Dermatomyositis associated with impairment in both muscle aerobic and anaerobic function

We report a 50-year-old Japanese woman with dermatomyositis in whom an anti-mitochondrial antibody was detected. Muscle biopsy demonstrated periodic acid Schiff- (PAS) positive vacuoles in addition to infiltrates of mononuclear cells. Histochemical analysis showed reduced phosphorylase activity. An aerobic exercise test demonstrated that the concentrations of serum lactate and pyruvate were elevated before corticosteroid therapy but decreased after the therapy. On the other hand, in forearm ischemic exercise tests, the responses of serum lactate and pyruvate were attenuated before corticosteroid therapy but recovered after the therapy. These findings indicate that an inflammatory mechanism interfered with myophosphorylase activity and muscle aerobic function.     

Delayed motor and sensory neuropathy in a patient with brainstem encephalitis

Bickerstaff's brainstem encephalitis (BBE), Miller Fisher syndrome (MFS) and Guillain-Barré syndrome (GBS) are thought to be closely related and to form a continuous spectrum. However, chronic polyneuropathy in BBE has not been reported. We report the temporal profile of anti-ganglioside antibody titer in a case of BBE-like brainstem encephalitis complicated with chronic polyneuropathy. A 71-year-old Japanese woman presented with drowsiness and cerebellar ataxia in addition to mild weakness in distal limb muscles. Anti-GalNAc-GD1a IgG and anti-GalNAc-GM1b IgG antibodies were positive in her serum. Brain magnetic resonance imaging revealed high-intensity signals in the midbrain, pons, and middle cerebellar peduncles on T2-weighted images. Central nervous system manifestations improved after immunomodulating therapy that included prednisolone, plasmapheresis and intravenous immunoglobulin. Nevertheless, the distal muscle weakness was exacerbated when the anti-GalNAc-GD1a IgG titer was elevated. Nerve conduction study indicated motor and sensory neuropathy which was developed motor dominant axonal damage. These findings suggest that anti-ganglioside antibodies, including anti-GalNAc-GD1a IgG, may be involved in a common autoimmune mechanism in BBE-like brainstem encephalitis and chronic motor dominant axonal neuropathy. However, the fact that the latter manifestation exacerbated after the improvement of former one possibly indicates different thresholds of neurologic symptoms mediated by anti-ganglioside antibodies in the present patient.     

Comparison of ropivacaine with bupivacaine and lidocaine for ilioinguinal block after ambulatory inguinal hernia repair in children

BACKGROUND: We have compared ropivacaine with bupivacaine and lidocaine for ilioinguinal block in thirty children undergoing ambulatory inguinal hernia repair. METHODS: Patients were assigned randomly to receive 0.5 ml.kg(-1) of 0.2% ropivacaine (Group R, n = 10), 0.25% bupivacaine (Group B, n = 10) or 1% lidocaine (Group L, n = 10). The patients' parents, who were not informed of the type of local anaesthetic employed, evaluated the postoperative pain at 2 h and 6 h after operation using the Wong-Baker FACES Pain Rating Scale. RESULTS: There was a significant difference in the face scale score between Group R and Group L, and Group B and Group L. There was no difference in the face scale score between Group R and Group B. There were no complications or clinical evidence of local anaesthetic toxicity. CONCLUSIONS: We have confirmed that bupivacaine and ropivacaine are more effective than lidocaine in the prevention of postoperative pain after children's inguinal hernia repair. We suggest that ropivacaine 0.2% is an alternative to bupivacaine 0.25% for ilioinguinal block in ambulatory paediatric surgery.     

Dermatomyositis associated with Sjögren's syndrome: VEGF involvement in vasculitis

Two patients with dermatomyositis complicated with Sj?gren's syndrome (SjS), are reported. Both patients exhibited sensory-dominant polyneuropathy, compatible with neurologic involvement in SjS. Vascular endothelial growth factor (VEGF) levels were increased in their plasma. Histological examination demonstrated vasculitic changes in biopsied specimens of muscle and salivary glands from the patients, and VEGF was overexpressed in the vasculitic lesions. These findings suggest that VEGF overexpression was associated with the development of vasculopathy in skeletal muscle and salivary glands and possibly in the peripheral nervous system.     

