A 59-year-old female was started on oral carbamazepine for her psychological disorder on April 21 2004. Four weeks later, she developed generalized erythemas on the trunk and extremities, and ran a high fever of 38°C. A physical examination showed erythroderma, and laboratory examination revealed liver dysfunction and hypereosinophilia. Three days after admission, a number of small pustules emerged on the erythrodermic backgrounds. The skin lesions were improved by the withdrawal of carbamazepine and systemic prednisolone (50mg per day). However, acute pancreatitis was also developed during the course. HHV-6 IgG was increased up to x1280 on June 17. Also, HHV-6 DNA was detected with two peaks during the therapy. DLST with carbamazepine showed a high titer of stimulation index. This case was unique in the acute generalized exanthematous pustulosis (AGEP)-like clinical appearance and the development of acute pancreatitis. HHV-6 DNA was increased with double peaks, which might suggest a “re-reactivation” of HHV-6 by carbamazepine.

Levetiracetam (LEV), a relatively new antiepileptic drug, is now frequently used for treating partial or generalized seizures. Among the adverse effects of LEV, rhabdomyolysis is rare. We describe here a case of LEV-induced rhabdomyolysis in a 26-year-old woman. The patient’s seizures had been controlled with carbamazepine and phenobarbital for the previous 7 years. However, LEV was initiated at the age of 26 years because her seizures control deteriorated with seizures occurring monthly. She experienced lower limb weakness with a high level of creatine kinase 15 days after starting LEV. When LEV was discontinued, her creatine kinase levels decreased and her symptoms gradually improved. This case provide another example of rhabdomyolysis during the early phase of LEV treatment.
Rhabdomyolysis

Objective : The purpose of this clinical research was to create an assessment for patients with muscle disease who wish to continue driving by investigating their motor function and driving experience. Methods : Twenty-four patients with muscle disease who visited our hospital from December 2009 to April 2010 were enrolled in our research. For patients who were still driving, physiatrists evaluated their motor functions, examined simulated driving motions and recorded their driving capabilities and techniques, their ability to get into and out of the vehicle and their ability to store and remove their wheelchairs. Patients no longer driving were asked why they had given up driving. Results : Fifteen patients who continued driving had enough upper limb strength and could simulate driving motions, though the location and degree of their muscle weakness were variable. Five of fifteen drove with the aid of a hand-operated brake and accelerator. Seven needed personal assistance: three to get into and out of the vehicles, six to store and remove wheelchairs. The nine patients who had stopped driving reported that the primary reason for discontinuing driving was that they recognized their muscles were insufficient to control the vehicle. Conclusions : We propose to evaluate muscle strength and to test simulated driving motions when assessing patients with muscle disease. A hand-operated brake and accelerator is efficient for patients with lower limb muscle weakness. However, since no efficient automobile modifications are available for those patients who cannot get into and get out of their vehicles or store and remove their wheelchairs by themselves, we suggest arranging personal assistance for such patients.

We report the operative treatment of a chronic contained rupture of a saccular abdominal aortic aneurysm with a retroperitoneal hematoma. A 62-year-oldman walked into our hospital complaining of a painless abdominal mass and intermittent claudication. He had an episode of severe abdominal pain about 2 years prior to admission. A giant retroperitoneal neoplasm was suspected initially based on computed tomography (CT). However, magnetic resonance imaging, angiography and color doppler sonography demonstrated a chronic contained rupture of an abdominal aortic aneurysm. At laparotomy, a punched out oval defect (width 3.5cm×length 4.5cm) that was thought to connect the thrombosed aneurysm to an organized retroperitoneal hematoma was discovered in the posterior wall of the bifurcation of the aorta. An infrarenal aorto-biexternal iliac Y-graft with a bypass to the left femoral artery was placed without removing the aneurysm or the hematoma. Uneventful recovery followed. About one year after the operation, the retroperitoneal hematoma appeared smaller on CT scan. This case fulfilled the criteria for a “chronic contained rupture” of an abdominal aortic aneurysm proposed by Jones and associates.

OBJECTIVETo establish a means for prenatal prediction of spinal muscular atrophy (SMA) through survival motor neuron (SMN) gene deletion analysis and genetic counseling in families with a child affected with SMA.

METHODSGenetic analysis for prenatal prediction of Werdnig-Hoffmann disease was performed in a at risk Chinese family by polymerase chain reaction (PCR)-single-strand conformation polymorphism (SSCP) in SMN gene exons 7 and 8.

RESULTSThe pregnancy was positive for the homozygous deletion of the SMN gene, thus the fetus was diagnosed as being affected and the pregnancy was terminated.

CONCLUSIONThis approach is fast and reliable for DNA-based prenatal diagnosis of Werdnig-Hoffmann disease.

