Normal pressure hydrocephalus represents a significant yet under-recognized neurological disorder, particularly among the elderly, and prior to this study, the exact incidence within Germany remained undefined. The condition's reversible nature contrasts starkly with its potential to lead to severe morbidity if not promptly and effectively managed [11]. This study is the first to harness data from the German Federal Database Statistics (Destatis) to evaluate the recent epidemiology of NPH in Germany, revealing a 48% increase in recorded cases between 2005 and 2022, from 5.5 per 100,000 population in 2005 to 8.0 per 100,000 population in 2022, with the most significant rise noted in the most vulnerable, namely the age group 80–89 years.
Our analysis shows that the incidence of NPH diagnosis in Germany has increased significantly over the last 18 years. Despite accounting for the ageing and growing population, we found that the number of cases rose by almost 50%. This is a much higher incidence rate than previously estimated in Germany, that ranged from 1.09-5/100,000/year. In comparison, the overall incidence of inpatient admission for iNPH was estimated to be 2.86/100,000 in the US [12]. The low incidence estimated rates reported in other studies may have been due to their study type, which is on selected subsample populations or are hospital-based [13]. Our nationwide registry study provides a more accurate depiction of iNPH diagnosis trends in Germany over the long term.
It is interesting to note that our study has shown only an increase in NPH diagnosis was observed in the three oldest age groups (70–79, 80–89 and 90+), accounting for the overall increase during the study period [22]. Conversely, groups below the age of 70 have had decreased incidences, indicating that NPH is increasingly becoming a condition exclusive to the elderly. Moreover, these age groups have also shown an increase in incidence that exceeded the growth attributable solely to expected demographic changes in their respective population size. These shifts could stem from improvements in diagnostic modalities, alterations in healthcare availability, and fluctuations in the prevalence of comorbidities [14]. Our results imply that factors beyond mere demographic shifts contribute to the increasing incidence of this condition, particularly among the elderly demographic, therefore highlighting a pivotal storyline in the epidemiology of normal pressure hydrocephalus.
The observed decline in NPH diagnoses from 2019 to 2021 warrants attention, as it potentially reflects the extensive impact of the COVID-19 pandemic on healthcare systems globally [15]. The pandemic has had a multifaceted effect on medical care, deterring individuals from seeking hospital treatment due to infection fears and overwhelming healthcare facilities, leading to a reduction in routine medical services [16]. Consequently, this period likely saw a significant number of undiagnosed or deferred NPH cases due to these capacity constraints [17]. Given that NPH requires timely diagnosis and management to prevent irreversible neurological deterioration [18], there is a concern for a substantial latent backlog of patients. These individuals remain untreated and may present a substantial 'grey number' within the German healthcare system, poised to place further strain [19] on already taxed neurological and neurosurgical services as restrictions ease. This backlog represents not only a looming clinical challenge but also emphasizes the necessity for robust healthcare strategies to address the anticipated surge in demand for NPH-related healthcare services in the coming years.
Over the duration of our study, a surge was observed in the number of patients undergoing surgical intervention as part of their normal pressure hydrocephalus management. This surge in surgical interventions reflects the burgeoning adoption of evidence-based practices, bolstered by several published guidelines, enhanced patient selection criteria and better awareness of the condition among referring physicians [20]. Mechanical complications associated with CSF shunt devices, exacerbated by patient-related factors, continue to be significant determinants for shunt revision surgeries [21]. Predominantly, the majority of revisions are attributed to underdrainage (57–66%) and shunt infections (12–17%) [22]. This overarching decline in revision rates seen in this study highlights the contemporary advancements in both surgical methodologies and shunt device technologies in Germany, and possibly improved patient selection [23]. Furthermore, the introduction of programmable valves and devices for gravitational control and refined antibiotic administration protocols, have markedly reduced the incidence of complications associated with inappropriate over-drainage (a risk factor for subdural hematomas) and CNS infection thereby reducing the need to remove or manipulate primary shunts [24].
While our study leverages extensive data from the Federal Statistical Office, it's essential to recognize potential biases due to varying reporting standards across institutions. This is especially important consider the variations and difficulties in diagnosing NPH, which is frequently mistaken for other neurodegenerative disorders [25]. The introduction of the DRG system in Germany in 2004 could have influenced the early years of our dataset, reflecting an adaptation in hospitals’ coding and reporting [26]. Such variations might lead to potential underrepresentation or misclassification of cases at certain centers. Although our data captures data from hospitals, other confounding factors, like regional healthcare disparities or demographic shifts, could also influence our results. ICD-10 and OCD codes are vital for standardizing recording medical procedures across different centers in a region and facilitate streamline retrieval identification for in retrospective analysis. It is important to acknowledge their inherent limitations including misclassification and missing/unclear data reported under ambiguous and non-specific codes. To mitigate this, we diligently incorporated these ambiguous codes in our data and analysis to offer for a comprehensive portrayal of the state of normal pressure hydrocephalus within Germany.