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A 72-year-old man with ascending colon cancer was admitted to our hospital. Right hemicolectomy and lymph node dissection(D3)were performed. The pathological diagnosis was signet-ring cell carcinoma, T4a(SE), N2b, M1a(LYM), Stage Ⅳ, R0, Cur B. Capecitabine was administered after surgery. Subcutaneous bleeding, thrombocytopenia, and a rapid increase in tumor marker levels occurred 9 months after surgery. He had already developed disseminated intravascular coagulation and was admitted to our hospital immediately. CT scan revealed metastasis in the thoracic vertebrae. Bone scintigraphy demonstrated multiple abnormal areas of uptake in the costal bones and the thoracic and lumber vertebrae. We made a final diagnosis of disseminated carcinomatosis of the bone marrow by histopathological examination. Unfortunately, before starting chemotherapy, his general condition deteriorated, and he died 14 days after hospitalization. We present here a case of colon cancer with disseminated carcinomatosis of the bone marrow.

Bisphosphonates (BPs) are chemically characterized by a P-C-P bond with two lateral side chains on the carbon atom, and have been widely used as anti-resorptive agents in various metabolic bone diseases. 4-[(methylthio) phenylthio] methanebisphosphonate (MPMBP) is a novel non-nitrogen-containing BP with an antioxidant side chain that possesses anti-inflammatory properties. Since inflammation is known to be a cause of the pathological bone resorption, we investigated the effects of MPMBP on bone metabolism both in vitro and in vivo. The results showed that: i) MPMBP dose-dependently increased alkaline-phosphatase activity in a culture of osteoblastic MC3T3-E1 cells, ii) MPMBP increased the synthesis of collagen (type-I) in an organ culture of mouse calvaria, iii) local injection of MPMBP to alveolar bone induced prominent increases in both the bone mass and thickness of alveolar bone at the local site of injection in rabbits, iv) MPMBP increased the mRNA expression of alkaline-phosphatase, type-I collagen, osteocalcin, and bone sialoprotein in MC3T3-E1 cells, v) MPMBP inhibited the translocation of NF-κB/p65 to the nuclei in osteoblasts of cultured mouse calvaria. Taken together, these findings suggest that MPMBP is a promising agent to prevent bone loss, or even accelerate new bone formation, through inducing an uncoupling between bone resorption and bone formation, which is preferable to maintain bone mass and quality.

The safety and feasibility of the chemotherapy for super-elderly patients over 85 years old have not been clarified yet. We report an extremely aged patient with recurrent rectal cancer that was successfully treated with chemotherapy. A 85-year-old woman underwent Hartmann procedure for rectal cancer. Nine months after surgery, CT scan revealed liver metastases in S5 and S7. We administered capecitabine plus bevacizumab chemotherapy. Liver metastases were disappeared after 6 courses. Although grade 2 hypertension was appeared, no other adverse event occurred. However, due to lung metastases, we attempted irinotecan plus bevacizumab as second line treatment. After 10 courses, general fatigue was gradually developed, so we changed the frequency of chemotherapy from biweekly to triweekly administration. The patient's performance status score has been kept 0, and she has been under treatment as an outpatient for 3 years. The chemotherapy for extremely aged patients with recurrent colorectal cancer was suggested to be safe and feasible under the adequate dose reduction and interval adjustment.

Curative treatment for unresectable colon cancer is difficult, and therefore, chemotherapy is often administered in an attempt to improve the prognosis. However, the safety andfeasibility of chemotherapy for elderly patients over 80-years-old have not yet been clarified. We report an elderly colon cancer patient with multiple liver metastases who was successfully treatedwith mFOLFOX6 andsLV5 FU2 chemotherapy. The patient was an 83-year-old-man who was referredto our hospital. After performing sigmoidectomy, we administered mFOLFOX6 chemotherapy. After 5 courses, the regimen was changed to sLV5FU2 owing to grade 3 neuropathy. Liver metastases disappearedanda complete response was obtained1 year after chemotherapy administration. Twenty-four courses of sLV5FU2 chemotherapy had been safely performed. Although grade 1 neutropenia developed, no other adverse event was observed. Currently, the patient is alive without recurrence. Chemotherapy for elderly patients is both feasible and safe.

A36 -year-old man with intellectual disabilities consulted a local physician complaining of a cough, and an abdominal mass was observed on palpation. The patient visited our hospital for close examination. Abdominal contrasting CT revealed a mass with a clear boundary with heterogeneous contrast on the left side of his abdominal cavity. We performed a laparotomy and observed that the tumor originated from the greater omentum. The tumor size was 9×8×6 cm and its weight was 200 g. Histopathologic examination showed hyperplastic spindle-shaped tumor cells with less nuclear fission. Immunohistochemical staining showed that the tumor was positive for CD34, CD99, and bcl-2, slightly positive for p53, and negative for S-100, a- SMA, c-kit, and desmin. Based on the results, a diagnosis of solitary fibrous tumor (SFT) was made. The patient has not shown any recurrence 8 months after surgery.