China ; Female ; Fetal Diseases ; diagnosis ; Gene Deletion ; Genetic Counseling ; Humans ; Pregnancy ; Prenatal Diagnosis ; Spinal Muscular Atrophies of Childhood ; diagnosis ; genetics

Objects：Sit-to-stand (STS) interventions are frequently used as part of stroke rehabilitation. This study aimed to clarify the kinematic and kinetic characteristics of patients recovering from stroke, who have difficulties with STS movement.Method：The participants included 26 stroke patients who struggled to complete the STS maneuver. Using a 3D motion analysis system, the kinematic and kinetic parameters during STS were retrospectively analyzed and compared between failed and successful trials. The kinematic parameters of the trunk and lower limbs were obtained within the seat-off phases, and the kinetic indices of the lower limbs were obtained from measurements taken before and after the seat-off phase. The weight-bearing ratio on the unaffected side was calculated from the vertical ground reaction forces.Results：In the successful trials, analyses of the parameters revealed more forward tilting of the pelvic and thoracic regions, a further forward and downward shift of the center of mass, and larger hip and knee extension moments than those of the failed trials. However, there was no difference in the weight-bearing ratio on the non-paralyzed side.Conclusion：This study revealed that the kinematic and kinetic properties of the STS maneuver differed between successful and failed trials among patients with stroke. The study therefore provides useful information for clinical evaluation and rehabilitation.

Objects：Sit-to-stand (STS) interventions are frequently used as part of stroke rehabilitation. This study aimed to clarify the kinematic and kinetic characteristics of patients recovering from stroke, who have difficulties with STS movement.Method：The participants included 26 stroke patients who struggled to complete the STS maneuver. Using a 3D motion analysis system, the kinematic and kinetic parameters during STS were retrospectively analyzed and compared between failed and successful trials. The kinematic parameters of the trunk and lower limbs were obtained within the seat-off phases, and the kinetic indices of the lower limbs were obtained from measurements taken before and after the seat-off phase. The weight-bearing ratio on the unaffected side was calculated from the vertical ground reaction forces.Results：In the successful trials, analyses of the parameters revealed more forward tilting of the pelvic and thoracic regions, a further forward and downward shift of the center of mass, and larger hip and knee extension moments than those of the failed trials. However, there was no difference in the weight-bearing ratio on the non-paralyzed side.Conclusion：This study revealed that the kinematic and kinetic properties of the STS maneuver differed between successful and failed trials among patients with stroke. The study therefore provides useful information for clinical evaluation and rehabilitation.

1. 5-HT inhibits spontaneous fluid secretion as well as stimulated secretion with secretin (cAMP mediated) or ACh (Ca2+ mediated) in the isolated guinea pig pancreatic ducts. 2. The inhibitory effect of 5-HT is reversible and is dependent on the concentration in the range 0.01-0.1 microM, which is much lower than those that affect intestinal motility and secretion. 3. The 5-HT3 receptor in duct cells appears to mediate the inhibitory effect of 5-HT. 4. [Ca2+]i is unlikely to mediate the inhibitory effect of 5-HT.
5-Methoxytryptamine/pharmacology ; Acetylcholine/pharmacology ; Animal ; Calcium/metabolism ; Guinea Pigs ; Pancreatic Ducts/metabolism* ; Pancreatic Ducts/drug effects ; Secretin/pharmacology ; Serotonin/pharmacology ; Serotonin/metabolism* ; Serotonin/analogs & derivatives* ; Vasodilator Agents/pharmacology

Few reports have yet investigated adequate cognitive rehabilitation for patients with chronic cognitive disorder. In this report, we describe a case of cognitive rehabilitation 10 months after onset of cerebral infarction in the right middle cerebral artery region, resulting in a good outcome. The patient was a 44-year-old right-handed male, with primary symptoms of inattention and left hemispatial neglect. The patient had not undergone any neuropsychological evaluations or cognitive rehabilitation until 10 months after disease onset. After 30 days of both direct attention training and metacognitive strategy training, including time pressure management, improvement of his cognitive disorder was achieved. The teaching of internal methods to improve metacognition with a high level of evidence was effective even 10 months after onset. Furthermore, it is important for therapists who provide cognitive rehabilitation using metacognitive strategy training to understand patients' remaining functions based on neuropsychological assessment, and to consider and teach compensatory methods so that they can be effectively used by patients in situations of daily life.

Few reports have yet investigated adequate cognitive rehabilitation for patients with chronic cognitive disorder. In this report, we describe a case of cognitive rehabilitation 10 months after onset of cerebral infarction in the right middle cerebral artery region, resulting in a good outcome. The patient was a 44-year-old right-handed male, with primary symptoms of inattention and left hemispatial neglect. The patient had not undergone any neuropsychological evaluations or cognitive rehabilitation until 10 months after disease onset. After 30 days of both direct attention training and metacognitive strategy training, including time pressure management, improvement of his cognitive disorder was achieved. The teaching of internal methods to improve metacognition with a high level of evidence was effective even 10 months after onset. Furthermore, it is important for therapists who provide cognitive rehabilitation using metacognitive strategy training to understand patients' remaining functions based on neuropsychological assessment, and to consider and teach compensatory methods so that they can be effectively used by patients in situations of daily life.