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Published on: May 22, 2020
Cerebrospinal fluid circulation and hydrocephalus
Ville Leinonen1, Ritva Vanninen2, Tuomas Rauramaa3
1Department of Neurosurgery, Institute of Clinical Medicine, University of Eastern Finland and Department of Neurosurgery, NeuroCenter, Kuopio University Hospital, Kuopio, Finland.
Insights
Hydrocephalus (HC) involves cerebrospinal fluid (CSF) imbalance, causing enlarged ventricles. Diagnosis and treatment depend on obstruction site, with idiopathic normal-pressure hydrocephalus (iNPH) being a common chronic form.
Area of Science:
- Neurology
- Neurosurgery
- Pathology
Background:
- Hydrocephalus (HC) is characterized by cerebrospinal fluid (CSF) production-absorption imbalance, leading to ventricular enlargement.
- Causative factors include brain tumors obstructing CSF flow, necessitating classification by obstruction site for treatment.
- Acute and chronic forms exist, with idiopathic normal-pressure hydrocephalus (iNPH) being the most frequent chronic type.
Purpose of the Study:
- To define hydrocephalus (HC) and its classifications.
- To discuss the incidence and diagnostic challenges of HC, particularly iNPH.
- To highlight the role of neuropathology in identifying HC causes and comorbidities.
Main Methods:
- Review of hydrocephalus (HC) definitions and classifications.
- Analysis of incidence data for congenital HC and idiopathic normal-pressure hydrocephalus (iNPH).
- Discussion of radiological and neuropathological diagnostic considerations.
Main Results:
- HC is defined by CSF imbalance and ventricular enlargement, with varied incidence rates.
- Congenital HC incidence is 0.4-0.6/1,000 newborns; iNPH incidence ranges from 0.5-5.5/100,000 annually.
- Radiological findings can be nonspecific, and differentiating iNPH requires careful neuropathologic examination.
Conclusions:
- Hydrocephalus classification guides treatment, such as endoscopic third ventriculostomy or CSF shunts.
- Accurate diagnosis, especially for iNPH, can be challenging due to nonspecific imaging.
- Neuropathologic examination is crucial for detecting comorbid diseases and potential etiologic factors in HC.
Abstract:
Hydrocephalus (HC) is classically defined as dynamic imbalance between the production and absorption of cerebrospinal fluid (CSF) leading to enlarged ventricles. Potential causative factors include various brain disorders like tumors causing obstruction of CSF flow within the ventricular system or the subarachnoid space. Classification of HC is based on the site of CSF flow obstruction guiding optimal treatment, with endoscopic third ventriculostomy in intraventricular obstruction and CSF shunt in communicating HC. Another clinically relevant classification is acute and chronic; the most frequent chronic form is idiopathic normal-pressure hydrocephalus (iNPH). The reported incidence of HC varies according to the study population and classification used. The incidence of congenital HC is approximately 0.4-0.6/1,000 newborns and the annual incidence of iNPH varies from 0.5/100,000 to 5.5/100,000. Radiologically, ventricular dilatation may be nonspecific, and differentiation of iNPH from other neurodegenerative diseases may be ambiguous. There are no known specific microscopic findings of HC but a systematic neuropathologic examination is needed to detect comorbid diseases and possible etiologic factors of HC. Depending on the etiology of HC, there are several nonspecific signs potentially to be seen.
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