Improvement trend for individual health guidance intervention according to Japan clinical guidelines by public health nurses for type 2 diabetes mellitus who visited for medical checkups regularly: a case-control preliminary report.

We conducted a retrospective case-control study to assess the efficacy of personalized health guidance interventions on individuals with type 2 diabetes mellitus and obesity. A selection was made of individuals in regular visits to the Takagi Hospital for medical checkups between January 2017, and October 2021. Totally, 108 subjects (cases) with health guidance were divided into 2 groups: one group without pharmacotherapy for diabetes mellitus in medical institutions (n = 92) and another group with pharmacotherapy (n = 116). Cases were provided with personalized health guidance interventions by public health nurses for 30 min, in accordance with the Japanese clinical guidelines for the prevention of lifestyle-related diseases. Sex- and age-matched controls were chosen from individuals with diabetes mellitus without health guidance. The intervention without pharmacotherapy resulted in improvements in health indicators, including body weight, waist circumference, diastolic blood pressure, triglyceride levels, and γ-glutamyl trans-peptidase. These positive effects were not observed in the control group without health guidance. The therapeutic effects of health guidance were observed in cases where pharmacotherapy was administered. In conclusion, the implementation of individual health guidance interventions may prove to be effective for individuals with type 2 diabetes mellitus and obesity who regularly attend medical checkups.

Internal structures of the globus pallidus in patients with Parkinson's disease: evaluation with quantitative susceptibility mapping (QSM).

OBJECTIVES: The aim of this study was to assess the susceptibility change in medial and lateral globus pallidus (GPm and GPl) related to age separately, using quantitative susceptibility mapping (QSM) and to determine whether QSM can depict GPm in Parkinson's disease (PD) patients. METHODS: QSM was performed in 19 PD patients and in 41 normal control (NC) subjects. First, we quantitatively analysed age-related changes in QSM value in NC for GPl and GPm by a manual region of interest (ROI) technique. Then, in PD patients and age-matched NC subjects, we evaluated the depiction of GPm on QSM images qualitatively. RESULTS: In NC, the QSM value within GPl significantly increased gradually with age (r = 0.32, p = 0.04), whereas it did not change with age in GPm. The average QSM value was significantly larger for GPl than for GPm (205 vs 191, p < 0.05). In both PD patients and age-matched NC, the depiction of GPm on QSM images was good in most cases (87 %, 33 of 38 sides in PD patients) mainly because of the differences in susceptibility between GPm and GPl. CONCLUSIONS: The QSM value in GPl increases gradually with age, which allows for the identification of GPm in elderly PD subjects.

Proposal: different types of alteration and loss of consciousness in epilepsy.

There are at least five types of alterations of consciousness that occur during epileptic seizures: auras with illusions or hallucinations, dyscognitive seizures, epileptic delirium, dialeptic seizures, and epileptic coma. Each of these types of alterations of consciousness has a specific semiology and a distinct pathophysiologic mechanism. In this proposal we emphasize the need to clearly define each of these alterations/loss of consciousness and to apply this terminology in semiologic descriptions and classifications of epileptic seizures. The proposal is a consensus opinion of experienced epileptologists, and it is hoped that it will lead to systematic studies that will allow a scientific characterization of the different types of alterations/loss of consciousness described in this article.

Limbic encephalitis associated with anti-voltage-gated potassium channel complex antibodies as a cause of adult-onset mesial temporal lobe epilepsy.

Recently, some reports have indicated that limbic encephalitis associated with anti-voltage-gated potassium channel complex antibodies (VGKC-Ab) is a cause of adult-onset mesial temporal lobe epilepsy (MTLE). We report a 53-year-old woman who had her first epileptic seizure at the age of 50 years old. Examination by 3-Tesla brain MRI revealed left hippocampal high signal intensity and swelling on fluid-attenuated inversion recovery (FLAIR) and T2-weighted imaging at 2 months after her first seizure. The patient received intravenous methylprednisolone and carbamazepine 300 mg/day. One month later, MRI revealed improvement of her left hippocampal abnormalities. Thereafter, she had no seizures, however, three years after her first seizure, EEG revealed a seizure pattern in the left temporal region. Brain MRI revealed left hippocampal high signal intensity and brain fluorodeoxyglucose positron emission tomography revealed hypermetabolism. Her serum VGKC-Ab levels were 118 pM(normal < 100 pM). Intravenous methylprednisolone therapy was reinitiated. Two months later, her hippocampal abnormalities had improved and 3 months later her VGKC-Ab levels decreased to 4.4 pM. Remission of the epileptic seizures was also observed. This MTLE in the middle age was considered as limbic encephalitis associated with anti- VGKC-Ab. In cases of unexplained adult-onset MTLE, limbic encephalitis associated with anti-VGKC-Ab, which responds well to immunotherapy, should be considered in the differential diagnosis.