A 75-year-oldman presenting with obstructive jaundice was referredto our hospital. Basedon a diagnosis of carcinoma of the ampulla of Vater, we performed pancreatoduodenectomy. Postoperative histopathological examination revealed a welldifferentiated papillotubular adenocarcinoma, T3, N0, M0, Stage III . Six months after surgery, an isolatedliver metastasis in S6 was identifiedon CT scan andMRI; therefore, we administeredgemcitabine plus cisplatin chemotherapy. After 6 courses of this regimen, a clinical complete response(CR)was obtained. After 12 courses, the clinical CR continued; however, grade 3 lower-extremity peripheral neuropathy appeared. Therefore, gemcitabine monotherapy was administered as second line chemotherapy. However, multiple liver metastases appearedandthe patient passedaway owing to exacerbation of the disease 2 years after initiating chemotherapy. Although recurrent ampullary carcinoma is difficult to treat, our patient had a long-term survival. Here we report the details of our case and review the relevant literature.

The safety and feasibility of chemotherapy for super-elderly patients (over 85 years old) has not been clarified yet. We report an extremely aged patient with recurrent rectal cancer that was successfully treated with capecitabine plus bevacizumab chemotherapy. An 85-year-old-woman underwent a Hartmann procedure for rectal cancer. Nine months after surgery, tumor markers were elevated. CT and MRI revealed liver metastases in S5 and S7. We administered capecitabine plus bevacizumab chemotherapy. Tumor makers were normalized after 2 courses, and the liver metastases disappeared after 6 courses. Although Grade 1 hypertension developed, no other adverse event occurred. Chemotherapy has been safely performed for 20 courses. The patient's PS score has been maintained at 0, and she has been under treatment as an outpatient. We suggest that capecitabine plus bevacizumab chemotherapy is an effective regimen for extremely aged patients with recurrent colorectal cancer.

A 67-year-old man had a gastric polyp diagnosed on screening. Atrophic changes in the upper gastric mucosa were seen on upper gastrointestinal endoscopy. In addition, endoscopy revealed in the middle area of the stomach wall a 10 mm polyp that was diagnosed as a carcinoid tumor through biopsy. Blood serum gastrin was elevated at 2,800 pg/mL.We diagnosed a Rindi Type 1 gastric carcinoid. The patient was planned to be treated with surgical laparoscopy assisted distal gastrectomy (LADG); however, the procedure was changed to intraoperative laparoscopy assisted total gastrectomy (LATG). Chromogranin-positive tumor pathological findings in the mucous membrane submucosa and in the muscularis mucosae endocrine cell micronest (ECM) were widespread. There was no obvious vascular invasion. After the surgery, the serum gastrin level normalized and the patient remains alive.

A 78 -year-old man with rectal cancer underwent abdominoperineal resection of the rectum. In the postoperative period, the patient experienced wound infection, leading to an abdominal wall hernia. Two years following surgery, a rise in the serum CEA level was seen. A metastatic tumor was detected in the right lung on chest CT. VATS right lung inferior lobe segmental resection was performed. After lobectomy, the serum CEA level continued to increase. Another metastatic tumor was detected in the right lung on chest CT. Chemotherapy with capecitabine, oxaliplatin, and bevacizumab was commenced. The erosive part of the abdominal wall scar hernia extended during the nine weeks of chemotherapy. The chemotherapy was then discontinued. In the follow-up CT scan, a right pleural recurrence, local recurrence in the pelvis, and a liver metastasis were detected. Chemotherapy was re-introduced 3 years after surgery. The erosive part of the abdominal wall hernia again began to spread with chemotherapy recommencement. Four months after restarting chemotherapy, the hernia ruptured, with a loop of the small intestine protruding out of it. The patient covered this with a sheet of vinyl and was taken by the ambulance to our hospital. The erosive part of the abdominal wall hernia had split by 10 cm, and a loop of the small intestine was protruding. As ischemia of the small intestine was not observed, we replaced it into the abdominal cavity, and performed a temporary suture repair of the hernia sac. Following this, bevacizumab was discontinued, and the erosive part reduced. We performed a radical operation for abdominal wall scar hernia repair 11 weeks after the discontinuation of bevacizumab.

