One patient thought to have NPH but had CBD

I found this abstract and the full paper a bit hard to understand. It seems that 10 patients were diagnosed during life as having NPH (normal pressure hydrocephalus), “characterized with a clinical triad of symptoms: cognitive impairment; gait difficulty; and urinary incontinence.” Each had biopsies during life to try to determine what sort of disease they had. The biopsies may’ve been performed during the placement of a shunt. All patients had brain autopsies upon death.

The main thing I’m unclear on is whether any of the 10 patients had pathologically confirmed NPH, or whether there’s even diagnostic criteria for pathologically-definitive NPH. I don’t think a single one was thought to have NPH upon brain autopsy.

But one patient had autopsy confirmed CBD and even responded to the surgical placement of a shunt in the brain. Here’s that patient’s info:

leading symptoms (prior to biopsy): gait difficulty
age at biopsy: 75
cognition at biopsy: moderate dementia
MMSE: 20
shunt response: improved
clinical diagnosis or symptoms: DM (diabetes mellitus), HTA (arterial hypertension), CI (cardiac insufficiency), psychiatric
symptoms 30y, balance disorder (sudden falls), hypokinesia, apraxia, apatia [Robin’s note: I assume this is “apathy”], ocular movement disorder

age at death: 81
cognition at death: severe dementia
post mortem neuropathological diagnosis: CBD
final cause of death: pneumonia

According to the article, there is one other study that included 38 NPH patients. One of those also had CBD upon brain autopsy.

That’s about it from this article. The abstract is below.


Neuropathology and Applied Neurobiology. 2011 Jun 23. [Epub ahead of print]

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

Leinonen V, Koivisto AM, Savolainen S, Rummukainen J, Sutela A, Vanninen R, Jääskeläinen JE, Soininen H, Alafuzoff I.
Department of Neurosurgery, Kuopio University Hospital, Finland, etc.


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.

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.

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-beta 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.

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 amyloid-beta aggregates but neuropathological characteristics of iNPH as a distinct disease still need to be discovered.

PubMed ID#: 21696417 (see for this abstract only)