[Clinical guidelines for epilepsy].

Many international guidelines for epilepsy from the countries in Europe, USA and Asia have been published since the introduction of evidence-based medicine. In Japan, the clinical guidelines for epilepsy management were published by the Japanese Society of Neurology (JSN) in 2002 and 2010. The clinical guideline for epilepsy 2010 primarily targets general practitioners treating epilepsy patients. The Japan Epilepsy Society has been publishing 16 guidelines for several topics since 2005. The clinical guideline for epilepsy 2010 recommends that carbamazepine can be regarded for new onset partial epilepsy and sodium valproate is for new onset generalized epilepsy as anti-epileptic drug (AED) monotherapy. The new AEDs received approval by the Ministry of Health, Labour and Welfare, Japan, mainly in the add-on treatment of adults with partial epilepsy. The clinical guideline for epilepsy 2010 will contribute to improvement in the management of epilepsy in Japan.

[Treatment of epilepsy in the elderly].

As the elderly constitute the most rapidly growing population, epilepsy in this group is an important health issue worldwide. We identified 70 patients who developed epilepsy after the age of 65 years at our institution. Complex partial seizures (CPS) without secondarily generalization were most frequent. The most frequent diagnosis was temporal lobe epilepsy. Etiological diagnosis was possible in nearly 50% patients, including those with cerebrovascular disease. Interictal EEG revealed focal epileptiform discharges in 66.7% of patients. Forty-two patients were on antiepileptic monotherapy and 52 patients had been seizure-free for more than 1 year. Epileptogenecity was relatively low in elderly patients and they responded well to antiepileptic medication.

Mesial temporal lobe epilepsy as a neuropsychiatric syndrome of systemic lupus erythematosus.

In this study, we aimed to investigate the types of seizures and epilepsy associated with systemic lupus erythematosus (SLE). We searched the medical records at a tertiary referral center to identify a cohort of epilepsy patients with SLE who were treated between January 2000 and August 2011. We analyzed the clinical and immunologic profiles of these patients, their seizure and epilepsy classifications, electroencephalography (EEG) and magnetic resonance imaging (MRI) assessments, and the treatment administered for epilepsy and SLE. As the result, 17 patients with SLE and epilepsy were identified. Seven patients had mesial temporal lobe epilepsy (MTLE), eight had epilepsy secondary to stroke, and two had generalized epilepsy. Of the seven patients with MTLE, anteriotemporal spikes were noted in all patients with EEG, and MRI findings suggesting hippocampal sclerosis were noted in four patients. Clobazam and levetiracetam were effective in treating three patients, and one patient underwent amygdalohippocampectomy. In conclusion, MTLE may be a characteristic manifestation of neuropsychiatric syndrome of systemic lupus erythematosus.

Parkinson's disease: diagnostic potential of high-resolution phase difference enhanced MR imaging at 3 T.

OBJECTIVES: To determine whether it is possible to diagnose patients with Parkinson's disease (PD) on an individual basis using magnetic resonance imaging with phase difference enhanced imaging (PADRE). METHODS: PADRE delineated the crural fibres as a layer of low signal intensity and the substantia nigra as a layer of medium signal intensity in a healthy volunteer, and showed a clear boundary between the crural fibres and the substantia nigra (BCS). Twenty-four PD patients and 24 control subjects were enrolled. Contrast ratios between the substantia nigra and occipital white matter were calculated, and two radiologists independently reviewed the PADRE findings regarding BCS obscuration. RESULTS: Mean contrast ratio in PD patients was significantly higher than in control subjects (0.56 vs 0.39, P < 0.01). The BCS on PADRE was obscured significantly more frequently in any subgroups with PD patients compared with control subjects (P < 0.01). The observation of BCS obscuration had a sensitivity, specificity and accuracy for the diagnosis of PD of 92 %, 88 % and 90 % for radiologist 1 and 83 %, 88 % and 85 % for radiologist 2, respectively. CONCLUSION: PADRE is able to identify PD in patients as a loss of delineation between the crural fibres and the substantia nigra on an individual basis.

