Opsoclonus, defined as rapid, involuntary, chaotic, repetitive, conjugate saccadic eye movements in all directions and persisting during sleep, is often accompanied by myoclonic jerks of the face and body, and at least as frequently by cerebellar ataxia. Opsoclonus, first described by Orzechowski in 1913, has been known by other names such as "dancing eyes" "acute ocular oscillation" "ataxic conjugate movements of the eyes" and "saccadomania". Opsoclonus has been ascribed to viral infection of the central nervous system or the remote effect of a visceral carcinoma. We present two cases of opsoclonus with brief review of literatures. The first case is 63-year-old man presented with 15 day history of fever, chill, dehydration and 4 days history of body tremulousness, ataxia, dizziness and opsoclonus. On physical examination, there were typical opsoclonus, intention tremor, cerebellar ataxia and myoclonus of extremities. Brain CT scan showed mild cerebral atrophy and calcification in the basal ganglia. Opsoclonus was completely disappeared 1 month later from onset of symptoms. The second case is 2 month-old girl presented with 4 months history of gait unbalance, opsoclonus and myoclonic jerk of extremities. The brain CT scan was normal. Certain viral infection was suspected as a cause of opsoclonus of our 2 cases.
Ataxia ; Atrophy ; Basal Ganglia ; Brain ; Central Nervous System ; Cerebellar Ataxia ; Dehydration ; Dizziness ; Extremities ; Female ; Fever ; Gait ; Humans ; Infant ; Middle Aged ; Myoclonus ; Ocular Motility Disorders* ; Physical Examination ; Saccades ; Tomography, X-Ray Computed ; Tremor

BACKGROUND AND PURPOSE: Although plasmapheresis is becoming standard practice as a rescue therapy for neuromyelitis optica (NMO), evidence for the therapeutic efficacy of plasmapheresis is limited, and the effect of plasmapheresis on anti-aquaporin-4 (AQP4) levels in patients with NMO has not been reported. Here, our objective was to evaluate the clinical efficacy of therapeutic plasmapheresis and its effect on anti-AQP4 antibody levels in patients with NMO spectrum disorder (NMOSD). METHODS: We retrospectively reviewed the medical records of 15 patients with NMOSD who had 18 acute attacks and received plasmapheresis because they did not respond to high-dose intravenous methylprednisolone (IVMP) therapy. Anti-AQP4 antibodies were measured before and after plasmapheresis. The primary outcomes were functional improvements immediately and 6 months after plasmapheresis, and the secondary outcome was the change in anti-AQP4 antibody serum levels following plasmapheresis. RESULTS: Plasmapheresis following IVMP therapy led to significant improvement in 50% of the 18 attacks in 15 patients immediately after the procedure was completed, and in 78% (14 attacks) after 6 months. Plasmapheresis was generally well tolerated in all patients. Anti-AQP4 antibody serum levels declined significantly following plasmapheresis, to a mean of 15% of the preplasmapheresis levels. Lower scores on the visual outcome scale recorded before an attack were associated with significant immediate improvement upon the completion of plasmapheresis (p=0.03). CONCLUSIONS: Plasmapheresis following IVMP therapy effectively removed anti-AQP4 antibodies and was accompanied by a substantial improvement in the neurological disability of patients with NMOSD. Lower levels of pre-existing neurological damage may be associated with an improved acute response to plasmapheresis.
Antibodies ; Humans ; Medical Records ; Methylprednisolone ; Neuromyelitis Optica ; Plasmapheresis ; Retrospective Studies