October 29, 2019, e-Pearl of the Week: Pisa syndrome

Pisa syndrome

Pisa syndrome (pleurothotonus) presents as lateral trunk dystonia greater than 10 degrees relieved by passive mobilization or supine position, although there is no unanimous definition.  Pisa syndrome is most commonly caused by drugs (e.g. neuroleptics, antiemetic, anticholinesterases)1 or neurodegenerative disorders (e.g., Parkinson, Multiple System Atrophy), though many cases remain idiopathic. The prevalence is 0.42%.2 Pathophysiology involves dopaminergic-cholinergic imbalance and/or serotonergic-noradrenergic dysfunction.3  Acute/subacute forms are usually reversible if there is early recognition and modification of anti-parkinsonian drugs.4 Chronic Pisa syndrome can occur in a combined fashion with camptocormia and deteriorate over time.4 Risk factors include age, female sex, or rapid increase in dosage of neuroleptics.  Anticholinergics are effective in 40% of cases.3 Supportive treatment, such as physical therapy with postural retraining, has the best outcome for recovery.5 

References

  1. Yokochi F. Lateral flexion in Parkinson’s disease and Pisa syndrome. J Neurol 2006; 253 Suppl 7:VII17-20.
  2. Lee Y. Antipsychotic-Induced Pisa Syndrome : A 2-Year Follow-up Study. Clin Neuropharmacol 2018;41:60–63.
  3. Suzuki T, Matsuzaka H. Drug-Induced Pisa Syndrome (Pleurothotonus): epidemiology and management. CNS Drugs 2002;16, 165–174.
  4. Michel SF, Arias Carrión O, Correa TE, Alejandro PL, Micheli F. Pisa Syndrome. Clin Neuropharmacol 2015;38:135–140.
  5. Miletić V. Pisa syndrome in Parkinson's disease: Diagnostic and management challenges. Journal of Parkinsonism and Restless Legs Syndrome 2016;6:29–35.

Submitted by Chisom Enebeke, Medical Student-Trinity School of Medicine, St Vincent and the Grenadines, and Faisal Khan, MD, Consultant Neurologist-Sugarland Neurology and Sleep, Texas

Dr. Enebeke and Dr. Khan report no disclosures.