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Subacute sclerotic panencephalitis (SSPE) is a refractory neurological disorder after exposure to measles virus. Recently, SSPE cases have been treated with antiviral therapies, but data on the efficacy are inconclusive. Abnormal tau accumulation has been reported in the brain tissue of SSPE cases, but there are few reports in which this is amply discussed. Five autopsied cases diagnosed as definite SSPE were included in this study. The subject age or disease duration ranged from 7.6 to 40.9 years old or from 0.5 to 20.8 years, respectively. Cases 3 and 4 had been treated with antiviral therapies. All evaluated cases showed marked brain atrophy with cerebral ventricle dilatation; additionally, marked demyelination with fibrillary gliosis were observed in the cerebral white matter. The brainstem, cerebellum, and spinal cord were relatively preserved. Immunoreactivity (IR) against measles virus was seen in the brainstem tegmentum, neocortex, and/or limbic cortex of the untreated cases but was rarely seen in the two treated cases. Activated microglia were broadly observed from the cerebrum to the spinal cord and had no meaningful difference among cases. Neurofibrillary tangles characterized by a combination of 3- and 4-repeat tau were observed mainly in the oculomotor nuclei, locus coeruleus, and limbic cortex. IR against phosphorylated tau was seen mainly in the cingulate gyrus, oculomotor nuclei, and pontine tegmentum, and tended to be observed frequently in cases with long disease durations but also tended to decrease along with neuronal loss, as in Case 5, which had the longest disease duration. Since the distribution of phosphorylated tau was independent from that of measles virus, the tauopathy following SSPE was inferred to be the result of diffuse brain inflammation triggered by measles rather than a direct result of measles virus. Moreover, antiviral therapies seemed to suppress measles virus but not the progression of tauopathy.

Aim Establishing the natural course of muscular impairment in Duchenne muscular dystrophy (DMD) is essential for defining the effect of treatments in clinical trials. We retrospectively investigated the change of thigh muscle volume in DMD using new indices, muscle volume index (MVI) and %MVI. Methods MVI is the estimated muscle volume contained within a given computed tomography (CT) slice and calculated using the CT histogram-based procedure after correcting for a partial volume effect. %MVI is calculated by dividing the MVI by the entire volume of the region of interest. We applied this method to 49 retrospectively selected CT images from 21 patients with DMD, aged 6–13 years, at a relatively early stage. Results Both MVI and %MVI were found to have a significant inverse correlation with the clinical disability stage. In most patients, the changes over time in MVI and %MVI could be regressed to the exponential function Y = Ae−rX. The rate constant of the approximation curve was 0.299 ± 0.072 for MVI, and 0.351 ± 0.088 for %MVI, respectively. Conclusion MVI and %MVI have proven useful for follow-up assessments of skeletal muscle volume in patients with DMD. Our retrospective longitudinal study should provide a basis for evaluating therapeutic interventions in future clinical trials.

Nine patients with multiple system atrophy (MSA) were studied based on MRI findings of cerebral hemispheric involvement. The age at onset was 56.4+/-8.6 (mean+/-S.D.) years, duration of illness at the first MRI study 2.1+/-1.1 years, duration of illness at the last study 9.7+/-2.6 years, and the follow-up duration 7.6+/-2.3 years. Controls were 85 neurologically intact persons (60.2+/-11.1 years age). In the MRI study, measurements of the ratio of each area to the intracranial area were performed for the cerebral hemisphere, frontal, temporal and parietal-occipital lobes. A significant progression of atrophy to under the normal limit was observed in the cerebrum, frontal and temporal lobes. Besides the typical pathological lesions in MSA, five autopsied patients revealed frontal lobe atrophy with mild gliosis, mild demyelination and glial cytoplasmic inclusions (GCIs). One of these patients showed remarkable frontal lobe atrophy with degenerative changes in the cerebral cortex. We observed the involvement of the cerebral hemisphere, especially the frontal lobe.

The adiabatic bulk and shear moduli, and their temperature derivatives, of polycrystalline modified spinel of composition (Mg 0.91 Fe 0.09 ) 2 SiO 4 were determined by means of the resonance sphere technique at ambient pressure. The adiabatic bulk and shear moduli, respectively, are 165.70 and 106.25 GPa at 300 K. The temperature derivatives of the adiabatic bulk and shear moduli, respectively, are −0.016(3) and −0.012(1) GPa/K at temperatures between 278 and 318 K, which do not agree with those of Li et al. [Science 281 (1998) 675].

