The clinical spectrum of hydrocephalus in adults: report of the first 517 patients of the Adult Hydrocephalus Clinical Research Network registry

Restricted access

OBJECTIVE

The authors describe the demographics and clinical characteristics of the first 517 patients enrolled in the Adult Hydrocephalus Clinical Research Network (AHCRN) during its first 2 years.

METHODS

Adults ≥ 18 years were nonconsecutively enrolled in a registry at 6 centers. Four categories of adult hydrocephalus were defined: transition (treated before age 18 years), unrecognized congenital (congenital pattern, not treated before age 18 years), acquired (secondary to known risk factors, treated or untreated), and suspected idiopathic normal pressure hydrocephalus (iNPH) (≥ age 65 years, not previously treated). Data include etiology, symptoms, examination findings, neuropsychology screening, comorbidities, treatment, complications, and outcomes. Standard evaluations were administered to all patients by trained examiners, including the Montreal Cognitive Assessment, the Symbol Digit Modalities Test, the Beck Depression Inventory–II, the Overactive Bladder Questionnaire Short Form symptom bother, the 10-Meter Walk Test, the Boon iNPH gait scale, the Lawton Activities of Daily Living/Instrumental Activities of Daily Living (ADL/IADL) questionnaire, the iNPH grading scale, and the modified Rankin Scale.

RESULTS

Overall, 517 individuals were enrolled. Age ranged from 18.1 to 90.7 years, with patients in the transition group (32.7 ± 10.0 years) being the youngest and those in the suspected iNPH group (76.5 ± 5.2 years) being the oldest. The proportion of patients in each group was as follows: 16.6% transition, 26.5% unrecognized congenital, 18.2% acquired, and 38.7% suspected iNPH. Excluding the 86 patients in the transition group, who all had received treatment, 79.4% of adults in the remaining 3 groups had not been treated at the time of enrollment. Patients in the suspected iNPH group had the poorest performance in cognitive evaluations, and those in the unrecognized congenital group had the best performance. The same pattern was seen in the Lawton ADL/IADL scores. Gait velocity was lowest in patients in the suspected iNPH group. Categories that had the most comorbidities (suspected iNPH) or etiologies of hydrocephalus that directly cause neurological injury (transition, acquired) had greater degrees of impairment compared to unrecognized congenital, which had the fewest comorbidities or etiologies associated with neurological injury.

CONCLUSIONS

The clinical spectrum of hydrocephalus in adults comprises more than iNPH or acquired hydrocephalus. Only 39% of patients had suspected iNPH, whereas 43% had childhood onset (i.e., those in the transition and unrecognized congenital groups). The severity of symptoms and impairment was worsened when the etiology of the hydrocephalus or complications of treatment caused additional neurological injury or when multiple comorbidities were present. However, more than half of patients in the transition, unrecognized congenital, and acquired hydrocephalus groups had minimal or no impairment. Excluding the transition group, nearly 80% of patients in the AHCRN registry were untreated at the time of enrollment. A future goal for the AHCRN is to determine whether patients with unrecognized congenital and acquired hydrocephalus need treatment and which patients in the suspected iNPH cohort actually have possible hydrocephalus and should undergo further diagnostic testing. Future prospective research is needed in the diagnosis, treatment, outcomes, quality of life, and macroeconomics of all categories of adult hydrocephalus.

ABBREVIATIONS ADL/IADL = Activities of Daily Living/Instrumental Activities of Daily Living; AHCRN = Adult Hydrocephalus Clinical Research Network; BDI-II = Beck Depression Inventory–II; HCRN = Hydrocephalus Clinical Research Network; iNPH = idiopathic normal pressure hydrocephalus; iNPHGS = iNPH grading scale; MoCA = Montreal Cognitive Assessment; mRS = modified Rankin Scale; OAB-q = Overactive Bladder Questionnaire; SDMT = Symbol Digit Modalities Test; SiNPH = suspected iNPH.

Downloadable materials

  • Supplemental Tables A and B (PDF 424 KB)

Article Information

Correspondence Michael A. Williams: University of Washington School of Medicine, Seattle, WA. maw99@uw.edu.

INCLUDE WHEN CITING Published online May 24, 2019; DOI: 10.3171/2019.2.JNS183538.

Disclosures Dr. Hamilton, Dr. Moghekar, and Dr. Williams report being a member of the Medical Advisory Board for the Hydrocephalus Association. Dr. Hamilton and Dr. Williams report being a member of the Hydrocephalus Association Board of Directors. Dr. Edwards reports receiving honoraria for delivering CME-accredited lectures/courses on hydrocephalus from Johnson & Johnson, Inc., and having an ownership stake in Integra LifeSciences and in B. Braun Miethke. Dr. Hamilton reports being a consultant for Integra Canada.

The AHCRN currently receives support from Codman/Integra for a prospective clinical trial of shunt surgery in iNPH. Support was received from the Hydrocephalus Association.

© AANS, except where prohibited by US copyright law.

Headings

Figures

  • View in gallery

    Cognitive, depression, gait, and bladder screening results for all adult hydrocephalus categories. A: MoCA scores: range 0–30; cutoff score ≥ 26 for the normal elderly population. B: SDMT scores: number of correct responses; normal population scores range from 33.31 (SD 9.02) to 69.91 (SD 12.64). Performance is sensitive to age and education. C: BDI-II scores: score of 0–13 is the minimal range, 14–19 is mild, 20–28 is moderate, and 29–63 is severe. D: 10-Meter Walk Test velocity in meters/second. E: Boon iNPH gait scale scores: range 0–20; normal elderly control group scores range from 3 to 10. The mean score is 5.8 ± 1.9. F: OAB-q Short Form symptom bother scores: range 0–100; control group score is 9.8 ± 11.7. *p < 0.05, ** p < 0.01, ***p < 0.001 for pairwise comparisons by t-test or Mann-Whitney U-test. Each comparison is denoted by a code, where T = transition, UC = unrecognized congenital, A = acquired, and SiNPH = suspected iNPH. For example, T** denotes that p < 0.01 comparing distributions of a factor with patients in the transition category. Figure is available in color online only.

  • View in gallery

    Functional and outcome scales and age distribution for all adult hydrocephalus categories. A: Lawton ADL/IADL total score: range 0–32. With self-rating scores, < 5 suggests functional independence. B: iNPHGS total score: range 0–12, with 0 indicating no symptoms. C: mRS scores: range 0–6, with 0 indicating no symptoms. D: Age in years. *p < 0.05, ** p < 0.01, ***p < 0.001 for pairwise comparisons by t-test or Mann-Whitney U-test. Each comparison is denoted by a code, where T = transition, UC = unrecognized congenital, A = acquired, and SiNPH = suspected iNPH. For example, T** denotes that p < 0.01 comparing distributions of a factor with patients in the transition category. Figure is available in color online only.

TrendMD

Metrics

Metrics

All Time Past Year Past 30 Days
Abstract Views 490 490 285
Full Text Views 89 89 63
PDF Downloads 62 62 46
EPUB Downloads 0 0 0

PubMed

Google Scholar