Normal Pressure Hydrocephalus in Children and Young Adults

Neurosurgery ◽  
1987 ◽  
Vol 20 (6) ◽  
pp. 904-907 ◽  
Author(s):  
Gene H. Barnett ◽  
Joseph F. Hahn ◽  
Joann Palmer

Abstract Normal pressure hydrocephalus (NPH) is generally considered to be a disorder of adult and geriatric patients. We report four patients who are children or young adults with chronic neurological disorders, recent deterioration of their levels of function, normal cerebrospinal fluid (CSF) pressures, and ventricular enlargement. All four patients improved after the placement or revision of a ventriculoperitoneal shunt. Frequent symptoms and signs included irritability (three patients), vomiting (three patients), and abnormal limb posturing (two patients). Correct diagnosis was hampered by two factors: (a) Multiple or prolonged recordings of CSF pressures were invariably well within the normal ranges with respect to age, and (b) the patients had chronic neurological deficits. After ventriculoperitoneal shunting, subjective and objective improvement was seen in all cases. Young patients with large ventricles may benefit from shunting procedures despite low CSF pressures. These patients may be clinically identified by symptoms of new neurological dysfunction in cases of previously static neurological disease or acceleration of slowly progressive neurological dysfunction. Some of these underlying neurological disorders may predispose children and young adults to NPH.

Neurosurgery ◽  
1987 ◽  
Vol 20 (6) ◽  
pp. 904???7 ◽  
Author(s):  
G H Barnett ◽  
J F Hahn ◽  
J Palmer

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.


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):  
Andrew Graham

Dementia in old age is usually due to Alzheimer’s disease, cerebrovascular disease, or mixed pathology. Dementia due to other neurological disorders is uncommon, but important to recognise because management may be very different to that in primary or vascular dementia. This chapter surveys five neurological conditions that may present with dementia in later life: idiopathic normal pressure hydrocephalus (INPH); Huntington’s disease (HD); multiple sclerosis (MS); autoimmune limbic encephalitis (LE); and prion disease. For each disorder the epidemiology, clinical features, investigations & treatment are reviewed, with examples of the characteristic brain imaging changes. Accurate diagnosis of these conditions can be challenging even for physicians with a special interest in dementia, and often requires a neurological referral.


2019 ◽  
Vol 19 (1) ◽  
pp. 53-56
Author(s):  
Adomas Bunevicius ◽  
Ashley Vernon ◽  
Alexandra Golby

Abstract BACKGROUND Ventriculoperitoneal shunting (VPS) is effective for the treatment of normal-pressure hydrocephalus (NPH) and sometimes requires laparoscopic assistance with abdominal insufflation. OBJECTIVE To evaluate the association of abdominal insufflation with opening pressure (OP) in NPH patients undergoing VPS implantation. METHODS Between March 2016 and April 2019, 52 consecutive patients who underwent first-time VPS implantation surgery were retrospectively identified by reviewing electronic health records. OP during the large volume lumbar tap test (OPLP) and VPS implantation surgery (OPSURGERY) were measured in 29 patients. RESULTS Laparoscopic assistance with abdominal insufflation was used in 20 (69%) cases. There were no differences in patient age (P = .589), gender (P = .822), body mass index (P = .289), weight (P = .789), height (P = .542), and OPLP (P = .476) in patients operated with and without laparoscopic assistance. When compared to patients operated without laparoscopic assistance, laparoscopic assistance was associated with a greater rate of OP increase during surgery relative to OPLP (40% vs 100%, P = .002), a greater increase in OPSURGERY relative to OPLP (−0.40 ± 5.38 vs 10.17 ± 5.53 cm H2O, P < .001), and a greater proportion of patients with OPSURGERY of ≥25 cm H2O during the VPS surgery (0% vs 78%, P < .001). CONCLUSION Abdominal insufflation is associated with an increase in intracranial pressure with OPs often exceeding 25 cm H2O. This should be considered when selecting optimal VPS pressure settings.


2020 ◽  
Vol 132 (1) ◽  
pp. 306-312 ◽  
Author(s):  
Tarek Y. El Ahmadieh ◽  
Eva M. Wu ◽  
Benjamin Kafka ◽  
James P. Caruso ◽  
Om J. Neeley ◽  
...  