The autopsy findings of a 78-year-old man mimicking primary lateral sclerosis (PLS) are reported. He showed slowly progressive spasticity, pseudobulbar palsy and character change, and died 32 months after the onset of symptoms. Autopsy revealed severe atrophy of the frontal and temporal lobes, remarkable neuronal loss and gliosis in the precentral gyrus, left temporal lobe pole and amygdala, mild degeneration of the Ammon's horn, degeneration of the corticospinal tract, and very mild involvement of the lower motor neurons. The anterior horn cells only occasionally demonstrated Bunina body by cystatin-C staining, and skein-like inclusions by ubiquitin staining. This is a peculiar case with concomitant involvement in the motor cortex and temporal lobe in motor neuron disease predominantly affecting the upper motor neuron.

Sirs: Several patients with pathologically proven amyotrophic lateral sclerosis (ALS) have been reported to run a benign course without need for a mechanical ventilator [5, 6, 9]. We observed autonomous breathing in a case of long-surviving ALS, and the effects of the metabolic control system on respiration were revealed. The patient in this report is a housewife with no family history of neuromuscular disease or consanguineous pedigree. She noticed difficulty in speech at age 53 years, followed by gradual muscular weakness in her upper and lower extremities. She was diagnosed as ALS based on the clinical and EMG findings. At age 56, her developing dyspnea led to a tracheostomy and mechanical ventilation control. Three months later, she was transferred to our hospital. At that time, she was alert and had no sensory deficits. Though her ocular movements were normal, there was both moderate weakness of her facial muscles together with marked tongue atrophy with fasciculation. Muscular atrophy and motor weakness were remarkable in the upper limbs and moderate in the lower limbs. Muscle stretch reflex was hyperactive in facial muscles, jaw jerk and lower extremities, but there was areflexia in the upper extremities. There was foot clonus but no Babinski’s reflex. In spite of being under mechanical ventilation when admitted to our hospital, spontaneous breathing with SpO2 of 97–98 % has been observed during these past three years since admission. Now, she is 60 years old and in a state of tetraplegia with marked facial muscle weakness. PTR and ATR are still hyperactive. She is alert, very intelligent, and communication is maintained via a computer-assisted communication system. Neuroimaging studies show normal finding. Her respiration states are minute volume of 3–4 l and PO2 92.7 Torr/PCO2 43.5 Torr without mechanical ventilator, but she cannot voluntarily control her breathing (Fig. 1A). Her attempt to hold her breath causes decreasing amplitude of her breathing while maintainig respiration rhythm, and her intention to hold her breathing merely increases the amplitude. Laughing causes irregular respiration rhythm and increases amplitude (Fig. 1B), and her cough reflex from tracheal stimulation is positive (Fig. 1C). She cannot speak via a tracheostomy tube equipped with a speech bulb. The hypercapnic response was measured by a modification of Read’s method [10], which was performed after obtaining the informed consent of the patient and her family. She rebreathed 5 % CO2 in oxygen from a 7 l anesthesia bag. The results revealed an augmentation of ventilation in response to the progressive increase of PtCO2 up to 55 Torr (Fig. 2). Breathing is controlled separately by the autonomic and voluntary pathways [1]. A discrete lesion in the central nervous system may cause a selective paralysis of one type of respiration, but spare the other. The respiratory findings in the present case are a loss of voluntary respiration but preservation of autonomous rhythm, metabolic control and evidence of activation in response to emotional stimuli. These findings have been reported in patients with lesions in the ventral pons [2, 3, 4, 8] and bilateral pyramidal tracts [7]. The reverse condition of an impaired autonomic but preserved volitional system is called central alveolar hypoventilation syndrome or Ondine’s curse [11, 12], which requires mechanical ventilation during sleep. Preservation of autonomous respiration suggests intact medullary respiratory neurons such as the ambiguous and retroambiguous nuclei of the vagus, and respiratory rhythm-generating neurons in the caudal ventral pons [1]. Also, this patient showed normal activity of CO2/H+ sensitive neurons which are located in the solitary nuclei and ventrolateral field of the caudal medulla oblongata [1]. The influence of emotional laughing on the autonomic respiration rhythm suggests the retained pathway of limbic cortex to these respiratory neurons [3, 8]. Descending bulbospinal respiratory pathways, which run in the anterior funiculus [1], and the anterior horn cell originating the phrenic nerve must be preserved to some degree to maintain spontaneous breathing. At the same time, a loss of volitional respiration indicates quite severe degeneration in the corticospinal tract, which is indicated by hyperactive tendon reflex in extremely weakened muscle. This case shows a peculiar symptom of impaired volitional but preserved autonomous respiration. To our knowledge, this is the first report of physiological study in ALS with a benign respiration course. Metabolic stimulation seems to be useful to reinforce the respiratory movement in such patients. LETTER TO THE EDITORS

