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Amyotrophic lateral sclerosis (ALS) is an adult-onset intractable motor neuron disease characterized by selective degeneration of cortical neurons in the frontotemporal lobe and motor neurons in the brainstem and spinal cord. Impairment of these neural networks causes progressive muscle atrophy and weakness that spreads throughout the body, resulting in life-threatening bulbar palsy and respiratory muscle paralysis. However, no therapeutic strategy has yet been established to halt ALS progression. Although evidence for clinical practice in ALS remains insufficient, novel research findings have steadily accumulated in recent years. To provide updated evidence-based or expert consensus recommendations for the diagnosis and management of ALS, the ALS Clinical Practice Guideline Development Committee, approved by the Japanese Society of Neurology, revised and published the Japanese clinical practice guidelines for the management of ALS in 2023. In this guideline, disease-modifying therapies that have accumulated evidence from randomized controlled trials were defined as "Clinical Questions," in which the level of evidence was determined by systematic reviews. In contrast, "Questions and Answers" were defined as issues of clinically important but insufficient evidence, according to reports of a small number of cases, observational studies, and expert opinions. Based on a literature search performed in February 2022, recommendations were reached by consensus, determined by an independent panel, reviewed by external reviewers, and submitted for public comments by Japanese Society of Neurology members before publication. In this article, we summarize the revised Japanese guidelines for ALS, highlighting the regional and cultural diversity of care processes and decision-making. The guidelines cover a broad range of essential topics such as etiology, diagnostic criteria, disease monitoring and treatments, management of symptoms, respiration, rehabilitation, nutrition, metabolism, patient instructions, and various types of care support. We believe that this summary will help improve the daily clinical practice for individuals living with ALS and their caregivers.

After the Cancer Control Act (Basic Act) was enforced in April of 2007, the Ministry of Health, Labour and Welfare and local governments nominated leading hospitals (1 chief center in every prefecture, and 1 core hospital in every secondary medical area) for comprehensive promotion of research, prevention and treatment of cancers. These hospitals are supposed to organize palliative care seminars at least once every year to conduct basic training on palliative care for all doctors engaged in cancer treatment, in order to implement palliative care from the early stage. The seminar contains lectures and role-plays regarding relief of pain and improving quality of life during recuperation for all cancer patients and their families. The author suggests that neurologists attend such seminars and introduce similar skills and knowledge to patients with neurological diseases, particularly intractable diseases, after the author had occasion to join such a seminar as an assistant facilitator. The training sessions for communication with patients and families, giving bad news, using opioids, and organizing support teams among local resources also seem useful for neurological fields, until similar teaching schemes are established by appropriate organizations, such as the Japanese Society of Neurology.

We report unique MRI abnormalities seen in a patient with Tolosa-Hunt syndrome (THS). A 60-year-old woman was admitted for left eye symptoms, including periorbital pain, numbness around the left eyebrow, blurred vision, delayed light reflex and impairment of abduction. Laboratory studies were unremarkable except for elevated erythrocyte sedimentation rates. T1-weighted MRI showed a mass lesion in the left orbital apex. Fat-suppressed T2-weighted MRI showed a hyperintense parallel linear lesion on the left optic nerve and a ring lesion around it, producing the "tram-track" sign on the axial view and the "donut configuration" on the coronal view. A diagnosis of THS was made, and corticosteroid therapy was started. Symptoms were improved rapidly, and MRI abnormalities disappeared. Reevaluation of MRI which had been taken at the previous episode of the right eye symptoms two years before also showed the "tram-track" sign and the "donut configuration" on the right. These signs are easy to be recognized and well reflect the stage of the disease. They are thus useful for diagnosing THS and evaluating the effect of the treatment. One should pay attention not only to the cavernous sinus and orbital apex, but also to the optic nerve for the MRI diagnosis of THS.

The Ministry of Health, Labour and Welfare (MHLW) of Japan has funded special research programs to investigate etiology and treatment of intractable neurodegenerative diseases. Individual health-care expenditures for registered patients with Parkinson's disease (PD) (Hoehn and Yahr Stage 3-5) have been evaluated by the government. The total number of patients registered with a diagnosis of degenerative parkinsonism including PD, progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) was 75,026 in 2004. We analyzed the data from a survey of application forms completed by 23,058 PD patients, which had been entered into the MHLW's computer. The male to female ratio was 1: 1.47, and the mean ages of all patients at registration and at onset were 71.3 years and 62.7 years, respectively. Incidence of young-onset Parkinson's disease was 2.7% of all PD patients. The percentage of PD patients at Hoehn and Yahr Stage 3 was 48.8%, at Stage 4, 23.1%, and at Stage 5, 25.2%. About 60% of patients resided at home, whereas 20% were admitted into the hospital and 20% into a nursing home. The percentages of patients with diurnal fluctuations, dyskinesia and psychotic symptoms were 37.3%, 16.8% and 18.4%, respectively. Ages of patients at registration and at disease onset who presented with diurnal fluctuations and dyskinesia were typically younger and the duration of disease was longer than for those presented without them (p < 0.0001). Patients with psychotic symptoms were older and the duration of disease was longer than those without them (p < 0.0001). Ages of patients receiving ablative surgery were younger than those who received deep brain stimulation (DBS) (57.4 versus 63.1 years, p < 0.01). Approximately 70% of all the patients who underwent stereotactic surgeries were treated within 10 years from onset. The percentage of patients with feeding tube was 6.6%, and the age at registration was older (75.6 versus 71.0 years, p < 0.0001) and duration of disease was longer (11.0 versus 8.4 years, p < 0.0001) in patients with feeding tube than in patients with oral feeding. This is the first analysis of results from the survey of questionnaire for PD provided by MHLW.

