| 1. |
- Andrén, Kerstin, 1980, et al.
(author)
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Early shunt surgery improves survival in idiopathic normal pressure hydrocephalus
- 2021
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In: European Journal of Neurology. - : Wiley. - 1351-5101 .- 1468-1331. ; 28:4, s. 1153-1159
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Journal article (peer-reviewed)abstract
- Background and purpose To examine the effect of delayed compared to early planning of shunt surgery on survival, in patients with idiopathic normal pressure hydrocephalus (iNPH), a long-term follow-up case-control study of patients exposed to a severe delay of treatment was performed. Methods In 2010-2011 our university hospital was affected by an administrative and economic failure that led to postponement of several elective neurosurgical procedures. This resulted in an unintentional delay of planning of treatment for a group of iNPH patients, referred to as iNPH(Delayed) (n = 33, waiting time for shunt surgery 6-24 months). These were compared to patients treated within 3 months, iNPH(Early) (n = 69). Primary outcome was mortality. Dates and underlying causes of death were provided by the Cause of Death Registry. Survival was analysed by Kaplan-Meier plots and a Cox proportional hazard model adjusted for potential confounders. Results Median follow-up time was 6.0 years. Crude 4-year mortality was 39.4% in iNPH(Delayed) compared to 10.1% in iNPH(Early) (p = 0.001). The adjusted hazard ratio in iNPH(Delayed) was 2.57; 95% confidence interval 1.13-5.83, p = 0.024. Causes of death were equally distributed between the groups except for death due to malignancy which was not seen in iNPH(Delayed) but in 4/16 cases in iNPH(Early) (p = 0.044). Conclusions The present data indicate that shunt surgery is effective in iNPH and that early treatment increases survival.
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| 2. |
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| 3. |
- Constantinescu, Clara, 1995, et al.
(author)
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Prevalence of Possible Idiopathic Normal Pressure Hydrocephalus in Sweden: A Population-Based MRI Study in 791 70-Year-Old Participants.
- 2024
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In: Neurology. - 1526-632X. ; 102:2
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Journal article (peer-reviewed)abstract
- Very divergent prevalence rates for idiopathic normal pressure hydrocephalus (iNPH) are reported, probably due to differences in study sample selection and diagnostic criteria. This MRI-based study aimed to determine the prevalence of iNPH and iNPH-specific radiologic changes and their association with clinical symptoms in a large, 70-year-old population-based cohort (Gothenburg H70).In this cross-sectional study, disturbances in gait and balance, cognition, and urinary continence were assessed using clinical examination and self-report. MRI was evaluated for iNPH-specific imaging markers. iNPH was diagnosed according to International Guidelines (I.G.). Based on radiologic findings, participants were allocated to 1 of 4 groups: (A) Evans index (EI) ≤0.3 (reference), (B) EI >0.3 without other iNPH-typical radiologic findings, (C) radiologically probable iNPH according to I.G., and (D) radiologically holistically probable (h-probable) iNPH fulfilling radiologic criteria according to I.G. plus highly iNPH-specific changes according to an experienced neuroradiologist.The Gothenburg H70 Studies include 791 individuals (377 men, 414 women) born in 1944 who underwent brain MRI. The prevalence of iNPH was 1.5% (2.1% for men, 0.96% for women) according to I.G. Ninety participants (11%) had EI >0.3 without other iNPH-typical radiologic findings, 29 (3.7%) fulfilled the I.G. radiologic probable iNPH criteria alone, and 11 (1.4%) were classified as radiologically h-probable iNPH. Forty participants (5.1%) had I.G. radiologic features of iNPH (70% men vs 30% women, p = 0.005). Gait disturbances were more common in participants with EI >0.3 without other radiologic iNPH features (B) (33%) compared with the reference group (A) (19%) (p = 0.006). All clinical symptoms were more common in participants with I.G. radiologic features of iNPH (C + D) than they were in the reference group (A) (p < 0.03).The iNPH prevalence of 1.5% among 70-year-olds, which is considerably higher than earlier reported in this age group, suggests that iNPH may be more common than previously assumed. This is supported by the 5.1% total prevalence of imaging signs of iNPH. Ventriculomegaly without other iNPH-typical radiologic findings may be an early sign of developing iNPH in some patients.
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| 4. |
- Hellberg, Clara, et al.
