[Epileptic Amnesia].

With the rise in the number of elderly individuals with dementia, the importance of the following two types of epileptic amnesia has become increasingly recognized. One is transient epileptic amnesia (TEA), which is known to have a high frequency of complications such as accelerated long-term forgetting and autobiographical amnesia; the definition and significance of the TEA complex syndrome (TEACS), an extension of the TEA concept, are described. The other is epileptic cognitive impairment resembling Alzheimer's disease (ECI-A), the definition and characteristics of which are presented along with cases encountered by the author, as reported for TEACS.

Two cases of IgG4-related disease accompanied by many cerebral microbleeds and a review of the literature: can IgG4-related disease cause cerebral small vessel vasculitis/vasculopathy?

IgG4-related disease (IgG4-RD) is a condition of unknown cause, which involves marked tissue infiltration by IgG4-positive plasma cells into various organs throughout the body. Histopathological examinations based on biopsy examinations are essential for obtaining a definitive clinical diagnosis of IgG4-RD. However, there are only a limited number of organs from which biopsy samples can be easily obtained. Furthermore, it is impossible even for recently developed imaging techniques to directly detect abnormalities affecting small organs, such as the cerebral small vessel system. Due to these limitations, the clinical diagnosis of so-called "IgG4-related cerebral small vessel vasculitis/vasculopathy" is very difficult. In this report, two cases of IgG4-RD involving elderly patients are presented, together with their cranial magnetic resonance imaging features, especially those seen on T2* imaging. Both patients exhibited many cerebral microbleeds (CMB) on T2* imaging. I consider that it is possible to indirectly detect abnormalities of the small cerebral vessels by searching for CMB because they are caused by the failure of small cerebral vessels. Of course, the fact that many CMB were seen in both cases might be considered to be a coincidence. However, the chances of this are low because a rapid increase in the number of CMB, as was seen in case 1, and the occurrence of so many CMB, as was seen in case 2, are rare. Based on my clinical experiences and the detailed findings of the IgG4-RD cases described in this report, I present the hypothesis that "IgG4-related cerebral small vessel vasculitis/vasculopathy" exists.

A proposal for a new clinical entity: transient epileptic amnesia complex syndrome (TEACS).

Transient epileptic amnesia (TEA) is a special type of temporal lobe epilepsy, the main symptom of which is recurrent amnesia attacks. In the late 1990s, Zeman et al. developed the following diagnostic criteria for TEA: (i) recurrent, witnessed episodes of amnesia; (ii) other cognitive functions remain intact during attacks; and (iii) evidence of epilepsy. It was subsequently reported that patients with TEA almost always demonstrate two other types of memory symptoms: accelerated long-term forgetting (ALF) and autobiographical amnesia (AbA). As a result, it has been recognised that TEA causes at least three characteristic types of amnesia, that is, amnesia attacks, ALF, and AbA. In this report, we present two clinical cases, in which the patients showed symptoms of ALF and/or AbA without suffering any type of epileptic seizure, including amnesia attacks, for a long time. We discuss a syndrome associated with TEA, particularly the relationship between TEA and ALF/AbA, based on our two cases and a review of the literature. In addition, we propose a new clinical entity, which we named 'transient epileptic amnesia complex syndrome (TEACS)' and will help to ensure that physicians recognise the existence of such cases and do not overlook this condition. Furthermore, the following diagnostic criteria for TEACS are proposed. (i) The patient is middle-aged to elderly at onset and has no history of epilepsy. (ii) ALF and/or AbA have been definitively diagnosed. (iii) The ALF and/or AbA precede TEA attacks and/or other epileptic seizures. (iv) Except for the ALF/AbA, the patient's cognitive functions are confirmed to be intact via clinical examinations. (v) There is evidence for a diagnosis of epilepsy. Such evidence can include: (i) wake or sleep electroencephalography; or (ii) a clear response to anti-epileptic drugs. Furthermore, we describe our hypotheses regarding the pathogenesis of ALF/AbA and discuss the relationships between TEACS and other epileptic amnesia-related syndromes.

Tactile hallucinations in dementia with Lewy bodies.

BACKGROUND: Visual hallucinations (VH) are one of the most common psychological symptoms of dementia with Lewy bodies (DLB). It is generally considered that the VH that occur in DLB usually disappear when patients try to touch imaginary objects. However, DLB patients also sometimes experience tactile hallucinations (TH). To the best of my knowledge, this is the first report to comprehensively describe the TH experienced by DLB patients. METHODS: I searched the literature for case reports that described TH in Parkinson's disease (PD), PD dementia, or DLB. There were four reports regarding TH in PD, one of which was a review article published in 2017. I described the clinical courses of five representative cases of DLB in which TH were associated with VH, and then compared the characteristics of the TH experienced in DLB with those of the TH encountered in previously reported cases of PD. RESULTS: The TH experienced by the DLB patients had very similar characteristics to the TH experienced by PD patients. However, the TH of the DLB patients differed from those of PD patients in the following respects: (i) when the DLB patients tried to touch the imaginary objects, they often did not disappear; (ii) the imaginary objects experienced by the DLB patients sometimes exhibited characteristic hardness and temperature; and (iii) the imaginary objects experienced by the DLB patients were often influenced by the patients' behaviour, which in turn led to further hallucinations. CONCLUSIONS: Based on the abovementioned characteristics of the hallucinations of DLB patients, I propose that these phenomena represent a novel psychopathological entity, which I shall temporarily refer to as, 'strengthening of VH by TH'.

