Enhancing Cerebral Amyloid Angiopathy-Related Inflammation Diagnosis With PET Using Pittsburgh Compound B.

ABSTRACT: Cerebral amyloid angiopathy-related inflammation is a rare encephalopathy characterized by inflammation against amyloid protein accumulated in cerebral small vessels. A 50-year-old man was presented with a subacute consciousness disorder. Brain MRI revealed high intensity lesions in the white matter of the right parietal and occipital lobes on fluid-attenuated inversion recovery sequences and cerebral microbleeds in the right parietal and occipital lobes on T2*-weighted images. Pittsburgh compound B-PET demonstrated accumulation in the right temporoparietal lobe, confirming a potential diagnosis of probable cerebral amyloid angiopathy-related inflammation without brain biopsy. Steroid pulse therapy was initiated, with good results.

First evidence of tick-borne encephalitis (TBE) outside of Hokkaido Island in Japan.

Tick-borne encephalitis (TBE) is an infection of the central nervous system caused by the tick-borne encephalitis virus (TBEV). TBE is endemic in parts of Europe and Asia. TBEV is transmitted to humans primarily by Ixodes ticks. There have been 5 TBE cases identified in Japan, all on the northern island of Hokkaido. Rodents with TBEV antibodies and Ixodes ticks have been identified throughout Japan, indicating that TBEV infection might be undiagnosed in Japan. Residual serum and cerebrospinal fluid (CSF) collected in 2010-2021 from 520 patients ≥1 year-of-age previously hospitalized with encephalitis or meningitis of unknown etiology at 15 hospitals (including 13 hospitals outside of Hokkaido) were screened by ELISA for TBEV IgG and IgM antibodies; TBEV infection was confirmed by the gold standard neutralization test. Residual serum was available from 331 (63.6%) patients and CSF from 430 (82.6%) patients; both serum and CSF were available from 189 (36.3%). Two patients were TBE cases: a female aged 61 years hospitalized for 104 days in Oita (2000 km south of Hokkaido) and a male aged 24 years hospitalized for 11 days in Tokyo (1200 km south of Hokkaido). Retrospective testing also identified a previous TBEV infection in a female aged 45 years hospitalized for 12 days in Okayama (1700 km south of Hokkaido). TBEV infection should be considered as a potential cause of encephalitis or meningitis in Japan. TBE cases are likely undiagnosed in Japan, including outside of Hokkaido, due to limited clinical awareness and lack of availability of TBE diagnostic tests.

18 F-Fluorodeoxy Glucose and 11 C-Methionine Accumulation in Demyelinating Lesions.

Background Few studies have evaluated the accumulation of 18 F-fluorodeoxyglucose (FDG), 11 C-methionine (MET), and other positron emission tomography (PET) tracers in patients with demyelinating disease. Purpose This study aimed to investigate the accumulation of FDG-PET/computed tomography (CT) and MET-PET/CT in demyelinating lesions. Material and Methods A retrospective search of the patient database in our hospital identified five patients with demyelinating disease in whom PET studies performed in the past 10 years revealed accumulation of FDG or MET. The clinical diagnoses were multiple sclerosis ( n =1), myelitis ( n =1), limbic encephalitis ( n =1), chronic inflammatory demyelinating polyneuropathy (CIDP; n =1), and acute demyelinating encephalomyelitis (ADEM; n =1). Two patients received FDG-PET/CT alone and three patients received both FDG-PET/CT and MET-PET/CT on the same day. Images were visually and conjointly reviewed by two radiologists. In semiquantitative evaluation, the maximum standardized uptake value (SUV max ) of the lesion was measured. The lesion-to-normal brain uptake ratio (L/N ratio) was calculated. Results FDG and/or MET accumulated to a part of the lesions seen on MRI. SUV max on FDG-PET/CT ranged from 3.8 to 10.3, and L/N ratio on MET-PET/CT ranged from 16.6 to 2.4. Conclusion It has been established that neoplastic and demyelinating lesions can be differentiated on the basis of FDG or MET uptake. However, as accumulation of FDG and MET can also occur in demyelinating lesions; knowledge of this possibility is of clinical importance.

Decreased circulating branched-chain amino acids are associated with development of Alzheimer's disease in elderly individuals with mild cognitive impairment.