(author)
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Nationwide prevalence of primary dystonia, progressive ataxia and hereditary spastic paraplegia
- 2019
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In: Parkinsonism & Related Disorders. - : Elsevier BV. - 1353-8020. ; 69, s. 79-84
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Journal article (peer-reviewed)abstract
- Objective: To determine the nationwide prevalence of primary dystonia, ataxia and hereditary spastic paraplegia (HSP) in Sweden. Methods: We extracted data on all patients who were registered in The National Patient Register (NPR) in Sweden (population 9.64 million) at least twice during five consecutive years with a diagnosis of primary dystonia, ataxia or HSP. We excluded patients with an additional diagnosis possibly indicating secondary causes, and determined the proportion of wrongly diagnosed patients at our own tertiary center by patient examination or chart review. We analyzed patients' age and disorder subtypes, geographical distribution of patients within Sweden and the country of birth of all patients. Results: Nationwide, we identified 4239 patients (31.6% male) with a diagnosis of primary dystonia. Of 347 patients with dystonia at our center, 20.2% may have had a different final diagnosis. Extrapolation of this uncertainty rate to the national population resulted in a prevalence for primary dystonia of 35.1/100,000. There were 672 patients (49.6% male) with ataxia in NPR, and the diagnostic uncertainty rate among 81 patients in our center was 13.6% (prevalence 6.0/100,000). HSP was diagnosed in 235 patients nationwide (52.3% male, prevalence 2.4/100,000). Patients were distributed relatively evenly throughout the country. The proportions of patients with these diagnoses who were born outside of Sweden were lower (8.0-12.7%) than the proportion of all Swedish residents born abroad (15.9%). Conclusions: In this large, nationwide study, the prevalence of dystonia was high compared to previous studies, which partly may be explained by the high coverage of NPR.
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| 5. |
- Jaraj, Daniel
(author)
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Epidemiology of Normal Pressure Hydrocephalus - Prevalence, Risk Factors, Diagnosis and Prognosis
- 2016
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Doctoral thesis (other academic/artistic)abstract
- The number of older persons and individuals with cognitive impairment is expected to increase dramatically in most parts of the world. It is therefore important to learn more about disorders that affect cognition. Idiopathic normal pressure hydrocephalus (iNPH) mainly occurs in older persons and symptoms include cognitive impairment, gait disturbance and urinary symptoms. The aim of this thesis was to examine various aspects regarding the epidemiology of iNPH. The sample comprised data from the Gothenburg population studies. Study participants underwent comprehensive clinical and neuropsychiatric examinations between 1986 and 2009. iNPH was diagnosed in concordance with criteria from international consensus guidelines. Study I: The prevalence of iNPH was higher than previously reported. More than one in twenty, among 80-year-olds, had signs and symptoms consistent with probable iNPH. Study II: Vascular risk factors and markers of cerebrovascular disease were associated with iNPH. Hypertension was related to an almost three-fold increased chance of having imaging signs of iNPH. For diabetes, it was more than four-fold. The strongest relation to iNPH was for cerebral white matter lesions, which were associated with a more than six-fold increased chance. Study III: More than one fifth of the sample had ventricular enlargement, defined by current cut-off values for Evans Index. In addition, men aged 80 years or more, had on average, values equal to or higher than what is currently considered pathological. Study IV: Persons who fulfilled criteria for probable iNPH had an almost four-fold increased risk of death. In those with radiological signs of iNPH, the risk of dementia was almost three-fold increased. iNPH is probably more common than previously supposed. Many older persons have clinical and imaging signs consistent with iNPH. These findings are important considering that iNPH is a treatable disorder. Vascular factors are probably involved in the pathophysiology. Current cut- off values for ventricular enlargement, using Evans Index, ought to be reappraised in order to improve diagnostic possibilities. Untreated iNPH is associated with a poor prognosis with a high risk of death or dementia. Radiological signs of iNPH may have a greater prognostic importance than previously presumed.
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| 6. |
- Jaraj, Daniel, et al.
(author)
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Estimated ventricle size using Evans index: reference values from a population-based sample.
- 2017
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In: European Journal of Neurology. - : Wiley. - 1351-5101 .- 1468-1331. ; 24:3, s. 468-474
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Journal article (peer-reviewed)abstract
- Background and purpose Evans index is an estimate of ventricular size used in the diagnosis of idiopathic normal-pressure hydrocephalus (iNPH). Values >0.3 are considered pathological and are required by guidelines for the diagnosis of iNPH. However, there are no previous epidemiological studies on Evans index, and normal values in adults are thus not precisely known. We examined a representative sample to obtain reference values and descriptive data on Evans index. Methods A population-based sample (n = 1235) of men and women aged ≥70 years was examined. The sample comprised people living in private households and residential care, systematically selected from the Swedish population register. Neuropsychiatric examinations, including head computed tomography, were performed between 1986 and 2000. Results Evans index ranged from 0.11 to 0.46. The mean value in the total sample was 0.28 (SD, 0.04) and 20.6% (n = 255) had values >0.3. Among men aged ≥80 years, the mean value of Evans index was 0.3 (SD, 0.03). Individuals with dementia had a mean value of Evans index of 0.31 (SD, 0.05) and those with radiological signs of iNPH had a mean value of 0.36 (SD, 0.04). Conclusions A substantial number of subjects had ventricular enlargement according to current criteria. Clinicians and researchers need to be aware of the range of values among older individuals.
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| 7. |
- Jaraj, Daniel, et al.
(author)
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Mortality and risk of dementia in normal-pressure hydrocephalus: A population study.