We report an autopsy case of a 67-year-old man clinicogenetically diagnosed as having spinal muscular atrophy (SMA) type III (Kugelberg-Welander disease), showing slowly progressive muscle wasting and weakness of the extremities. His brother showed similar manifestations. Autopsy revealed neuronal loss and severe gliosis in the anterior horns of the spinal cord, a marked neurogenic change of skeletal muscles and mild degeneration of cardiomyocytes. Chromatolytic change was seen in the anterior horn, but not in the Clarke's and thalamic nuclei. The anterior spinal roots were atrophic, and there was loss of myelinated fibers with abundant glial bundles. In addition, degeneration was also observed in the posterior column and dentate nucleus. The pathological features were essentially similar to those of SMA I. Chronic change was prominent while acute change was mild in degree, corresponding to a very long clinical course.

Background: Cardiac dysfunction is one of the major prognostic factors in patients with Duchenne muscular dystrophy (DMD). Minor myocardial damage assessed by plasma cardiac troponin I (cTnI) is often observed in patients with DMD. However, it is unclear that how the minor myocardial damage occurs to which patient with DMD and how it relates to LV dysfunction. Therefore, we assessed the hypothesis that minor myocardial damage is associated with LV dysfunction, the evaluation of plasma cTnI helps the prediction of LV functional deterioration, and carvedilol prevents elevation of plasma cTnI in patients with DMD. Methods: Plasma cTnI were repeatedly (every 3 months) measured for 2 years and LV function was assessed by echocardiography in 58 patients with DMD. Carvedilol (2.5–5 mg/day) was orally administered for a year to the patients who have shown plasma cTnI elevation (positive cTnI, cut off 0.06ng/mL). Results: There were 3 differential groups regarding the progression rate of LV systolic dysfunction, i.e. rapid (19% of total, LVEF < 50% in their 10 th ), slow (50% of total, LVEF > 50% in their 20 –30 th ), and unchanged group (31% of total, LVEF < 50% in their 20 –30 th ). The episode of positive cTnI was observed in 27 (46%) of total patients with DMD. LVEF was lower in patients with positive cTnI than that with negative cTnI (42 ± 2 vs. 52 ± 2%, p < 0.05). Positive cTnI was observed in all patients in rapid group, 84% of patients in slow group, and only 6% of patients in unchanged group. Fourteen differential dystrophin gene mutations were recognized in 48 patients but they were not associated with those 3 differential groups or patients with positive cTnI. Administration of carvedilol in 13 patients (LVEF 40 ± 3) decreased the cTnI detection rate (from 44 ± 5% to 26 ± 10%, p < 0.05), while it was unchanged in 14 patients (LVEF 41 ± 3) without carvedilol treatment (from 44 ± 7% to 39 ± 6%) during same observation period. Conclusions: The elevation of plasma cTnI was associated with LV systolic dysfunction in patients with DMD. The prediction of LV dysfunction in patients with DMD may be feasible with combinations of age, LVEF, and plasma cTnI elevation. Carvedilol could be a new therapeutic strategy to prevent minor myocardial damage in patients with DMD.

In this paper, we describe a case of mutiple sclerosis (MS) with diagonistic dyspraxia and the callosal lesions in MRI. The patient was a 54-year-old woman with 12 year-history of suffering from MS. Her clinical symptoms were left alien hand, mild euphoria, right blindness, left visual deficit (0.06), mild weakness of right upper limb, complete paraplegia of lower limbs, total sensory deficit below middle sternal level and neurogenic bladder. She was right-handed person and her alien hand was such a manner; when she intended to use spoon with right hand, her left hand aimlessly began to hold and release a cup or dish. Then, she was diagnosed as diagnostic dyspraxia. Neuropsychological examinations disclosed left hemispheric dysfunction including left hand agraphia and disconnection of the callosum. MRI showed patchy lesions in the callosum, right optic radiation, both side thalamus (leftright), left cerebral peduncle, and spinal cord of cervical to the thoracal portion. Although the functional disorders and the radiological atrophy of the callosum, the clinical manifestation of the callosal disconnection in MS cases has been scarcely reported, and this case seems to be a quite rare condition to be described.

