scholarly journals Secondary Normal-Pressure Hydrocephalus in Rheumatoid Meningitis

2021 ◽  
pp. 434-440
Author(s):  
Micaela Owens ◽  
Na Tosha Gatson ◽  
Gino Mongelluzzo ◽  
Oded Goren ◽  
Eric Newman ◽  
...  

Normal-pressure hydrocephalus (NPH) is a common cause of gait apraxia, cognitive impairment, and urinary incontinence in the elderly. It is usually a primary idiopathic disorder but can be secondary. We present a case of secondary NPH due to biopsy-confirmed rheumatoid meningitis initially refractory to intravenous (IV) immunotherapy. Our patient reported an excellent response right after shunting. Her gait remains normal one and a half years later. We searched PubMed for similar cases of rheumatoid meningitis with gait abnormality for additional clinicopathologic discussion. The patient’s movement disorder initially improved with steroid taper. However, she developed progressive symptoms, later on, refractory to IV solumedrol and rituximab. She underwent ventriculoperitoneal shunting (VPS) and reported an outstanding outcome. This is the first reported biopsy-confirmed case of rheumatoid meningitis causing NPH to undergo shunting for immediate improvement. Previous cases of rheumatoid meningitis-associated Parkinsonism have improved with steroid induction. Although our patient’s rheumatoid arthritis is now controlled, her case illustrates that NPH in autoinflammatory conditions may not recover with immune suppression alone. VPS is an option for a faster response in secondary NPH due to rheumatoid meningitis or other inflammatory disorders with progressive symptoms despite standard induction therapy.

2021 ◽  
Vol 12 ◽  
Author(s):  
Rui Yin ◽  
Junxian Wen ◽  
Junji Wei

Normal-pressure hydrocephalus is a clinical syndrome that mainly targets the elderly population. It features dementia, impaired walking, and the malfunction of sphincters. The rapid identification and large-scale screening of patients with normal-pressure hydrocephalus (NPH) are of great significance as surgical interventions can greatly improve or even reverse the symptoms. This review aims to summarize the traditional parameters used to diagnose NPH and the emerging progression in neuroimaging of the disease, hoping to provide an up-to-date overall perspective and summarize the possible direction of its future development.


2019 ◽  
Vol 13 (2) ◽  
pp. 133-143 ◽  
Author(s):  
Louise Makarem Oliveira ◽  
Ricardo Nitrini ◽  
Gustavo C. Román

ABSTRACT. Normal-pressure hydrocephalus (NPH) is a potentially reversible syndrome characterized by enlarged cerebral ventricles (ventriculomegaly), cognitive impairment, gait apraxia and urinary incontinence. A critical review of the concept, pathophysiology, diagnosis, and treatment of both idiopathic and secondary NPH was conducted. We searched Medline and PubMed databases from January 2012 to December 2018 using the keywords “normal-pressure hydrocephalus” / “idiopathic normal-pressure hydrocephalus” / “secondary normal-pressure hydrocephalus” / “NPH” / “ventriculoperitoneal shunt”. The initial search produced 341 hits. After careful selection, a total of 54 articles were chosen and additional relevant studies were included during the process of writing this article. NPH is an important cause of potentially reversible dementia, frequent falls and recurrent urinary infections in the elderly. The clinical and imaging features of NPH may be incomplete or nonspecific, posing a diagnostic challenge for medical doctors and often requiring expert assessment to minimize unsuccessful surgical treatments. Recent advances resulting from the use of non-invasive MRI methods for quantifying cerebral blood flow, in particular arterial spin-labeling (ASL), and the frequent association of NPH and obstructive sleep apnea (OSA), offer new avenues to understand and treat NPH.


2018 ◽  
Vol 2018 ◽  
pp. 1-8
Author(s):  
A. Cucca ◽  
M. C. Biagioni ◽  
K. Sharma ◽  
J. Golomb ◽  
R. M. Gilbert ◽  
...  

Idiopathic normal pressure hydrocephalus (iNPH) is the most common cause of hydrocephalus in adults. The diagnosis may be challenging, requiring collaborative efforts between different specialists. According to the International Society for Hydrocephalus and Cerebrospinal Fluid Disorders, iNPH should be considered in the differential of any unexplained gait failure with insidious onset. Recognizing iNPH can be even more difficult in the presence of comorbid neurologic disorders. Among these, idiopathic Parkinson’s disease (PD) is one of the major neurologic causes of gait dysfunction in the elderly. Both conditions have their peak prevalence between the 6th and the 7th decade. Importantly, postural instability and gait dysfunction are core clinical features in both iNPH and PD. Therefore, diagnosing iNPH where diagnostic criteria of PD have been met represents an additional clinical challenge. Here, we report a patient with parkinsonism initially consistent with PD who subsequently displayed rapidly progressive postural instability and gait dysfunction leading to the diagnosis of concomitant iNPH. In the following sections, we will review the clinical features of iNPH, as well as the overlapping and discriminating features when degenerative parkinsonism is in the differential diagnosis. Understanding and recognizing the potential for concomitant disease are critical when treating both conditions.


Neurosurgery ◽  
1997 ◽  
Vol 40 (1) ◽  
pp. 67-74 ◽  
Author(s):  
Joachim K. Krauss ◽  
Jens P. Regel ◽  
Werner Vach ◽  
Freimut D. J??ngling ◽  
Dirk W. Droste ◽  
...  

Neurosurgery ◽  
2006 ◽  
Vol 59 (4) ◽  
pp. 847-851 ◽  
Author(s):  
J Mocco ◽  
Matthew I. Tomey ◽  
Ricardo J. Komotar ◽  
William J. Mack ◽  
Steven J. Frucht ◽  
...  