The advent of a super aging society is causing a rapid increase in the number of patients with dysphagia, and, in response, the use of percutaneous endoscopic gastrostomy (PEG) has become markedly widespread over the past decade. As the result of its rapid spread, PEG is controversial both ethically and economically, and, in the revision of medical treatment fees for 2014, the preoperative deglutition usability test of all cases is fixed for the full amount request requirements of gastrostomy. Thus, the demands on dysphagia practice are rising. Therefore, we conducted a survey of the dysphagia practice of otolaryngologists, doctors, and speech therapists in charge of dysphagia practice in hospitals, home medical care clinics, and dental offices in Itabashi-ku, Tokyo, and we considered the role of otolaryngologists in dysphagia practice. According to the survey, the roles that are expected of otolaryngologists in dysphagia practice are the evaluation of swallowing function using videoendoscopic examination of swallowing in over 50% of home medical care clinics and dental offices. On the other hand, surgical treatment is expected of otolaryngologists in over half in the hospital group. A total of 64% of the home care clinic group and 47% of the dental group did not coordinate with otolaryngologists in dysphagia practice because there are no otolaryngologists to consult. The home care clinic group indicated a demand of the swallowing function test in short-term admission or home practice. In the survey of departments of otolaryngology in hospitals or otolaryngology clinics, 40% of these institutions evaluate deglutition, while the other institutions did not perform evaluations because of the lack of human resources or deficient facilities. The otolaryngologist specializes in the laryngopharynx where aspiration occurs, and not only diagnoses local organic disease, but also directs the patient to a doctor in an appropriate department on the basis of understanding the patient's background diseases. We conclude that organized participation and leadership in this region are urgent tasks of otolaryngologist.

A 64 -year-old woman was referred to our hospital with a diagnosis of advanced rectal cancer with metastases to the left supraclavicular lymph nodes and paraaortic lymph nodes. Alow anterior resection was performed because of the symptoms of ileus. Subsequently, chemotherapy consisting of XELOX with bevacizumab was initiated as the first-line regimen, over 6 courses. Asecond -line regimen of FOLFIRI with bevacizumab was selected due to multiple lung metastases and the progression to both left supraclavicular and paraaortic lymph nodes. During the first 3 courses, the patient had no harmful side effects. Although the patient received adequate prophylactic antiemetic therapy and supportive treatment, grade 4 delayed emesis induced by irinotecan (CPT-11) occurred at 7 days after the fourth course of FOLFIRI chemotherapy. The patient was given total parenteral nutrition, after which she recovered substantially from the emesis. Delayed emesis is occasionally seen with irinotecan therapy and can be efficiently managed with adequate prophylactic antiemetic therapy. However, delayed emesis occurring one week after administration is rarely observed. Delayed emesis and subsequent therapy affect the quality of life (QOL) of the patient and subsequent therapy therefore, adequate attention and prompt management are required for delayed emesis.

We investigated the clinicopathological findings of 13 patients with perforated colorectal cancer. In 6 patients, the primary region affected by the cancer was the sigmoid or rectosigmoid colon, and 9 out of 13 patients had perforations at the location of the tumor itself. The Hartmann operation was performed in 5 patients, and D2 or D3 lymph node dissection was performed in 6 patients. The final stages of the 13 patients were as follows: 1 patient with stage II cancer, 5 patients with stage III cancer, and 7 patients with stage IV cancer. Postoperative death occurred in 1 patient. Five out of 7 patients with curative operations had recurrences; 2 patients had peritoneal disseminations, 2 patients had lung metastases, and 1 patient had paraaortic lymph node metastases. Even if patients underwent a curative operation, a high frequency of recurrence, especially of peritoneal dissemination, was observed. Therefore, we conclude that a careful follow-up is required.

A 61-year-old man was referred to our hospital because of gastric pain and weight loss.Upper gastrointestinal endoscopy revealed a superficial depressed (Type 3) tumor with pyloric stenosis.The tumor was diagnosed as tubular adenocarcinoma by pathological examination.Abdominal computed tomography showed enlarged paraaortic and No. 8a lymph nodes.The patient underwent distal gastrectomy (D0)and Roux-en-Y reconstruction.After surgery, chemotherapy combined with molecular targeted therapy (S-1+cisplatin[CDDP]+trastuzumab), based on overexpression of the HER2 protein in the primary tumor as assessed by immunostaining, was administered.After the molecular targeted chemotherapy, the carcinoembryonic antigen (CEA )levels decreased to the normal range and the enlarged lymph nodes were remarkably decreased in size. The patient is currently alive without progressive disease.

Dropped head syndrome is seen in various diseases. We investigated its pathophysiological mechanisms with physical and radiological examination, surface EMG and responses to physiotherapy. Subjects had dropped head as a complaint, but their primary diagnoses were various. We investigated 16 cases: 5 cases of Parkinson disease, 5 cases of multiple system atrophy predominant parkinsonism, 3 cases of cervical spondylosis and 3 cases with other diagnoses. We found that patients had common findings such as bulging of cervical muscles, and tonic EMG activities mainly in the extensors in the sitting and standing position, but in the flexors of the neck only in the supine position. Of the 16 cases, 14 were treated with physiotherapy to improve the alignment of the pelvis and whole vertebral column; 6 of the 14 cases (63%) showed remarkable improvement. We conclude that the primary reason of dropped head syndrome is unknown in Parkinson disease and cervical spondylosis, but also that many of the patients have secondary changes in alignment of the skeletomuscular system which could be treated with physiotherapy.

