Levodopa/carbidopa/entacapone-induced acute Pisa syndrome in a Parkinson’s disease patient

doi:10.1016/j.jns.2008.07.017

Journal of the Neurological Sciences

Volume 275, Issues 1–2, 15 December 2008, Pages 154-156

https://doi.org/10.1016/j.jns.2008.07.017 Get rights and content

Abstract

Pisa syndrome (PS) is a dystonic lateroflexion of the trunk with a postural disturbance resembling the leaning tower of Pisa. Initially reported as a side effect related to antipsychotic therapy, this original dystonic posture is also manifested in neurodegenerative disorders such as Alzheimer’s disease and multiple system atrophy, or in rare idiopathic cases. Recent observations have described the onset of PS with subchronic course in patients affected by Parkinson’s disease (PD). Here, we report on the acute development of PS in a parkinsonian patient during treatment with entacapone/levodopa/carbidopa combination. This case illustrates how, in contrast to previously well-known chronic/subchronic forms, this axial dystonic posture may occur in PD as an acute onset reversible type, related to levodopa treatment.

Introduction

Pisa syndrome (PS) also known as pleurothotonus, is a type of axial dystonia originally described by Ekbom et al. [1] as a side effect related to antipsychotic therapy. Typical clinical features of PS are characterized by abnormal and severe lateral flexion of the trunk accompanied by a slight backward rotation, with a postural disturbance resembling the leaning tower of Pisa, which becomes more manifest on standing or walking.

Recently, we were among the first to report the onset of PS in patients with Parkinson’s disease (PD) who had not been prescribed neuroleptics, antiemetics or cholinesterase inhibitors. Here, we report the acute development of PS in a parkinsonian patient during treatment with entacapone/levodopa/carbidopa combination. We moreover illustrate how PS may present with an acute onset subtype different from the previously well-recognized chronic/subchronic patterns.

Section snippets

Case report

A 62-year-old male came to our observation. At the age of 54, he had developed a slight resting tremor of the left hand with subsequent manifestation of rigidity and motor slowness. His past medical history was unremarkable, and no family history of PD was referred. Brain MRI findings were normal. At the age of 55, the patient was diagnosed with PD according to clinical criteria for PD [2], 1 year later started on low doses of standard levodopa/benserazide (200/50 mg a day) leading to marked

Discussion

Dystonia is not an uncommon complication in patients with idiopathic PD [3] and can involve the trunk and result in abnormalities of body posture and skeletal deformities. PD patients may present a typical involuntary stooped posture (bent spine), leading to extreme trunk flexion of the thoracolumbar spine (camptocormia) [4], or show an abnormal neck flexion (dropped head) [5]. The finding of lateral flexion of the trunk in PD is not an uncommon observation, although few reports have been

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Reversible istradefylline-induced pleurothotonus in a patient with Parkinson's disease: A case report and literature review
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Citation Excerpt :
These cases showed an acute course, and most were cured with appropriate treatment. These acute PS cases were reported with the introduction of pergolide [4,5], pramipexole [5,6], high-dose levodopa [5,7], rasagiline [8], or ropinirole [9]. This is the first case report of an acute course of reversible PS after istradefylline introduction, and the mechanism of reversible PS with PD is discussed.
Pleurothotonus, commonly known as Pisa syndrome (PS), is characterized by abnormal lateral flexion of the trunk. The precise mechanism of this disease is unknown. Istradefylline was administered to a 68-year-old male patient with Parkinson's disease (PD) to treat wearing-off; however, PS appeared 4 months after the first istradefylline treatment. Despite drug adjustments for 9 months, no improvement was observed. Finally, istradefylline was discontinued, and PS symptoms gradually improved over the subsequent 4 months and eventually disappeared. From 2005 to 2014, six studies appeared in the literature on dopaminergic therapy for PS patients with PD. The period between PS appearance after drug introduction until PS recovery with appropriate treatment differs among drugs. This study aimed to identify the drugs that initiate PS and assess the period between PS appearance and disappearance, respectively, after the drug is first administered and later discontinued.
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Most of these studies were reports of anecdotal cases with no discussion of associated drug-induced parkinsonism. In parkinsonian patients, Pisa syndrome has been associated with the initiation or change in dose of dopamine agonists,36,39,41 variation in levodopa regimen,38,39 and addition of rasagiline and COMT inhibitors to levodopa treatment.40,42,43,99 Accordingly, it has been hypothesised that a cholinergic–dopaminergic imbalance is involved in the development of drug-induced Pisa syndrome.11
Pisa syndrome is defined as a reversible lateral bending of the trunk with a tendency to lean to one side. It is a frequent and often disabling complication of Parkinson's disease, and has also been described in several atypical forms of parkinsonism and in neurodegenerative and psychiatric disorders after drug exposure and surgical procedures. Although no consistent diagnostic criteria for Pisa syndrome are available, most investigations have adopted an arbitrary cutoff of at least 10° of lateral flexion for the diagnosis of the syndrome. Pathophysiological mechanisms underlying Pisa syndrome have not been fully explained. One hypothesis emphasises central mechanisms, whereby Pisa syndrome is thought to be caused by alterations in sensory–motor integration pathways; by contrast, a peripheral hypothesis emphasises the role of anatomical changes in the musculoskeletal system. Furthermore, several drugs are reported to induce Pisa syndrome, including antiparkinsonian drugs. As Pisa syndrome might be reversible, clinicians need to be able to recognise this condition early to enable prompt management. Nevertheless, further research is needed to determine optimum treatment strategies.
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This study was designated to quantitatively evaluate the effect of levodopa on spinal posture in patients with PD using a computer-assisted handheld SpinalMouse device.
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Short communicationLevodopa/carbidopa/entacapone-induced acute Pisa syndrome in a Parkinson’s disease patient

Abstract

Introduction

Section snippets

Case report

Discussion

Parkinsonism Relat Disord

New dystonic syndrome associated with butyrophenone therapy

Z Neurol

Accuracy of clinical diagnosis of idiopathic Parkinson’s disease: a clinicopathological study of 100 cases

J Neurol Neurosurg Psychiatry

Dystonia in Parkinson’s disease

J Neurol

Camptocormia: pathogenesis, classification, and response to therapy

Neurology.

Dropped head syndrome in Parkinson’s disease

Mov Disord

Pisa syndrome in a cohort of Alzheimer’s disease patients

Acta Neurol Scand

Pisa syndrome in a patient with multiple system atrophy

Mov Disord

Short communication
Levodopa/carbidopa/entacapone-induced acute Pisa syndrome in a Parkinson’s disease patient