Alleles With Tau and Aβ Pathology in Patients With Amyotrophic Lateral Sclerosis and Parkinsonism-Dementia Complex in the Kii Peninsula.

Background And Objectives: To examine the association of the ε4 and ε2 alleles with the pathologic features of patients with amyotrophic lateral sclerosis and parkinsonism-dementia complex cases in the Kii peninsula of Japan (Kii ALS/PDC).

Methods: We analyzed variants in 18 autopsy patients with ALS/PDC, consisting of 9, 8, and 1 patient with PDC, ALS, and PDC followed by ALS, respectively. Moreover, we revealed the relationship between variants and Aβ and tau pathologies.

Recovery of Platinum Group Metals from Spent Automotive Catalysts Using Lithium Salts and Hydrochloric Acid.

The recovery of platinum group metals (PGMs) from waste materials involves dissolving the waste in an aqueous solution. However, since PGMs are precious metals, their dissolution requires strong oxidizing agents such as chlorine gas and aqua regia. In this study, we aimed to recover PGMs via the calcination of spent automotive catalysts (autocatalysts) with Li salts based on the concept of "spent autocatalyst + waste lithium-ion batteries" and leaching with only HCl.

[Recurrent myelitis in a case of IgG4-related respiratory disease].

We report an 80-year-old man with IgG4-related pleuritis who had been treated with a low dose oral steroid for two years and developed recurrent myelitis. He was admitted to our hospital with gradually worsening numbness in the lower body and difficulty in walking due to mild weakness and loss of proprioception in the legs. T-weighted MR images of the spinal cord showed a high signal intensity lesion, located centrally in the spinal cord at the Th2-4 spine levels.

An immigrant family with Kii amyotrophic lateral sclerosis/parkinsonism-dementia complex.

Objectives: Amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) is a unique endemic on Guam island of the USA, the Kii Peninsula of Japan, and Papua state of Indonesia. The pathomechanism of ALS/PDC remains to be solved, although interaction between some environmental factors and genetic background is plausible. This is the first autopsy-proven immigrant family of ALS/PDC of the Kii Peninsula.

"Endemic paraplegia of Koza in Kii" in Honcho Koji Innen Shu published in 1689 is probably the earliest description of amyotrophic lateral sclerosis of Kii Peninsula: Presentation of the original and investigation of factuality.

High prevalence of amyotrophic lateral sclerosis (ALS) in Kii Province (Kii) located in southern Kii Peninsula was first pointed out by Kinnosuke Miura in 1911, and epidemiological studies by Kiyoshi Kimura et al. verified extremely high incidence after World War II. In 1970s, Yoshiro Yase pointed out that "endemic paraplegia of Koza in Kii" in Honcho Koji Innen Shu published in 1689 would mean the same disorder as that of ALS and be the earliest description of Kii ALS although he gave no clear grounds.

Expression of Mutant Ubiquitin and Proteostasis Impairment in Kii Amyotrophic Lateral Sclerosis/Parkinsonism-Dementia Complex Brains.

Kii amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) is a progressive neurodegenerative disorder that is endemic to the Kii peninsula of Japan. The disorder is clinically characterized by a variable combination of parkinsonism, dementia, and motor neuron symptoms. Despite extensive investigations, the etiology and pathogenesis of ALS/PDC remain unclear.

Brain Transcriptome Analysis Links Deficiencies of Stress-Responsive Proteins to the Pathomechanism of Kii ALS/PDC.

Amyotrophic lateral sclerosis and Parkinsonism-dementia complex (ALS/PDC) is a unique endemic neurodegenerative disease, with high-incidence foci in Kii Peninsula, Japan. To gather new insights into the pathological mechanisms underlying Kii ALS/PDC, we performed transcriptome analyses of patient brains. We prepared frozen brains from three individuals without neurodegenerative diseases, three patients with Alzheimer's disease, and 21 patients with Kii ALS/PDC, and then acquired microarray data from cerebral gray and white matter tissues.

[Lessons from the 116 years (1902-2018) of history of the Japanese Society of Neurology in establishing its identity].

The Japanese Society of Neurology and Psychiatry was founded in 1902 as a joint society of Neurology and Psychiatry, but was renamed the Japanese Society of Psychiatry and Neurology in 1935 because of the stagnation of activities of Neurology and the rise of those of Psychiatry. After World War II, activities of Neurology were restored and the Japanese Society of Neurology (JSN) independent from the Societies of Internal Medicine and Psychiatry was established in 1960 after overcoming many difficulties. In 1975, neurology was approved by law as one of the specialized fields of medicine.

Ultra-high-dose methylcobalamin in amyotrophic lateral sclerosis: a long-term phase II/III randomised controlled study.

Objective: To evaluate the efficacy and safety of intramuscular ultra-high-dose methylcobalamin in patients with amyotrophic lateral sclerosis (ALS).

Methods: 373 patients with ALS (El Escorial definite or probable; laboratory-supported probable; duration ≤36 months) were randomly assigned to placebo, 25 mg or 50 mg of methylcobalamin groups. The primary endpoints were the time interval to primary events (death or full ventilation support) and changes in the Revised ALS Functional Rating Scale (ALSFRS-R) score from baseline to week 182.

Nitrative Stress and Tau Accumulation in Amyotrophic Lateral Sclerosis/Parkinsonism-Dementia Complex (ALS/PDC) in the Kii Peninsula, Japan.

The Kii Peninsula of Japan is known to be a high incidence area of amyotrophic lateral sclerosis/parkinsonism-dementia complex (Kii ALS/PDC) with tauopathy. Nitrative stress and oxidative stress on ALS/PDC and their relationship to tau pathology were clarified. Seven patients with Kii ALS/PDC (3 males and 4 females, average age 70.