This is a review of non-clinical and clinical study results of rifabutin (Mycobutin, RBT) which was approved as a new anti-mycobacterial agent 38 years after rifampicin (RFP) was approved in Japan. The anti-bacterial actions of RBT were similar to those of RFP, but its potency was stronger (4 to 32 times in MIC90). RBT showed excellent penetration in cells (9 times in neutrophil, 15 times in monocyte, against plasma concentration) and in tissues (5 to 10 times in pulmonary tissue). Clinical efficacy of RBT (150 mg, as well as 300 mg daily) was comparable to that of RFP 600 mg daily, in the treatment of newly diagnosed tuberculosis, drug-resistant tuberculosis, and the NTM diseases. In addition, RBT 300 mg showed significant prophylactic effect on the development of disseminated MAC infection in HIV positive subjects. Most of the adverse events of RBT were the same as those of RFP, including drug-drug interactions related to the induction of CYP3A4. The concomitant use of RBT (over 450 mg) with clarithromycin induces uveitis, which warrants special attention. It is expected that the efficacy and safety of RBT in Japanese subjects will be evaluated in Japan through the accumulation of clinical experience.

The prevalence of depression has been reported to be higher in cancer patients, especially those of advanced stage, compared to normal controls. However, depression is often under-recognized in clinical oncology settings. And this psychological problem is not routinely assessed even in patients with inoperable metastatic cancer who often have psychological disorders. Psychological distress including depression, is affected by physical, psychosocial, and clinical factors. In order to detect psychiatric problems at the early stage, we assessed the mental conditions of 47 inpatients with metastatic gastric and colorectal cancerusing the Japanese version of Zung's Self Rating Depression Scale(SDS)and analyzed the relationships between these factors and SDS scores. While SDS scores of our patients did not differ according to their gender, age, performance status (PS), ortypes of patients' character, they were significantly higher in Group B(cancer patients with palliative care alone), compared to Group A(those receiving chemotherapy)(p<0. 001). As the disease in the four identical patients progressed to the terminal stage, their scores were significantly increased, respectively(p<0. 05). These results suggest that psychological intervention should be more critical for terminally ill patients without any indication of chemotherapy.

Considering the various studies conducted on spasticity, it is quite evident that the signs and symptoms of this disorder vary. In some cases of spasticity, spasms may be noted. An increase in velocity-dependent stretch reflexes has been proposed as a cardinal feature of spasticity. There are 2 possible mechanisms underlying increased stretch reflex of spasticity: one is the changes in gamma innervations and the other is one the changes in spinal motoneurons. Changes in gamma innervations for spasticity are not evident while the muscle are at rest. On the other hand, changes are clear in normal subjects during voluntary contraction of muscles, but not in sbjects with spasticity. We have discussed the changes in membrane properties caused by presynaptic inhibition, reciprocal Ia inhibitory function, recurrent inhibition, and autogenic Ib inhibition as the other candidate spinal mechanisms for spasticity.

It has been reported that high intensity on diffusion-weighted image (DWI) in magnetic reasonance imaging (MRI) accompanying a reduction of the apparent diffusion coefficient (ADC) can be detected at the ictal or postictal stage of epileptic seizure. However, it remains unclear whether such a change results from persisting systemic convulsive seizure or from certain physiological changes such as recurrent epileptic discharge prior to the occurrence of obvious convulsion. We report here a case of symptomatic epilepsy displaying a high intensity area on DWI in MRI before convulsive seizure was initiated. A 64-year-old man was admitted to our hospital due to complaints of dizziness and motor weakness. CT and conventional MRI scans failed to reveal any new lesions except for the scar of a ventricular tube in the right parietal lobe, which had been removed due to shunt infection. The DWI, however, demonstrated an apparent high intensity in the right parietal cortex, and the ADC was significantly reduced as compared to that on the contralateral side. Five days after admission, the patient showed convulsive seizures beginning from the right face and upper extremity which subsequently developed to status epilepticus. Following recovery from the convulsions with administration of anticonvulsants, the high intensity of the right parietal lobe on DWI appeared to be diminished. The present case indicates that the manifestation of a high intensity on DWI concomitant with ADC reduction at the epileptic focus can be readily induced by the occurrence of epileptic discharges before convulsive seizure is evident.

A new test method for boric acid in agar was developed. After digestion with nitric acid, the concentration of boron was measured by ICP-AES or ICP-MS with internal standards. Collaborative studies involving 5 laboratories were conducted to evaluate the new method by using a finely powdered agar sample and a standard sample (NIST SRM SRM1570a). The repeatability and reproducibility were very good. Moreover, HORRAT(R) values were less than 2. Therefore, it was considered that the new method is acceptable for the determination of boric acid in agar.

