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In the northern Perth Basin (Western Australia), the Early Triassic Kockatea Shale is the primary petroleum source rock. Possible source rocks in the Northern Carnarvon Basin are more varied and include the Upper Jurassic Dingo Claystone as well as the Early Triassic Locker Shale. Biomarker analyses were conducted on petroleum samples from these basins to understand the nature of the petroleum systems. Many of the analysed petroleum samples contain carotenoids (okenane, chlorobactane and isorenieratane) derived from photosynthetic sulfur bacteria, suggesting that their source rocks were deposited under conditions of photic zone euxinia (PZE) and/or derived from microbialites. In the northern Perth Basin, the major lithofacies contributing to the source rock are dark coloured mudstones deposited under PZE conditions and/or derived from microbialites. In the southern Perth Basin, the potential source rock is either Permian, Jurassic or Cretaceous in age as indicated by the low concentrations or absence of carotenoids and the Triassic biomarker n -C 33 alkylcyclohexane. There is also a possibility that the Lower Triassic Locker Shale is the source rock of petroleum in the Tubridgi field on the Peedamullah Shelf of the Northern Carnarvon Basin, based on the similarity of biomarkers to Perth Basin petroleum sourced from the Kockatea Shale. However, the possibility of charge from the Upper Jurassic Dingo Claystone cannot be entirely excluded. Supplementary material: biomarker dataset is available at https://doi.org/10.6084/m9.figshare.c.6452153

Summary Biomarker analysis has been conducted on rock samples of the Late Permian to Early Triassic Kockatea Shale containing microbial mats in the northern Perth Basin. The northern wells of the northern Perth Basin exhibit darker shale horizons and contain microbial mats. These horizons contain abundant carotenoid biomarkers attributed to PZE and microbial mats. Such biomarkers are however, not identified in the southern wells of the northern Perth basin which are deposited under more oxic conditions in a tidal regime. In the northern wells, there are fluctuations of PZE conditions intruded by oxic conditions, caused by fluctuations of the chemocline. Elevated 2α-methylhopanes index and higher renieratane and renierapurpurane/isorenieratane ratio are identified in the microbial mats formed under oxic conditions. In addition, the higher proportion of both okenane against chlorobactane and isorenieratane and C33 n-alkylcyclohexane ratio (n-C33 ACH ratio) are identified in the microbial mat facies. The combination of organic geochemistry (biomarker analysis) and geology (core observation, well analysis and seismic interpretation) show a strong relationship between basin morphology, paleogeography and paleoenvironmental conditions. The southern wells experienced higher influence of fluvial input (oxic conditions). The northern wells, which are far north of the fluvial input show evidence for PZE conditions.

Identification of hydrocarbon generating source rocks and evaluation of their potential are essential in the exploration and development of hydrocarbon resources. For an offshore oil field in Abu Dhabi, we conducted geochemical study using crude oil and core samples from Upper Cretaceous Cenomanian carbonate rocks. The study objectives are 1) correlation of crude oil and source rock with biomarker, and 2) evaluation of the source rock potential. The Cenomanian carbonate rocks of the oil field are composed of shallow marine porous limestone and deep marine lime mudstone. This Cenomanian lime mudstone was believed as source rock of the crude oil in the interfingered Cenomanian porous limestone reservoirs. However, the origin of crude oil has been poorly constrained with geochemistry yet. In this study, we carried out geological description and RockEval pyrolysis analysis of core samples to evaluate source rock potential of the lime mudstone. Then, biomarkers such as hopane, sterane and compound specific isotopic ratio of n-alkane were analyzed to correlate the source rock and the crude oil samples with GC/MS, GC/MS/MS and GC/C/IRMS for high resolution biomarker measurements and robust interpretation. As a result, the biomarker fingerprints of the crude oil in porous limestone and the organic material in the lime mudstone show significant similarity. It proves that the crude oil in the porous limestone is migrated from interfingered organic rich Cenomanian lime mudstone. In addition, the lime mudstone shows excellent source rock property (Total Organic Carbon exceeding 4%, Hydrogen Index > 600mg/g TOC) and categorized as Type I/II source rock deposited in marine environment. Furthermore, the biomarkers effectively constrain the maturity of source rock which is difficult to evaluate with Vitrinite Reflectance and RockEVAL analysis. Consequently, the timing of hydrocarbon generation and the area of effective source rock will be interpreted based on our study result with higher confidence. This study deepens understanding of Cenomanian petroleum system in offshore Abu Dhabi. The result suggests the advantage of biomarker application not only in oil-source correlation but also in source rock maturity analysis.

