Case

A 42 year old gentleman presented with rest tremors, bradykinesia and rigidity for 10 years. He never had any symptoms suggestive of autonomic dysfunctions. On examination there was no cognitive decline and cerebellar signs were absent. He was prescribed levodopa/carbidopa preparation with trihexphenidyl. He responded very well to the treatment [UPDRS scale (off 40, on 24)]. After three years of onset of the disease, family members noticed that while walking he was having lateral flexion of trunk and was leaning on left side (see video). Along with this he also developed striatal hand (Figure 1). During supine position his trunk posture used to improve completely. He also showed significant improvement in symptoms with levodopa/carbidopa. His abnormal posture was worse during ‘off’ period in comparison to ‘on’ period. He was never given any typical or atypical neuroleptic agents. X-rays whole spine, MRI brain and paraspinal electromyography (EMG) were normal.

Discussion

This patient of PD was diagnosed to have Pisa syndrome due to lateral flexion and axial dystonia on standing which used to completely improve in supine position. Normal EMG and spine X-rays rule out any other possibilities.

Pisa syndrome is a combination of lateral deviation of spine and corresponding tendency to lean on one side.3 Exact pathogenesis of PS is still not known. Different hypothesis have been discussed for understanding the mechanism of this syndrome. It is believed that cholinergic excess in patients of AD receiving cholinesterase inhibitor cause PS. In patients of PD it may be possible that striatal dopamine deficiency or imbalance in dopaminergic-cholinergic level is responsible for PS.4 This imbalance seems to be asymmetrical and is responsible for the lateral flexion on one side. Some authors have described improvement in this phenomenon following levodopa/carbidopa treatment. Our patient also had significant improvement in lateral flexion on standing during on period, however striatal hand symptoms showed no response to levodopa/carbidopa. It has been described that PD subjects in advanced stage of disease (HY III & IV) have more postural abnormality and striatal deformities while our patient manifested in early part of disease (HY IIb).3 Dopa agonists and amantidine has been tried with variable response. Neurosurgical interventions like pallidotomy and deep brain stimulation has also been tried in 13 subjects with good results.5

Notes

The authors have no financial conflicts of interest.

Figure 1

Striatal hand deformity in Parkinson’s disease patient.

REFERENCES

• 1. Ashour R, Tintner R, Jankovic J. Striatal deformities of the hand and foot in Parkinson’s disease. Lancet Neurol 2005;4:423–431.ArticlePubMed
• 2. Patel S, Tariot PN, Hamill RW. Pisa syndrome without neuroleptic exposure in a patient with dementia of the Alzheimer type. J Geriatr Psychiatry Neurol 1991;4:48–51.ArticlePubMed
• 3. Cannas A, Solla P, Floris G, Tacconi P, Serra A, Piga M, et al. Reversible Pisa syndrome in patients with Parkinson’s disease on dopaminergic therapy. J Neurol 2009;256:390–395.ArticlePubMed
• 4. Villarejo A, Camacho A, García-Ramos R, Moreno T, Penas M, Juntas R, et al. Cholinergic-dopaminergic imbalance in Pisa syndrome. Clin Neuropharmacol 2003;26:119–121.ArticlePubMed
• 5. Umemura A, Oka Y, Ohkita K, Yamawaki T, Yamada K. Effect of sub-thalamic deep brain stimulation on postural abnormality in Parkinson disease. J Neurosurg 2010;112:1283–1288.ArticlePubMed

Figure & Data

References

Citations

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