Homovanillic acid and 5-hydroxyindole acetic acid as biomarkers for dementia with Lewy bodies and coincident Alzheimer's disease: An autopsy-confirmed study.

Dementia with Lewy bodies (DLB) and Alzheimer's disease (AD) are the two most common causes of dementia. Both pathologies often coexist, and AD patients with concomitant neocortical LB pathology (referred to as the Lewy body variant of AD) generally show faster cognitive decline and accelerated mortality relative to patients with pure AD. Thus, discriminating among patients with DLB, AD, and coincident DLB and AD is important in clinical practice. We examined levels of homovanillic acid (HVA), 5-hydroxyindole acetic acid (5-HIAA), tau, phosphorylated tau (p-tau), and beta-amyloid (Aß) 1-42 in cerebrospinal fluid (CSF) to evaluate their viability as biomarkers to discriminate among different forms of dementia. We obtained a total of 3498 CSF samples from patients admitted to our hospital during the period from 1996 to 2015. Of these patients, we were able to carry out a brain autopsy in 94 cases. Finally, 78 neuropathologically diagnosed cases (10 AD, six DLB, five DLB with AD, five controls without neurological diseases, and 52 cases with other neurological diseases) were studied. CSF levels of HVA and 5-HIAA were consistently decreased in pathologically advanced Lewy body disorder (LBD; Braak LB stages >3) compared with pathologically incipient LBD (Braak LB stages <2). These results suggest that if an individual has LB pathology in the central nervous system, CSF levels of HVA and 5-HIAA may decrease after the onset of clinical symptoms. In addition, CSF levels of HVA and 5-HIAA decreased with LB pathology, and were especially low in cases of DLB and DLB with AD. Furthermore, the combination of HVA, 5-HIAA, and brain specific proteins t-tau, p-tau, and Aß 1-42 in CSF were useful for discriminating among DLB, DLB with AD, and AD with high diagnostic accuracy.

[A Case of Corticobasal Syndrome Complicated with Hypopituitarism and Hashimoto's Disease].

We report the case of an individual with corticobasal syndrome (CBS), hypopituitarism due to a post-traumatic leptomeningeal cyst, and Hashimoto's disease. A 71-year-old woman was admitted to our hospital because of cognitive dysfunction and bradykinesia. Following a primary diagnosis of hypopituitarism and hypothyroidism, she was given hormone replacement therapy, and her clinical symptoms appeared to improve. However, some cognitive impairment and extrapyramidal symptoms remained. The results of careful neurological examinations, as well as magnetic resonance, single-photon emission computed tomography, and positron emission tomography images, suggested a diagnosis of CBS-CBD (corticobasal degeneration). Because parkinsonism and cognitive impairment can be caused by endocrinopathy, it was initially difficult to reach the complete diagnosis that included CBS. Thus, it is important to understand that complicated neurological presentations can be caused by several different disorders.

[Steroid responsive chronic meningoencephalitis reminiscent of rheumatoid meningitis: a case report].

A 62-year-old woman presented at our hospital with a headache, cognitive decline, and fever that had persisted for 3 months. On admission, fever, headache, and mild cognitive dysfunction were all clearly evident, suggesting chronic meningoencephalitis. Laboratory examination showed mild neutrophilia as well as an increase in her erythrocyte sedimentation rate and serum C-reactive protein levels. MRI showed multiple small hyperintense lesions on T2 weighted image and diffusion weighted image (DWI) in the cerebral cortex and white matter. Contrast-enhanced T1 weighted image showed the abnormal pial enhancement along the cerebral sulci. Systemic evaluations for infectious organisms, autoantibodies, and malignant tumors were all negative. Her fever and neurological symptoms continued. As a result of worsening MRI findings, a brain biopsy was carried out. Neuropathological analysis revealed neutrophilic infiltration in the subarachnoid space and multinucleated giant cells. However, there was no vasculitis on the histological sections. This pathological finding was reminiscent of rheumatoid meningitis despite articular findings of rheumatoid arthritis, as well as rheumatoid factor (RF) and anti-CCP antibody tests being negative. After oral steroid therapy, her fever and inflammatory reactions by laboratory test diminished and her cognitive function improved remarkably.