マツノマダラカミキリの蛹室内温湿度環境を調べるため，人工蛹室を内蔵したマツ材線虫病被害丸太を直射日光の当たる裸地および日陰の林内に設置し，それぞれの場所での人工蛹室内と外気の気温および湿度を測定した。平均気温，最低気温および曇天日の最高気温に丸太の設置場所や人工蛹室内外による違いはみられなかったが，晴天日の日中には裸地の人工蛹室内気温は高温になりやすく，最高39.9oCに達した。人工蛹室内湿度に日周変動はみられず，日数の経過に伴う丸太の乾燥により徐々に低下した。これらの結果をもとに，材内の温湿度環境がマツノマダラカミキリの羽化脱出に及ぼす影響について考察した。

The martensitic transformation in a Ti 50 Ni 34 Cu 16 alloy was studied by in situ X-ray diffraction experiment upon cooling, utilizing synchrotron radiation beams with great intensity. As a result the two-stage B2–B19–B19′ martensitic transformation was directly confirmed, and the lattice parameters of both B19 and B19′ martensites were accurately determined as a function of temperature. Based on the results, the origin of the broad internal friction peak was discussed.

Direct X-ray diffraction experiments with an intense synchrotron radiation beam were carried out, to confirm whether or not the successive B2–B19–B19′ transformation occurs in a solution-treated Ti 50 Ni 30 Cu 20 alloy. Consequently, the second B19–B19′ transformation was found with M s = 192 K. The lattice parameters of both martensites were also determined as a function of temperature.

We report a 37-year-old Japanese woman with hereditary progressive dystonia with marked diurnal fluctuation and dopa-responsive dystonia. She developed dystonia in the lower limbs at the age of 11 years, followed by spasmodic torticollis and resting tremor of the feet, which responded remarkably to low doses of levodopa (100 mg/day). Concentrations of biopterin and neopterin in CSF were decreased. Polymerase chain reaction analysis of the guanosine 5'-triphosphate cyclohydrolase I gene revealed a novel mutation (Thr186--Lys).

We report the case of a 60-year-old man with autopsy-proven dementia with motor neuron disease (D-MND) and Alzheimer's disease lesion. The patient presented with clumsiness of his right hand at the age of 55 years old and subsequently developed dysarthria, weakness and atrophy of his upper limbs. He was unaffectionate towards his family, repeated the same phrase, and showed severe disorientation of time and place. Neurological examination on admission showed not only diffuse lower motor neuron signs, such as weakness, atrophy, fasciculation and areflexia in both upper limbs, but also dementia (HDS-R 9/30). He died of respiratory insufficiency. Neuropathological examination showed mild atrophy of the frontal and temporal lobes and anterior spinal roots. Microscopic examination of cortical sections revealed degenerative changes with simple atrophy and gliosis, and these changes were predominant in layers 1 and 2 of the frontal and temporal cortices. Using immunohistochemical staining, ubiquitin-positive but tau-negative inclusions were frequently found in neurons of the hippocampal granular cell layers and temporal lobes. Many senile plaques and neurofibrillary tangles were present in all sections of the brain. Our final diagnosis was dementia with motor neuron disease accompanying Alzheimer's disease lesion, because of hypoperfusion in the parietal lobe as well as the frontal lobe demonstrated by SPECT, and the presence of many senile plaques and neurofibrillary tangles in the cerebral cortex. Overlapping of pathologically-proven D-MND and Alzheimer's disease lesion is extremely rare, and this case may improve our understanding of the process of neurodegeneration.

