Massimiliano Todisco1, Nicolò Gabriele Pozzi2, Roberta Zangaglia1, Brigida Minafra1, Domenico Servello3, Roberto Ceravolo4, Enrico Alfonsi5, Alfonso Fasano6, Claudio Pacchetti7. 1. Parkinson's Disease and Movement Disorders Unit, IRCCS Mondino Foundation, Pavia, Italy. 2. Parkinson's Disease and Movement Disorders Unit, IRCCS Mondino Foundation, Pavia, Italy; Department of Neurology, University Hospital Würzburg and Julius-Maximilians-University, Würzburg, Germany. 3. Unit of Functional Neurosurgery, Department of Neurology and Neurosurgery, IRCCS Galeazzi Institute, Milan, Italy. 4. Unit of Neurology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. 5. Department of Neurophysiopathology, IRCCS Mondino Foundation, Pavia, Italy. 6. Morton and Gloria Shulman Movement Disorders Centre and the Edmond J. Safra Program in Parkinson's Disease, Toronto Western Hospital, University Health Network, Toronto, Ontario, Canada; Krembil Research Institute, Toronto, Ontario, Canada. 7. Parkinson's Disease and Movement Disorders Unit, IRCCS Mondino Foundation, Pavia, Italy. Electronic address: claudio.pacchetti@mondino.it.
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.
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.