Objectives: Incomplete stent apposition (ISA) is frequently observed after sirolimus-eluting stent (SES) implantation. This study investigated the incidence, morphological features, and possible mechanisms of this phenomenon., Methods: Fifty-two lesions in 47 eligible patients were treated with SES and serial intravascular ultrasound (IVUS) assessment at the time of post-intervention and 8-month follow-up. ISA was carefully identified from the IVUS images of these lesions. Specifically, quantitative two dimensional IVUS analysis was performed if the lesions demonstrated ISA, including routine IVUS parameters as well as other measurements related to ISA., Results: Overall, ISA was observed in 13 lesions (25.0%) at follow-up. Persistent ISA (n = 6, 11.5%), defined as ISA consistently observed both at post-intervention and follow-up, and late-acquired ISA (n = 7, 13.5%)were systematically compared. Eighty-three percent of cases of persistent ISA were located around the stent edges, whereas all cases of late-acquired ISA were in the stent body. In the persistent ISA group, no serial changes were observed in the lumen area or external elastic membrane area (EEMA) from post-intervention to follow-up. However, in the late-acquired ISA group, EEMA and lumen area significantly increased from post-intervention to follow-up (EEMA: 13.4 +/- 3.2 vs 17.6 +/- 3.3 mm2, respectively, p < 0.0001 ; lumen area: 6.7 +/- 1.4 vs 9.2 +/- 1.8 mm2, respectively, p = 0.004). No adverse clinical events were observed in either group., Conclusions: ISA was frequently observed during and after SES implantation in clinical practice. No clinical disadvantages were observed during 16 month clinical follow-up periods. Positive remodeling may potentially cause late-acquired ISA.

Oxaliplatin (L-OHP) has been established as a key drug for advanced colorectal cancer, and combination therapy with 5-FU/Leucovorin (LV)(FOLFOX regimen) is commonly used in Europe and the US. A phase I study of modified (m) FOLFOX 6 therapy was conducted to determine the recommended dose (RD) of 5-FU infused for 46 hours. Inclusion criteria were unresectable advanced colorectal cancer,measurable lesions, performance status (PS; ECOG) 0-2, age 20-75 years, and adequate organ functions. L-OHP and l-LV was administered over 2 hours and followed by bolus injection and continuous infusion of 5-FU for 46 hours every 2 weeks. Two cycles of mFOLFOX 6 therapy were performed. Doses of L-OHP, l-LV, and bolus injection of 5-FU were fixed at 85 mg/m(2), 200 mg/m(2), and 400 mg/m(2), respectively. The dose of continuous infused 5-FU was escalated from 1,600 mg/m(2), (level 1), 2,000 mg/m(2), (level 2), 2,400 mg/m(2), (level 3), and 2,800 mg/m(2), (level 4). RD was determined in a dose escalation manner, and safety was evaluated according to NCI-CTC Ver 2.0. A total of 13 patients were enrolled. Male/female=7/6, PS 0/1/2=2/4/7, mean age 64 years (range 55-75). Thrombocytopenia was not observed, and grade 2 of neutropenia and peripheral neuropathy was observed in 4 and 6 out of 13 patients. No dose-limiting toxicity (DLT) was observed at level 1 (n=3), 2 (n=4), and 3 (n=4), but at level 4 (n=2), 2 patients experienced DLT; grade 3 easy fatigue and anorexia required treatment delay over 7 days. Level 3 was therefore determined as RD. A phase II study is ongoing to evaluate the efficacy of mFOLFOX 6 therapy.

Two cases of bilateral multiple primary lung cancers are presented. One is synchronous, and another is metachronous. Case 1: An abnormal shadow in the left lower lobe was found on the chest radiograph of 40-year-old female during a regular check-up. Chest computed tomography (CT) film showed a micronodule in the right S2. Left lower lobectomy was performed, followed by second-staged right S2 segmentectomy 1 month after the initial operation. Case 2: A 62-year-old female underwent left lower lobectomy for lung cancer in April 1997. Follow-up CT showed a nodule in right S8. In March 2000, right basal segmentectomy was performed for second lung cancer. Moreover, follow-up CT showed a nodule in right S3. Right wedge resection was performed for third lung cancer in July 2002. It is important to carefully examine a synchronous lesion before the operation of a primary lung cancer and to perform close follow-up surveillance for early detection of a metachronous lesion.

Unlabelled: The PDQ-39 (Parkinson's Disease Questionnaire-39) is a specific quality-of-life (QOL) scale for patients with Parkinson's disease (PD). It evaluates the dysfunction of daily living and quality of life. We developed the Japanese-translated version of PDQ-39 and examined the validity of the PDQ-39 in Japanese PD patients., Subjects: A total of 156 patients with PD were studied (age 35-86 years, Hoehn-Yahr stage 1 to 4). None of the patients had dementia., Methods: Semi-structured interviews were conducted by one trained interviewer. The PDQ-39, SF-36 (36-item short form of the Medical Outcome Study questionnaire), EQ5D (Euro Qol), and NAS-J (Nottingham Adjustment Scale Japanese version) were used for evaluation. The attending physician of each patient rated the patient's condition by using the UPDRS (Unified Parkinson's Disease Rating Scale)., Results: Exploratory factor analysis, Cronbach alpha, and construct validity were fair, and there were significant correlations between the results of the PDQ-39 and the corresponding parts of the UPDRS and SF-36, and the Hoehn-Yahr stage, respectively., Conclusion: The Japanese version of the PDQ-39 is valuable for evaluating the QOL of Japanese patients with PD. Results of comparison of PDQ-39 with other scales of the QOL such as NAS-J suggest the possibility that acceptance of the disease and feelings of self-achievement improve the QOL of patients with Parkinson's disease.