The patient was 66 year-old man with no family history of neurological diseases. At age 51, he showed initial symptom of parkinsonism, and was revealed a cerebellar atrophy by CT at age 52. He was suffered from malignant syndrome followed by renal dysfunction, which needed hemodialysis therapy. At age 54, he admitted to our hospital, when he showed parkinsonism, ataxia and dysautonomia. Neuroimaging study disclosed typical findings of multiple system atrophy (MSA), and dilatation of inferior horn of the lateral ventricle. In the next year, he lost of amburatory function, and showed low cognitive function of 5 scores in HDSR. At age 57, he was tracheostomised because of complete paralysis of the vocal cord abductor muscles. MRI study disclosed marked temporal lobe atrophy. He was complicated with bladder carcinoma, and died of multiple organ failure at age 66. The brain weight was 1,115 g. Gross neuropathological findings were temporal lobe dominant cerebral atrophy and marked pontocerebellar atrophy. Meningitis and sepsis were seen in the cerebrum, and some infarctions in the temporal and frontal lobes. Besides the typical degenerative findings of MSA, remarkable temporal lobe atrophy with enlargement of the inferior horn of lateral ventricle was observed. There were numerous number of neuron containing neuronal inclusion body (NCI) in the hippocampal cortex, dentate fascia and parahippocampal gyrus. In spite of small amount of NFT in the parahippocampal gyrus, there were no se- nile plaque, tau-positive structure except NFT, argyrophilic grain or Pick body. This case was a long-survived MSA with remarkable atrophy of the temporal lobe. The characteristic neuropathological finding was numerous numbers of neurons containing NCI in the cotices arround the inferior horn. Although frontal lobe dominant cerebral lobe atrophy is common in the long-survived MSA cases, extreme temporal lobe atrophy is rare condition. There may be a MSA subtype strongly affecting temporal lobe with numerous NCI.

We report two cases of Duchenne muscular dystrophy (DMD) complicated with dilated cardiomyopathy (DCM), who were affected with cerebral infarction. Case 1 suddenly developed dysarthria and right facial weakness at age 21. Cranial CT study disclosed a low density area in the left basal ganglia and internal capsule. Case 2 had a history of transient ischemic attack (TIA) at age 21. Five months after the TIA, he developed right hemiplegia and dysarthria, and a low density area in the corona radiate in left cerebral hemisphere was observed in cranial CT. These two cases showed the radiographic cardiomegaly with cardio thoracic ratio (CTR) of 72.8% and 66.6%, the decreased echocardiographic left ventricular ejection fraction below 20%, and the elevated titer of thrombin-anti-thrombin III complex (TAT) and D-dimer. The autopsy of Case 2 at age 26 disclosed the remarkable degeneration and fibrosis of myocardium and old ischemic lesion in the left cerebral frontal cortex. Despite the negative finding of the emboli in the left heart, cardiogenic cerebral infarction secondary to DCM was strongly suspected in both cases.

We report siblings with xeroderma pigmentosum group A (XP-A) showing mild cutaneous and late-onset severe neurological manifestations. The elder brother first noticed unstability in walking at 16 years of age. Subsequently slowly progressive mental deterioration developed with cerebellar ataxia, spasticity, sensory disturbance, urinary dysfunction and vocal cord paralysis. His younger sister presented with dysarthria at 18 years of age. She showed manifestations similar to her brother's. Both of them suffered from sensitivity to the sun but no malignant skin tumor. They were diagnosed as XP-A by the measurement of unscheduled DNA synthesis and complementation analysis. Gene analyses revealed compound heterozygote for G--C substitution at the 3' splicing acceptor site of intron 3 and insertion of 4 bases in exon 6 of XPA gene. It is suggested that transcription-coupled repair is dominantly affected with relative sparing of global genome repair in these siblings.