We report a 61-year-old man with vitamin E deficiency, presenting with, myopathy as an only clinical symptom. In 1997, at 59 years of age, he noted mild proxymal-muscle weakness and atrophy in the four extremities, nine years after he received a Billroth II partial gastrectomy for a gastric ulcer. His muscle weakness slowly exacerbated, and he was admitted to our hospital in 1999. On admission, neurological examination confirmed mild proximal-muscle weakness and atrophy in the four extremities. Intelligence, cranial nerves, coordination, sensation and tendon reflexes were all normal. Laboratory examination showed normochromic anemia (Hb 9.9 g/dl, Ht 30.9%, MCV 97.5 fl, MCHC 31.2 pg), hypoproteinemia (5.0 g/dl), and hypocholesterolemia (107 mg/dl). The levels of serum CK, lactate and pyruvate were normal. The serum vitamin E level was markedly reduced (0.17 mg/dl; normal 0.75-1.41). Cerebrospinal fluid was normal. Nerve conduction, sensory evoked potentials (SEP), electromyography (EMG), head CT and electroencephalography (EEG) were all normal. Muscle biopsy from the right deltoid muscle showed both mild myogenic and neurogenic changes. Remarkably, type 1 muscle fiber predominance and granular accumulation of autofluorescent lipofuscin granules in the muscle fibers were found. These pathological findings were compatible with those of vitamin E-deficient myopathy. Thus, he was diagnosed as having vitamin E-deficient myopathy, which was confirmed by apparent effective supplementation of vitamin E. Interestingly, our present case did not show any other neurological manifestations such as deep sensory disturbance, sensory ataxia or polyneuropathy. A long-term workload due to hard physical labor and smoking in our patient may have accelerated oxidative muscle damage, resulting in amyotrophy mainly due to vitamin E deficient myopathy.

We report a novel type of hereditary sensory and autonomic neuropathy (HSAN) with adult onset in a Japanese family. One male and 2 females of 6 siblings were affected. They developed anosmia initially at the ages of 20-50 years, followed by anhidrosis and sensory loss. Skin ulceration was absent. Both superficial and deep sensation were impaired in the most distal parts of all 4 limbs. Orthostatic hypotension was present in all patients. This is a unique subtype of HSAN distinct from the HSAN I-V described by Dyck.

PET has been proved to be a powerful tool for exploring the brain function. We discussed the advantages and limitations of PET for analyzing the brain function on the basis of our clinical and experimental experiences of functional imaging. A multimodality PET study measuring cerebral energy metabolism (CMRO2 and CMRglc), cerebral blood flow (CBF), oxygen extraction fraction (OEF) and neurotransmitter function (presynaptic and postsynaptic) opens up a closer insight into a precise pathophysiology of the brain dysfunction: In cerebral infarction, it reveals a state of "misery perfusion" in the acute stage, "luxury perfusion" in the intermediate stage, and proportionately decreased CBF and CMRO2 in the chronic stage. Neurotransmitter function may identify specifically a neuronal subgroup of dysfunction. Owing to the low temporal resolution of PET, a neuronal activity may propagate transsynaptically to remote areas during the period of scanning, resulting in an obscured primary site of the neuronal activity. Uncoupling between neuronal activities and cerebral energy metabolism/CBF may occur under a certain state of brain pathology, particularly after an acute destructive lesion, according to our experimental studies. Neurotransmitter function may reveal the effect of drugs on the brain function, and may be useful for developing a new method of drug therapy for brain diseases in the future.

A 65-year-old woman was operated for gastric adenocarcinoma in 1989. Six years later, peritonitis carcinomatosa, swelling of periaortic lymphnodes and high serum CA-125 were discovered. She received chemotherapy with 5-FU and cisplatin resulting in reduction of ascites. In September, 1998, the swelling of left supraclavicular lymphnodes and the elevation of serum CA-125 reappeared. Pathological diagnosis of supraclavicular lymphnodes was adenocarcinoma. Serum CA-125 was normalized by chemotherapy using cisplatin, farumorubicin and endoxan. However, unsteadiness appeared since December 10, 1998 followed by dysarthria and involuntary movement of neck and upper limbs. These symptoms progressed subacutely. The physical examination on admission revealed swelling of left suraclavicular lymphnodes, nystagmus on lateral gaze, saccadic eye movement on smooth pursuit and severe cerebellar ataxia. In addition, resting tremor of 3-4 Hz was observed at right hand, left wrist and neck which tended to increase amplitude by calculation. Similar movements were seen in the left first toe, though the frequency was lower. Brain MRI revealed mild cerebellar atrophy. She was diagnosed as paraneoplastic cerebellar degeneration (PCD) by serum anti Yo antibody and clinical course. The study of HLA showed positive link to A4 without A24. The primary focus of adenocarcinoma in cervical lymphnodes was suggested to be ovary rather than stomach due to the pattern of immunostaining for cytokeratin, CEA and CA125, although no carcinoma was found in ovarium clinically. The feature of this case is a PCD with resting tremor of frequency of 3-4 Hz and negative link to HLA-A24 in Japanese.

