Pisa syndrome in Idiopathic Normal Pressure Hydrocephalus

Massimiliano Todisco, Nicolò Gabriele Pozzi, Roberta Zangaglia, Brigida Minafra, Domenico Servello, Roberto Ceravolo, Enrico Alfonsi, Alfonso Fasano, Claudio Pacchetti

Research output: Contribution to journalArticle

Abstract

INTRODUCTION: Idiopathic Normal Pressure Hydrocephalus (iNPH) is a complex syndrome of ventriculomegaly that can include parkinsonian-like features besides the classical triad of cognitive decline, urinary incontinence, and gait/balance disturbances. Pisa syndrome (PS) is a postural abnormality often associated with parkinsonism and defined as lateral trunk flexion greater than 10° while standing that resolves in the supine position. We reported a case series of classical "fixed" PS and one case of "Metronome" recurrent side-alternating PS in iNPH, displaying opposite electromyographic patterns of paraspinal muscles.

METHODS: Eighty-five iNPH patients were followed longitudinally for at least one year through scheduled clinical and neuropsychological visits.

RESULTS: Five (5.9%) subjects revealed PS. None of them had nigrostriatal dopaminergic involvement detected by [123I]FP-CIT SPECT. Among these patients, four had "fixed" PS, whereas one showed a recurrent side-alternating PS which repeatedly improved after ventriculo-peritoneal shunt and following adjustments of the valve-opening pressure of the shunt system.

DISCUSSION: This is the first case series of PS in iNPH and the first report of "Metronome" PS in iNPH. The prompt response of the abnormal trunk postures through cerebrospinal fluid (CSF) shunt surgery suggests a causative role of an altered CSF dynamics. PS and gait disorders in iNPH could be explained by a direct involvement of cortico-subcortical pathways and subsequent secondary brainstem involvement, with also a possible direct functional damage of the basal ganglia at the postsynaptic level, due to enlargement of the ventricular system and impaired CSF dynamics. The early detection of these cases supports a proper surgical management.

Original languageEnglish
Pages (from-to)40-44
Number of pages5
JournalParkinsonism and Related Disorders
Volume 66
Early online dateJun 28 2019
DOIs
Publication statusPublished - 2020

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Normal Pressure Hydrocephalus
Hydrodynamics
Gait
Cerebrospinal Fluid
Cerebrospinal Fluid Shunts
Paraspinal Muscles
Social Adjustment
Ventriculoperitoneal Shunt
Supine Position
Urinary Incontinence
Parkinsonian Disorders
Basal Ganglia
Single-Photon Emission-Computed Tomography
Posture
Brain Stem

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Pisa syndrome in Idiopathic Normal Pressure Hydrocephalus. / Todisco, Massimiliano; Pozzi, Nicolò Gabriele; Zangaglia, Roberta; Minafra, Brigida; Servello, Domenico; Ceravolo, Roberto; Alfonsi, Enrico; Fasano, Alfonso; Pacchetti, Claudio.

In: Parkinsonism and Related Disorders, Vol. 66, 2020, p. 40-44.

Research output: Contribution to journalArticle

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T1 - Pisa syndrome in Idiopathic Normal Pressure Hydrocephalus

AU - Todisco, Massimiliano

AU - Pozzi, Nicolò Gabriele

AU - Zangaglia, Roberta

AU - Minafra, Brigida

AU - Servello, Domenico

AU - Ceravolo, Roberto

AU - Alfonsi, Enrico

AU - Fasano, Alfonso

AU - Pacchetti, Claudio

N1 - Copyright © 2019 The Authors. Published by Elsevier Ltd.. All rights reserved.

PY - 2020

Y1 - 2020

N2 - INTRODUCTION: Idiopathic Normal Pressure Hydrocephalus (iNPH) is a complex syndrome of ventriculomegaly that can include parkinsonian-like features besides the classical triad of cognitive decline, urinary incontinence, and gait/balance disturbances. Pisa syndrome (PS) is a postural abnormality often associated with parkinsonism and defined as lateral trunk flexion greater than 10° while standing that resolves in the supine position. We reported a case series of classical "fixed" PS and one case of "Metronome" recurrent side-alternating PS in iNPH, displaying opposite electromyographic patterns of paraspinal muscles.METHODS: Eighty-five iNPH patients were followed longitudinally for at least one year through scheduled clinical and neuropsychological visits.RESULTS: Five (5.9%) subjects revealed PS. None of them had nigrostriatal dopaminergic involvement detected by [123I]FP-CIT SPECT. Among these patients, four had "fixed" PS, whereas one showed a recurrent side-alternating PS which repeatedly improved after ventriculo-peritoneal shunt and following adjustments of the valve-opening pressure of the shunt system.DISCUSSION: This is the first case series of PS in iNPH and the first report of "Metronome" PS in iNPH. The prompt response of the abnormal trunk postures through cerebrospinal fluid (CSF) shunt surgery suggests a causative role of an altered CSF dynamics. PS and gait disorders in iNPH could be explained by a direct involvement of cortico-subcortical pathways and subsequent secondary brainstem involvement, with also a possible direct functional damage of the basal ganglia at the postsynaptic level, due to enlargement of the ventricular system and impaired CSF dynamics. The early detection of these cases supports a proper surgical management.

AB - INTRODUCTION: Idiopathic Normal Pressure Hydrocephalus (iNPH) is a complex syndrome of ventriculomegaly that can include parkinsonian-like features besides the classical triad of cognitive decline, urinary incontinence, and gait/balance disturbances. Pisa syndrome (PS) is a postural abnormality often associated with parkinsonism and defined as lateral trunk flexion greater than 10° while standing that resolves in the supine position. We reported a case series of classical "fixed" PS and one case of "Metronome" recurrent side-alternating PS in iNPH, displaying opposite electromyographic patterns of paraspinal muscles.METHODS: Eighty-five iNPH patients were followed longitudinally for at least one year through scheduled clinical and neuropsychological visits.RESULTS: Five (5.9%) subjects revealed PS. None of them had nigrostriatal dopaminergic involvement detected by [123I]FP-CIT SPECT. Among these patients, four had "fixed" PS, whereas one showed a recurrent side-alternating PS which repeatedly improved after ventriculo-peritoneal shunt and following adjustments of the valve-opening pressure of the shunt system.DISCUSSION: This is the first case series of PS in iNPH and the first report of "Metronome" PS in iNPH. The prompt response of the abnormal trunk postures through cerebrospinal fluid (CSF) shunt surgery suggests a causative role of an altered CSF dynamics. PS and gait disorders in iNPH could be explained by a direct involvement of cortico-subcortical pathways and subsequent secondary brainstem involvement, with also a possible direct functional damage of the basal ganglia at the postsynaptic level, due to enlargement of the ventricular system and impaired CSF dynamics. The early detection of these cases supports a proper surgical management.

U2 - 10.1016/j.parkreldis.2019.06.024

DO - 10.1016/j.parkreldis.2019.06.024

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VL - 66

SP - 40

EP - 44

JO - Parkinsonism and Related Disorders

JF - Parkinsonism and Related Disorders

SN - 1353-8020

ER -