We report an autopsy case of a 51-year-old man clinically diagnosed with a complicated type of hereditary spastic paraplegia. His sister showed similar manifestations. Gait disturbance was manifested at 14 years of age. Subsequently, slowly progressive spastic tetraplegia developed with mental deterioration, neuropathy and amyotrophy. Marked cerebral atrophy with thin corpus callosum was shown by cranial MRI. Autopsy revealed a severely atrophic brain with extreme thinning of the whole corpus callosum. Microscopically, neurodegeneration was found in the corticospinal tract, thalamus, cerebral white matter and substantia nigra, as well as in the anterior horn and posterior column of the spinal cord. The remaining neurons contained large amounts of lipofuscin and eosinophilic granules. Unique to this patient was the severe gliosis in the cerebral white matter and substantia nigra, suggesting that sufficient development had been established when the degenerative process occurred. The predominant feature of the present case is the neurodegeneration process rather than hypoplasia.

We have been introducing the intermittent positive pressure ventilation (IPPV) therapy in Duchenne muscular dystrophy (DMD) since 1990. Then, the changes of life-span and causes of mortality by IPPV were investigated in this study. The subjects were 157 patients of long-term followed up DMD in our hospital. The Kaplan-Meier's survival curve of 73 IPPV treated patients showed a median survival time of 31.0 years of age, whereas that of 20.4 years in 84 patients not treated by IPPV. The actual mean age of death in 29 IPPV treated patients was 25.68 +/- 5.18 years-old (M +/- SD), which was significantly higher than that in 74 patients with not-IPPV treated of 19.76 +/- 3.47. The IPPV treatment changed the major causes of death; 59.5% to 3.5% for respiratory failure, 12.2% to 37.9% for cardiac failure, and 0% to 10.3% for repiratory trouble. There were certain number of patients who died of repiratory infection, respiratory tract disorder, digestive organ disorders and sudden death. Thus, IPPV therapy is significantly effective for prolongation of life-span in DMD, and it seems to be necessary to establish the treatment strategy for cardiac failure and other potentially fatal complications.

We investigated neuro-respiratory function of a 60-year-old woman with a 7-year-history of amyotrophic lateral sclerosis. She was trachomized 4 years ago, however, spontaneous respiration had been observed. Plethysmography disclosed autonomous and abolished volitional control on breathing. Cough reflex and emotional laughing influenced on respiration pattern. Positive reaction of respiration to hypercapnia was observed. Then, it seemed to be preserved respiratory neurons in the medulla oblongata and bulbospinal tract, and remarkable impairement of the corticospinal tract.

We present the clinical and genetic characteristics of a Japanese patient with neuropathologically confirmed familial amyotrophic lateral sclerosis/parkinsonism dementia complex (ALS/PDC). The 68-year-old proband with an 8-year history of parkinsonism and neurogenic amyotrophy and her three siblings suffering from parkinsonism associated with dementia originated from the Kii Peninsula of Japan. The proband's brain exhibited mild frontal lobe atrophy, moderate atrophy of the pes hippocampi, decoloration of the substantia nigra and locus coerules, and atrophy of the anterior root of the spinal cord. Microscopic examinations revealed degeneration of the CA1 portion of the hippocampus to the parahippocampus gyrus, substantia nigra, locus coerules and the spinal anterior horn with Bunina bodies. Neurofibrillary tangles (NFTs) were observed in widespread regions of the central nervous system through the cerebral cortex to the spinal cord. The predominant distribution of NFTs in the the third layer of the cerebral cortex was compatible with the characteristic feature of ALS/PDC in Guam. No tau mutation was found in the proband. The lack of mutations in the tau gene not only in this patient but also in earlier reported cases of ALS in the Western Pacific seems to suggest that other genetic factors may be contributing to ALS/PDC.

