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Progressive supranuclear palsy

Definition

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

Manifestations:

  1. Parkinson ? like rigidity, no tremor

  2. Loss of postural reflexes

  3. Postural dystonia increased on extension of the head.

  4. Progressive external ophthalmoplegia 1st to voluntary conjugate vertical gaze then to lateral gaze is diagnostic.

  5. Full range oculo-cephalic reflexes are deranged on arousal.

  6. Some degree of dementia.   

Treatment

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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