A more realistic approach, using dynamic stimuli, to test facial emotion recognition impairment in temporal lobe epilepsy.

To explore potentially impaired social functioning in patients with mesial temporal lobe epilepsy (MTLE), we evaluated facial emotion recognition (FER) using dynamic facial stimuli. We evaluated FER in 88 patients with MTLE, including 25 posttemporal lobectomy (PTL) patients, when they watched videos of actors expressing the six basic emotions of happiness, sadness, anger, fear, surprise, and disgust. Thirty-two healthy subjects were examined as controls. The relationships between task, performance, and neurophysiological and radiological variables potentially affecting the ability to recognize moving facial emotions were examined by multivariate analysis. Both the patients with MTLE and the PTL subset demonstrated significantly impaired FER compared with healthy controls. Of the six emotions, they showed impaired recognition of sadness, fear, and disgust. Facial emotion recognition was impaired in patients with chronic MTLE, particularly those with bilateral damage. Failure to recognize emotional expressions, particularly fear, disgust, and sadness, may contribute to difficulties in social functioning and relationship building.

Clinical features and haplotype analysis of newly identified Japanese patients with gelsolin-related familial amyloidosis of Finnish type.

Familial amyloidosis of the Finnish type (FAF) is an autosomal dominant form of systematic amyloidosis characterized by lattice corneal dystrophy, cranial neuropathy, and cutis laxa. Although FAF has been frequently found in the Finnish population, FAF is a considerably rare disorder in other regions. In this study, we examined the clinical characteristics as well as the haplotypes of six Japanese patients with FAF from five families. They showed the typical clinical presentations of FAF, but we found a broad range of ages at onset of neurological symptoms. All members had the c.654G>A mutation in GSN. To evaluate the disease haplotypes, high-density single-nucleotide polymorphism (SNP) arrays were used and disease-relevant haplotypes were reconstructed. Haplotype analysis in the four apparently unrelated families suggested a common founder haplotype. In a sporadic FAF patient, however, the haplotype was dissimilar to the founder haplotype. The present study demonstrated that a founder mutation in most of the Japanese families with FAF, except for a sporadic patient in whom a de novo mutation event was suggested as the origin of the mutation.

Modern technology calls for a modern approach to classification of epileptic seizures and the epilepsies.

In the last 10-15 years the ILAE Commission on Classification and Terminology has been presenting proposals to modernize the current ILAE Classification of Epileptic Seizures and Epilepsies. These proposals were discussed extensively in a series of articles published recently in Epilepsia and Epilepsy Currents. There is almost universal consensus that the availability of new diagnostic techniques as also of a modern understanding of epilepsy calls for a complete revision of the Classification of Epileptic Seizures and Epilepsies. Unfortunately, however, the Commission is still not prepared to take a bold step ahead and completely revisit our approach to classification of epileptic seizures and epilepsies. In this manuscript we critically analyze the current proposals of the Commission and make suggestions for a classification system that reflects modern diagnostic techniques and our current understanding of epilepsy.

[Advances in research on cognitive function related to temporal lobe epilepsy: focus on social cognitive function].

Research on cognitive function related to temporal lobe epilepsy has thus far focused on memory, language, and general intelligence. Recently, however, the concept of social cognitive function has been proposed in the field of neuropsychology. Social cognitive function refers collectively to the higher cognitive functions that are essential in our social lives, and its representative aspects are facial expression recognition and decision-making. Emotional processing centered around the amygdala is thought to play a key role in the neural mechanism of this function. We conducted a study on the social cognitive function (decision-making) of patients with temporal lobe epilepsy, and found that this function is reduced in these patients, and that the right amygdalo-hippocampal complexes play an important role. In order to ensure the best possible treatment for epilepsy patients, it is necessary not only to make an accurate diagnosis and provide appropriate treatment, but also to provide support for enabling a smoother social life from the perspective of social cognitive function. Future research developments in this field are expected to contribute to total management in medical care for epilepsy patients.

