Parkinson-plus Syndromes

There are four main Parkinson-plus syndromes, which present as Parkinsonism (triad of resting tremor, hypertonia, and bradykinesia) with additional clinical features

Progressive supranuclear palsy

  • Parkinsonism and vertical gaze palsy (dysfunction of the muscles involved in looking upwards)
  • Tend to present with a more symmetric and tremor-negative parkinsonism

Multiple system atrophy

  • Rare condition where the neurones of multiple systems in the brain degenerate
  • Affects the basal ganglia as well as multiple other areas
    • The degeneration of the basal ganglia lead to a Parkinson’s presentation
    • The degeneration in other areas lead to autonomic dysfunction (causing postural hypotension, constipation, abnormal sweating and sexual dysfunction) and cerebellar dysfunction (causing ataxia)
  • Tend to present with a more symmetric and tremor-negative parkinsonism

Cortico-basal degeneration

  • Parkinsonism and involves spontaneous activity by an affected limb, or akinetic rigidity of that limb - ‘alien hand syndrome’
  • Patients sometimes present with apraxia - the inability to conceptulise movement i.e. description of not being able to follow instructions, but being able to do them automatically

Lewy body dementia

  • Parkinsonism and fluctuations in cognitive impairment and visual hallucinations, often before Parkinsonian features occur

Others

Drug-induced parkinsonism

  • Dopamine-blocking or depleting drugs, particularly neuroleptics (with the exception of clozapine), induce parkinsonism or worsen symptoms in affected patients, and may precipitate symptoms in elderly patients in the presymptomatic phase
  • Antimuscarinic drugs reduce these symptoms, although tardive dyskinesia may be made worse

Wilson’s disease

  • Rare and treatable disorder of copper metabolism inherited as an autosomal recessive trait
  • Copper deposition occurs in the basal ganglia, cornea and liver where it can cause cirrhosis
  • Neurological manifestations:
    • An akinetic-rigid syndrome similar to Parkinson's disease
    • Pseudosclerosis dominated by tremor
    • Ataxia
    • A dystonic syndrome, which often leads to severe contractures
  • All young patients (below age 50) with any hyperkinetic movement disorder or with liver cirrhosis should be screened for Wilson’s disease (serum copper and caeruloplasmin should be checked)
  • Intellectual impairment develops
  • Neurological damage is reversible with early treatment
  • Management - chelating agent penicillamine