The efficiency of high-dose vitamin C therapy for inclusion body myositis (IBM) was assessed.The subjects were five patients with IBM confirmed pathologically. After the intravenous administration of 40 mg/kg vitamin C five times/week for four weeks, muscle weakness was found to improve in three cases. The average muscle score improved from 8.1 to 8.8, from 7.0 to 8.1 and from 6.2 to 6.8. Magnetic resonance imaging (MRI) demonstrated a reduction in the size of T2 high lesions and gadolinium enhancement in the thigh muscles in one case. Based on our findings, high-dose vitamin C therapy is considered to be effective in some cases of IBM.

We report a 63-year-old man who presented with amoxapine-induced tardive dystonia. At 49 years of age, he developed depression and was administrated 50 mg amoxapine, 4 mg cloxazoram and 3 mg biperiden per day. The daily dose of amoxapine was gradually increased up to 150 mg at 58 years of age. At 61 years of age and after having been taking amoxapine for twelve years, he noticed a rotating left arm and muscle pain in his left shoulder and arm while walking. At 62 years of age, he stopped taking these three drugs. However, the dystonic movements and pain both continued to get worse. Neurological findings revealed no abnormality except for a dystonic posture and movements in the neck and bilateral arms while sitting, standing and walking. Positron emission tomography with C-11 raclopride revealed a mild decrease in the dopamine D 2 receptor numbers in the bilateral striatum. However, two dopamine agonists, pergolide and bromocriptine, worsened his dystonia. In contrast, the daily administration of 2 mg of trihexyphenidyl, an anti-cholinergic agent, markedly ameliorated the dystonia symptoms. As a result, the long-term co-administration of biperiden, an anti-cholinergic agent, may mask the toxicity of amoxapine, which may induce tardive dystonia.

A 63-years-old woman noticed unsteady gait at the age of 56 years and then developed dysarthria two years later. A general physical examination at age 56 revealed mild hypertrophy of both Achilles tendons. On neurological examination, she had scanning speech, moderate limb and truncal ataxia, and moderate hyperreflexia of all limbs. A soft tissue X-ray examination disclosed hypertrophy of both Achilles tendons with multiple punctate calcification. Brain MRI showed diffuse cerebellar atrophy. Motor evoked potentials in the right limb disclosed a prolonged central conduction time. Blood chemistry showed familial type IIa hypercholesterolemia (cholesterol 320 mg/dl, and LDL-cholesterol 245 mg/dl), yet cholestanol level was normal. A examination of CTX gene mutation at hot spots revealed no mutation. Her mother and two siblings also had hypertrophy of Achilles tendons as well as type IIa hypercholesterolemia. In addition, the one sibling showed mild ataxia of lower limbs, respectively. This report suggests a possible link between familial type IIa hypercholesterolemia and cerebellar degeneration syndrome clinically mimicking CTX.

We report a case of a 63-year-old man suffering from anti-Ri-associated paraneoplastic cerebellar degeneration (PCD) with gastric cancer. The neurologic presentation was limited to severe cerebellar ataxia without opsoclonus. The gastric cancer was composed of both poorly differentiated adenocarcinoma and neuro-endocrine carcinoma. The patient's serum reacted with recombinant Ri antigen and the neuroendocrine tumor component. It is thus considered that PCD without opsoclonus in the present case was related to the gastric neuroendocrine tumor and anti-Ri antibody.

A 22-year-old woman noticed an acute onset muscle weakness in her right leg after an asthmatic attack. A neurological examination showed moderate muscle weakness from the right iliopsoas muscle to the toe muscles, a decreased deep tendon reflex in her right lower limb and mild hypesthesia in her right L2-S1 segments. Needle EMG revealed fibrillation potentials in the right gastrocnemius muscle and a positive sharp wave in the right anterior tibialis muscle. The findings of motor nerve conduction studies and sensory nerve conduction studies were normal. The lesion was considered to be located at the posterior horns, as well as at the anterior horns at the L2-S1 levels and also at their roots on the right side. The anterior horn cells appeared to be the most severely affected. Polio-, echo-, entero- and coxsackie-virus antibody titers showed no significant changes on the 36th and 64th days of the disease. The serum IgE level was elevated and mite antigen-specific IgE was strongly positive. MRI revealed no abnormalities in either the thoracic or lumbar spinal cord. Although the sensory disturbance did rapidly improve after corticosteroid therapy, no improvement was seen in her muscle weakness which thus resulted in the atrophy of the affected muscle. While undergoing the corticosteroid therapy, she suffered another asthmatic attack. Nine days after the second attack, she further developed weakness in her right deltoid, biceps brachii, triceps brachii, wrist extensor, wrist flexor, digits extensor and digits flexor muscles with hyperreflexia in her left upper limb. Cervical MRI disclosed a high intensity area at the C3-6 level on the T2-weighted images and also a gadolinium enhancement of the lesion. Since monoplegia had appeared twice previously after bronchial asthma attacks in this case, Hopkins syndrome was suggested. Hopkins syndrome has so far been exclusively reported in children, and no recurrent cases have ever been reported with this condition. This is therefore considered to be the first case of Hopkins syndrome occurring in an adult and also demonstrating recurrence.