We report a 75-year-old man with parkinsonism who died suddenly. The patient was well until 64 years of the age when he had an onset of tremor in his left hand. He was treated with a medicine in another hospital, and his tremor subsided. Five years after the onset, he started to note difficulty in fine finger movements and gait disturbance. He tended to lean backward with frequent falls. He was treated with bromocriptine, trihexyphenydil, and L-dops without apparent improvement. He visited our out patient clinic on November 11, 1993 when he was 75 years of the age. Neurologic examination at that time revealed an alert and well oriented man in no acute distress. Higher cerebral functions were intact. In the cranial nerves, he showed restriction in the upward as well as down ward gaze (40% of normal). He showed masking of the face and spoke in small voice. He walked in a stooped posture with small steps; retropulsion was present. Muscle rigidity was moderately positive in the neck, however, no rigidity was noted in the limbs. No abnormal involuntary movements were seen. He showed moderate bradykinesia and difficulty in finger tapping. Muscle stretch reflexes were normally elicited and the plantar response was flexor bilaterally. Sensation was intact. The autonomic nervous system appeared intact. He was treated with 300 mg/day of Sinemet with marginal improvement in his balance. In February 4, 1994, he had a common cold. On the next day, his parkinsonism worsened and he became unable to walk by himself. He was found unconscious in the bathroom on the same day. He was brought to our hospital by an ambulance. Upon arrival, he was unresponsive and was not breathing. Blood pressure could not be measured. Pupils were dilated without reaction to light. Cardiac resuscitation was attempted, however, ventricular fibrillation appeared on an EEG monitor, and he was pronounced dead at eleven o'clock in the morning. The patient was discussed in a neurological CPC, and the chief discussant arrived at the conclusion that the patient had progressive supranuclear palsy because of vertical gaze palsy, axial rigidity, and poor response to levodopa. Regarding the cause of his sudden death, the chief discussant thought that he developed pulmonary embolism. Postmortem examination revealed non-bacterial thrombotic endocarditis in the heart, but this did not appeared to be related to his sudden death. Multiple disseminated small emboli were found occluding small arteries of the left lung; this was consistent with acute pulmonary embolism, and this was thought to be the cause of his sudden death. In the central nervous system, marked atrophy of the globus pallidus was noted; both internal as well as external segments showed marked atrophy; no myelinated fibers were seen in the globus pallidus. Neuronal cell loss was marked in the globus pallidus, the subthalamic nucleus, and the substantia nigra. No Lewy bodies or tangles were seen. The histologic diagnosis was consistent with pallido-nigro-luysian atrophy. Brownish pigments such as seen in Hallervorden-Spatz disease were seen in the globus pallidus. In addition, formy spheroids were seen in the substantia nigra. However, iron deposits were not so strong as to suggest Hallervorden-Spatz disease. Pallido-nigro-luysian atrophy is a rare neurodegenerative disorder. It is interesting to note that this condition may mimic progressive supranuclear palsy or pure akinesia clinically.

Zinc finger motif is a novel DNA binding motif characterized by the unique role of zinc; the protein folding and the DNA binding ability are governed by the coordination of a zinc ion. In C2H2-type zinc finger, two cysteines and two histidines contribute to form a globular domain through zinc coordination. The zinc finger of C2H2-type gives promise of recognition for any DNA sequences because of its recognition mode. A C2H2-type zinc finger protein Sp1 has been converted into artificial site-specific nuclease with an attached Ni-based DNA cleavage unit (Gly-Gly-His). This protein cleaved DNA at a single site near the Sp1 recognition sequence. The zinc finger-based nuclease is applicable to chromosome mapping and sequencing.