- 2017
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In: Alzheimer's & Dementia. - : Wiley. - 1552-5260 .- 1552-5279. ; 13:8, s. 850-857
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Journal article (peer-reviewed)abstract
- Introduction We examined mortality, dementia, and progression of hydrocephalic symptoms among untreated individuals with idiopathic normal-pressure hydrocephalus (iNPH) in a population-based sample. Methods A total of 1235 persons were examined between 1986 and 2012. Shunted individuals were excluded. We examined 53 persons with hydrocephalic ventricular enlargement (probable iNPH: n = 24, asymptomatic or possible iNPH: n = 29). Comparisons were made with individuals without hydrocephalic ventricular enlargement. Results The 5-year mortality was 87.5% among those with probable iNPH. The hazard ratio (HR) for death was 3.8 (95% confidence interval [CI]: 2.5–6.0) for probable iNPH. Those with possible iNPH and asymptomatic hydrocephalic ventricular enlargement had increased risk of developing dementia, HR 2.8 (95% CI: 1.5–5.2). Only two individuals with hydrocephalic ventricular enlargement remained asymptomatic. Discussion In the present sample, persons with clinical and imaging signs of iNPH had excess mortality and an increased risk of dementia. The data also suggest that radiological signs of iNPH might be more important than previously supposed.
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| 8. |
- Jaraj, Daniel, et al.
(author)
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Prevalence of idiopathic normal-pressure hydrocephalus
- 2014
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In: Neurology. - : Ovid Technologies (Wolters Kluwer Health). - 0028-3878 .- 1526-632X. ; 82:16, s. 1449-1454
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Journal article (peer-reviewed)abstract
- Objectives:The aim of this study was to determine the prevalence of idiopathic normal-pressure hydrocephalus (iNPH) in elderly persons in a large population-based sample using radiologic and clinical examinations.Methods:We examined representative elderly populations aged 70 years and older that had undergone neuropsychiatric evaluations and CT of the brain between 1986 and 2000 (n = 1,238). Gait was evaluated by clinical examination and history of walking difficulty. Cognitive function was evaluated with the Mini-Mental State Examination and urinary incontinence by self-report. iNPH was diagnosed in concordance with the American-European iNPH guidelines. Exclusion criteria were history of meningitis, severe head trauma, and subarachnoid hemorrhage.Results:The prevalence of probable iNPH was 0.2% in those aged 70-79 years (n = 2) and 5.9% (n = 24) in those aged 80 years and older, with no difference between men and women. Only 2 of these persons had been treated for iNPH. Hydrocephalic ventricular enlargement, i.e., a CT image consistent with NPH, was found in 56 persons (4.5%). An Evans Index >0.3 was found in 256 (20.7%) and occluded sulci at the high convexity in 67 persons (5.4%). All of these findings were more common in the older age groups.Conclusions:Many elderly possess clinical and imaging features of iNPH, especially those older than 80 years. The number of persons with iNPH is probably much higher than the number of persons currently treated.
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| 9. |
- Jaraj, Daniel, et al.
(author)
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Vascular factors in suspected normal pressure hydrocephalus. A population-based study
- 2016
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In: Neurology. - 0028-3878. ; 86:7, s. 592-599
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Journal article (peer-reviewed)abstract
- Objective: We examined clinical and imaging findings of suspected idiopathic normal pressure hydrocephalus (iNPH) in relation to vascular risk factors and white matter lesions (WMLs), using a nested case-control design in a representative, population-based sample. Methods: From a population-based sample, 1,235 persons aged 70 years or older were examined with CT of the brain between 1986 and 2000. We identified 55 persons with hydrocephalic ventricular enlargement, i.e., radiologic findings consistent with iNPH. Among these, 26 had clinical signs that fulfilled international guideline criteria for probable iNPH. These cases were labeled suspected iNPH. Each case was matched to 5 controls from the same sample, based on age, sex, and study cohort. Data on risk factors were obtained from clinical examinations and the Swedish Hospital Discharge Register. History of hypertension, diabetes mellitus (DM), smoking, overweight, history of coronary artery disease, stroke/TIA, and WMLs on CT were examined. Risk factors associated with iNPH with a p value <0.1 in χ2 tests were included in conditional logistic regression models. Results: In the regression analyses, suspected iNPH was related to moderate to severe WMLs (odds ratio [OR] 5.2; 95% confidence interval [CI]: 1.5–17.6), while hydrocephalic ventricular enlargement was related to hypertension (OR 2.7; 95% CI: 1.1–6.8), moderate to severe WMLs (OR 6.5; 95% CI: 2.1–20.3), and DM (OR 4.3; 95% CI: 1.1–16.3). Conclusions: Hypertension, WMLs, and DM were related to clinical and imaging features of iNPH, suggesting that vascular mechanisms are involved in the pathophysiology. These findings might have implications for understanding disease mechanisms in iNPH and possibly prevention.
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