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A 66-year-old woman suddenly experienced unsteadiness while walking; she had experienced the same symptom before, but it had resolved immediately. Her neurological findings showed cerebellar ataxia, absence of tendon reflex in the extremities, and orthostatic hypotension. MRI with DWI of the brain showed linear high-intensity areas at the white matter just below the cerebral cortex. Therefore, we suspected neuronal intranuclear inclusion disease (NIID). In her cutaneous skin biopsy, intranuclear inclusion bodies, which tested positive for an anti-ubiquitin antibody and anti-p62 antibody, were observed in sweat gland cells and fibroblasts; therefore, we diagnosed her with NIID. As no one in her family had similar symptoms, this was a case of sporadic NIID. Adult-onset NIID with the main presentation of cerebellar ataxia is rare; in our case, this repeated acute-onset symptom was a unique manifestation of the condition.

A 75-year-old woman presented with a 4-month history of repetitive loss of consciousness, dizziness, and ear fullness. Fluid attenuation inversion recovery magnetic resonance imaging showed high-intensity areas in the right occipital lobe, both frontal lobes, and parietal lobes, and the patient was therefore admitted to our department for further examination. Neurologic findings included a positive Barré sign (right upper extremity), impaired tandem gait, and positive left-sided Babinski reflex. The patient was diagnosed with Takayasu's arteritis owing to a difference in blood pressure between the left and right upper extremity, weakened pulse in the left upper extremity, as well as thickened arterial wall, and delayed enhancement on a contrast-enhanced computed tomography image. The head magnetic resonance imaging findings disappeared within 4 months and the patient was diagnosed with posterior reversible encephalopathy syndrome. Posterior reversible encephalopathy syndrome associated with elderly female Takayasu's arteritis is rare. ; the present case therefore offers valuable information.

A 69-year-old man experienced general malaise, weight loss, amnesia, gait disturbance, and restlessness a month prior to admission. Brain MRI showed high intensity areas in the bilateral medial temporal lobes and insular cortices on FLAIR images, and therefore, he was diagnosed with limbic encephalitis. After admission, quadriplegia and respiratory failure progressed rapidly, and he needed ventilatory management. A nerve conduction study revealed low compound muscle action potential amplitude with loss of sensory nerve action potential, which indicated axonal sensorimotor neuropathy. We administered intravenous immunoglobulin and methylprednisolone pulse therapy, but he did not recover. Although no tumor was found on CT, his serum was positive for anti-Hu antibody; therefore, we diagnosed him with paraneoplastic neurological syndrome. An FDG-PET study showed accumulation at lesions on two hilar lymph nodes. Small cell lung carcinoma was detected by endobronchial ultrasound-guided transbronchial needle aspiration. Although paraneoplastic acute sensorimotor neuropathy with respiratory failure resembling Guillain-Barré syndrome is rare, identification of antibodies and servey of tumors aids accurate diagnosis.

A 69-year-old male was admitted to hospital with clouded consciousness and abnormal behavior. His body temperature was 38.2 degree Celsius upon admission and he was somnolent. Herpes zoster was observed along the first division of the trigeminal nerve on the right side of the face. The right palpebra was severely swollen, and the right eye showed a dilated pupil, loss of light reflex, and total ophthalmoplegia. A spinal tap revealed pleocytosis and increased proteins, and a DNA-PCR test for varicella-zoster virus (VZV) was positive. Optic neuritis was diagnosed based on fundoscopy. Following acyclovir administration, the patient regained full consciousness and the rash was alleviated; however, visual acuity did not recover. VZV-induced meningoencephalitis complicated with orbital apex syndrome is rarely observed. We suspect that VZV initially infected the nasociliary nerve at the distal end of the first division of trigeminal nerve and spread to the adjacent optic, oculomotor, trochlear, and abducens nerves, resulting in VZV-induced meningoencephalitis complicated with orbital apex syndrome.

The healthcare system surrounding surgeons is collapsing due to Japan's policy of limiting health expenditure, market fundamentalism, shortage of healthcare providers, unfavorable working environment for surgeons, increasing risk of malpractice suits, and decreasing number of those who desire to pursue the surgery specialty. In the USA, nonphysician and mid-level clinicians such as nurse practitioners (NPs) and physician assistants (PAs) have been working since the 1960s, and the team approach to medicine which benefits patients is functioning well. One strategy to avoid the collapse of the Japanese surgical healthcare system is introducing the NP/PA system. The division of labor in medicine can provide high-quality, safe healthcare and increase the confidence of the public by contributing to: reduced postoperative complications; increased patient satisfaction; decreased length of postoperative hospital stay: and economic benefits. We have requested that the Ministry of Health, Labor and Welfare establish a Japanese NP/PA system to care for patients more efficiently perioperatively. The ministry has decided to launch a trial profession called "tokutei (specifically qualified) nurse" in February 2010. These nurses will be trained and educated at the Master's degree level and allowed to practice several predetermined skill sets under physician supervision. We hope that all healthcare providers will assist in transforming the tokutei nurse system into a Japanese NP/PA system.