We present a 93-year old female who abruptly developed left hemiplegia on the way to the wash room. She was admitted to another hospital where her consciousness deteriorated and artificial respiration was necessary. She was transferred to our hospital 20 days after the onset of her stroke. On admission, her BP was 60/40 mmHg, and wheezing râle was heard in both lung fields. No heart murmur was present. She was in comatose state, but a small amount of spontaneous movements were present in her eyelids and the jaw. Neurologic examination revealed no papilledema, small non-reactive pupils with the right side being slightly larger than the left, absence of ocular movements, and loss of oculocephalic response and corneal reflex. An intratracheal tube was in situ, and the observation of the pharynx was impossible. No stiff neck was present. No spontaneous movement was observed in her limbs which were flaccid, and all the deep reflexes were lost except for jaw jerk which was weakly positive. Cranial CT scans revealed low density areas in the right anterior and middle cerebral artery territories with patchy areas of high density being consistent with a hemorrhagic infarction. In addition, a small high density mass lesion was noted in the left frontal lobe adjacent to the falx, which was thought to be a small meningioma. Her clinical course was complicated by pneumonia, hypotension and cardiac failure. She expired 2 months after the onset of her stroke. Postmortem examination revealed an extensive hemorrhagic infarction involving the territory of the right internal carotid artery.(ABSTRACT TRUNCATED AT 250 WORDS)

We report a 46-year-old female who presented progressive ophthalmoplegia and limb weakness. She was well until the age of 15 years when there was an onset of bilateral deafness. She became completely deaf by 20 years of age. She noted an onset of weakness in her legs when she was 27-years-old and of ptosis at 34 years of age. She was admitted to our hospital when she was 41-years-old. Neurological examination revealed near total ophthalmoplegia, bilateral ptosis, dysphagia, generalized muscle atrophy and weakness of approximately 4/5 degree, facial grimacing, athetotic movements in four limbs. Laboratory examinations revealed increase in blood lactate and pyruvate levels and diffuse low density change in the cerebral white matter in CT scans. She was thought to have a mitochondrial encephalomyopathy. She was discharged for follow-up, but her clinical course was that of a relentless deterioration. She was readmitted to our service in December 1989. She showed further progress in her weakness and muscle atrophy. Otherwise neurological examination was essentially similar to the previous one. Her cranial CT scans showed low density changes in striatum, thalamus and midbrain in addition to the white matter. Enzyme activities of the electron transport complexes revealed a moderate decrease in the succinatecytochrome c reductase activity, and the Southern blot analysis of mtDNA revealed multiple deletions in mitochondrial genomes. Two months after her admission, she developed bronchopneumonia, and expired on March 13th, 1990. Post-mortem examination revealed diffuse pallor of myeline in the cerebral white matter in K-B staining. A marked neuronal loss and gliosis were observed in putamen bilaterally. Skeletal muscles showed typical changes of mitochondrial myopathies with ragged-red fibers in Gomori-Trichrome staining, and crystalline inclusion bodies by electron microscopic observations. Some neurogenic atrophies were also seen. Oculomotor nuclei appeared intact. It was thought that she had an incomplete form of Kearns-Sayre syndrome. The patient was discussed in a neurological CPC of the departments of Neurology and Pathology of Juntendo University School of Medicine.

Occipital horn syndrome (OHS; Ehlers-Danlos syndrome type IX) belongs to the category of the copper metabolism disorders and is at present being investigated biochemically as is Menkes disease. We report a case of OHS in a 34-year-old male, which we believe to be the first Japanese case. He had been noted to have psychomotor retardation since his early childhood and now presents severe psychomotor retardation and muscle atrophy. He shows characteristic facial appearance, hyperelasticity of the skin, joint subluxation and generalized muscular atrophy. Laboratory investigations revealed a low serum copper and ceruloplasmin level as well as intestinal non-absorption of copper. Radiologic imagings showed occipital exostoses and bladder diverticula. The activity of lysyl oxidase, a copper-dependent enzyme involved in cross-link formation in collagen, was decreased in a skin-biopsied specimen. Electronmicroscopic investigation of a muscle biopsy showed irregularity of the myofibrillar network and accumulation of the concentric laminated bodies in the subsarcolemmal regions.

A 9-year-old girl with type I hyperprolinemia, who also had photogenic epilepsy, was reported. She showed epileptic discharges and the regression in speech and motor activities, since 7 years of age. Her plasma proline levels were 3 to 4 times higher than control levels. In urine, iminoglycinuria appeared, when plasma proline value exceeded 0.80 mM. The proline oxidase activity of the liver tissues obtained by biopsy in the patient was about 23.5%, compared to that of controls. In spite of the restriction of proline and protein intake, she showed progressive speech and motor retardation.

X-linked ichthyosis is an inborn error of metabolism due to the deficiency of steroid sulfatase. We reported two cases of the patients associated with myopathies, which are Duchenne muscular dystrophy (DMD) and myotonic dystrophy (MyD), respectively. In addition to DMD and MyD, they showed corneal opacities, lack of steroid-sulfatase activities in peripheral leukocytes and massive accumulation of cholesterol sulfate in plasma. Such cases were not reported, previously. Assay of steroid sulfatase and cholesterol sulfate in the patients having ichthyosis is important to elucidate the wide clinical spectrum of steroid sulfatase deficiency.