Effectiveness of educational program using printed educational material on care burden distress among staff of residential aged care facilities without medical specialists and/or registered nurses: Cluster quasi-randomization study.

AIM: Behavioral and psychological symptoms of dementia are an important source of distress for caregivers. The aim of the present study was to evaluate the effectiveness of educational intervention using printed educational material for reducing distress induced by behavioral and psychological symptoms of dementia among caregivers working at facilities without medical specialists and/or registered nurses. METHODS: A cluster quasi-randomized, controlled comparative trial was carried out at 17 facilities in Japan. Our intervention was an educational program administered at baseline using printed educational material for the care staff. The primary outcome was evaluated using the Japanese version of the Neuropsychiatric Inventory Questionnaire. The secondary outcome measures were caregiver burnout evaluated using the Japanese version of the Maslach Burnout Inventory and the care dependency of residents measured using the Japanese version of the Care Dependency Scale. RESULTS: The total Neuropsychiatric Inventory Questionnaire score decreased significantly in the intervention group (F [1355] = 6.57, P = 0.01), and the difference between the intervention and control groups was also significant (F [1355] = 4.78, P = 0.03). There were no significant changes in the Maslach Burnout Inventory or Care Dependency Scale scores in the intervention group, while the Maslach Burnout Inventory subscale (personal accomplishment) score decreased significantly in the control group. CONCLUSIONS: Our intervention achieved a significant reduction of distress among caregivers working at care homes without medical specialists and/or registered nurses. The findings of this research show that educational intervention can make a valuable contribution to training programs for care staff. Geriatr Gerontol Int 2018; 18: 487-494.

Possible effectiveness of low-dose pregabalin in a patient suffering from Lewy body disease and chronic pruritus.

Many patients with Lewy body disease (LBD) complain of pain, although the detailed mechanism responsible for LBD-related pain remains to be clarified. Moreover, there does not appear to be any reports about LBD patients suffering from an itching sensation. In this report, the case of an LBD patient who experienced severe itching that persisted for over 6 months, which was treated successfully with low-dose pregabalin therapy, is described. This is the first study to report the effects of low-dose pregabalin, which has been used to treat LBD-related pain, to treat itching in a patient with LBD.

Effectiveness of low-dose pregabalin in three patients with Lewy body disease and central neuropathic pain.

Many patients with Lewy body disease complain of pain, and their pain may be associated with this disease. Recently, pain has become a focus of attention in Parkinson's disease, but there is little information regarding pain in patients who have dementia with Lewy bodies. We used pregabalin to treat three Lewy body disease patients with chronic pain that may have been related to degeneration of central neurons. All three patients responded well to pregabalin at 25-50 mg/day. To our knowledge, there have been no previous reports of pregabalin showing efficacy for central neuropathic pain in Parkinson's disease or Lewy body disease.

Similarity of symptoms between transient epileptic amnesia and Lewy body disease.

Epilepsy with the main symptom of amnesia is known as transient epileptic amnesia (TEA). Dementia with Lewy bodies (DLB) is the second most common form of neurodegenerative dementia. The concept that Lewy body disease includes Parkinson's disease with dementia and dementia with Lewy bodies was proposed in the 2005 revision of the Clinical Diagnostic Criteria. Here, we describe a woman with cognitive impairment, olfactory dysfunction, and reduced 123 I-meta-iodobenzylguanidine uptake on myocardial scintigraphy. The patient and her family and friends were unaware of parkinsonism, visual hallucinations, or epilepsy for a long period. After syncope occurred twice within a short interval, electroencephalography revealed sharp waves from the bilateral frontal to parietal lobes, indicating a diagnosis of TEA. The present case prompted us to compare the symptoms of TEA with the clinical diagnostic criteria for dementia with Lewy bodies, revealing their similarities. We also discuss whether Lewy body disease may cause TEA rather than having an incidental association with it.

Diffuse neurofibrillary tangles with calcification (Kosaka-Shibayama disease) in Japan.

Diffuse neurofibrillary tangles with calcification (DNTC) is a rare, pre-senile type of dementia. The term 'DNTC' was initially proposed by Kosaka in 1994. Although 26 autopsies and 21 clinical patients with DNTC have been described in Japan to date, DNTC has rarely been reported in the European and North American published work. We speculate that DNTC has been overlooked in other countries. Herein, we review all known reports of DNTC in Japan and propose clinical diagnostic criteria for DNTC.

Long-term efficacy of donepezil for relapse of visual hallucinations in patients with dementia with Lewy bodies.