An open study in the Netherlands have investigated the efficacy of combination therapy with methylprednisolone(mPSL) and high-dose immunoglobulin (IVIg) for Guillain-Barré syndrome (GBS) patients. The study suggested that the combination therapy is more effective than treatment with IVIg alone. Following the study, randomized trial of the combination therapy for the GBS patients was performed by the Dutch GBS study group in 2004. The randomized trial revealed that it was associated with a slightly, but not significantly, higher percentage of patients who recovered by more than grade 1 in the disability score. In addition, the long-term outcome (52 weeks) of the combination did not significantly differ from that of non-combination therapy; however, the median time to independent ambulation tended to be shorter in patients undergoing the combination therapy. We have summarized the recent advances in our understanding of the therapy for the patients with GBS. GBS consists of at least two subtypes of acute peripheral neuropathy, such as acute motor axonal neuropathy (AMAN) and acute inflammatory demyelinating polyneuropathy (AIDP). It has been reported that the subtype of AMAN is morecommon in Japan, as compared with the Western countries. Further studies are needed to clarify the effectiveness of the combination therapy, because of the difference of the subtype between Japan and the Western countries.

We report a patient with syrinx formation secondary to cervical epidural abscess, cervical spondylitis, cervical discitis and meningitis. A 53-year-old woman developed sudden fever, headache and neck pain. On admission, neurological evaluation showed limitation of cervical motion, meningeal irritation, and exaggerated muscle stretch reflexes in all four extremities. Cerebrospinal fluid cell count was 832/mm3 and protein was 771 mg/dl. Bacterial culture of the cerebrospinal fluid showed Staphylococcus aureus. A cervical MRI scan with Gd-enhancement revealed focal high intensity signal in the T2-weighted and FLAIR images, at the anterior meninges of the C3-8 segments, the vertebral bodies of C5-6 and the intervertebral disks of C5-6 segment. Her diagnosis was cervical epidural abscess, cervical spondylitis, cervical discitis and meningitis. Antibiotics and steroids improved her symptoms. Six weeks after the onset of symptoms, a cervical MRI scan showed narrowing and synarthrosis in the intervertebral space between C5 and C6. MRI and myelo CT scans demonstrated a newly-formed syrinx from C3-C 7. This was an interesting case of syrinx formation secondary to cervical epidural abscess.

We have reported a very rare case of a patient with callosal disconnection syndrome due to acute disseminated enchephalomyelitis (ADEM). A 54-year-old right-handed woman developed sudden consciousness disturbance and fever after 2 weeks when she had common cold. She did not have a history of stuttering. On admission, the neurological finding showed consciousness disturbance, and exaggerated muscle stretch reflex in four extremities without meningeal irritation. The cerebrospinal fluid examination revealed the cell counts of 273/mm3, the protein of 348 mg/dl and the myelin basic protein 18.3 pg/ml. The brain MRI demonstrated a focal high signal intensity in the right cerebellar peduncle, right temporal lobe, left occipital lobe and corpus callosum on the T2-weighted and FLAIR images. The lesion of corpus callosum was the trunk and splenium, sparing the dorsal side of splenium. Her diagnosis was ADEM. The steroid pulse therapy (metylpredinisolone 1 g/day for 3 days) improved the consciousness disturbance. After 6 weeks from the onset, the neuropsycological examination was performed. The remarkable findings included pure alexia without color naming defects, the acquired stuttering and one-way disturbance of somesthetic transfer from the left hand to the right. This patient may contribute to the study of functional localization of the corpus callosum.

We report here a 65-year-old man with ptosis, diplopia, and progressive lower limb muscle weakness without nasal symptoms. CA19-9 and CEA were elevated in the serum and cerebrospinal fluid (CSF), but Squamous Cell Carcinoma Antigen (SCC) was not. CA19-9-positive atypical cells were found in the CSF. A right ethmoidal tumor was discovered by paranasal CT and 18F-Deoxyglucose Positron Emission Tomography (FDG-PET) studies. The ethmoidal tumor invaded to the orbits and disseminated into the CSF space through the cribriform plate. Biopsy demonstrated that the paranasal tumor consisted of mucoepidermoid carcinoma. We report here the first case of meningeal carcinomatosis due to mucoepidermoid carcinoma in the ethmoid sinus.

