Objective: To describe Pisa syndrome as an early presenting feature of CBGD and add to literature on CBGD so that neurologists can suspect CBGD when they encounter Pisa syndrome.

Background: Pisa syndrome or pleurothotonus or lateral trunk flexion is an axial dystonia which has been rarely described in association with CBGD. It has been reported mainly in patients chronically taking antipsychotic medications. It has also been described in only two probable cases of Alzheimers disease and Multiple system atrophy.

Method: 52-year-old right-handed male presented with complaints of left upper limb tremor since 2020 and extra-pyramidal symptoms in from of slowing of all movements of daily activities since 2021. It was noted that the patients torso was tilted towards the left side when he was upright. His wife reported that he had a habit of driving his scooter in this posture since 2017 and she had difficulty sitting behind him on scooter owing to his bending his body to left. On detailed questioning , she told she felt that the bending had increased but not very significantly. An image of re-enactment using front and back riders on a scooter is shown to document how the wife would have easily picked up even minimal bending of the trunk of her husband which otherwise could have gone unnoticed[Fig.1]. On clinical examination of the patient, left alien hand was present. Patient had rigidity – both limb and axial and frontal release signs. Stimulus sensitive upper limb myoclonus was present. MRI [Fig. 2] demonstrated right parietal lobe atrophy with normal swallow tail sign in midbrain. Patient was given a trial of levodopa-carbidopa.

Results: Patient did not show any therapeutic response to levodopa-carbidopa and there was no improvement in his clinical symptoms. This in addition to his clinical signs and neuroimaging led us to make a diagnosis of CBGD.

Conclusion: Pisa Syndrome which has its origin in parietal lobe can occur as the presenting feature of CBGD. A recent hypothesis claims that verticality misperception is associated with the generation of Pisa syndrome ¹. More studies on parietal lobe functions and pathology can answer this question.

Figure 1

Figure 2

References: 1. Young Eun Huh, Kunhyun Kim, Won-Ho Chung, Jinyoung Youn, Seonwoo Kim, & Jin Whan Cho, Pisa Syndrome in Parkinson’s Disease : Pathogenic Roles of Verticality Perception Deficits : www.nature.com/scientific reports

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MDS Abstracts - https://www.mdsabstracts.org/abstract/pisa-syndrome-as-an-early-presenting-feature-of-corticobasal-ganglionic-degeneration-cbgd/