular nucleus bilaterally, particularly prominent in the left globus pallidus and extending toward internal capsules ( fig. 1 c, d). Cerebrospinal fluid analyses were normal except for mildly elevated total proteins (0.68 g/l). EEG demonstrated generalized slow dysrythmia. An extensive search for opportunistic infections proved negative, HCV antibodies were positive. HIV encephalopathy was diagnosed and HAART associating lamivudin 300 mg/day, zidoDear Sir, A large variety of movement disorders have been reported in HIV-infected patients including tremor, hemichoreahemiballism, paroxysmal dyskinesia, tic, myoclonus, dystonia, and parkinsonism [1–4] . In most cases, hyperkinesias have been related to AIDS-defining opportunistic infections involving the basal ganglia, in particular toxoplasmosis, whereas parkinsonism and tremor have been linked to a deleterious effect of HIV-related pathology on the dopaminergic system [1, 3] . Only a few patients with focal or generalized dystonia have been reported so far [2, 4–8] , only one of them possibly related to HIV infection itself [5] . We here report a unique patient who rapidly developed a severe blepharospasm-oromandibular dystonia syndrome corresponding to Brueghel syndrome as the revealing manifestation of HIV encephalopathy, which entirely resolved when treated by highly active antiretroviral therapy (HAART).