[A Case of Wernicke's Encephalopathy Presenting with Acute Deterioration of Consciousness Caused by Peripheral Parenteral Nutrition].

We report the case of a 67-year-old woman with Wernicke's encephalopathy(WE), who had been suffering from repeated vomiting and poor oral intake due to both reflux esophagitis and atrophic gastritis. She presented with altered of consciousness, horizontal nystagmus, and gait disturbance, and acute deterioration of consciousness was observed after starting peripheral parenteral nutrition (PPN). Brain MRI showed bilateral high intensity lesions in the medial thalamus and the dorsal midbrain on FLAIR and T2-weighted images. Although brain MRI characteristics are useful for diagnosing WE, it is possible that there are no abnormal MRI findings in its early stages. In addition, only 10-20% of WE cases present with the classical clinical triad of confusion, ophthalmoplegia, and ataxia. Therefore, confirming the diagnosis can be challenging. In general, rapid improvement of symptoms is observed with prompt vitamin B1 supplementation. However, delays in treatment can result in irreversible amnesia and ataxia. Furthermore, in the state of vitamin B1 deficiency, even PPN, not just total parenteral nutrition, can worsen symptoms, and this deterioration is attributed to the glucose load.

[The study of spinal abnormalities associated with cloacal exstrophy].

The treatment of spinal abnormalities with cloacal exstrophy is controversial. Ten cases of this complex treated at Hyogo Prefectural Kobe Children's Hospital between 1991 and 2010 are presented. In our series, all 10 patients had tethered spinal cords. In addition, there were 3 terminal myelocystoceles, 2 meningoceles, 7 lipomas, 5 thickened filums and 3 syrinxes. Eight of 10 patients underwent surgery, and no patient deteriorated. All 3 patients with terminal myelocystocele had lower extremity weakness, but motor functions in two patients improved after surgery. All 4 patients with lipoma and/or thickened filum were asymptomatic. Two of them were conservatively treated, and they remain asymptomatic. Terminal myelocystocele and symptomatic syrinx should be surgically treated.

[Spinal subdural abscess in the cervical region: a case report].

The authors present a patient with a spinal subdural abscess (SSA) in the cervical region and review the relevant literature. A 48-year-old man suffering from intractable high fever and back pain was admitted to our hospital with a diagnosis of meningitis. Despite antibiotic therapy, his condition deteriorated and he developed neurological deficits including left hemiparesis, sensory disturbance and bladder dysfunction. MR images of the cervical spine with gadolinium contrast revealed a circumferentially enhancing lesion anterior to the spinal cord that extended from the C4 to C6 level and compressed the spinal cord. After an urgent laminectomy extending from C4 to C6, the subdural abscess that consisted of purulent material and a thick capsule was irrigated and drained. Staphylococcus aureus was cultured from the abscess and he received antibiotic therapy postoperatively for 14 weeks. The high fever and the back pain subsided immediately and his neurologic condition gradually recovered. The majority of SSA cases involve the thoracic or lumbar region and are rarely found in the cervical region. Because they are associated with a high morbidity, early diagnosis with MRI and urgent surgical interventions including decompressive laminectomy, copious irrigation and drainage followed by appropriate antibiotic therapy are vital.

Three-dimensional relationship between the conus branch and the precordial leads confirmed by 64-multidetector-row computed tomography.

A 65-year-old man with effort angina pectoris underwent percutaneous coronary intervention of the proximal right coronary artery. The lesion was dilated with a bare-metal stent under wire protection of the conus branch (CB). However, the jailed CB was occluded. Electrocardiogram with conventional precordial leads (V(1) through V(6)) accompanied with the supplementary leads (V(1) through V(6)) positioned 1 intercostal space higher showed marked ST elevation in V(1) through V(3) that was more prominent in V(1) through V(3). The 64-multidetector-row computed tomographic coronary angiography showed recanalization of the CB located just in the center of the V(1), V(2), V(1), and V(2) electrodes.

A case of cerebellar capillary hemangioma with multiple cysts.

Intracranial capillary hemangiomas are very rare, though several spinal capillary hemangiomas have recently been reported. We report here a case of intracranial capillary hemangioma with multiple cysts and review the current literature of similar cases. A 4-month-old girl was referred to our hospital for treatment of hydrocephalus and a cerebellar mass lesion. She presented with hemangiomas distributed widely over the body, as well as disseminated hemangiomas in the pleura, liver, spleen, pancreas, kidneys and vagina. Pathological examination of the specimen from the vagina confirmed the diagnosis of a capillary hemangioma made at another hospital. Radiological examination of the brain revealed a cystic mass lesion in the left cerebellar hemisphere with subsequent obstructive hydrocephalus. The cysts extended upward into the bilateral ventricle. Following neuroendoscopic fenestration of the cysts, resection of the left cerebellar mass was performed. Histological examination of the lesion demonstrated similarly sized capillaries, and the pathological diagnosis was confirmed as capillary hemangioma. There was no recurrence postoperatively. Our patient was treated successfully by surgical resection and neuroendoscopic procedures. Surgical intervention may therefore be indicated in intracranial capillary hemangiomas.

