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[Pathophysiology and Diagnosis of Idiopathic Normal Pressure Hydrocephalus (Hakim's Disease)]
Madoka Nakajima1, Kaito Kawamura, Chihiro Akiba
1Department of Neurosurgery, Juntendo University.
Abstract:
Idiopathic normal-pressure hydrocephalus (iNPH) is a disease that can be detected early and treated promptly through neurological screening evaluations and brain imaging. Diagnosis is established based on characteristic clinical and imaging findings demonstrating not only ventricular enlargement but also tight high cortical subarachnoid spaces, along with enlargement of the Sylvian fissures and basal cisterns. Additionally, iNPH frequently coexists with neurodegenerative disorders such as Alzheimer's disease and dementia with Lewy bodies, suggesting that the iNPH brain environment is prone to abnormal protein accumulation. Recent advances in blood-based biomarker technology have enabled the detection of amyloid-β pathology in peripheral blood samples. Therefore, in the near future, the combined use of imaging studies and blood biomarker testing in neurodegenerative disease screening may support a more comprehensive diagnosis of iNPH, including its associated neurodegenerative comorbidities.
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