A novel tract imaging technique of the brainstem using phase difference enhanced imaging: normal anatomy and initial experience in multiple system atrophy.

OBJECTIVES: To develop a new tract imaging technique for visualising small fibre tracts of the brainstem and for detecting the abnormalities in multiple system atrophy of the cerebellar type (MSA-C) using a phase difference enhanced (PADRE) imaging technique, in which the phase difference between the target and surrounding tissue is selectively enhanced. METHODS: Two neuroradiologists compared the high-spatial-resolution PADRE imaging, which was acquired from six healthy volunteers, three patients with MSA-C, and 7 patients with other types of neurodegenerative diseases involving the brainstem or cerebellum. RESULTS: Various fine fibre tracts in the brainstem, the superior and inferior cerebellar peduncles, medial lemniscus, spinothalamic tract, medial longitudinal fasciculus, central tegmental tract, corticospinal tract and transverse pontine fibres, were identified on PADRE imaging. PADRE imaging from MSA-C demonstrated the disappearance of transverse pontine fibres and significant atrophy of the inferior cerebellar peduncles, while the superior cerebellar peduncles were intact. PADRE imaging also demonstrated that the transverse pontine fibres and inferior cerebellar peduncle were not involved in the other neurodegenerative diseases. CONCLUSION: PADRE imaging can offer a new form of tract imaging of the brainstem and may have the potential to reinforce the clinical utility of MRI in differentiating MSA from other conditions.

[Quality of life and burden in caregivers for ALS patients in Japan].

The present study was conducted with 20 ALS patients and their caregivers with the aim of examining whether caregiver burden and the caregiver's quality of life were correlated to the patient's degree of functional impairment. Patients were divided into a relatively mild functional impairment group (score of 14-18 on the ALS Functional Rating Scale (ALSFRS)) and a severe ALS group (score of 0-3 on ALSFRS). For those in the high-score ALSFRS group, caregiver burden increased as the patient's degree of functional impairment progressed, but there was no correlation in the low-score group. Furthermore, caregivers in the high-score ALSFRS group had significantly more mental health problems. These findings suggest the need for mental health care and reduction of caregiver burden due to progression of functional impairment for caregivers of ALS patients still at a relatively early stage of the disease.

Partial loss of hippocampal striation in medial temporal lobe epilepsy: pilot evaluation with high-spatial-resolution T2-weighted MR imaging at 3.0 T.

PURPOSE: To determine whether partial loss of the hippocampal striation (PLHS) at 3.0 T is more accurate than the currently accepted methods of using conventional magnetic resonance (MR) imaging to detect hippocampal sclerosis in medial temporal lobe epilepsy (MTLE). MATERIALS AND METHODS: This retrospective study had institutional review board approval, and informed consent was waived. Fluid-attenuated inversion-recovery (FLAIR) MR images and T2-weighted MR images in the oblique coronal plane in 22 consecutive patients (10 men, 12 female patients; mean age, 41.0 years; range, 14-76 years) (25 hemispheres) with a clinical diagnosis of MTLE were retrospectively evaluated. Twenty-five hippocampi in 15 subjects without epilepsy were evaluated as age-matched controls. The volumes and thicknesses of the four anatomic sections of the hippocampi were quantitatively measured on the T2-weighted images. Two radiologists independently reviewed the MR imaging findings of the hippocampus regarding atrophy, abnormal signal intensity, and PLHS on each side separately, without comparing both sides. Sensitivity and specificity were calculated among the MR imaging findings. RESULTS: Signal intensity abnormality on FLAIR images had a sensitivity of 36%, a specificity of 96%, and an accuracy of 66% for the diagnosis of hippocampal sclerosis. PLHS on T2-weighted MR images had a sensitivity of 76% and a specificity of 80% for the diagnosis of hippocampal sclerosis. The sensitivity for PLHS was higher than that for atrophy (44%) and abnormal signal intensity (48%) of the hippocampus on T2-weighted MR images. Although the mean volume of the hippocampus and the thickness of the hippocampal body were significantly smaller for patients with MTLE than for control subjects (P < .001 for both), there was no clear distinguishing threshold value between abnormal and normal hippocampi. CONCLUSION: PLHS showed the highest sensitivity for MTLE. This MR imaging feature might improve the accuracy of the diagnosis of bilateral hippocampal sclerosis, although further research is required.

