Brain death and organ transplantation: knowledge, attitudes, and practice among Japanese students.

Objective: To investigate the knowledge, attitudes and practice of Japanese students regarding brain death and organ transplantation.

Methods: A 22-item questionnaire was handed out among 383 Japanese students during the 2002 academic year. The data was finally analyzed using a statistical package for social sciences, SPSS.

The model and moral justification for organ procurement in Japan.

Organ replacement therapy is a part of medical practice in today's world and many countries have adopted the required guidelines and regulations. Establishing the basis on which organs can be removed, is still one of the most controversial issues of health policy making in the debate. The critical disparity between supply and demand in organ replacement therapy, even with the existence of social acceptance and organ transplantation law, turns attention towards the importance of an appropriate model of organ procurement.

Upward gaze-evoked nystagmus with organoarsenic poisoning.

The authors report assessment of abnormal ocular movements in three patients after organoarsenic poisoning from diphenylarsinic acid. The characteristic and principal sign is upward gaze-evoked nystagmus. Moreover, vertical gaze holding impairment was shown by electronystagmography on direct current recording.

Beta-site APP cleaving enzyme 1 (BACE1) is increased in remaining neurons in Alzheimer's disease brains.

Alzheimer's disease (AD) is characterized by the extensive deposition of amyloid beta protein (Abeta) in the brain cortex. Abeta is produced from beta-amyloid precursor protein (APP) by beta-secretase and gamma-secretase. beta-Secretase has been identified as beta-site APP cleaving enzyme1 (BACE1).

Bladder and bowel dysfunction in chronic inflammatory demyelinating polyradiculoneuropathy.

We present a patient with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who developed severe bladder and bowel dysfunction (BBD) as evidenced by constipation, voiding difficulty, and urinary urgency. These symptoms appeared 10 years after onset of CIDP. Cystometry showed disturbance of bladder sensation and detrusor areflexia.

Diphenylarsinic acid poisoning from chemical weapons in Kamisu, Japan.

We noted a new clinical syndrome with prominent cerebellar symptoms in apartment building residents in Kamisu, Japan. The well that provided drinking water contained diphenylarsinic acid, a degradation product of diphenylcyanoarsine or diphenylchloroarsine, which were developed for use as chemical weapons, inducing severe vomiting and sneezing. Characteristics of diphenylarsinic acid poisoning include brainstem-cerebellar and cerebral symptoms.

A clinical and genetic study in a large cohort of patients with spinocerebellar ataxia type 6.

In order to clarify the clinical and genetic features of SCA6, we retrospectively analyzed 140 patients. We observed an inverse correlation between the age of onset and the length of the expanded allele, and also between the age of onset and the sum of CAG repeats in the normal and the expanded alleles. The ages of onset of four homozygous patients correlated better with the sum of CAG repeats in both alleles rather than with the expanded allele calculated from heterozygous SCA6 subjects.

[A case of hair loss induced by carbamazepine].

We report a 52 year-old man presenting with an acute considerable hair loss induced by carbamazepine (CBZ). The remarkable scalp hair loss started within a week after CBZ administration. There was no evidence of dermatitis or allergic reaction, or other cause for the hair loss.

Early vestibular dysfunction in Machado-Joseph disease detected by caloric test.

The selective vulnerability of distinct neuronal structures is a major feature of Machado-Joseph disease (MJD), also known as spinocerebellar ataxia 3 (SCA3). Vestibular dysfunction is known to be a symptom of MJD, but little is known about precisely when the vestibular system becomes impaired. Using a caloric test, we evaluated vestibular function in 2 MJD patients.

Frontotemporal dementia with ubiquitinated neuronal inclusions presenting with primary lateral sclerosis and parkinsonism: clinicopathological report of an autopsy case.

We report a case displaying upper motor sign, parkinsonism, and behavioral abnormality, with marked degeneration of the precentral cortex, neostriatum and frontotemporal lobes, as well as ubiquitinated neuronal inclusions. The patient was a 66-year-old male at the time of death. At age 57, he noticed progressive difficulties in speaking and swallowing.

Pravastatin at 10 mg/day does not decrease plasma levels of either amyloid-beta (Abeta) 40 or Abeta 42 in humans.

It has been assumed that statins work as a preventative drug for Alzheimer's disease (AD). Although some epidemiological observations raise doubts to the effectiveness of statins for AD, many in vitro and clinical studies insist on the effectiveness of statins decreasing amyloid-beta (Abeta) levels in medium or blood. To explore the effect of pravastatin on Abeta production, we followed the longitudinal plasma levels of both Abeta 40 and Abeta 42 during the allocation of pravastatin in 46 patients with hyperlipidemia.

[A case of sensory perineuritis with Bowen disease].

A 60-year-old man was admitted because of subacutely progressive sensory disturbance characterized by fluctuating dysesthesia and light pain on the skin of his hands and feet. He exhibited mononeuritis multiplex, and a plaque with sloughing was observed on the left side of his back. Sural nerve biopsy revealed marked thickening of the perineurium with vascularization, and no necrotizing vasculitis.

Association of Lewy bodies and glial cytoplasmic inclusions in the brain of Parkinson's disease.

We report the histopathological and immunohistochemical findings from the brain of an elderly patient diagnosed with Parkinson's disease (PD). Neuropathological examination revealed moderate neuronal cell loss and astrocytosis in the substantia nigra. Lewy bodies were found in many sites characteristic for PD, including the substantia nigra, locus coeruleus, hypothalamus, substantia innominata, pontine raphe nucleus, and dorsal motor vagal nucleus, cingulate and insular cortices.

Chrysamine G and its derivative reduce amyloid beta-induced neurotoxicity in mice.

The neurotoxicity of amyloid beta (Abeta) is widely believed to play a seminal role in neurodegeneration in Alzheimer's disease. We examined the effect of Chrysamine G (CG) on such neurotoxicity using the specific measurement of surviving neurons. CG was found to reduce the neurodegeneration induced by both the active short fragment of Abeta(25-35) and full-sized Abeta(1-40).

Chemical chaperones reduce aggregate formation and cell death caused by the truncated Machado-Joseph disease gene product with an expanded polyglutamine stretch.

Machado-Joseph disease/spinocerebellar ataxia-3 (MJD/SCA-3) is an inherited neurodegenerative disorder caused by expansion of the polyglutamine stretch in the MJD gene-encoded protein ataxin-3. The truncated form of mutated ataxin-3 causes aggregation and cell death in vitro and in vivo. Abnormal conformation and misfolding of the pathological protein are assumed critical to pathogenesis.

The expression of dystrophin, alpha-sarcoglycan, and beta-dystroglycan during skeletal muscle regeneration: immunohistochemical and western blot studies.

We evaluated re-expression of dystrophin, alpha-sarcoglycan and beta-dystroglycan in regenerating skeletal muscles of rats after cardiotoxin-induced myonecrosis in order to understand the dynamic behaviour of these proteins during the regeneration process. Immunohistochemical staining of these proteins almost disappeared in the sarcolemma of necrotic fibers on the 1st day, and was obscured due to non-specific staining on the 3rd day. Dystrophin was labeled faintly at the sarcolemma of regenerating muscle fibers on the 5th day.