AlphaB-crystallin is known as a small heat shock protein with a cytoprotective function. This study was undertaken to investigate the relationship between alphaB-crystallin and changes seen in Alzheimer's disease. The distribution and immunohistochemical characteristics of alphaB-crystallin positive neurones in the cerebral cortices of 4 patients with Alzheimer's disease were examined. AlphaB-crystallin positive neurones were mainly distributed in the limbic and paralimbic regions, namely parahippocampal gyrus, fusiform gyrus, cingulate cortex, middle and superior frontal gyrus, and insular cortex, which corresponded to commonly affected regions in Alzheimer's disease. Moreover, such neurones were present predominantly in the III or V layer of the cerebral cortex. The number of alphaB-crystallin positive neurones increased in parallel with the neuronal loss. Logistic regression analysis revealed a significant relation between the density of alphaB-crystallin positive neurones and that of extracellular neurofibrillary tangles (NFTs), with a correlation coefficient (r) of 0.57 and P < 0.0001 in 14 regions of the cerebral cortex. In contrast, the relation was not statistically significant between the density of alphaB-crystallin positive neurones and that of classical senile plaques, diffuse plaques or intracellular NFTs. Modified Gallyas-Braak (GB) staining on alphaB-crystallin positive neurone demonstrated several patterns of the structures: faint GB positive structures in the swollen perikaryon with straight neurites, fine granules compressed and contorted into fuzzy bundles, intensely GB positive filamentous structures together with fine granules and very intensely GB positive ring-like NFTs in a swollen perikaryon with curved neurites. In positive neurones, the density of ring-like NFTs correlated with that of atrophic perikaryon, or bent neurites and a decrease in the immunoreactivity of alphaB-crystallin. These data suggest that a close relationship exists between the appearance of alphaB-crystallin in neurones, extracellular NFTs, and neurofibrillary formation in alphaB-crystallin positive neurones in Alzheimer brain.

Clinical features of mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) resemble those of cerebral infarcts, but the pathogenesis of infarct-like lesions is not fully understood. To characterise these infarct-like lesions, we studied two patients with MELAS using diffusion-weighted (DWI) MRI before and after stroke-like episodes and measured the apparent diffusion coefficient (ADC) in the new infarct-like lesions. These gave high signal on DWI and had much higher ADC than normal-appearing regions. The ADC remained high even 30 days after a stroke-like episode then decreased in lesions, with or without abnormality as shown by conventional MRI. We speculate that early elevation of ADC in the acute or subacute phase reflects vasogenic rather than cytotoxic edema. The ADC of the lesions, which disappeared almost completely with clinical improvement, returned to normal levels, which may reflect tissue recovery without severe damage. To our knowledge, this is the first study of DWI in MELAS.

In an autopsy case of sporadic rapidly progressive lower motor neuron disease (MND), Gallyas-positive argyrophilic and ubiquitinated filamentous intracytoplasmic inclusions were found in the neurons. Clinically, 7 months prior to death, a 68-year-old woman experienced a history of rapidly progressive muscle weakness of all four extremities and bulbar sign, without sensory and autonomic disturbance. Two months later, she became unable to stand or walk. Four months after onset, she needed respiratory support, and subsequently died due to cardiorespiratory arrest. Neuropathological examinations revealed neuronal loss and associated gliosis in the lower motor neurons, except for ocular motor nuclei, Clark’s column, and accessory cuneate nucleus, and tract degeneration was observed in the middle root zone of the posterior column and spinocerebellar tract. No Bunina bodies or Lewy body-like hyaline inclusions were found in the anterior horns. Gallyas-positive argyrophilic filamentous inclusions were found in the lower motor neurons and in nerve cells of the Clark’s column, intermediate zone, posterior horn and accessory cuneate nucleus. These were positive with anti-ubiquitin antibody but negative with anti-tau (tau-2 and AT8) and neurofilament antibodies. Electron microscopic examinations disclosed randomly arranged tubular-like filamentous profiles, with a diameter of 12–14 nm, sometimes with amorphous granules in the perikaryon. This is the first report on the Gallyas-positive argyrophilic and ubiquitinated filamentous inclusions in neurons in MND.

We have studied Gallyas- and tau-positive glial structures in three autopsied cases of motor neuron disease with dementia (MND-D). Gallyas-positive, tau-immunoreactive thread-like structures in the neuropil and crescent/coiled inclusions in the glial cells were mainly observed in the hippocampus, parahippocampal gyrus, and amygdaloid nucleus. Double staining using Gallyas staining and carbonic anhydrase 2 (CA2) immunohistochemistry revealed that some crescent/coiled inclusions occurred in the CA2-immunopositive cytoplasm of the oligodendroglia. Electron microscopic study with the Gallyas-Braak method revealed that the inclusion was a reticular, partly compact mass, containing 15 nm fibrils around round or oval nuclei. Since the regions where these structures appeared exhibited neuronal loss with gliosis, these data suggest that a cytoskeletal abnormality involving tau protein in glia might be associated with the degenerative process of MND-D.