We herein report a Japanese case of Poland-Möbius syndrome. The patient was a 19-year-old female. She was the product of a full-term forceps delivery. Birth weight was 2500 g. She had a defect of the right pectoral muscle, and syndactyly of the right hand. When she was 10 days old, facial diplegia, bilateral abducens nerve palsy, and bilateral ptosis were also noted. She was admitted to our hospital at 19 years of age. On physical examination, she had microsyndactyly of the right hand, and her right pectoralis major muscle was absent. Neurological examination revealed bilateral abducens nerve paresis, mild impairement of the upward and adducting movement of both eyes and bilateral facial weakness and atrophy of the left side of her tongue. Her karyotype was normal. Neither R 1 nor R 2 response was evoked in the blink reflex on either side. Brain MRI disclosed thin facial nerves and atrophy of the pons and medulla. Therefore, she was diagnosed as a case of Poland-Möbius syndrome. In this case, the facial nerves were considered to be hypoplastic.

A 49-year-old woman, with a two-year-history of multiple screlosis (MS), noticed postural dizziness, intractable hiccups and vomiting. On admission, she had mild quadriparesis, hypesthesia below the C5 level, and a girdle sensation at the T5 and L1 levels. A CSF examination showed slight increases in the protein level (48 mg/dl) and cell count (7/mm3). Brain MRI demonstrated no obvious lesion in the medulla oblongata. The head-up-tilting test showed a decrease in the blood pressure from 105/63 mmHg to 70/55 mmHg. The pulse rate, however, increased from 57/min to 72/min. The cold pressure test also revealed a mild impairement in her blood pressure response. The R-R interval variation (coefficient of variation: CVRR) during normal breathing was 2.58 (normal:1.66). The valsalva ratio was 1.84 (normal: 1.4-2.0). The Aschner eye-ball pressure test, the blood pressure response to the injection of epinephrine, and the sweating response to the injection of acetylcholine were all normal. She was thus administered domperidone and chlorpromazine. Only domperidone effectively improved the nausea and vomiting. All symptoms, including orthostatic hypotension, hiccups and vomiting, disappeared about one month after admission. The remission of her symptoms was considered to reflect the natural course of MS. The results of autonomic nervous system function tests and her clinical features suggest that an irritable lesion in the medullary tegmentum, including the nucleus tractus solitarii, most likely caused her symptoms. The above findings indicate that autonomic symptoms, such as orthstatic hypotension, hiccups and vomiting, may sometimes be the only symptoms observed in a relapse of MS.

A 34-year-old woman presented with numbness in both lower limbs and weakness of right lower limb twenty six days after a primary varicella infection (chickenpox) associated with fever and rash. Neurological examination revealed numbness of both lower limbs, more severe on the right side, mild paresis of the right lower limb, particularly in the tibialis anterior muscle, and absent ankle jerk on the right. After admission, hyperalgesia appeared at the thoracic 10-12 levels. The T2-weighted MRI of the spinal cord revealed a high signal intensity lesion at the Th 9 level and gadolinium enhancement was seen in that lesion as well as in the bilateral posterior radicles and the left anterior radix at the Th 9 level. On needle electromyography, fasciculation was found in the right tibialis anterior and gastrocnemius muscles. The temporal dispersion of F-wave was seen in the right peroneal nerve. We diagnosed the patient suffered from radiculomyelitis following the primary varicella infection. The secondary immunological mechanism rather than direct viral invasion is most likely in our patient, because (1) neither VZV DNA, nor anti-VZV antibody was positive in the CSF, and (2) the duration was relatively long between the development of skin rash and that of neurological symptoms.