[Management of chiasmatic-hypothalamic gliomas in children: report of nine pediatric cases].

Radical resection of chiasmatic-hypothalamic glioma (CHG) carries a significant risk of morbidity and the optimum treatment remains undecided. The authors reported 9 children with CHG, who were treated with surgical resection with or without postoperative chemotherapy. Age at the time of diagnosis ranged from 4 months to 7.7 years (mean 3.1 years), and no patient had evidence of neurofibromatosis type 1. Surgical resections of the tumors were performed in all patients because of severe visual impairment or intracranial hypertension caused by large tumors. All of the surgical interventions resulted in partial resections. Pathological examination revealed pilocytic astrocytomas in 7 patients, low grade astrocytoma in 1 and anaplastic astrocytoma in 1. Seven patients with residual tumors received postoperative chemotherapy consisting of cisplatin, cyclophosphamide, etoposide and vincristine. Reduction in tumor size was noticed in 5 patients, although 2 patients had no response and switched to local radiotherapy. Although minor complications of chemotherapy were noticed in 5 patients, severe sequelae such as neuropsychological deficits or endocrinopathies did not occur, and all patients completed chemotherapy programs. Additional treatments are recommended in case of incomplete tumor resections, because our experience demonstrates that the majority of the residual tumors have potential to progress. Our present data suggests that the chemotherapy of the aforementioned regimen is effective in controlling CHGs after partial resections and is relatively well tolerated even in young children who are vulnerable to radiotherapy.

[Ganglioglioma of the medulla oblongata: case report and review of the literature].

The authors present a pediatric case of ganglioglioma occurring in the medulla oblongata. A 7-year-old boy was referred to our hospital with complaints of ataxia, seizure and sleep apnea. MRI of the brain disclosed a large tumor occupying the medulla oblongata, and the upper portion of the cervical spinal cord was also involved. The patient underwent midline suboccipital craniotomy and laminectomy of C1 to attempt radical resection of the tumor, which resulted only in partial removal of the tumor due to severe bradycardia during the operation. The histological diagnosis was ganglioglioma, WHO grade 2. Although both radiotherapy and chemotherapy were performed following the operation, the tumor remained unchanged. The patient died of respiratory arrest five months after the operation. Gangliogliomas usually occur in the supratentorial region, which permits easy surgical access and good prognosis. Only 3% of gangliogliomas occur in the brain stem, and its management can be challenging because of the difficulty of radical resection and poor response to both radiotherapy and chemotherapy.

Intracranial hemorrhage and vitamin K deficiency associated with biliary atresia: summary of 15 cases and review of the literature.

Biliary atresia (BA) is a rare disease, characterized by progressive and obliterative cholangiopathy, and is one of the major causes of secondary vitamin K deficiency in infancy. We describe 15 infants (10 female, 5 male) with BA, presenting with intracranial hemorrhage (ICH), including 10 subdural hemorrhages, 4 subarachnoid hemorrhages, 2 intraventricular hemorrhages, and 1 intraparenchymal hemorrhage. The age at onset of ICH ranged from 26 to 79 (mean 54.2) days. Eight patients underwent successful surgical evacuation of ICH, following administration of vitamin K. All 15 patients underwent Kasai portoenterostomy for BA 8-30 days after onset. During a mean follow-up period of 86.8 (range 2-352) months, 4 patients died of liver failure despite lack of neurological sequelae. Two patients underwent living-related donor and 1 patient living-unrelated donor liver transplantation. Only 2 patients suffered neurological signs and symptoms, including mental retardation and epilepsy, whereas 3 were noted to have temporary hemiparesis which recovered completely during the follow-up period. The possibility of BA should be considered in the treatment of ICH due to vitamin K deficiency, since it is reported to be one of the major causes of secondary vitamin K deficiency. Urgent surgical intervention for ICH can be performed successfully following sufficient administration of vitamin K or fresh frozen human plasma. Moreover, early performance of Kasai portoenterostomy is possible even for patients who have undergone craniotomy.

[A pediatric case of multiple sclerosis mimicking malignant brain tumor].