Neurofibrillary tangles (NFT) and senile plaques (SP) contain various pathological structures, and the majority of these pathological structures are argyrophilic. To investigate the glial reactions of the argyrophilic substance, we performed immunohistochemistry for microglia or for astroglia after Gallyas-Braak staining, which is one of the most sensitive silver impregnation techniques detecting argyrophilic structures in NFT and SP. We found that extracellular argyrophilic structures in NFT and SP showed glial reactions, and we observed reactive microglia in the center of NFT and SP in contrast to astroglia, which were situated in the periphery. These findings suggest that the exposed argyrophilic components in the extracellular space stimulate both glial reactions, but that there is a striking difference in localization between microglia and astroglia.

Since Young et al 1described a patient with acute loss of sympathetic and parasympathetic functions in 1969, many similar cases have been reported as being acute idiopathic autonomic neuropathy.2 Apart from acute cholinergic neuropathy, there are four types of acute idiopathic autonomic neuropathy, classified according to somatic nerve involvement: acute pandysautonomia, which has minimal or no motor or sensory dysfunctions3;acute autonomic and sensory neuropathy in which sensation is seriously involved4-6; Guillain-Barre syndrome with prominent dysautonomia, manifesting autonomic and somatic motor dysfunctions; and acute autonomic sensory and motor neuropathy, characterised by prominent dysautonomia and severe motor and sensory impairment. Despite minimal or no sensory disturbance, reduction of myelinated fibres in biopsied sural nerve has been found in several cases with acute pandysautonomia.3However, the exact site of pathological involvement in the sensory system remains unknown, because postmortem examinations have seldom been performed. We report a case of acute pandysautonomia with no evidence of significant somatic nerve involvement, accompanied by dorsal root ganglionopathy shown by postmortem examinations. An 18 year old male student experienced low grade fever, vomiting, and tingling pain in the limbs on 26 February 1987. Orthostatic syncopy occurred three times on the next day. Difficulty in emptying the bladder, alternate diarrhoea and constipation, and lack of sweating over the whole body developed rapidly. He was admitted to the Third Department of Internal Medicine, Medical College of Oita on 24 April 1987. On physical examination, he weighed 46 kg, with a recent weight loss of 14 kg. Blood pressure was 108/78 mm Hg in the supine position and heart rate was 88/min. When changing from the supine to theupright position, he …

We report an autopsy case of a 51-year old man who showed slowly progressive pure akinesia: freezing phenomenon and festination during 21 years of illness without tremor, rigidity, upward gaze palsy, bradykinesia and dementia, which were not responded to L-DOPA clinically. Neuropathological findings revealed the circumscribed regions in the substantia nigra and middle portion of the internal globus pallidus (GPi), without neurofibrillary tangles, neuropil threads, and glial fibrillary tangles. So this case was clearly distinguished with progressive nuclear palsy and pallidonigroluisian atrophy. It was first reported to describe that L-DOPA nonresponsive pure akinesia can arise from nigopallidal atrophy.

A 61-year-old man developed mononeuritis multiplex accompanied by eosinophilia in 1993. Approximately 3 years later, acute renal dysfunction, a subendocardial tumor, and a high peripheral anti-neutrophil cytoplasmic antibody titer were also detected. Renal biopsy revealed glomerular crescents and interstitial infiltration of eosinophils, so allergic granulomatosis and angiitis was diagnosed. These clinical abnormalities regressed with steroid therapy. He had no history of asthma. This was therefore considered to be an atypical form of Churg-Strauss syndrome with rapidly progressive glomerulonephritis.

Experiments were performed to determine the effect of salinity level on the melting heat transfer characteristics of a horizontal ice cylinder immersed in quiescent saline water. Emphasis was placed on interpreting the heat transfer mechanism which dominates the solid-liquid interface situation. Measurements were carried out for saline water of 0.5–3.5 wt% in salinity, while the ambient temperature ranged from 1.8 to 24.0°C. Flow visualization was employed to investigate the transient flow patterns and corresponding solid-liquid interface locations. It was found that the flow patterns around the ice cylinder were a strong function of the saline water concentration, which then considerably affected the local heat transfer coefficient along the melting ice cylinder.

We investigated a patient with mitochondrial myopathy accompanied by cardiomyopathy. Molecular analysis disclosed a C-to-G substitution at nucleotide position 3254 of the mitochondrial tRNA(Leu)(UUR). Pedigree analysis revealed that this mutation was inherited maternally. Mutation C3254G may also be a candidate for genetic defects in mitochondrial myopathy.