BACKGROUND: The use of cholinesterase inhibitors is recommended for the treatment of dementia with Lewy bodies (DLB) in the guidelines of the DLB Consortium. However, no consensus regarding therapeutic approaches for DLB-related visual hallucinations has been reached. To the best of our knowledge, an appropriate dose of donepezil for the treatment of DLB at each stage has not been discussed. METHODS: Eight patients suffering from DLB with visual hallucinations were treated with donepezil. We summarize the effects of donepezil on these visual hallucinations chronologically in all cases and discuss its efficacy and characteristics. RESULTS: Donepezil contributed to the complete disappearance of visual hallucinations in all cases, and its effects were maintained for more than 6 months. However, relapses of visual hallucinations also occurred in all cases. Against these relapses, an increased dose of donepezil was very effective in resolving them again in almost all cases in this study. CONCLUSIONS: Donepezil was highly effective against visual hallucinations in DLB patients, but there were some issues regarding pharmacotherapy for DLB.

Effectiveness of low-dose milnacipran for a patient suffering from pain disorder with delusional disorder (somatic type) in the orofacial region.

Glossodynia is chronic pain localized around the tongue, with no perceivable organic abnormalities. In the fields of oral and maxillofacial surgery, it is categorized as an oral psychosomatic disease. In contrast, psychiatric nosology classifies glossodynia as a pain disorder among somatoform disorders, per the DSM-IV. The patient was a 71-year-old woman who developed symptoms of glossodynia, specifically a sore tongue. In the decade before she presented to us, she had had bizarre symptoms of oral cenesthopathy such as the sensation that her teeth had become 'limp and floppy' and that she needles in her mouth. Treatment was attempted using several psychotropic drugs, but no satisfactory response was noted. Because the patient was referred to our outpatient clinic, we tried psychotropic therapy again. Additionally, valproic acid, tandospirone and sertraline were administered (in this order), but the patient still showed no response. However, when sertraline was changed to milnacipran, all symptoms disappeared in a short period. We suggest that a small dose of milnacipran can be effective for controlling oral cenesthopathy as well as glossodynia.

Total palliative care for a patient with multiple cerebral infarctions that occurred repeatedly in association with gastric cancer (Trousseau's syndrome).

OBJECTIVE: Malignancy-related thromboembolism, also referred to as Trousseau's syndrome, can present as acute cerebral infarction, nonbacterial thrombotic endocarditis (NBTE), and migratory thrombophlebitis. Therefore, many physical, neurological, and psychological symptoms associated with Trousseau's syndrome may occur in the clinical course. METHOD: To illustrate this, we report a case of a male patient in his 50s with carcinomatous peritonitis caused by gastric cancer, with multiple cerebral infractions that developed during disease progression. The patient was admitted to our hospital for the treatment of side effects of chemotherapy, although he strongly hoped to go home as soon as possible. In addition to making social supports plans, we were required to perform intensive total palliative care, because of his physical pain, general fatigue, anorexia, abdominal and neck pain, and psychological issues (insomnia, delirium, depression, suicidal thoughts, self-mutilation, panic attacks, agoraphobia, fear of death, and feelings of hopelessness). RESULTS: To the best of our knowledge, based on the literature search, this is the first reported case of Trousseau's syndrome described in the context of total palliative care, especially psychological care. SIGNIFICANCE OF RESULTS: We propose that neurological symptoms of Trousseau's syndrome cause these extensive mental disorders. Furthermore, because of the prognosis of Trousseau's syndrome, we should utilize our expertise fulfill the patient's wishes.

A case of dementia with Lewy bodies that temporarily showed symptoms similar to Creutzfeldt-Jakob disease.

We discuss a case of a 67-year-old male with dementia with Lewy bodies (DLB) that was initially suspected as Creutzfeldt-Jakob disease (CJD) or another type of encephalopathy, because he showed rapidly progressive deterioration, myoclonus, gait disturbance and a decline in activities of daily living. The present study describes a clinically atypical case with probable DLB and reviews similar cases in the literature, and we propose a rapidly progressive clinical subtype of DLB.

Distribution of neurofibrillary tangles in diffuse neurofibrillary tangles with calcification.

AIMS: In this study, the appearance and distribution of neurofibrillary tangles (NFT) in diffuse neurofibrillary tangles with calcification (DNTC) were investigated neuropathologically in order to elucidate the detailed distribution pattern in this disease. METHODS: The distribution of NFT in six cases neuropathologically diagnosed as DNTC (two men and four women) was studied using Gallyas-Braak silver stain. The age at death ranged from 56 to 73, with an average of 63.5 +/- 7.5 years. RESULTS: NFT were seen throughout the cerebral cortex, and were especially marked in the temporal and limbic cortices. The distribution pattern of NFT in the limbic lobe was similar to that in Alzheimer's disease as reported in the previous studies. In the temporal lobe, more NFT were distributed in the anterior than in the posterior area, which was confirmed in all six cases. The temporal pole showed the highest density of NFT including ghost tangles. CONCLUSIONS: The diffuse appearance of NFT in the cerebral cortex with the highest severity in the temporal pole was found to be a neuropathological characteristic of DNTC.