This report concerns a Japanese family with neuropathological findings consistent with amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) in the Island of Guam. The proband was a 68-year-old woman with an 8-year history of parkinsonism which was followed by psychiatric symptoms and neurogenic amyotrophy 5 years after the onset. She had a family history of parkinsonism associated with dementia in all of her three siblings. They grew up in the Hobara village, a focus of amyotrophic lateral sclerosis in the Kii Peninsula of Japan in their childhood. Their parents were not consanguineous nor natives of the Kii Peninsula. The brain weight was 1040 g and there were mild frontal lobe atrophy, moderate atrophy of pes hippocampi, decoloration of the substantia nigra and locus coeruleus, and atrophy of the anterior root of the spinal cord. The microscopic examinations revealed degeneration of CA1 portion of the hippocampus to the parahippocampus gyrus, substantia nigra, locus coeruleus and spinal anterior horn with Bunina bodies. The spinal pyramidal tracts also mildly degenerated. Neurofibrillary tangles (NFT) were observed in the cerebral cortex, especially in the cortices from hippocampus to lateral occipitotemporal gyri, basal nucleus of Mynert, basal ganglia, thalamus, substantia nigra and widespread regions of the central nervous system through the brainstem to spinal cord including the nucleus of Onufrowitcz. In spite of a small amount of the senile plaques in the cerebral cortex and Lewy bodies in the substantia nigra and locus coeruleus, abundant NFT were distributed mainly in the third layer of the cerebral cortex, which is the characteristic feature of ALS/PDC. Thus, this was likely to be an ALS/PDC case outside the Guam Island. A tau mutation was not found on DNA analysis.

The progression of muscle damage was studied in the patients with dystrophinopathy (Duchenne and Becker muscular dystrophy) using CT in order to describe natural history and investigate genotype–phenotype correlations. Muscle volume index (MVI) is the estimated muscle volume contained within a given CT slice and calculated using the CT histogram-based procedure after correcting for a partial volume effect. %MVI is calculated by dividing the MVI by the entire volume of the region of interest. We applied this method to CT scans of skeletal muscles retrospectively selected from 44 patients with DMD and 22 patients with BMD in our hospital. Diagnosis was based on clinical aspects, muscle biopsy and gene analysis. In DMD, %MVI declined exponentially with age. We subdivided DMD patients according to the mutation sites (proximal/distal to exon 45). DMD patients carrying proximal mutations showed a higher degree of muscular impairment than DMD with distal mutations. In BMD, there was a correlation between disease duration and ln (%MVI). BMD patients carrying deletion of exon 45–47 showed more rapid progression of muscle damage than BMD with deletion of exon 45–48. This study provided novel data on genotype–phenotype correlations in dystrophinopathy and suggested that location of the mutation might influence motor outcome. Those findings may be useful in the clinical management of patients and clinical trials.

In this paper, atypical pathological findings in a genetic diagnosed case of dentato-rubro-pallido-luysian atrophy (DRPLA) with mild degeneration in the common lesions of the disease is reported. The patient was 59-year-old woman with 31-year history of involuntary movement, ataxia and psychiatric disorders. Her CAG repeat number of DRPLA gene was 68/14. Besides the spongy degeneration in the third and fourth layers of the occipital cortex, severe degenerations were observed in the cerebellar cortex and white matter, inferior olive, posterior funicular nuclei of the medulla oblongata, posterior and lateral funicles of the spinal cord, and Clarke's nucleus. A wide spread distribution of the intracellular polyglutamine aggregation was also showed including both common and uncommon lesions. Genetic diagnosis disclosed a DRPLA case with lesions different from the conventional cases.

The clinico-pathological study of a new type of familial parkinsonism with striatal degeneration is reported. The inheritance mode was autosomal recessive, and three out of four offspring of married cousins developed parkinsonism in their early adulthood. Their clinical signs were rigidity, bradykinesia, postural instability and dysarthria. These symptoms were slowly progressive and responsive to levodopa therapy to a variable degree. On cerebral magnetic resonance imaging, T2 and proton density-weighted images showed hyperintensity in the bilateral putamina. The neuropathological study of one case revealed atrophy of the bilateral putamina and caudate nuclei, and a severe neuronal loss and gliosis in the putamina. Patchy mosaicism of normal and degenerated tissue was observed in the putamina. A similar mode of the degeneration was mildly seen in the caudate nuclei. The substantia nigra showed atrophy of the pars reticulata, and mild to moderate neuronal loss of the pars compacta with rostral dominance, but no Lewy bodies were observed. These neuropathological findings differed from those of Parkinson's disease or juvenile parkinsonism, but mimic to those of X-linked dystonia parkinsonism (Lubag). It seems that this familial bilateral striatal degeneration is a new variant of familial parkinsonism.