To advocate patient's right is an inevitable assignment of all neurologists who have clinical activities for patients with neurological diseases, though the assignment has very broad fields and is impossible to perform by one. The author summarized present issues of guardianship, the program to protect the social welfare rights in communities, and advance directives for patients with neurological diseases. The new guardianship for adults in Japan started in 2000 and has been increasingly used mainly for the demented. Proxy decision making for a medical care by the guardian is still unsettled. The author raised a possible question on identification of the person who wants medical certification for guardianship and his her intention to obtain such a document, in order to avoid unexpected troubles in the family or involved parties. Replies about the identification from an inspector at a family court, lawyers, judicial scriveners, social workers and Japan National Council of Social Welfare were shown on the table. In May 2007, the Ministry of Health. Labour and Welfare presented the guideline of process to decide the end-of-life-care. But advance directives for medical cares are still controversial in Japan. Multidisciplinary team to advocate patients' rights was stressed in this presentation.

Amyotrophic lateral sclerosis (ALS), Parkinson's disease (PD) and spinocerebellar degeneration (SCD) are included in the 45 intractable diseases" acknowledged by the Ministry of Health, Labour and Welfare of Japan. The registration of applicants is based on the attending doctors' diagnosis, and medical costs of registered patients are covered by the government. However, the accuracy of the diagnoses has not been investigated previously. The aim of the present study is to investigate the accuracy of the diagnoses of patients registered as PD, SCD and ALS in Mie prefecture of Japan. The study design was cross-sectional and the survey was carried out using an anonymous questionnaire. We asked the attending doctors the most probable clinical diagnosis in each applicant for PD, SCD or ALS at the annual renewal of application form of the specified diseases by the Ministry. The questionnaires were sent to the 57 neurologists in 20 general hospitals and 2 neurology clinics in Mie prefecture, Japan. The survey was carried out from July to September, 2003. For each disorder (PD, ALS, SCD), the accuracy rates were calculated as the ratio of the number of patients with the most probable diagnosis of the consistent disease to the number of all patients registered each disease. Questionnaires on 678 patients replied from 31 neurologists, 27 of whom were the neurology specialists approved by the Japanese Society of Neurology, were retrieved and analyzed. They covered 41.1% of the total registered PD cases, 45.4% of total SCD cases, and 54.0% of total ALS cases in Mie prefecture. The final clinical diagnosis were made by such specialists in 641 cases (94.5%). The accuracy rate of PD was 97.3% for all degenerative parkinsonisms and 90.2% for idiopathic PD, 96.0% for SCD, and 77.8% for typical ALS and 81.5% for ALS and Kennedy-Alter-Sung syndrome. Although the most probable diagnoses were performed by the attending physicians without verification by other raters, the accuracy rates were excellently high in these diseases, partly because most of the attending physicians were neurology specialists. The relatively low accuracy rate in ALS may be caused partly by the difficulty in differentiating it from spine diseases, neuropathies or myopathies at the early stage of the disease when the patients had been registered, or by inclusion of other motor neuron diseases such as Kennedy-Alter-Sung syndrome.

We report transient dementia in a 71-year-old woman, that was caused by hyperthyroidism of painless thyroiditis. Her friends, who found her very forgetful and performing abnormal behavior, brought her to our hospital. She looked alert and cooperative. There was neither goiter nor pain of the thyroid. Neurological examination revealed impairment of recent memory with a low score of memory test (MMSE: 20/30) indicating mild dementia. EEG showed slow alpha waves (8 Hz) with random theta and delta waves in the frontotemporal regions bilaterally. MRI showed mild diffuse atrophy of the brain, and SPECT revealed diffuse decrease in the cerebral blood flow most predominantly in the frontotemporal regions bilaterally. Abnormal findings included low levels of thyroid stimulating hormone (TSH), high levels of free triiodothyronine and free thyroxine. Antithyrogloblin antibody was positive and TSH receptor antibody was negative, while CRP was normal. Radioactive Tc uptake of the thyroid gland was low. A diagnosis of painless thyroiditis was made. Thyroid function turned to euthyroid state spontaneously in four weeks without antithyroid therapy, and subsequently memory disturbance and abnormal behavior disappeared. SPECT after clinical recovery showed improvement in the cerebral blood flow of the frontotemporal lobes. To our knowledge, dementia-like symptoms secondary to hyperthyroidism of painless thyroiditis have not been reported previously, and should be kept in mind as one of the causes of treatable dementia.