Polysialic acid and HNK-1 are expressed in the adult rat vestibular endorgans

Polysialic acid (PSA) and human natural killer (HNK)-1 carbohydrate epitopes are expressed mainly in developing neurons but also in restricted areas, even in adulthood. In the present study, we demonstrated the expression of PSA and HNK-1 epitopes in adult primary vestibular afferent neurons. In addition, we confirmed the presence of two distinct polysialyltransferases, PST and STX, that form PSA, as well as two types of glucuronyltransferases, GlcAT-P and GlcAT-S involved in the biosynthesis of HNK-1 epitopes in the vestibular endorgans. These results combined suggest that both PSA and HNK-1 carbohydrate epitopes are synthesized and may have an important role in the adult peripheral vestibular endorgans.     

Percutaneous radiofrequency ablation for unresectable large hepatic tumours during hepatic blood flow occlusion in four patients

AIM: To evaluate percutaneous radiofrequency (RF) ablation therapy for unresectable large hepatic tumours combined with regional interruption of hepatic blood flow, and to assess the safety and efficacy of this procedure. MATERIALS AND METHODS: Four patients with hepatic tumours were enrolled in this study. Patients were treated by a single session of RF ablation during occlusion of both hepatic artery and hepatic vein. Tumour size ranged from 45-57 mm (mean 50.2 mm). Initial therapeutic efficacy was evaluated with helical computed tomography (CT) performed within 9 days after the treatment. CT or magnetic resonance imaging (MRI) was performed every 2-3 months thereafter. RESULTS: The largest axis of coagulated lesions after the ablation was 50-60 mm (mean 56.5 mm) in diameter. The ablation therapy was considered complete in three patients; after a mean follow-up of 12.7 months, CT and MRI revealed complete destruction of their tumours. One patient required further treatment. No severe complications occurred. CONCLUSION: Although further studies are needed, in this limited clinical trial a local ablation area exceeding 50 mm in diameter was achieved safely.     

Isolated cranial neuropathy associated with anti-glycolipid antibodies

We describe seven patients with isolated cranial neuropathy in whom serum anti-glycolipid antibodies were detected. Trigeminal sensory neuropathy was found in four patients, who had exhibited symptoms for 2 months to 4 years. The other three patients showed facial nerve palsy with or without ophthalmoparesis. Temporal profile analysis of anti-glycolipid antibodies revealed that titers of anti-glycolipid IgM antibodies against GM2 and LM1 gradually decreased in patients having chronic trigeminal sensory neuropathy. In patients with acute trigeminal sensory neuropathy, elevation of anti-LM1 antibody titers continued over 12 months although anti-GalNAc-GD1a antibody disappeared. On the other hand, titers of anti-glycolipid antibodies rapidly decreased in patients with acute facial nerve palsy with or without ophthalmoparesis. We conclude that anti-glycolipid antibodies may play an important role in the development of isolated cranial neuropathy in some patients.     

Differential gene expressions during immortalization of normal human fibroblasts and endothelial cells transfected with human telomerase reverse transcriptase gene

It is widely accepted that telomerase, which compensates for telomere shortening, is finally activated in almost all kinds of human malignant neoplasms, and ectopic expression of telomerase may endow some kinds of human somatic cells with indefinite proliferation capacity, i.e., immortality. To clarify the intrinsic responses required in acquiring immortality, we investigated the chronological changes in the expression levels of the cell cycle and apoptosis-related genes by real-time RT-PCR in human normal fibroblasts and endothelial cells after hTERT transfection. We found that fibroblast MJ90 required intrinsic responses including reversible upregulation of cell-cycle promoting genes and down-regulation of apoptosis-inducing genes in early phase after transfection, whereas the endothelial cell HUE142-2 did not. In addition, the microarray analysis of the fibroblast strains revealed that the dysregulated genes during cellular immortalization were different from those reported in fibroblasts probably having acquired telomere maintenance mechanism concomitant with hTERT induction. These findings indicate that cell-type specific differential gene expression after telomerase activation may be important to acquire telomere-maintenance capacity and immortality in some non-cancerous human cells. Investigation of these molecules may elucidate the differences in the capacity of acquiring immortality in cancer and normal somatic cells in future.