We report a 60-year-old man with myelodysplastic syndrome (MDS) who developed polymyositis and cutaneous vasculitis. He noticed difficulty in climbing up stairs 4 months before admission. On admission, he showed brownish skin pigmentation in the distal positions of the four extremities. Neurological examination revealed muscle weakness of the neck flexor and proximal muscles of four extremities. Serum myogenic enzymes including creatine kinase increased, and electromyography showed denervation potentials and MUPs were myogenic, which was compatible with polymyositis. Muscle biopsy indicated perivascular mononuclear cell infiltration and muscle fiber necrosis with cytoplasmic bodies and rimmed vacuoles in the muscle fibers. Complete blood cell counts revealed macrocytic normochromic anemia and bone marrow puncture disclosed marked hyperplasia of megakaryocytes, erythroblasts and myeloblasts, suggesting a refractory anemia type of MDS. In addition, skin biopsy showed chronic cutaneous vasculitis. Serum protein electrophoresis and immunoelectrophoresis revealed the presence of IgM gamma type monoclonal gammopathy. With immunosuppressive therapy, serum creatine kinase level was decreased. Since immunological abnormalities and the presence of autoimmune diseases have been reported in patients with MDS, both polymyositis and chronic cutaneous vasculitis in the present patient may be caused by the similar autoimmune mechanisms related to MDS.

We report a 63-year-old man who died of respiratory failure. He was well until 1992 (57 years of his age), when he had an onset of progressive weakness of the bilateral upper limbs. He showed no improvement with TRH administration in other hospital. On January 12, 1994, he admitted to our department because of the progressive muscle weakness. Neurologic examination revealed a muscular atrophy associated with severe weakness and hyporeflexia in both upper limbs, and fasciculation were seen in his tongue. Electrophysiological studies revealed mild conduction block in the left medial nerve, and F-waves were not evoked in the left ulnar nerve and bilateral median nerves. After an administration of 25 g/day of human gamma-immunoglobulin for 5 days, conduction block as well as F-wave abnormalities in the left median and left ulnar nerve were improved, yet no improvement of muscle weakness was seen. The anti-GM1 IgG titer was transiently elevated in the patient's serum after gamma-immunoglobulin therapy. On September 8, 1994, subtotal gastrectomy was performed because of the early stage gastric cancer. Histological examination showed poorly differentiated adenocarcinoma (signet-ring cell carcinoma). His muscle weakness had been gradually extended to the lower limbs and he couldn't walk himself on January, 1998. On March, 1998, he developed tetraplegia, mild dysphagia, dysuria and the respiratory disturbance. On April 12, 1998, he admitted to our department for the second time. Neurologic examination revealed a muscular atrophy and fasciculation associated with severe weakness in all of his limbs, tongue and musclus masseter. Neither deep tendon reflex nor pathologic reflex was evoked in his upper and lower extremities. His ocular movements and sensations were well preserved. He died of respiratory failure on May 1, 1998. The patient was presented in a neurological CPC. Neurological and laboratory findings suggested a spinal progressive muscular atrophy (SPMA). However, there were several unusual points as a typical SPMA in this case, that is, an improvement of the electrophysiological abnormalities by gamma-globulin treatment, as well as transient elevation of anti-GM1 antibody. The clinical neurologists have arrived at the conclusion that the patient had lower motor neuron syndrome associated with anti-ganglioside antibody and cause of death was ascribed to the respiratory failure. We discussed whether this case was SPMA or multifocal motor neuropathy. Postmortem examination revealed numerous diverticulums in the ascending colon and lymphothyroiditis. No recurrent carcinoma was detected. Neuropathologically, both severe atrophy of the anterior spinal roots, and severe gliosis and neuronal loss in the anterior horn of the spinal cord were observed. Onuf nuclei were not affected. Neurogenic muscular atrophy was detected in the tongue, diaphragm, and limb muscles. Motor neurons of the brainstem were relatively preserved, but skein-like inclusions as detected by anti-ubiquitin antibody, were present in the facial and hypoglossal nuclei. Neither motor cortex nor cortico-spinal tracts were affected. Demyelination, remyelination or cellular infiltrations were not apparent in the right median nerve and sciatic nerves. The neuropathologic features were compatible with SPMA.

