Post-mortem findings in 10 patients with presumed normal-pressure hydrocephalus and review of the literature

• Published in: Neuropathology and applied neurobiology Vol. 38; no. 1; pp. 72 - 86
• Main Authors: Leinonen, V., Koivisto, A. M., Savolainen, S., Rummukainen, J., Sutela, A., Vanninen, R., Jääskeläinen, J. E., Soininen, H., Alafuzoff, I.
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01-02-2012; Blackwell
• Subjects: Aged; Aged, 80 and over; Alzheimer's disease; amyloid-β; Autopsy; beta -Amyloid; Biological and medical sciences; Biopsy; Blood Vessels - pathology; Brain; Brain - pathology; brain biopsy; Cognitive ability; Dementia disorders; Differential diagnosis; Female; Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. Cerebral palsy; Humans; Hydrocephalus; Hydrocephalus, Normal Pressure - pathology; Immunohistochemistry; Male; Medical sciences; Nervous system; Nervous system (semeiology, syndromes); Neurodegeneration; Neurodegenerative diseases; Neurokirurgi; Neurology; Neuropathology; Neurosurgery; normal-pressure hydrocephalus; Pathology; Patologi; Pressure; vascular lesions; Human; Normal pressure hydrocephaly; Nervous system diseases; vascular lesions; brain biopsy; Central nervous system; amyloid-β; Review; Cerebral disorder; Encephalon; Biopsy; β Amyloid protein; Central nervous system disease; normal-pressure hydrocephalus; Lesion; neuropathology
• ISSN: 0305-1846; 1365-2990; 1365-2990
• DOI: 10.1111/j.1365-2990.2011.01195.x

V. Leinonen, A. M. Koivisto, S. Savolainen, J. Rummukainen, A. Sutela, R. Vanninen, J. E. Jääskeläinen, H. Soininen and I. Alafuzoff (2012) Neuropathology and Applied Neurobiology38, 72–86
Post‐mortem findings in 10 patients with presumed normal‐pressure hydrocephalus and review of the literature
Aims: Neuropathological features of idiopathic normal‐pressure hydrocephalus (iNPH) are poorly characterized. Brain biopsy during life may help in the differential diagnosis of dementia, but post‐mortem validation of biopsy findings is scarce. Here we review and report brain biopsy and post‐mortem neuropathological findings in patients with presumed NPH. Methods: We evaluated 10 patients initially investigated by intraventricular pressure monitoring and a frontal cortical biopsy for histological and immunohistochemical assessment as a diagnostic procedure for presumed NPH. Results: Out of the 10 patients, eight were shunted and seven benefited. Until death, six had developed severe and two mild cognitive impairment. One was cognitively unimpaired, and one was mentally retarded. Three subjects displayed amyloid‐β (Aβ) aggregates in their frontal cortical biopsy obtained at the initial procedure. One of these patients developed Alzheimer's disease during a follow‐up time of nearly 10 years. One patient with cognitive impairment and NPH suffered from corticobasal degeneration. In six patients various vascular lesions were seen at the final neuropathological investigation. Five of them were cognitively impaired, and in four vascular lesions were seen sufficient in extent to be considered as causative regarding their symptoms. Conclusions: The frequent finding of vascular pathology in NPH is intriguing, suggesting that vascular alterations might be causative of cognitive impairment in a notable number of patients with NPH and dementia. Brain biopsy can be used to detect Aβ aggregates, but neuropathological characteristics of iNPH as a distinct disease still need to be discovered.