Sedentary stage

At this stage patients are able to sit unsupported but cannot walk. Attempted walking with support leads to a high steppage, wide-based gait with reeling instability, and flinging arm movements in an attempt to maintain posture. Hyper-reflexia is seen although plantar responses usually remain flexor with intact abdominal reflexes. Clonus is characteristically short-lived. Athetoid and choreiform movements and parkinsonian tremors may occur. There is no paralysis, although muscle power is reduced. Obesity is common at this stage but may be present in early disease associated with bulimia. Characteristically, there is emotional lability and bizarre uncontrollable laughter, which has led to the disease being referred to as 'laughing death'. There is no sensory impairment. In sharp contrast to CJD, myoclonic jerking is rarely seen. EEG is usually normal or may show non-specific changes.(43) This stage lasts around 2 to 3 months. When truncal ataxia reaches the point where the patient is unable to sit unsupported, the third or tertiary stage is reached.

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