Hydrocephalic Parkinsonism: lessons from normal pressure hydrocephalus mimics

Starr, Brian; Hagen, Matthew; Espay, Alberto J.

doi:10.1186/2054-7072-1-2

Cited by 39 publications

(37 citation statements)

References 13 publications

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“…As in our study, neurodegenerative diseases are frequent causes of poor outcome after VP-shunting [22]. The extrapyramidal symptoms of these diseases can mimic the symptoms of patients with NPH [36]. Even these patients can improve temporarily after VP-shunting [10].…”

Section: Risk Factorssupporting

confidence: 62%

Secondary deterioration in patients with normal pressure hydrocephalus after ventriculoperitoneal shunt placement: a proposed algorithm of treatment

Gutowski

Rot

Fritsch

et al. 2020

Fluids Barriers CNS

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Background: After ventriculoperitoneal shunt surgery for idiopathic normal pressure hydrocephalus (iNPH) with adjustable gravitational valves, a certain proportion of patients develop secondary clinical worsening after initial improvement of clinical symptoms. The aim of this study was to analyze this group of patients with secondary deterioration and to evaluate the performed shunt management. Methods: For this investigation, we retrospectively reviewed our NPH registry for patients included between 1999 and 2013 with a decrease by a minimum of two points in the Kiefer score in the first year of follow up and an increase of two points in the Kiefer score between the second and the fifth year after shunt surgery (secondary deterioration). Then, we analyzed the patient's shunt management (adapting the valve pressure setting, shuntography, valve replacement, catheter replacement, implant an adjustable gravitational unit). Additionally, we searched for risk factors for secondary deterioration. Results: Out of 259 iNPH patients, 53 (20%) patients showed secondary deterioration on an average of 2.7 (2-4 years) years after shunt surgery. Fourteen (26%) patients with secondary deterioration improved after shunt or valve management and 58% remained without clinical benefit after management. We had a drop-out rate of 15% due to incomplete datasets. Our shunt management reduced the rate of secondary deterioration from 20 to 15%. On the basis of our findings, we developed an algorithm for shunt management. Risk factors for secondary deterioration are the age of the patient at the time of shunting, newly diagnosed neurodegenerative diseases, and overdrainage requiring adjusting the valve to higher-pressure levels. Conclusion: Twenty percent of patients with iNPH were at risk for secondary clinical worsening about 3 years after shunt surgery. About one-fourth of these patients benefited for additional years from pressure level management and/or shunt valve revision. Our findings underline the need for long-term follow-ups and intensive shunt management to achieve a favorable long-term outcome for patients with iNPH and VPS.

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Section: Risk Factorssupporting

confidence: 62%

Secondary deterioration in patients with normal pressure hydrocephalus after ventriculoperitoneal shunt placement: a proposed algorithm of treatment

Gutowski

Rot

Fritsch

et al. 2020

Fluids Barriers CNS

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“…However, with the help of the recent reports including pathological examinations, many discussions and hypotheses have been suggested about this topic. 1,9,10 For instance, Starr et al postulated that previously reported cases of "dual" pathology (e.g., NPH and PSP) actually represent the hydrocephalic presentation of selected neurodegenerative disorders. 9 Moreover, in his crucial report, Saper remarked the controversies regarding the definition of iNPH.…”

Section: Discussionmentioning

confidence: 99%

“…1,9,10 For instance, Starr et al postulated that previously reported cases of "dual" pathology (e.g., NPH and PSP) actually represent the hydrocephalic presentation of selected neurodegenerative disorders. 9 Moreover, in his crucial report, Saper remarked the controversies regarding the definition of iNPH. 10 He mentioned that in many related studies on shunting for iNPH had required only two or even one of these symptoms to include the patient in the series.…”

Section: Discussionmentioning

confidence: 99%

Progressive Supranuclear Palsy: A Crucial Co-occurrence in Normal Pressure Hydrocephalus or an Etiology of Hydrocephalic Presentation?

