Rapid-Onset Dystonia Parkinsonism
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Rapid-onset dystonia parkinsonism (RDP) is a rare autosomal dominant disorder that usually affects adolescents and young adults but occasionally young children. It begins abruptly and evolves very rapidly over days to weeks. A typical onset is with orofacial dystonia, severe dysarthria, dysphagia, and muscle cramps in upper extremities associated with variable degrees of bradykinesia, rigidity, and postural instability. Cranial and upper limb muscles are more affected than the lower limbs. Bulbar involvement is prominent manifest by an open mouth, drooling, and near inability to speak or swallow. Initial deficits usually stabilize over the first few weeks but may then persist unchanged or show mild improvement.
KeywordsOpen Mouth Limb Muscle Postural Instability Muscle Cramp Typical Onset
Rapid-onset dystonia-parkinsonism.mp4 (MP4 5,861KB)
Clip 1: the patient has a stiff gait with reduced arm swing. Facial expression is markedly reduced. Clip 2: the same patient exhibits fixed flexion dystonia of left hand. (Video contribution from Dr. Nutan Sharma, Massachusetts General Hospital, Boston.)