Progressive supranuclear palsy
Progressive degenerative changes in the basal ganglia, basal nuclei and cerebellum.
It is an uncommon condition, however it is the commonest form of Parkinsonism plus. It affects patients in their middle age / older
Parkinson ? like rigidity, no tremor
Loss of postural reflexes
Postural dystonia increased on extension of the head.
Progressive external ophthalmoplegia 1st to voluntary conjugate vertical gaze then to lateral gaze is diagnostic.
Full range oculo-cephalic reflexes are deranged on arousal.
Some degree of dementia.
Dopamine agonists (bromocryptine) brings some relief but progress is invariable. The poor response to treatment is explained by the loss of D2 receptor.
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