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A 52-year-old woman was admitted to our hospital with muscle pain and an elevated creatine kinase level. She had experienced wrist pain at onset seven years ago. The initial possible diagnoses were rheumatoid arthritis and adult-onset Still disease. The patient received corticosteroid and immunosuppressant therapy but experienced deterioration of symptoms. The symptoms of muscle pain and mild creatine kinase elevation emerged four years prior to her visit. Further elevation of creatine kinase was observed for three months before her visit despite adjusting the immunosuppressant dose. On admission, she presented with muscle moderate weakness of the trunk and extremities and pain of the shoulder and medial thigh muscles. Elevation of muscle enzymes and inflammatory response were also detected, and the anti-PL7 antibody was positive. Muscle biopsy from biceps brachii revealed necrotizing myopathy with necrotic and regenerated muscle fibers. The final diagnosis was anti-PL7 antibody positive myositis. The patient was treated with a higher dose of prednisolone and an adequate dose of tacrolimus. Following this treatment, the symptoms were improved. Anti-ARS (aminoacyl t-RNA synthetase) antibodies such as anti-PL7 antibody are useful in diagnosis and for prognostic prediction. Further investigation of patients with anti-ARS antibodies positive myositis is required.

Objective: Percutaneous endoscopic gastrostomy (PEG) serves as a major artificial hydration/feeding method in the terminal care of elderly patients. We surveyed the state of patients who underwent PEG at our hospital and investigated the clinical characteristics of patients in the long-term survival group, and the short-term survival group., Methods: The subjects were 125 patients who underwent PEG at our hospital between January 2007 and June 2011. Two kinds of examination were performed. In the first examination, subjects were divided into a short-term group of 37 patients who survived <181 days and a long-term group of 88 patients who survived ≥181 days. In the second examination, subjects were divided into 2 purpose-oriented groups: a prolongation of life group of 76 patients and a being able to eat group of 49 patients. We analyzed the clinical records of these patients and conducted a telephone-based survey., Results: In the short-term group, there were many cases of PEG after hospitalization due to medical diseases such as pneumonia. In the long-term group, many subjects were young, female, suffered from recurrence of cranial nerve disease, or slowly developed dysphagia. The involvement of rehabilitation doctors, the rate of undergoing rehabilitation for dysphagia, and cases who were able to achieve oral ingestion were significantly high. Of 49 patients who underwent PEG with the aim of being able to eat, 24 achieved oral ingestion., Conclusion: Our findings suggest that involving rehabilitation doctors and undergoing rehabilitation for swallowing effectively promote long-term survival after PEG.

A 45-year-old man was admitted to our hospital with flaccid paraplegia. Neurological examination at a local hospital, 2 months before admission to our hospital, showed sensory impairment of the right posterior surface of the thigh and a decreased Achilles tendon reflex. Spinal magnetic resonance imaging (MRI) showed a T₂ weighted high-intensity area at the Th10-11 level that was more pronounced in the gray matter. The patient developed flaccid paraparesis and urinary retention. No improvement was observed after 2 rounds of methylprednisolone (mPSL) pulse therapy. Spinal cord biopsy showed demyelinated axons and myelinophagia without any tumorous lesion. Myelopathy exacerbated, and hence, plasma exchange was performed. However, this was ineffective. We suspected that myelopathy was caused by intravascular lymphoma (IVL) because of the presence of a low-grade fever, weight loss, and elevated serum soluble IL-2 receptor titers. Random biopsies, including skin, rectal, bone marrow, muscle, and renal biopsies, and splenectomy were performed to make a definite diagnosis of IVL myelopathy. Among these biopsies, the diagnosis of IVL myelopathy was confirmed from the renal specimen. The patient underwent chemotherapy at our hospital, and the IVL remitted. The results of this study confirm that sufficient systemic investigation by using tissue biopsy specimens should be performed in order to confirm the diagnosis of IVL myelopathy.

Tuberculous meningitis (TbM) is a neurological emergency condition that requires prompt initiation of treatment. The standard initial treatment for TbM is often insufficient for producing remission because the anti-tuberculosis agent may cause severe side effects, or vasculitis and hydrocephalus may induce an intractable state. Moreover, it is difficult to distinguish paradoxical expansion from its own deterioration. We treated 2 cases of adult TbM by using multidisciplinary therapy, including methyl prednisolone pulse and intrathecal isoniazid administration. Both cases had not been diagnosed as pulmonary or other tuberculosis, and cerebrospinal fluid (CSF) culture and polymerase chain reaction at approximately 1 week after hospitalization identified the cases as TbM. We administered the standard initial treatment recommended by the British Infection Society guidelines for adults, but both cases deteriorated and showed elevation of intracranial pressure. We indwelled a lumbar drainage for Case 1 and an Ommaya reservoir for Case 2. We removed CSF and administrated isoniazid regularly using each of the drainage devices, added streptomycin, and increased the steroid dose including addition of steroid pulse therapy. Both cases improved, and their neurological dysfunction did not persist. After the induction of an intractable state occurs due to TbM, we are likely to assume poor prognosis and neurological sequelae. However, our experience in these cases showed amelioration of the symptoms leading to the rehabilitation of these patients in society.