The nigrostriatal dopaminergic function and regional glucose metabolism were evaluated in patients suffering from various disorders of basal ganglia by using positron emission tomography with 18F-dopa and 18F-FDG, respectively. The 18F-dopa uptake in the striatum (the caudate head and the putamen) decreased in patients with Parkinson's disease but was relatively unaffected in the caudate. The cerebral glucose metabolism was normal in patients with Parkinson's disease. The 18F-dopa uptake in the striatum also decreased in cases of multiple system atrophy and progressive supranuclear palsy, but there was no difference in the uptake between the caudate and the putamen. The glucose metabolism decreased in the cerebral cortices and the striatum: this finding was also different from those of Parkinson's disease. A normal 18F-dopa uptake with a markedly decreased striatal glucose metabolism was observed in cases of Huntington's disease. The 18F-dopa uptake increased and the glucose metabolism was normal in cases of idiopathic dystonia. Various patterns of 18F-dopa uptake and glucose metabolism were thus observed in the various disorders of basal ganglia. These results suggest that the measurements of the 18F-dopa uptake and glucose metabolism would be useful for evaluating the function of the basal ganglia in various disorders of basal ganglia.

Here we report a case of acute cerebellitis, in which the patient developed right peripheral facial palsy during the recovery phase of cerebellar ataxia. A 67-year-old man developed truncal and limb ataxia following a fever, general fatigue and anorexia. He was diagnosed to have acute cerebellitis. While the ataxia symptoms were improving without any treatment, right peripheral facial nerve palsy developed and an MRI revealed an enhancement of the right facial nerve proximal to the geniculate ganglion. After treatment with acyclovir and corticosteroids, his facial nerve palsy and ataxia both gradually improved. There has been no previous report of an adult case who developed peripheral facial nerve palsy during the recovery phase of acute cerebellitis. This case indicates that a wide spectrum of neurological complications may develop in association with a varicella-zoster virus infection.

A 19-year-old man developed progressive unsteady gait and speech disturbance two years ago. He recently noticed muscle twitches in the left limb. Neurological examination revealed scanning speech, saccadic ocular movement, ataxia of trunk and limbs predominant on the left side. Rhythmic myoclonus was present at rest around the left shoulder and arm, and amplified by raising the left arm. There was no myoclonus in the soft palate. MRI revealed only a cerebellar atrophy. This patient was diagnosed as having cortical cerebellar atrophy with rhythmic skeletal myoclonus (RSM). Tc-99m ECD SPECT showed a decrease in the blood flow of the right thalamus when the myoclonus was enhanced by raising the left upper limb, which suggests that the cerebello-thalamo-cortical system as well as Guillain-McIlaret triangle is involved in the development of RSM.

A 55-year-old woman, who had two episodes of difficulty in putting a key into a keyhole probably due to optic ataxia at age 52 and 54 years old, developed speaking errors and was admitted to our hospital. She was 152.5 cm in height and 52.5 kg in weight. Neurological examination revealed right homonymous hemianopsia and sensory aphasia. A CSF examination revealed lymphocytic pleocytosis of 88/microliter. Serum lactate and pyruvate were remarkably increased after an aerobic exercise test. A few ragged-red fibers were present in the biopsied brachial biceps muscle. Brain MRI by FLAIR method showed scattered high signal lesions in the left temporal lobe, bilateral parieto-occipital lobes, left insular cortex and left thalamus. The left superficial temporal lesion was enhanced by gadolinium-DTPA. The proton MRS demonstrated the lactic acid peak as well as the decrease of NAA/choline ratio (0.38) in the left parieto-occipital region. Thus, she was diagnosed as a case of MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes) and successfully treated with ubidecarenone (150 mg/day). Six months later, she again developed seizure, right hemiparesis and deterioration of aphasia and presented again CSF lymphocytic pleocytoses of 15/microliter. Brain MRI demonstrated new lesions in the left temporoparietal lobes, left insular cortex and left corona radiata. Therefore, CSF pleocytosis appeared to be associated with stroke-like episodes in this case. Although the mechanism of CSF pleocytosis remains to be elucidated, it may involve the breakdown of blood-brain barrier caused by mitochondrial dysfunction. Otherwise, an inflammatory process similar to that in cases of Leber disease, who developed multiple sclerosis-like additional lesions in the central nervous system, may also take place in MELAS.