We report a pediatric case of multiple sclerosis mimicking malignant brain tumor. A 11-year-old male presented with high fever, vomiting and partial convulsion of left lower extremity followed by left visual impairment. Magnetic resonance imaging (MRI) revealed a mass lesion in the right frontal lobe with significant peripheral edema. Incomplete ring-like enhancement by gadolinium administration was also noted. Open biopsy of the lesion was undertaken that provided pathological confirmation of demyelinating changes. Luxol fast blue stain and Bodian stain demonstrated extensive myelin loss and good preservation of axons. The patient was given intravenous high-dose methyl-prednisolone and sequential oral low-dose prednisone, which improved his neurological status progressively. The lesion was dramatically decreased in size and finally disappeared. Although many tumor-like demyelinating lesions were reported previously, pediatric cases of multiple sclerosis mimicking malignant brain tumor are extremely rare. Pathological examinations including Luxol fast blue stain and Bodian stain are essential for exact diagnosis.

Adenovirus-mediated overexpression of diacylglycerol kinase-zeta inhibits endothelin-1-induced cardiomyocyte hypertrophy.

BACKGROUND: Diacylglycerol (DAG) is a lipid second messenger that transiently accumulates in cells stimulated by endothelin-1 (ET-1) and other Galphaq protein-coupled receptor agonists. Diacylglycerol kinase (DGK) is thought to be an enzyme that controls the cellular levels of DAG by converting it to phosphatidic acid; however, the functional role of DGK has not been examined in cardiomyocytes. Because DGK inactivates DAG, a strong activator of protein kinase C (PKC), we hypothesized that DGK inhibited ET-1-induced activation of a DAG-PKC signaling cascade and subsequent cardiomyocyte hypertrophy. METHODS AND RESULTS: Real-time reverse transcription-polymerase chain reaction demonstrated a significant increase of DGK-zeta mRNA by ET-1 in cardiomyocytes. To determine the functional role of DGK-zeta, we overexpressed DGK-zeta in cardiomyocytes using a recombinant adenovirus encoding rat DGK-zeta (Ad-DGKzeta). ET-1-induced translocation of PKC-epsilon was blocked by Ad-DGKzeta (P<0.01). Ad-DGKzeta also inhibited ET-1-induced activation of extracellular signal-regulated kinase (P<0.01). Luciferase reporter assay revealed that ET-1-mediated increase of activator protein-1 (AP1) DNA-binding activity was significantly inhibited by DGK-zeta (P<0.01). In cardiomyocytes transfected with DGK-zeta, ET-1 failed to cause gene induction of atrial natriuretic factor, increases in [3H]-leucine uptake, and increases in cardiomyocyte surface area. CONCLUSIONS: We demonstrated for the first time that DGK-zeta blocked ET-1-induced activation of the PKC-epsilon-ERK-AP1 signaling pathway, atrial natriuretic factor gene induction, and resultant cardiomyocyte hypertrophy. DGK-zeta might act as a negative regulator of hypertrophic program in response to ET-1, possibly by controlling cellular DAG levels.

Dynamic 123I-BMIPP single-photon emission computed tomography in patients with congestive heart failure: effect of angiotensin II type-1 receptor blockade.

BACKGROUND: Heart failure is a major and growing public health problem with a high mortality rate. Although recent studies have demonstrated that a variety of metabolic and/or neurohumoral factors are involved in the progression of this syndrome, the precise mechanisms responsible for this complex condition are poorly understood. HYPOTHESIS: To examine 123I-beta-methyl-iodophenylpentadecanoic acid (BMIPP) kinetics in the early phase soon after tracer injection in patients with congestive heart failure (CHF), we performed dynamic single-photon emission computed tomography (SPECT). METHODS: Twenty-six patients with CHF and eight control subjects were examined. The consecutive 15 images of 2-min dynamic SPECT were acquired for 30 min after injection. In the early phase after injection (0-4 min), a significant amount of radioactivity existed in the blood pool. After 6 min, the myocardial 123I-BMIPP image was clear and thus the washout rate of 123I-BMIPP from 6 to 30 min was calculated. RESULTS: The washout rate of 123I-BMIPP from the myocardium was faster in patients with CHF than in the controls (8 +/- 4 vs. -5 +/- 3%, p < 0.01). The washout rate of 123I-BMIPP demonstrated positive correlation with left ventricular (LV) end-diastolic volume index (R = 0.54, p < 0.02) and inverse correlation with LV ejection fraction (R = 0.53, p <0.02). Patients were given the angiotensin II type-1 receptor antagonist candesartan for 6 months, and dynamic SPECT was repeated. The enhanced washout rate of 123I-BMIPP in CHF was reduced after treatment with candesartan (p < 0.05). CONCLUSION: These data suggest that (1) enhanced washout of 123I-BMIPP was observed soon after injection in patients with CHF, (2) the activation of angiotensin II signaling pathway is involved as an intracellular mechanism for enhanced 123I-BMIPP washout in heart failure, and (3) improvement in fatty acid metabolism may represent a new mechanism for beneficial effects of angiotensin II receptor blockade on cardiac function and survival in patients with heart failure. 123I-BMIPP washout in the early phase obtained from dynamic SPECT may be a new marker for evaluating the severity of heart failure and the effects of medical treatment.