Early shunt surgery improves survival in idiopathic normal pressure hydrocephalus.

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 iNPHDelayed (n = 33, waiting time for shunt surgery 6-24 months). These were compared to patients treated within 3 months, iNPHEarly (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 iNPHDelayed compared to 10.1% in iNPHEarly (p = 0.001). The adjusted hazard ratio in iNPHDelayed 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 iNPHDelayed  but in 4/16 cases in iNPHEarly (p = 0.044). CONCLUSIONS: The present data indicate that shunt surgery is effective in iNPH and that early treatment increases survival.

Mortality and risk of dementia in normal-pressure hydrocephalus: A population study.

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.

Prevalence of Possible Idiopathic Normal Pressure Hydrocephalus in Sweden: A Population-Based MRI Study in 791 70-Year-Old Participants

BACKGROUND AND OBJECTIVES: 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). METHODS: 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. RESULTS: 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). DISCUSSION: 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.

Nationwide prevalence of primary dystonia, progressive ataxia and hereditary spastic paraplegia.

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.

Prevalence and symptoms of intracranial arachnoid cysts: a population-based study.

To investigate the prevalence of intracranial arachnoid cysts in a large population-based sample. We also aimed to assess the association between arachnoid cysts and cognitive impairment, depression, epilepsy, headache, dizziness, previous head trauma, hip fractures, and mortality. A population-based cohort and nested case-control study. The sample comprised representative populations (n = 1235) aged ≥ 70 years. All participants underwent baseline neuropsychiatric examinations, including computed tomography (CT) of the brain, between 1986 and 2000. All CT scans were examined for arachnoid cysts. Headache, dizziness, history of head trauma, dementia, depression, epilepsy, and hip fracture were assessed using data from clinical examinations, interviews and the Swedish hospital discharge register. Cognition was assessed using the Mini-Mental Status Examination, and depressive symptoms using the Montgomery-Åsberg Depression Rating Scale. Date of death was obtained from the National Swedish Death Registry. The prevalence of arachnoid cysts was 2.3 % (n = 29), with no significant difference between men and women. Probands with and without cysts had the same frequency of headache, dizziness, previous head trauma, cognitive impairment, and depressive symptoms. Furthermore, there were no differences regarding the prevalence of dementia, depression, epilepsy, or previous hip fracture. Arachnoid cysts were not associated with increased mortality. Arachnoid cysts are common incidental finding, with the same rate in men and women, and are probably asymptomatic. The lack of relation with symptoms like headache, dizziness and cognitive impairment suggest caution in ascribing symptoms to incidentally discovered arachnoid cysts and a restrictive attitude to treatment.

Vascular factors in suspected normal pressure hydrocephalus: A population-based study.

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.

Prevalence of idiopathic normal-pressure hydrocephalus.

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.