In order to understand the pathogenesis and improve the treatment of tarsal tunnel syndrome, we investigated the tarsal region anatomically on 62 feet of 31 cadavers and five freshly amputated feet. The following results were thus obtained: 1. The bifurcation into the medial and lateral plantar nerves mostly occurred within the flexor retinaculum. 2. The medial calcaneal branch showed many anatomical variations. 3. The flexor retinaculum was not clearly demarcated at its superior and inferior borders, and it was not as thick as previously thought. 4. The neurovascular bundle was separated from other tendon sheaths, and enclosed in its own tunnel. 5. A fibrous septum found at the entrance of the abductor hallucis muscle, may represent an entrapment point of the medial plantar nerve. 6. The ganglion from the talocalcaneal joint tended to compress only the medial plantar nerve.

The incidence of multiple primary bronchogenic carcinomas has increased in Japan as the incidence of bronchogenic malignancy has increased. The patient was a 71-year-old man with simultaneous multiple bronchogenic carcinomas. He had large cell carcinoma in the left upper lobe and adenocarcinoma in the left lower lobe. We reviewed 66 reported Japanese cases with multiple lung cancers with special reference to sex distribution, age, simultaneous or metachronous, location, histological types, treatment and prognosis.

In 1959, this disorder was first reported by one of authors (K.H.) (6). Since then, many Japanese cases have been reported and recently some cases are also reported in other countries. Its cause and pathology are still unknown, because no autopsy case was obtained. In this report, some electromyographic findings are described and discussed. This series contain 26 cases (male 23, female 3). Age of onset: 13-33 years old (mean 19.4 years old). Clinical duration: 2-32 years old (mean 9.0 years). Only 6 cases (23%) showed trauma as the past history (Table 2). We classified 26 cases in three groups (UL: unilateral, BP: bilateral, one-side predominant, BS: bilateral symmetrical) based on the laterality of physical findings (amyotrophy, weakness, finger tremor, etc.) (Table 3). Conventional needle EMG were done in all cases and these findings were judged by the criteria which we defined as Fig. 2. In some cases, conduction velocity (MCV & SCV) were measured. Needle EMG showed more selective and localized distribution of NGP (neurogenic pattern) in the hand muscles, the forearm muscles except for M. brachioradialis, and M. triceps brachii on the atrophied sides of the arm in all clinical groups (UL, BP, BS) (Table 4.5). On the normal side of bilaterally examined cases of UL group, NGP was shown in 9/11 cases, buts its distribution seemed to be slightly restricted in the more distal part. Thus, in this disorder, 22/26 cases (77%) were electromyographically affected on the bilateral sides, although the clinical feature was mostly unilateral. The clinical features and pathological process seemed to be established in a few years after the onset without the apparent tendency of further progression. Afterwards, no apparent extension of NGP distribution and increment of grading were observed in the Clinical course. No delay was observed on MCV and SCV. The pathological lesions responsible for these electromyographic changes are discussed, either on spinal segmental origin or on brachial plexus origin, but some ambiguous portions are still remained.

We describe a 74 year old man who showed the jaw opening phenomenon by painful stimuli from two months after the onset of basilar artery thrombosis. He was admitted to our hospital because of consciousness disturbance and paralysis of all extremities. Soon after admission, he was in a state of impending herniation but with conservative therapy he recovered slightly, and then fell into an akinetic and mute state. Two months after the onset of the stroke, he began to open his mouth in response to painful stimuli, and five months after the stroke palatal myoclonus also appeared. Neurological signs and symptoms five months after admission were as follows; he was akinetic, mute and always kept his eyes closed because of complete blepharoptosis due to oculomotor nerve palsy. Pupils were dilated and adducted. Bilateral light reflexes were absent and the oculocepharic reflex could not adduct the eyes inwardly. Bilateral corneal reflexes were present, facial reflexes were exaggerated and jaw reflexes were also active. All limbs were spastic and paralyzed, and no voluntary movement was observed. Deep tendon reflexes were active in all extremities, and bilateral plantar responses were extensor. Palatal myoclonus was recognized in his soft palate, lips, sternocleidomastoid and diaphragm. Its frequency was about 150 cycles per minute. CT scan revealed severe low density areas in the midbrain and bilateral posterior lobes. In cerebral angiography, the upper part of the basilar artery was completely occluded.(ABSTRACT TRUNCATED AT 250 WORDS)

A case of epilepsy with myoclonic absences is reported. A boy, 3 years and 8 months old, had the first attack at the age of 1 year and 8 months. He was mentally retarded, but had no evidence of organic brain damages. He had been said to have "absence" at another hospital for 2 years until he was referred to our hospital. The attack was characterized clinically by sudden loss of consciousness accompanied with rhythmical massive myoclonias. The ictal EEGs showed synchronous diffuse 3 c/s spike-wave discharges. There was a strict and constant relation between spike-wave discharges and the myoclonia. The polygraphy recording (EEG and EMG of various muscles) was very helpful for the diagnosis of epilepsy with myoclonic absences. We think that epilepsy with myoclonic absences should be considered in any case of "absence" with concomitant 3 c/s spike-wave discharges which is resistant to appropriate treatment, or is accompanied with mental retardation.