OBJECTIVEA short-term lumbar drain (LD) trial is commonly used to assess the response of normal pressure hydrocephalus (NPH) patients to CSF diversion. However, it remains unknown whether the predictors of passing an LD trial match the predictors of improvement after ventriculoperitoneal shunting. The aim of this study was to examine outcomes, complication rates, and associations between predictors and outcomes after an LD trial in patients with NPH.METHODSThe authors retrospectively reviewed the records of 254 patients with probable NPH who underwent an LD trial between March 2008 and September 2017. Multivariate regression models were constructed to examine predictors of passing the LD trial. Complications associated with the LD trial procedure were recorded.RESULTSThe mean patient age was 77 years and 56.7% were male. The mean durations of gait disturbance, cognitive decline, and urinary incontinence were 29 months, 32 months, and 28 months, respectively. Of the 254 patients, 30% and 16% reported objective and subjective improvement after the LD trial, respectively. Complications included a sheared LD catheter, meningitis, lumbar epidural abscess, CSF leak at insertion site, transient lower extremity numbness, slurred speech, refractory headaches, and hyponatremia. Multivariate analyses using MAX-R revealed that a prior history of stroke predicted worse outcomes, while disproportionate subarachnoid spaces (uneven enlargement of supratentorial spaces) predicted better outcomes after the LD trial (r2 = 0.12, p < 0.05).CONCLUSIONSThe LD trial is generally safe and well tolerated. The best predictors of passing the LD trial include a negative history of stroke and having disproportionate subarachnoid spaces.


2020 ◽  
pp. 10.1212/CPJ.0000000000001018
Author(s):  
Lealani Mae Y. Acosta ◽  
Kassandra Stubblefield ◽  
Trisha Conwell ◽  
Kiersten Espaillat ◽  
Heather Koons ◽  
...  

ABSTRACTBackground:The work-up for idiopathic normal pressure hydrocephalus (INPH) can be difficult to coordinate and determining appropriate patients for ventriculoperitoneal shunting can be challenging, so we hypothesized that implementing a formalized protocol can improve patient selection for shunt. In conjunction with neurology and neurosurgery, we instituted a standardized means of assessing patients whose presentation is concerning for INPH and compared their work-up with similar patients seen without the protocol (i.e., “pre-protocol” or PP) regarding baseline characteristics, assessment, and outcomes.Methods:Twenty-six PP patients were compared with 40 protocol patients on measures, including baseline deficits, work-up, neurosurgical evaluation, and response to shunt.Results:Average age was similar between groups and percentage of patients who had a decline in gait, cognition, and/or incontinence was not statistically different (p>0.05). Significantly more protocol patients underwent high-volume lumbar puncture (HVLP; 97.5%; PP, 61.5%; p<0.001) and received formalized gait assessment with The Gait Scale (90%; PP, 0%, p<0.001) and standardized cognitive testing (95%; PP, 38.5%; p<0.001). Significantly more protocol patients had no improvement after HVLP (33.3%; PP 6.25%, p<0.045); subsequently, fewer got shunted (57.5%; PP, 84.6%; p<0.030). More protocol patients who were shunted reported gait improvement (100%; PP, 72.7%; p=0.009), though there was no difference in cognition (59.2%; PP, 82.6%, p=0.108) or incontinence (18.2%; PP, 39.1%, p=0.189).Conclusions:Implementing an INPH protocol leads to standardized and more extensive assessment and better patient selection for and subsequent outcomes from shunting, specifically regarding gait.


Neurosurgery ◽  
1983 ◽  
Vol 12 (1) ◽  
pp. 108-110 ◽  
Author(s):  
Kazuyoshi Korosue ◽  
Norihiko Tamaki ◽  
Kiyoshi Fujiwara ◽  
Satoshi Matsumoto

Abstract The authors report a patient with the syndrome of normal pressure hydrocephalus who had an arachnoid cyst in the 4th ventricle. Ventriculoperitoneal shunting followed by removal of the cyst resulted in a complete recovery.


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