Background: Nutritional epidemiology has shown that inadequate dietary protein intake is associated with poor brain function in the elderly population. The plasma free amino acid (PFAA) profile reflects nutritional status and may have the potential to predict future changes in cognitive function. Here, we report the results of a 2-year interim analysis of a 3-year longitudinal study following mild cognitive impairment (MCI) participants. Method: In a multicenter prospective cohort design, MCI participants were recruited, and fasting plasma samples were collected. Based on clinical assessment of cognitive function up to 2 years after blood collection, MCI participants were divided into two groups: remained with MCI or reverted to cognitively normal ("MCI-stable," N = 87) and converted to Alzheimer's disease (AD) ("AD-convert," N = 68). The baseline PFAA profile was compared between the two groups. Stratified analysis based on apolipoprotein E ε4 (APOE ε4) allele possession was also conducted. Results: Plasma concentrations of all nine essential amino acids (EAAs) were lower in the AD-convert group. Among EAAs, three branched-chain amino acids (BCAAs), valine, leucine and isoleucine, and histidine (His) exhibited significant differences even in the logistic regression model adjusted for potential confounding factors such as age, sex, body mass index (BMI), and APOE ε4 possession (p < 0.05). In the stratified analysis, differences in plasma concentrations of these four EAAs were more pronounced in the APOE ε4-negative group. Conclusion: The PFAA profile, especially decreases in BCAAs and His, is associated with development of AD in MCI participants, and the difference was larger in the APOE ε4-negative population, suggesting that the PFAA profile is an independent risk indicator for AD development. Measuring the PFAA profile may have importance in assessing the risk of AD conversion in the MCI population, possibly reflecting nutritional status. Clinical trial registration: [https://center6.umin.ac.jp/cgi-open-bin/ctr/ctr_view.cgi?recptno=R000025322], identifier [UMIN000021965].

A patient with McLeod syndrome showing involvement of the central sensorimotor tracts for the legs.

BACKGROUND: McLeod syndrome is a rare X-linked recessive acanthocytosis associated with neurological manifestations including progressive chorea, cognitive impairment, psychiatric disturbances, seizures, and sensorimotor axonal polyneuropathy. However, no studies have investigated the functioning of central sensorimotor tracts in patients with McLeod syndrome. CASE PRESENTATION: A 66-year-old man had experienced slowly progressive chorea and gait disturbance due to lower limb muscle weakness since his early fifties. Blood examinations showed erythrocyte acanthocytosis and the reduction of Kell antigens in red blood cells. Brain magnetic resonance imaging showed atrophy of the bilateral caudate nuclei and putamen. The diagnosis of McLeod syndrome was confirmed by the presence of a mutation of the XK gene on the X chromosome. Somatosensory-evoked potential and transcranial magnetic stimulation studies demonstrated that the central sensory and motor conduction times were abnormally prolonged for the lower extremity but normal for the upper extremity. CONCLUSIONS: This is the first report of the involvement of the central sensorimotor tracts for the legs in a patient with McLeod syndrome. The clinical neurophysiological technique revealed the central sensorimotor tracts involvements clinically masked by neuropathy.

Severe aquaporin 4-IgG-positive neuromyelitis optica with disseminated herpes zoster in a pregnant woman successfully treated with intravenous immunoglobulin.

A 26-year-old, 17-week pregnant woman developed aquaporin-4-IgG-positive severe longitudinally extensive transverse myelitis during the course of disseminated herpes zoster and became quadriparetic. She was unresponsive to high-dose intravenous methylprednisolone but became able to walk without assistance after intravenous immunoglobulin. One and a half months later, left optic neuritis developed but her vision improved with intravenous immunoglobulin. The only sequela was left T5 girdle sensation, and she delivered a healthy baby. Intravenous immunoglobulin may be a rescue therapy in aquaporin-4-IgG-positive neuromyelitis optica attacks in pregnant women, especially those with severe infections.

[NMDA-GluR Subunit Antibody-Positive Encephalitis: A Clinical Analysis of Five Cases].

Five consecutive cases of anti-NMDA-receptor encephalitis that we encountered were marked by a rapidly fluctuating level of consciousness associated with psychotic and delirious mental states. Opisthotonus, catatonia, and rhythmic and non-rhythmic involuntary movements of the mouth and jaw were also characteristic features of these particular cases. Serious and potentially fatal problems included epilepsia partialis continua, partial and generalized seizures, and respiratory depression, resembling the symptoms of encephalitis lethargica. An epidemic of encephalitis lethargica, also known of Economo encephalitis, occurred around 1917. Magnetic resonance imaging revealed edema of the neocortex in two cases and electroencephalography showed polymorphic and monomorphic delta slowing in the acute stage, although electroencephalographic seizure activity were not apparent. Routine cerebrospinal fluid analyses revealed lymphocyte-dominant pleocytosis in three cases, but antibodies against the NMDA-GluR subunit, GluN2B N-terminal, were at a high level in the fluid. All patients recovered without apparent sequelae. Two patients found to have ovarian teratoma underwent surgery for tumor removal. Treatments included pulse intravenous methylprednisolone, high-dose immunoglobulin, and plasma exchange together with seizure control and respiratory support. However, rituximab and or cyclophosphamide pulse therapy should also be considered for intractable cases, as indicated by recent reports. (Received February 16, 2016; Accepted May 2, 2016; Published September 1, 2016).