Polymyositis (PM) is an autoimmune inflammatory muscle disease of unknown cause in which cellular immunity is thought to play an important pathogenic role. Class II major histocompatibility complex (class II MHC: human leukocyte antigen (HLA)-DR operates as a cofactor of antigen presentation in immunological responses. There has been a major debate over whether muscle fibers themselves synthesize and express HLA-DR molecules and play a role in antigen presentation in PM pathogenesis. In this study, we demonstrated that most muscle fibers from patients with PM synthesized and expressed HLA-DR molecules on their surface. Human leukocyte antigen-DR expression was highly specific to PM. In addition, class II transactivator (CIITA), human leukocyte antigen DM (HLA-DM), and invariant chain (Ii), which are indispensable for expression of mature HLA-DR molecules and for antigen processing and presentation, were co-expressed. One of the cytokines that could induce this expression is interferon-gamma (IFN-gamma), released by activated lymphocytes. Our results indicate that in PM muscle fibers synthesize and express HLA-DR molecules and may contribute to the inflammatory responses together with lymphocytes.

The autopsy findings of a multiple system atrophy (MSA) patient with remarkable frontal lobe atrophy are described. The patient was a 65-year-old woman with a 13-year history of untreatable parkinsonism, dysautonomia and progressive motor aphasia. The brain weight was 810 g, and there was remarkable atrophy of the cerebrum predominantly in the frontal lobe, striatum, pons and cerebellum. Microscopic examination revealed a preserved cortical structure with laminar gliosis in the sixth layer of the precentral and superior frontal gyri of the frontal lobe, and postcentral gyrus and inferior parietal lobule of the parietal lobe. The second layer of the cortices of these regions were also revealed to be in a spongy state, and mild cell loss was seen in the fifth and six layers. The frontal lobe white matter showed a mild loss of myelinated fibers and axons, and mild gliosis. Glial cytoplasmic inclusions (GCIs) were abundantly observed in the deep layer of the cortex in the regions mentioned above, and were more abundant in the white matter of the frontal and parietal lobes, callosal body, and internal, external and extreme capsules. There was severe degeneration in the olivopontocerebellar and striatonigral systems, and GCIs in widespread regions of the brain. No Pick bodies, Lewy bodies, ballooned neurons, senile plaques, or significant amounts of neurofibrillary tangles were detected. There were no vascular changes. Thus, this was a verified MSA patient with progressive aphasia and remarkable frontal lobe atrophy. We indicate a possible involvement of the cerebral lobes in MSA.

Photoelectron transfer from tris (hydroxymethyl) aminomethane (Tris) to methyl orange (MO) or methyl yellow (MY) as an electron acceptor was studied with α, β, γ, δ-tetraphenyl porphyrin (TPP) and α, β, γ, δ-tetraphenyl porphyrin trisulfonic acid (TPPS) as sensitizers. The reaction efficiency was reduced when the sensitizer was solubilized in heptaethyleneglycol dodecyl ether (HED) micelles. The partitioning of the sensitizers (TPP, TPPS) and the electron acceptors (MO, MY) was estimated by means of absorption spectral measurements in the micellar solution. At HED concentrations above 0.5 mM in aqueous solution, the sensitizers (TPP, TPPS) were completely solubilized in the micellar phase and thus the reaction environment was transferred from the aqueous phase to the micellar phase. The decrease of the reaction rate in the micellar environment can be explained in terms of the lower polarity in the micellar phase which inhibits charge separation in the sensitizer-acceptor excited complex (exciplex). The reaction rate of the TPPS-MO system was the highest and that of the TPP-MY system was the lowest in the micellar phase, because the former system was accommodated in a rather polar micellar environment, while the latter system was located in a less polar micellar environment. It was also observed that an increase in micellar concentration resulted in a decrease of the reaction rate. This was found to be caused by the decreases in the local concentrations of sensitizer and acceptor, not by a change of the reaction environment.

Preparation d'une microemulsion eau-huile, a l'aide d'heptakis-(ethyleneglycol) mono-n-laurylether, d'hexanol, de n-decane et d'eau. Etudes des etats solubilises du dichlorure de tris-(bipyridine-2,2')-ruthenium(II) dans la microemulsion, a l'aide de mesures de fluorescence

It has been observed that oxygen participates in the photodegradation of methyl orange in aqueous micellar solutions. The specific superoxide ion scavenger, superoxide dismutase, is not concerned in the reaction. The replacement of H2O by D2O results in a tenfold acceleration in the reaction rate. It is concluded that the singlet oxygen generated by the photosensitizer, tetraphenyl porphyrin, has an important role in the reaction.