Year : 2022 | Volume : 5 | Issue : 1 | Page : 68--70
Pisa syndrome due to shunt malfunction in normal pressure hydrocephalus with comorbid Parkinson's disease
Halil Onder
Neurology Clinic, Yozgat City Hospital, Yozgat, Turkey
Dr. Halil Onder
Neurology Clinic, Yozgat City Hospital, Yozgat
Pisa syndrome (PS) is characterized by tonic flexion of the trunk and head to one side of the body and generally occurs in patients with Parkinson's disease (PD) taking antipsychotic drugs. Recently, only a few cases of PS caused by normal pressure hydrocephalus (NPH) have been reported. Here, we report a case of PS associated with NPH. We believe that the presentation of this rare case may provide an interesting perspective regarding the pathophysiology of PS. Future studies involving a large number of patients are warranted to clarify the mechanisms underlying PS in distinct neurological diseases such as PD, NPH, and multiple system atrophy.
Onder H. Pisa syndrome due to shunt malfunction in normal pressure hydrocephalus with comorbid Parkinson's disease.Ann Mov Disord 2022;5:68-70
Onder H. Pisa syndrome due to shunt malfunction in normal pressure hydrocephalus with comorbid Parkinson's disease. Ann Mov Disord [serial online] 2022 [cited 2023 Jan 27 ];5:68-70
Available from: https://www.aomd.in/text.asp?2022/5/1/68/330931
Pisa syndrome (PS) is a postural abnormality characterized by tonic flexion of the trunk and head to one side of the body. It generally occurs in patients with Parkinson's disease (PD) and atypical parkinsonism, especially multiple system atrophy.[1] Although the exact pathophysiology of PS remains to be elucidated, its underlying mechanism can be explained via the dysfunction of sensorimotor integration, abnormal basal ganglia function, and peripheral abnormalities.[2] A crucial hypothesis has been postulated to explain PS in PD: an asymmetric trunk posture develops due to asymmetric impairment of the nigrostriatal dopaminergic system, which, in turn, leads to lateral flexion toward the side least affected by parkinsonism.[3] However, other authors disagree with this theory and emphasize the cases of PS without clear asymmetry of parkinsonian symptoms.[4] We believe that PS cases that are associated with distinct etiologies may substantially contribute to our understanding of the pathophysiology of PS from different perspectives. Notably, only a few PS cases caused by normal pressure hydrocephalus (NPH) have been previously reported.[1],[3],[5] Here, we present rare case of PS associated with NPH pathophysiology.
A 73-year-old man was admitted to the neurosurgery clinic due to deterioration of gait and tonic flexion of the trunk and head to the right side. He had a medical history of ventriculoperitoneal (VP) shunt surgery 2 years ago for the treatment of NPH. The patient presented with symptoms of gait impairment [Video 1], cognitive impairment, and urinary incontinence. Cranial magnetic resonance imaging (MRI) confirmed hydrocephalus [Figure 1]. After a positive cerebrospinal fluid (CSF) tap test response, a programmable VP shunt was inserted, which markedly improved his symptoms. However, parkinsonian symptoms such as those mildly prominent on his left side, bradykinesia, and rigidity in his upper extremities were detected during the polyclinic visits, 6 months after shunt surgery. In addition, Myerson's sign and bradykinesia were noted. On suspicion of comorbid PD, a levodopa trial was attempted, which markedly improved his gait and upper extremity movement. Levodopa/benserazide was titrated up to a dosage of 4 × 100/25 mg, and rasagiline 1 × 1 mg was added to his treatment regimen. However, in the first year of follow-up, the patient was admitted to our polyclinic due to progressive headache and disturbance in his gait. Cranial computed tomography (CT) showed subdural hematoma. After immediate consultation with the neurosurgery department, shunt overdrainage was considered and the shunt pressure was increased from 80 mm H2O to 120 mm H2O. Subsequent serial CT images revealed that subdural hematoma had gradually disappeared and his headache resolved completely. However, in the next month, the patient was re-admitted to our clinic due to deterioration of gait and newly emerging tonic flexion of the trunk and head to the left side with slight rotation of the body (progressively emerging over the last two months), consistent with PS [Video 2]. There was no history of antipsychotic medication or change in PD treatment. The patient had a broad-based, short-stepped gait with prominent postural instability. Postural instability was prominent. Cranial CT showed resolution of subdural hematoma; however, enlargement of ventricles was noted in the follow-up period. Shunt malfunction was considered to be the cause of the patient's symptoms and the CSF tap test provided a moderate temporary recovery of his posture. Shunt revision was suggested; however, the patient and his relatives did not consent to the surgery.{Figure 1}
[SUPPORTING:1]
Studies on PS in association with NPH pathophysiology are extremely rare.[1],[5],[6] Leon-Sarmiento et al.[5] presented an initial report of a 26-year-old man presenting with cognitive abnormalities, gait disturbances, and urinary incontinence. The patient was diagnosed with PS, which was found to be associated with NPH. He had a remarkable recovery of PS following a CSF tap test. However, the patient died during the follow-up period and no shunt surgery could be performed. Pandey[7] reported the case of a 17-year-old boy presenting with gait difficulty, psychiatric symptoms, and PS toward the right side. After establishing the diagnosis of NPH from MRI findings showing hydrocephalus and a positive CSF tap test response, VP shunt surgery was performed, which resulted in complete recovery of PS. However, these were juvenile cases and did not meet the full criteria of probable NPH.[1],[5] Todisco et al.[6] presented a unique case series of PS in association with NPH in adults. In conclusion, the authors emphasized the causative role of the changes in CSF dynamics in PS. They discussed the direct involvement of cortico-subcortical pathways as the main mechanism of PS and hypothesized that subsequent brainstem disturbance is a contributory factor. Furthermore, they deliberated the role of functional damage to the basal ganglia at the postsynaptic level, possibly due to enlargement of the ventricular system. In our patient, there was no remarkable asymmetry in ventricular enlargement; however, the tap test yielded a moderate amelioration in leaning to the left side. However, we cannot exclude the involvement of possible functional damage to the basal ganglia due to the enlargement of the ventricular system.
A crucial discussion is that PD pathophysiology may have solely influenced or played a contributory role in the development of PS in our case. Notably, our patient had not taken any antipsychotic medication and there was no recent change in PD treatment. Moreover, the CSF tap test yielded a moderate recovery in PS, associating this manifestation with NPH pathophysiology. However, a major drawback of our case was that the patient did not accept shunt revision surgery, which could have resulted in possible persistent recovery. Moreover, dihydroxyphenylalanine imaging such as N-omega-fluoropropyl-2beta-carboxymethoxy-3beta-(4-iodophenyl) tropane single-photon emission computed tomography or fluoro-3,4-dihydroxyphenylalnine results were unavailable in our patient, preventing further deliberations at the level of neurotransmitter systems. Future studies involving more patients are warranted to understand the underlying pathophysiology. Furthermore, the results of these reports may provide additional perspectives regarding the pathophysiology of PS in other etiologies of neurodegenerative diseases, such as PD and multiple system atrophy.
Compliance with ethical standards
The patient consent was obtained for the publication.
There are no conflicts of interest to declare.
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