Time to presenting to hospital and associated factors in stroke patients. A hospital-based study in Japan.

OBJECTIVE: To investigate multifaceted factors that constrain early stroke treatment, from both clinical and social standpoints. METHODS: This was a cross-sectional study conducted at a general hospital in northern Japan. Data on time to presenting to hospital after stroke onset and its potential associated factors were retrospectively extracted from the medical records of hospitalised stroke patients and analysed. RESULTS: Median time of presenting to hospital after stroke onset for 287 patients was 12 hours (range, 0.4-347 hours), and 16.7% of cases presented within 2 hours of onset. Multivariate analysis revealed a significant level of association between a Japan Coma Scale score of ≥1 (OR=0.41; 95% CI, 0.20-0.86) and cardiogenic cerebral embolism (OR=0.36; 95% CI, 0.15-0.85). The proportions of patients treated with recombinant tissue plasminogen activator, those with a Modified Rankin Scale score of ≥2, and those in a rehabilitation hospital/long-term care ward or who died were significantly higher among patients who presented within 2 hours. CONCLUSION: Less than one fifth of our stroke patients presented to hospital within 2 hours. Although our results are preliminary given their limited generalisability and the retrospective nature of data collection, medical rather than social factors were found to be associated with time of presenting to hospital. Patients with severe symptoms presented to the hospital at an earlier stage after stroke onset. It is important to promote the earliest possible presentation to hospital after a stroke.

Comparison of characteristics and care-needs certification proportion between participants and non-participants in a geriatric health examination over a 3-year follow-up.

The purpose of this study was to compare the care-needs certification proportion of participants and non-participants in a geriatric health examination targeting community-dwelling seniors aged 70 years and older over 3 years. We implemented a geriatric health examination for 1347 community-dwelling adults aged 70 years and older in a local region of Japan in 2004. We followed the occurrence of new care-needs certification for 3 years in 443 subjects who participated in the health examination and in the 395 non-participants. Among the 838 subjects, there were 94 new certifications (11%) during the observation period. Non-participants had a significantly higher proportion of dependent, required assistance with walking and bathing, a history of stroke, poor self-reported health, tendency for depression and outdoors less than once a week than participants. Non-participants had a significantly lower average score of the motor fitness scale (MFS), their standing time from a long sitting position on the floor and the Tokyo Metropolitan Institute of Gerontology Index of Competence (TMIG Index of Competence). The proportion of care-needs certification over 3 years was significantly higher for non-participants (63/395, 16%) than for participants (31/443, 7%, p<0.05). Non-participants have a higher risk of care-needs certification. It is necessary to investigate current data gathering methods for seniors who do not undergo these examinations.

Evaluation of whole-body vibration by the category judgment method.

The purpose of this research is to establish a scale for comfort with regard to whole-body vibration by the category judgment method. Experiments were conducted with random signals as stimuli. These stimuli consisted of three types of signal, namely stimulus F, with flat PSD (Power Spectrum Density) ranging from 1 to 100 Hz, stimulus H with PSD, which became 20 dB higher at 100 Hz than at 1 Hz, and stimulus L that had a PSD 20 dB lower at 100 Hz. These signals were modified by Wk frequency weighting in accordance with ISO 2631-1, and the R.M.S. values were adjusted to be equal. In addition, the signal levels were varied over a range of five steps to create 15 kinds of individual stimuli. The subjects sat on a flat, horizontal metal plate mounted directly on the vibrator and were exposed to vertical vibrations before being asked to choose a numerical category to best indicate their perceived level of comfort (or otherwise) during each stimulus. The creation of this assessment scale, including the aforementioned categories, enabled not only clarification of the relationship between the vibration stimuli and the degree of comfort but also discovery of the connection between the frequency-weighted R.M.S. acceleration and the corresponding categories representing each degree of comfort without overlap. Moreover, it became clear that the subjects' assessment of the degree of comfort perceived differed with differences in the vibration spectrum.