Antibodies against a glutamate receptor in the central nervous system are reported to be an important autoimmune factor in infantile or childhood refractory epilepsy and in adult-onset paraneoplasstic cerebellar ataxia. We here reported a patient with glutamate receptor antibodies-positive elderly-onset intractable epilepsy and advanced-stage stomach cancer. A 77-year-old man with a history of resected bladder cancer suddenly developed an attack of epilepsia partialis continua in the right arm, followed by a generalized epileptic status. The ictal EEG showed spikes in the left parietal region with diffuse slow wave backgrounds. The brain MRI demonstrated a focal high signal intensity in the left postcentral gyrus on the T2-weighted and FLAIR images. The SPECT showed a high uptake in the focal area corresponding to the MRI lesion, suggesting a seizure focus. The routine analysis in the CSF was normal, but the glutamate receptor antibodies (IgG, IgM and C-terminal) were all positive. The status epilepticus was refractory to anticonvalsants, and the barbiturate anesthesia was introduced. Subsequently, the epilepsy has been fairly controlled by valproic acid and phenobarbital. The post ictal MRI demonstrated a focal atrophy in the left postcentral gyrus. The systemic evaluations revealed an advanced-stage stomach cancer. The glutamate receptor antibodies may play a role in late-onset refractory epilepsy, and may be an autoimmune factor in paraneoplstic neurologic syndrome presenting with epilepsy.

Pharmacokinetics, clinical efficacy and safety of teicoplanin (TEIC) were evaluated in pediatric and neonate patients with MRSA sepsis in the dosages approved in overseas. The administrated dose for pediatrics patients was 10 mg/kg once at hour 0, 12 and 24, followed by every 24 hours intervals. In neonates patients, first dose was 16 mg/kg, then 8 mg/kg every 24 hours intervals. 1. Pharmacokinetic results. All 17 patients (9 neonates and 8 pediatrics) who received TEIC were evaluated for pharmacokinetics. Trough concentrations were analyzed in 16 patients (9 neonates and 7 pediatrics) excluding one patient for lack of measurement of drug concentration at day 7. No patient with a concentration exceeding 60 micrograms/mL in peak or trough concentrations were reported. Mean concentrations in trough at day 3, 4 and 7 in neonates were 15.2, 14.7 and 17.8 micrograms/mL, and in pediatrics were 12.5, 12.2 and 13.1 micrograms/mL, respectively. These results were similar to those reported in foreign pediatrics and neonates patients. 2. Efficacy and safety results. Since no patient was excluded, all patients were evaluated for efficacy and safety. Microbiological efficacy as well as clinical cure were secondarily evaluated in 2 patients for whom MRSA was isolated from blood. Clinical efficacy rate was 76.5% (13/17) and number of cases in judgments of excellent, good, fairly improved and no change were 12, 1, 3 and 1 cases respectively. The patients for whom MRSA was isolated from blood were judged as MRSA eradicated case and cured without any additional anti-MRSA drugs. Adverse events were reported in 2 neonates and 3 pediatric patients. Possibly related adverse events to study drug (adverse drug reactions) were: 1 case of respiratory disorder, thrombocythemia, gamma-GTP increased, GOT increased and GPT increased in 3 pediatrics. These results suggest that an application of overseas dose regimen of TEIC for neonate and pediatrics is appropriate in Japan.

Transmyocardial laser revascularization (TMLR) has been approved as an indirect coronary revascularization measure through angiogenesis around created channels in patients who are not amenable to direct revascularization methods such as coronary artery bypass grafting (CABG) or percutaneous transluminal coronary angioplasty (PTCA). TMLR is less invasive and there are no contraindications in terms of left ventricular function. Therefore all patients who have untreatable lesions by CABG or PTCA with reversible ischemia are candidates for TMLR therapy. In a Japanese clinical trial, improvement of left ventricular function associated with relief of persistent angina and improved perfusion was seen in 54% of patients. Significant reduction of operative risk in adjunctive TMLR (combination of TMLR with CABG) compared with isolated CABG has been also demonstrated in a randomized trial. These results indicate the usefulness of adjunctive TMLR in multivessel-disease patients with left ventricular dysfunction. Because TMLR is a simple and less-invasive technique, combined use of TMLR with off-pump CABG or MIDCAB is also an attractive revascularization strategy in ischemic cardiomyopathy patients.