Subgroup differences in "brain-type" transferrin and α-synuclein in Parkinson's disease and multiple system atrophy

博士（医学）福島県立医科大

Evaluation of blood-brain barrier function by quotient alpha2 macroglobulin and its relationship with interleukin-6 and complement component 3 levels in neuropsychiatric systemic lupus erythematosus.

Although quotient of alpha2 macroglobulin (Qα2MG) was previously reported to be useful for the evaluation of blood-brain barrier (BBB) function, it is not commonly used. We therefore evaluated BBB function among the various subsets of neuropsychiatric systemic lupus erythematosus (NPSLE) using quotient Q α2MG. Furthermore, we determined the correlation between Q α2MG and cerebrospinal (CSF) interleukin (IL)-6 level and quotient complement component 3 (Q C3). To determine intrathecal production of C3, the C3 index (Q C3/Q α2MG) was also calculated. Fifty-six patients with SLE were included in this study. Of these, 48 were diagnosed with NPSLE, consisting of 30 diffuse NPSLE patients (acute confusional state (ACS): n = 14, non-ACS: n = 16) and 18 patients with focal NPSLE. CSF IL-6 concentration, and paired serum and CSF levels of α2MG and C3, were measured by enzyme-linked immuno solvent assay (ELISA). The Q α2MG, Q C3, and C3 index were then calculated. Q α2MG, Q C3, and IL-6 concentrations in the CSF were significantly elevated in NPSLE compared with non-NPSLE. Among the subsets of NPSLE, significant increases in Q α2MG, CSF IL-6, and Q C3 were observed in ACS compared with non-ACS or focal NPSLE. There was a positive correlation between CSF IL-6 level and Q α2MG, as well as between Q C3 and Q α2MG, in diffuse NPSLE. There were no significant differences in C3 index between NPSLE and non-NPSLE, as well as among the subgroups of NPSLE. Our study suggests that BBB disruption is present in ACS, and elevated levels of IL-6 and C3 in CSF in diffuse NPSLE, especially in ACS, might result from their entry to the CSF from the systemic circulation through the damaged BBB, as well as increased intrathecal production. Furthermore, Q α2MG might be useful for the evaluation of BBB integrity

高齢発症てんかんが示唆された門脈大循環シャント脳症

高齢者に発症するてんかんは，焦点意識減損発作が多く，少量の抗てんかん薬が奏功し，脳卒中が原因であることが多いなどの特徴があり，有病率は1%程度と推定されている。非肝硬変性の門脈大循環シャント脳症は，中高年に多く発症し多彩な症状が出現するが，てんかん発作で発症することはまれである。今回，てんかん発作で発症した高齢の門脈大循環シャント脳症例を経験した。79歳女性がてんかん発作を来し搬送された。抗てんかん薬を投与し症状は速やかに改善したが高アンモニア血症が持続した。ウレアーゼ産生Klebsiella oxytocaが尿から検出されST合剤を投与したが高アンモニア血症は持続した。脳波で持続する全般性徐波を認めた。頭部MRIで両側淡蒼球にT1強調画像高信号域を認め，腹部CTで門脈から分枝し下大静脈へ直接流入する門脈下大静脈シャントを認めた。門脈大循環シャント脳症と診断し内科的治療で血中アンモニア値は正常化した。てんかん発作が高齢で初発した場合には，てんかん発作を来すさまざまな疾患と高齢発症てんかんとを鑑別する必要がある。超高齢社会の日本において，高齢者のてんかんに対する理解を深めることは重要である。Abstract: In elderly onset epilepsy, focal impaired awareness seizures are often observed, small doses of antiepileptic drugs are effective, and stroke is the most common cause. In addition, the prevalence of elderly onset epilepsy is estimated to be about 1%. Non-cirrhotic portal-systemic encephalopathy often develops in middle-aged and elderly people. It presents with various symptoms, but rarely with epileptic seizures. We experienced a case of portal-systemic encephalopathy with epileptic seizures in an elderly patient. A 79-year-old woman presented with epileptic seizures. The symptom improved rapidly with the administration of antiepileptic drugs, but hyperammonemia was present. Urease-producing Klebsiella oxytoca was detected in urine culture, and trimethoprim-sulfamethoxazole combination was administered, but hyperammonemia persisted. Electroencephalography showed a persistent generalized slow wave. Head MRI showed a high-intensity area in the bilateral globus pallidus on T1-weighted images, and abdominal CT showed a portal-systemic shunt that branched from the portal vein and flowed directly into the inferior vena cava. The patient was diagnosed with portal-systemic encephalopathy and the ammonia level was normalized by conservative treatment. When treating patients with the first epileptic seizure occurring at older ages, it is necessary to distinguish between elderly onset epilepsy and other diseases that cause epileptic seizures. In Japan, a super-aging society, understanding elderly onset epilepsy is important

Cerebrospinal fluid interleukin-6 levels in patients with neuropsychiatric systemic lupus erythematosus.

<p>(A) Comparison of cerebrospinal fluid (CSF) interleukin (IL)-6 level between neuropsychiatric systemic lupus erythematosus (NPSLE) and non-NPSLE. (B) Comparison of CSF IL-6 level among subsets of NPSLE. ACS, acute confusional state; non-ACS, diffuse NPSLE other than ACS; Focal, focal NPSLE. Data are shown as box plots. The boxes indicate the upper and lower interquartile range (IQR), the lines within the boxes indicate the median, the whiskers indicate the minimum and maximum IQR Each dot represents an individual sample. Statistical analysis was performed using the Mann-Whitney <i>U</i>-test.</p

Complement component 3 (C3) index (quotient of complement component 3 (Q C3)/quotient of alpha2 macroglobulin (Q α2MG)).

<p>(A) Comparison of C3 index between NPSLE and non-NPSLE. (B) Comparison of C3 index among the subsets of NPSLE. ACS, acute confusional state; non-ACS, diffuse NPSLE other than ACS; Focal, focal NPSLE. Data are shown as box plots. The boxes indicate the upper and lower interquartile range (IQR), the lines within the boxes indicate the median, the whiskers indicate the minimum and maximum IQR. Each dot represents an individual sample. Statistical analysis was performed using the Mann-Whitney <i>U</i>-test.</p

Complement component 3 levels in cerebrospinal fluid and serum.

<p>(A) Comparison of cerebrospinal fluid (CSF) and complement component 3 (C3) level between neuropsychiatric systemic lupus erythematosus (NPSLE) and non-NPSLE. (B) Comparison of CSF C3 level among the subsets of NPSLE. ACS, acute confusional state; non-ACS, diffuse NPSLE other than ACS; Focal, focal NPSLE. (C) Comparison of serum C3 level between NPSLE and non-NPSLE. (D) Correlation between serum C3 level and CSF C3 level. In (A), (B), and (C), data are shown as box plots. The boxes indicate the upper and lower interquartile range (IQR), the lines within the boxes indicate the median, the whiskers indicate the minimum and maximum IQR. Each dot represents an individual sample. Statistical analysis was performed using the Mann-Whitney <i>U</i>-test. In (D), statistical analysis was performed using Spearman’s rank correlation test.</p