Amyotrophic lateral sclerosis and parkinsonism-dementia complex of the Hohara focus of the Kii Peninsula: A multiple proteinopathy?

The high incidence of amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia complex (PDC) has been previously known in the Kii Peninsula of Japan and in Guam. Recently, the accumulation of various proteins, such as tau, trans-activation response DNA binding protein 43 kDa (TDP-43), and alpha-synuclein (αSyn), was reported in the brains of patients with ALS/PDC in Guam. To confirm whether similar findings are present in Kii ALS/PDC, we neuropathologically examined the brains and spinal cords of 18 patients with ALS/PDC (clinical diagnoses: eight ALS and 10 PDC) in Hohara Village, which is the eastern focus of Kii ALS.

A migration case of Kii amyotrophic lateral sclerosis/parkinsonism dementia complex with the shortest stay in the endemic area and the longest incubation to develop the disease.

Amyotrophic lateral sclerosis/parkinsonism dementia complex (ALS/PDC) is an endemic disease observed in the Kii peninsula, Guam, and Papua. We report a case of a 76-year old man with ALS/PDC of the Kii peninsula of Japan (Kii ALS/PDC). The patient was born and grew up in the Kii peninsula.

The Improvement of the Outcome of Osmotic Demyelination Syndrome by Plasma Exchange.

A 71-year-old Japanese woman presented with progressive fatigue, lethargy, dysarthria and a gait disorder. Her laboratory data revealed hyponatremia (Na 101 mEq/L), and we started correcting her serum sodium level. Within a few days, she became comatose, bedridden, and was intubated.

Neutron activation analysis of scalp hair from ALS patients and residents in the Kii Peninsula, Japan.

The aim of this study was to evaluate the accumulation of transition metals in the scalp hair of amyotrophic lateral sclerosis (ALS) patients in the Koza/Kozagawa/Kushimoto (K) area (K-ALS) in the Kii Peninsula, Japan. Metal contents were measured in the unpermed, undyed hair samples of 88 K-residents, 20 controls, 7 K-ALS patients, and 10 sporadic ALS patients using neutron activation analysis at the Research Reactor Institute, Kyoto University. A human hair standard and elemental standards were used as comparative standards.

[Robert Wartenberg: the neurologist, the teacher, and the man].

Wartenberg was born in 1887 in Grodno, old Lithuania (present-day Belarus). He received his medical degree from the University of Rostock in 1919, and worked at the University of Freiburg until 1935, when he immigrated to the United States to escape Nazi Germany. He then worked at the University of California, San Francisco, and was appointed Clinical Professor of Neurology in 1952.

Neuropsychological study of amyotrophic lateral sclerosis and parkinsonism-dementia complex in Kii peninsula, Japan.

Background: The Kii peninsula of Japan is one of the foci of amyotrophic lateral sclerosis and parkinsonism-dementia complex (ALS/PDC) in the world. The purpose of this study is to clarify the neuropsychological features of the patients with ALS/PDC of the Kii peninsula (Kii ALS/PDC).

Methods: The medical interview was done on 13 patients with Kii ALS/PDC, 12 patients with Alzheimer's disease, 10 patients with progressive supranuclear palsy, 10 patients with frontotemporal lobar degeneration and 10 patients with dementia with Lewy bodies.

Amyotrophic lateral sclerosis and parkinsonism in Papua, Indonesia: 2001-2012 survey results.

Objective: Only one previous follow-up study of amyotrophic lateral sclerosis (ALS) and parkinsonism in Papua, Indonesia has been carried out since a survey undertaken in 1962-1981 by Gajdusek and colleagues. Therefore, to clarify the clinical epidemiology of ALS and parkinsonism in the southern coastal region of Papua, the clinical characteristics and prevalence of the diseases in this region were examined and assessed.

Methods: Cases of ALS and parkinsonism were clinically examined during a 2001-2012 survey in Bade and other villages along the Ia, Edera, Dumut and Obaa rivers in Papua, Indonesia.

EIF4G1 gene mutations are not a common cause of Parkinson's disease in the Japanese population.

Pathogenic mutations in the EIF4G1 gene were recently reported as a cause of autosomal dominant parkinsonism. To assess the frequency of EIF4G1 mutations in the Japanese population we sequenced the entire gene coding region (31 exons) in 95 patients with an apparent autosomal dominant inherited form of Parkinson's disease. We detected three novel point mutations located in a poly-glutamic acid repeat within exon 10.

Environmental characteristics and oxidative stress of inhabitants and patients with amyotrophic lateral sclerosis in a high-incidence area on the Kii Peninsula, Japan.

Objective: Although Oshima, in the Kii Peninsula of Japan, is located within a high incidence area of amyotrophic lateral sclerosis (ALS) (Koza/Kozagawa/Kushimoto area, K area), no patients with ALS were detected between 1960 and 1999. However, the incidence recently increased between 2000 and 2009. On Oshima, the source of drinking water was changed from a regional river/wells to the Kozagawa River in the K area in 1975.

[Effects of using audiovisual materials in gerontological nursing exercises].

The purpose of this study was to analyze the effects of using audiovisual materials in gerontological nursing exercises. Sixty-five second year nursing students watched audiovisual materials of scenes of communication between simulated patients (SP) with functional difficulties and other nursing students, and then presented reports on this exercise. We analyzed the reports of the nursing students, and we could determine several categories of description in their reports, which suggest that they could perceive indescribable facial expressions of the SP and mutual communication by unspoken messages between the SP and students, and that they could understand the patients' nonverbal messages through the patient's expressions and reactions.