[New international classification of epilepsy and new antiepileptic drugs for medically intractable epilepsy].

The task force of the international league against epilepsy (ILAE) proposed the "Diagnostic scheme for people with epileptic seizures and epilepsy" in 2001. The 2001 diagnostic scheme was updated in 2006. The ILAE Core Group recommended the new classification to replace the previous one (ILAE, 1981, 1989). However, the new classification is too complex to use for clinicians except expert epileptologists. About 10 new antiepileptic drugs are launched recently. Among them, gabapentine, topiramate, and lamotrigine have been approved in Japan as adjunctive therapy for medically intractable seizures. The advantage of the new antiepileptic drugs includes newer mechanism of action, broad spectrum of anti-seizure effects, fewer side effects, and lesser drug interactions. The rational polytherapy is necessary for refractory epilepsy. The majority of elderly patients with new onset epilepsy become seizure free on antiepileptic monotherapy, often with modest dose. We are now in the new era of epilepsy treatment.

[Case of sporadic hemiplegic migraine with cerebellar ataxia].

A 34-year-old man was admitted with his unsteady gait, difficulty in speech and a paroxysmal severe headache accompanied with sensori-motor disturbance of the right extremities and aphasic symptom. His family history was unremarkable. His unsteadiness has progressed very slowly from childhood. He noted to be inarticulate at the age of 18 years. At the age of 33 years, he suddenly had an attack of severe throbbing headache, which was mainly left parietal, with nausea and photophobia. During the headache, his right extremities were paralyzed and he became aphasic. He had lost a partial memory of the event All these symptoms had gone within 24 hours. Thereafter, the same headache occurred about once a month. Neurological examination revealed a mild truncal ataxia and ataxic dysarthria. Electroencephalography (EEG) showed intermittent delta waves restricted over the left fronto-temporal region. Brain MRI showed a moderate atrophy of superior cerebellar vermis and anterior cerebellar lobe. The diagnosis of sporadic hemiplegic migraine (SHM) with cerebellar ataxia was made. Our case was very similar to familial hemiplegic migraine (FHM) 1, of which some families are accompanied with transient amnesia, cerebellar ataxia and EEG abnormality. Although we did not detect any mutations in CACNA1A gene previously reported in FHM1, our case might share same pathogenesis with FHM1.

Prevalence of adult epilepsy in a general Japanese population: The Hisayama study.

The aim of the present study was to examine the prevalence and causes of adult epilepsy in a general Japanese population. We examined a total of 3333 Japanese residents in the town of Hisayama aged ≥40 years in 2012-2013. The examination was performed mainly at the municipal center for health promotion, but some subjects were examined in their homes, hospitals, or nursing homes. Twenty-three subjects had a diagnosis of epilepsy. The prevalence (95% confidence interval [CI]) of epilepsy per 1000 was 6.9 (4.1-9.7) in total, 4.9 (1.3-8.5) in men, and 8.4 (4.3-12.5) in women (P = 0.23 between sexes). The prevalence of epilepsy was significantly higher in the elderly (aged ≥65 years; 10.3 per 1000 [95% CI 5.4-15.1]) than in the middle-aged (aged 40-64 years; 3.6 per 1000 [95% CI 0.7-6.4]; P = 0.02). The major cause of epilepsy was cerebrovascular diseases (n = 11; 48% of the epilepsy patients). More than half of the epilepsy patients experienced the first episode of seizure in older age (≥65 years; n = 13; 57%). The findings of this study suggest the clinical importance of the prevention of cerebrovascular diseases to reduce the burden of epilepsy in the future.