Aim: In Japan, it is rare for patients to confirm their intentions regarding terminal care treatment in hospital by signature. To maintain respect for the autonomy of elderly patients, we encouraged confirmation by signature regarding terminal care treatment in elderly patients, after repeated discussions. The purpose of this study was to clarify the status of confirmation of intention by signature., Method: The subjects were 98 patients who signed documents signaling their intention regarding terminal care (signature group), and 165 patients who did not sign such documents (non-signature group), all of whom died in our hospital between April 2009 and March 2010. We surveyed and examined their backgrounds and treatment experiences based on the clinical records. Furthermore, we gave a questionnaire survey to these patients. A total of 31 (35.2%) patients from the signature group and 58 (35.2%) patients from the non-signature group responded., Results: In the signature group 32 (32.7%) patients judged to be in a terminal state were discharged alive. Those in the signature group had a significantly higher age, higher ratio of dementia, lower activity level in their daily life and were hospitalized with respiratory illnesses. In addition, the frequency of the informed consent discussions was greater than that of the non-signature group. A total of 90% of the signature group was able to accept the confirmation of their intention regarding terminal care by signature. In both groups, the doctors' explanations were understood, and the patients' wishes were successfully conveyed., Conclusion: In Japan, confirmation of intention regarding terminal care by signature can be effective and useful as one of the methods to determine intention regarding terminal care treatment in elderly patients.

Background: Senior citizens who have multiple diseases often receive multiple medications (polypharmacy). Because of the increased risk of adverse drug reactions with polypharmacy, trying to minimize the number of medications is important to medical care. Some rehabilitation physicians often treat multiple diseases alone, and hospitalization for rehabilitation provides a good opportunity to improve prescription practices. Our rehabilitation department has implemented a program to minimize the quantity of medications prescribed to hospitalized patients., Aim: To examine reductions in medication consumption through suitable prescribing patterns during rehabilitation., Methods: We investigated prescription practices and the reduction of medications from the clinical records of 203 patients who were admitted to our rehabilitation department from January to December, 2009., Results: Of the 203 patients, 131 (64.5%) were 75 years old or more, and 77 (37.9%) patients were less than 45 kg in weight. Patients took an average of 6.49 medications on admission and 6.02 on discharge. Ninety-two (45.3%) patients experienced a reduction in medication within one month. After a month, 77 (37.9%) patients experienced an increase in medication and only seven (3.4%) a decrease. No patient whose medications were reduced experienced adverse drug withdrawal reactions. The 14 patients whose antihypertensive medication was reduced experienced an excellent course. Similarly, those whose narcoleptic medications were reduced also did well., Conclusions: Medications should be optimized, and reduced if possible, in the early stages of hospitalization. In the absence of adverse events, it appears possible to decrease the quantity of analgesic and gastrointestinal drugs by one-third.

Background: The International Cooperative Ataxia Rating Scale (ICARS) is widely used as a scale for the assessment of the severity of cerebellar ataxia. However, this scale comprises several items; thus, making the application of this scale is not sufficiently practical to perform daily assessment of ataxic patients. A new rating scale--Scale for the Assessment and Rating of Ataxia (SARA)--was shown to provide highly reliable assessments; further, the scores on SARA correlated with the ICARS score and the Barthel index. After obtaining the permission, original SARA was translated into Japanese., Objective and Methods: To examine the reliability and internal consistency of the Japanese version of the SARA for the assessment of cerebellar ataxia in 66 patients with spinocerebellar degeneration., Results: Intraclass coefficients (ICC) were observed to be greater than 0.8 except in the case of the inter-rater "finger chase" and "fast alternating hand movement" tests., Conclusions: The Japanese version of SARA is highly reliable and very useful for the assessment of cerebellar ataxia on a daily basis.

A 67-year-old man was diagnosed with primary gastric malignant lymphoma by an endoscopic examination. Endoscopy revealed irregular ulcerative regions from body to antrum of the stomach. With a diagnosis of diffuse large B-cell lymphoma based on the biopsy finding, the patient was treated with R-CHOP chemotherapy. After three cycles of chemotherapy, a tight stenosis was located at the antrum of the stomach. Total gastrectomy was performed due to an obstruction. Pathological diagnosis was complete response. It was thought that the tumor organization rapidly disappeared due to the effectiveness of the chemotherapy, and that the origin of the stricture caused fibrosis.

To determine the frequency of cerebellar ataxia patients with autoantibodies for Hashimoto's disease, we analyzed 68 patients who were examined serum test for autoantibodies of Hashimoto disease among 178 cerebellar ataxia patients who visited our neurology clinic from January 2005 until December 2007. In these 68 patients, 8 had autoantibodies for Hashimoto's disease. Five of these 8 patients were diagnosed with hereditary spinocerebellar ataxia by genetic analysis. Moreover, one patient was diagnosed with probable multiple system atrophy by neurological examination. Cerebellar ataxic disease of known causes was ruled out for the remaining two cases; they were euthyroid and their cerebellar ataxia was slowly progressive and were diagnosed with cortical cerebellar atrophy. Although Hashimoto's disease may associate with cerebellar ataxia because cortical cerebellar atrophy is a heterogeneous condition, this association is not clear at present.

A 25-year-old woman was admitted to our hospital because of convulsions after delivering a baby. Her neurological examination revealed a disturbance of consciousness and weakness in both arms. A brain magnetic resonance imaging scan (MRI) showed thrombosis of the left transverse sinus and many patchy high signals at bilateral basal ganglia and subcortical areas. A digital subtraction angiogram (DSA) of the brain revealed a defect at the left transverse sinus, congestion of the cerebral venous flow at the vein of Galen and vasospastic changes at both posterior cerebral arteries. After treating the patient with a venous infusion of heparin, nicardipine and phenytoin, her neurological deficits improved within a day. When MRI and DSA were repeated 2 weeks later, the abnormal lesions had disappeared. These findings suggest that venous thrombosis and vasospasm had the pathogenesis of eclampsia in this case.