Abstract OBJECTIVE: Idiopathic normal pressure hydrocephalus (INPH) is characterized by a classic clinical triad of symptoms, including dementia, urinary incontinence, and gait disturbance. Recent work has demonstrated that the maximal midbrain anteroposterior (AP) diameter is significantly smaller in patients with INPH than in healthy, age-matched controls. The current study was undertaken to determine the effect of ventriculoperitoneal shunt placement on midbrain dimensions in INPH patients. METHODS: Twelve consecutive INPH patients undergoing ventriculoperitoneal shunt placement with pre- and postoperative computed tomographic scans at the Columbia University Medical Center were enrolled. Each patient's pre- and postoperative maximum AP and left-to-right diameters of the midbrain at the pontomesencephalic junction were independently measured in a blinded fashion by two of the authors. The average value of each dimension was computed by calculating the mean values of the measurements of the two observers. RESULTS: Both the mean AP diameter (preoperative mean, 2.06 ± 0.04 cm; postoperative mean, 2.27 ± 0.05; P = 0.0007) and left-to-right diameter (preoperative mean, 2.80 ± 0.07; postoperative mean, 3.03 ± 0.08; P = 0.0029) increased from pre- to postoperative imaging. The approximate cross-sectional area determined as the product of AP and left-to-right diameters also increased from pre- to postoperative images (preoperative mean, 5.79 ± 0.22 cm2; postoperative mean, 6.90 ± 0.25 cm2; P = 0.00049). CONCLUSION: This study provides supportive evidence that midbrain cytoarchitecture may play a role in the pathophysiology and post-ventriculoperitoneal shunt gait improvement of INPH patients.


Neurosurgery ◽  
2009 ◽  
Vol 64 (5) ◽  
pp. 919-926 ◽  
Author(s):  
Graeme F. Woodworth ◽  
Matthew J. McGirt ◽  
Michael A. Williams ◽  
Daniele Rigamonti

Abstract INTRODUCTION Because of the difficulty in distinguishing idiopathic normal pressure hydrocephalus (INPH) from other neurodegenerative conditions unrelated to cerebrospinal fluid (CSF) dynamics, response to CSF shunting remains highly variable. We examined the utility of CSF drainage and CSF pressure (Pcsf) dynamics in predicting response to CSF shunting for patients with INPH. METHODS Fifty-one consecutive INPH patients underwent continuous lumbar Pcsf monitoring for 48 hours followed by 72 hours of slow CSF drainage before ventriculoperitoneal shunting. Response to CSF drainage and B-wave characteristics were assessed via multivariate proportional-hazards regression analysis. RESULTS Improvement in 1, 2, or all 3 INPH symptoms was observed in 35 (69%), 28 (55%), and 11 (22%) patients, respectively, after CSF shunt implantation by 12 months after surgery. A positive response to CSF drainage was found to be an independent predictor of shunt responsiveness (relative risk, 0.30; 95% confidence interval, 0.09–0.98; P = 0.05). There was no difference in Pcsf wave characteristics between the shunt-responsive and -nonresponsive groups, regardless of whether 1-, 2-, or 3-symptom improvement was used to define response to CSF shunting. CONCLUSION In this study of 51 INPH patients who underwent Pcsf monitoring with waveform analysis and CSF drainage followed by shunt surgery, there was no correlation between specific Pcsf wave characteristics and objective symptomatic improvement after shunt placement. Pcsf monitoring with B-wave analysis contributes little to the diagnostic dilemma with INPH patients. Clinical response to continuous CSF drainage over a 72-hour period suggests a high likelihood of shunt responsiveness.


Author(s):  
Andreas Eleftheriou ◽  
◽  
Christina Koumantzia ◽  

Idiopathic normal pressure hydrocephalus (iNPH) is a gradually progressive disease affecting the elderly population. The diagnosis of probable iNPH is based on clinical history, physical findings, brain imaging. INPH involves non-obstructive enlargement of the cerebral ventricles combined with one or more symptoms of cognitive decline, impaired gait and balance, and urinary urgency. Our case describes an unusual coexistence of radiological and clinical iNPH symptoms with paroxysmal paraparesis.


2021 ◽  
Author(s):  
Fernando Hakim ◽  
Daniel Jaramillo-Velásquez ◽  
Martina González ◽  
Diego F. Gómez ◽  
Juan F. Ramón ◽  
...  

Normal pressure hydrocephalus syndrome is the most common form of hydrocephalus in the elderly and produces a dementia which can be reversible surgically. It is characterized by ventriculomegaly and the classic triad of symmetric gait disturbance, cognitive decline and urinary incontinence, also known as Hakim’s triad. To date, the exact etiology of the disease has not been elucidated and the only effective treatment is a cerebrospinal fluid shunting procedure which can be a ventriculoatrial, ventriculoperitoneal or lumboperitoneal shunt. The most important problem is the high rate of underdiagnosis or misdiagnosis due to similarities in symptoms with other neurodegenerative disorders, and in some cases, coexistence. Hence, increasing awareness amongst the community and medical professionals in order to increase clinical suspicion, timely diagnosis and treatment are paramount. The best way to achieve this is by having a structured protocol with patient-centered tests that evaluates the entire myriad of alterations a clinician might encounter whenever treating patients with this disorder. Recent advances in imaging technology as well as cerebrospinal fluid biomarkers have given interesting insight into the pathophysiology of the disease and will certainly contribute greatly in diagnostic advancements. We finally present an institutional protocol which has been accredited by international peers with promising results in diagnostic and outcome rates.


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