Önder

2020

Indian Journal of Neurosurgery

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Co-occurrences of neurodegenerative diseases in normal pressure hydrocephalus (NPH) is a remarkable topic of discussion, which has been mentioned in recent studies and it was also emphasized by the International Society for Hydrocephalus and Cerebrospinal Fluid Disorder. In this report, I illustrate two remarkable cases which were initially diagnosed with idiopathic normal pressure hydrocephalus (iNPH) and ventriculoperitoneal (VP) shunt surgery was performed, which yielded significant improvement in the clinic of both patients in the short-term period. However, in the long-term follow-up, both of them deteriorated, and re-evaluation of the neurological examination and neuroimaging data yielded the full clinical picture of progressive supranuclear palsy (PSP). Via the illustration of these cases and related literature data, I rediscuss some related hypotheses about the co-occurrence of these two conditions of hydrocephalus and PSP. I also suggest some distinct discussions to be clarified in future studies.

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“…Indeed, the clinical picture of iNPH often overlaps with the symptoms of other neurodegenerative diseases, including dementias such as Alzheimer’s disease (AD) and dementia with Lewy bodies, or extrapyramidal spectrum disorders including Parkinson’s disease (PD), progressive supranuclear palsy (PSP), and corticobasal syndrome [1, 6]. This is not unexpected given that iNPH is also considered a neurodegenerative disorder [6–8], and that symptoms of different neurodegenerative diseases often coexist. Indeed, these problems can make precise diagnosis impossible before death and postmortem analysis.…”

Section: Introductionmentioning

confidence: 99%

“…Indeed, these problems can make precise diagnosis impossible before death and postmortem analysis. Even then, a coexistence of multiple types of pathological processes can be found in the brain [6–13]. Thus, identification of specific biomarkers is important to improve accurate diagnosis and for use of most appropriate treatmentstrategies.…”

Section: Introductionmentioning

confidence: 99%

Decreased Expression of hsa-miR-4274 in Cerebrospinal Fluid of Normal Pressure Hydrocephalus Mimics with Parkinsonian Syndromes

Jurjević

Miyajima

Ogino

et al. 2017

JAD

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Background: Patients presenting with the classical idiopathic normal pressure hydrocephalus (iNPH) triad often show additional parkinsonian spectrum signs. Accurate differential diagnosis strongly influences the long-term outcome of cerebrospinal fluid (CSF) shunting.Objective: The aim of this study was to find potential CSF microRNA (miRNA) biomarkers for NPH mimics with parkinsonian syndromes that can reliably distinguish them from iNPH patients.Methods: Two cohorts of 81 patients (cohort 1, n = 55; cohort 2, n = 26) with possible iNPH who were treated in two centers between January 2011 and May 2014 were studied. In both cohorts, CSF samples were obtained from patients clinically diagnosed with iNPH (n = 21 and n = 10, respectively), possible iNPH with parkinsonian spectrum (PS) (n = 18, n = 10, respectively), possible iNPH with Alzheimer’s disease (AD) (n = 16), and non-affected elderly individuals (NC) (n = 6). A three-step qRT-PCR analysis of the CSF samples was performed to detect miRNAs that were differentially expressed in the groups.Results: The expression of hsa-miR-4274 in CSF was decreased in both cohorts of PS group patients (cohort 1: p < 0.0001, cohort 2: p < 0.0001), and was able to distinguish PS from iNPH with high accuracy (area under the curve = 0.908). The CSF concentration of hsa-miR-4274 also correlated with the specific binding ratio of ioflupane (123I) dopamine transporter scan (r = –0.494, p = 0.044). By contrast, the level of hsa-miR-4274 was significantly increased in the PS group after CSF diversion.Conclusion: Levels of CSF hsa-miR-4274 can differentiate PS from patients with iNPH, AD, and NC. This may be clinically useful for diagnostic purposes and predicting shunt treatment responses.

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Hydrocephalic Parkinsonism: lessons from normal pressure hydrocephalus mimics

Cited by 39 publications

References 13 publications

Secondary deterioration in patients with normal pressure hydrocephalus after ventriculoperitoneal shunt placement: a proposed algorithm of treatment

Secondary deterioration in patients with normal pressure hydrocephalus after ventriculoperitoneal shunt placement: a proposed algorithm of treatment

Progressive Supranuclear Palsy: A Crucial Co-occurrence in Normal Pressure Hydrocephalus or an Etiology of Hydrocephalic Presentation?

Decreased Expression of hsa-miR-4274 in Cerebrospinal Fluid of Normal Pressure Hydrocephalus Mimics with Parkinsonian Syndromes

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