Anterior Cerebral Artery Dissection in a Patient With Autosomal Dominant Polycystic Kidney Disease.

Intracranial artery dissection secondary to autosomal dominant polycystic kidney disease is far less common than cerebral aneurysm. A 55-year-old man presented a sudden onset of headache and disturbed consciousness caused by ischemic stroke in the bilateral frontal lobes with minor subarachnoid hemorrhage. The bilateral anterior cerebral arteries were firstly occluded and re-perfused with irregular narrowing and dilation in 3 days after stroke onset, indicating dissection. He was diagnosed with autosomal dominant polycystic kidney disease by abdominal CT findings and by his family history though his renal function was almost normal. Dissection in the anterior cerebral artery has not been reported previously, while some cases with dissection in the vertebral and extracranial arteries were reported in autosomal dominant polycystic kidney disease. His family also had a history of aortic dissection and subarachnoid hemorrhage. Intracranial artery dissection may be a manifestation of systemic arteriopathy with familial clustering in autosomal dominant polycystic kidney disease. Strict antihypertensive treatment is needed in these cases.

Association between cerebral small vessel diseases and mild parkinsonian signs in the elderly with vascular risk factors.

INTRODUCTION: The aim of this study was to examine the association between mild parkinsonian signs (MPS), cerebral small-vessel disease (SVD), and total SVD burden in patients with vascular risk factors. METHODS: We performed a cross-sectional study among 268 patients with vascular risk factors but without parkinsonism or dementia (71.0 ± 7.8 years, 63% male). MPS was evaluated via Unified Parkinson's Disease Rating Scale Part III. Brain MRI was used to determine SVD (cerebral microbleeds [CMBs], lacunar infarctions [LIs], and white matter hyperintensities [WMH]). The presence of each SVD feature was indicated by the total SVD score. Logistic regression analyses were performed adjusting for age, sex, history of stroke, hypertension, diabetes mellitus, and dyslipidemia. RESULTS: In a multivariate analysis, we found that the presence of CMBs, deep CMBs, mixed (in the basal ganglia and thalamus) LIs, periventricular hyperintensities (PVH), and deep WMH (DWMH), and total SVD score were significantly associated with MPS, whereas strictly lobar CMBs and other LIs (in strictly basal ganglia or strictly thalamus) were not. We also found a significant association between mixed LIs, PVH, DWMH and total SVD score and gait/balance function, between PVH and rigidity, and between mixed LIs and bradykinesia. Among elderly participants (≥73years), the association of total SVD score, deep CMBs, mixed LIs, and PVH, with MPS remained significant. CONCLUSION: Our results provide additional evidence that SVD including CMBs, and especially total SVD burden, might be a surrogate marker for MPS and support the contribution of hypertensive microangiopathy as the underlying etiology.

Prevalence of Sjögren's syndrome with dementia in a memory clinic.

INTRODUCTION: Sjögren's syndrome (SS) is an autoimmune disorder involving the exocrine glands, which affects 1.9-3.0% of the elderly population. Approximately 20% of all patients with SS have CNS involvement, including dementia, as a result of angiitis. AIMS: The aim of the study was to clarify the prevalence and impact of SS among patients in a memory clinic. METHODS: This study prospectively recruited patients with cognitive dysfunction in a memory clinic from 2007 to 2010. In addition to the examinations for dementia, the patients' levels of anti-SSA and SSB antibodies were measured. Schirmer's test and/or a lip biopsy were added if required. SS was diagnosed based on the American European consensus criteria. RESULTS: Out of 276 cases who completed the examinations, 265 (97/168 males/females, mean age: 77.9, median MMSE score: 23) did not demonstrated cognitive decline. Sixteen (6.3%) and seven (2.7%) patients were positive for anti-SS-A and SS-B antibodies, respectively. Twenty patients (7.5%) were diagnosed with primary SS (mean age: 77.2 years old, median MMSE: 21). Seven of these patients had previously been diagnosed with MCI (VCIND: 5, aMCI: 2), and 13 had been diagnosed with dementia. All had asymmetrical focal hypoperfusion on SPECT, and eighteen had subcortical lesions on MRI. Twelve were treated for dementia (median time: 2.1 years), and their MMSE significantly improved (median MMSE: 26, p=0.0019), while the non-SS subjects' MMSE declined (n=126, median: 22). CONCLUSION: The patients with SS accounted for 7.5% of those with a cognitive decline as determined at a memory clinic, and are characterized by subcortical white matter lesions and asymmetric hypoperfusion.