Epistemis

Rigidity in gear wheel

Specialty: Neurology.

  • extrapyramidal rigidity
  • toothed limb phenomenon
  • intermittent plastic hypertonia

Why it occurs

  • Idiopathic Parkinson's disease
  • Atypical Parkinsonisms (Multiple System Atrophy, Progressive Supranuclear Palsy, Corticobasal Degeneration)
  • Parkinsonism secondary to dopamine receptor blocking drugs (neuroleptics, metoclopramide)
  • Vascular parkinsonism (multiple lacunar infarcts in basal ganglia)
  • Toxicity due to heavy metals or carbon monoxide

Initial workup

Evaluation of passive mobility in the wrist, elbow and neck joints to identify constant resistance fractionated by the underlying tremor (Black phenomenon); high-resolution brain magnetic resonance imaging (MRI) to rule out structural lesions or patterns of mesencephalic or striatal atrophy; DaTscan (SPECT with ioflupane I-123) to detect loss of presynaptic dopamine transporters in the striatum.

red flags

Extrapyramidal rigidity of acute or subacute onset associated with high fever, autonomic instability, altered mental status and marked elevation of creatine kinase (CK), compatible with Neuroleptic Malignant Syndrome induced by antipsychotic drugs.

Standard management

  • Levodopa/Carbidopa — start with 50/12.5 mg three times a day, increasing according to tolerance and clinical response
  • Pramipexole or Rotigotine — dopamine agonists
  • Biperiden or Trihexyphenidyl — anticholinergics useful for extrapyramidal rigidity and tremor, with caution in the elderly due to risk of delirium
  • Immediate withdrawal of the offending drug if it is pharmacological parkinsonism.

Educational guidance for study. It is NOT a prescription recommendation. The actual choice depends on the cause, the patient, and current guidelines.

Area
Neurology
Listed causes
5
Treatment options
4
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