We have shown previously that pigment epithelium-derived factor (PEDF) acts as a survival factor for cerebellar granule cell neurons in culture, as well as protecting them against glutamate toxicity. In this study we have examined effects of PEDF on apoptotic cell death. We find that the granule cells die of apoptosis throughout the culture period, what we have termed "natural" apoptosis. PEDF prevents this natural apoptosis if added to immature cells, within the first 2 days in vitro (DIV), and the effect is maintained for up to DIV12. However, PEDF has no effect if added to mature cells at DIV5. Similar results are obtained when apoptosis is induced by shifting the cells from a serum- and 25 mM KCl-containing medium to serum-free medium with 5 mM KCl. PEDF most effectively blocks induced apoptosis in immature cells (DIV2) when added 24 hr prior to the change of medium, but still provides some protection when added simultaneously. However, 24 hr pretreatment with PEDF has a minimal effect when apoptosis is induced in mature DIV6 cells; addition at the same time is completely ineffective. Two polypeptide fragments of PEDF, only one of which contains the serine-protease inhibitory site, are equally active, supporting previous results which suggest that the neurotrophic effects of PEDF are not mediated by protease inhibition. We conclude that PEDF protects immature but not mature granule cells against both natural and induced apoptosis.

A specific subset of neuropeptides are expressed in astrocytes, with specificity in terms of both brain region and gene, as well as with specific developmental patterns: the overall pattern of expression is thus heterogeneous. These neuropeptides have been demonstrated to have a number of trophic effects, including regulation of cell number, of length of axons and dendrites, and of neuronal phenotype. The developmental regulation of expression of neuropeptides in astrocytes further supports functional roles for the neuropeptides as trophic modeling factors. Not all the trophic effects have to be stimulatory and evidence suggests that opioid peptides may function to prune dendrites or decrease cell numbers in order to modulate or shape the nervous system. In addition to roles during development, preliminary experiments suggest that astrocyte neuropeptide expression turns back on in the adult brain following injury, suggesting that the neuropeptides may play a role in supporting survival or recovery of damaged neurons. The heterogeneity of neuropeptide expression in astrocytes must therefore contribute to the establishment and maintenance of the network of communication among neurons and glia in the nervous system in ways we have yet to understand fully.

PAOSCLE consists of 5 % phenol in almond oil, effect of which on hemorrhoids and other anal diseases was clinically studied. The effectiveness of the agent was judged on the basis of the calculating table devised by, Sakabe and others.The agent was remarkably effective for bleeding from hemorrhoids. The rate of effectiveness on the first trial was 97.0 %, 66.0 % and 66.7 % for the first, the second and the third grade hemorrhoids respectively. The rate of effectiveness on the second trial improved up to 100.0 %, 88.7 % and 93.3 % respectively. The data show bleeding from hemorrhoids is suspended by the agent in almost every case of hemorrhoids. The result was especially good when the amount of injection was 5 cc or more at one trial.The effect was also noticed as for the mild prolapse of hemorrhoids, although in that case repeated injection was required. The bleeding from prolapsed mucosa in the cases of so-called " Whitehead-anus " also favorably responded to the injection. However, no effect was noticed in rectal prolapse.Any influence on liver functions or hematologies were not observed. The side-effect was nil except only a case of temporary malaise. Sclerotherapy with such a low-density agent as PAOSCLE is quite free from such harmful results as induced by conventional necrotherapy.Sclerotherapy with PAOSCLE is recommended as a measure of choice before the trial of operation.

A 17-year old woman noted myalgia after taking a long distance walk at the age of 10. In adolescence, she had several episodes of myalgia and pigmenturia after athletic activity or infection. At age 17, myoglobinuria and abnormally increased serum creatine kinase were documented after one of these episodes. The neurological examination revealed mild proximal muscle weakness of upper extremities. Electromyography showed myogenic patterns, such as brief, small abundant potentials on them. Venous lactate was raised normally on the ischemic exercise test. During prolonged fasting, plasma ketone bodies increased normally but there were abnormal elevations of plasma creatine kinase and myoglobin. Morphometric analysis of electron microscopy in muscle showed few lipid deposits and that of light microscopy revealed no abnormality. CPT activity in muscle was only 15% of normal value by the isotope-exchange assay. These results were consistent with the diagnosis of CPT deficiency. Although several cases of CPT deficiency with recurrent myoglobinuria have been reported in Western countries, our patients is the first case of Japanese showing recurrent myoglobinuria. CPT deficiency should be considered as a differential diagnosis in cases of recurrent myoglobinuria.