Atypical extraventricular neurocytoma

Extraventricular neurocytoma (EVN) is a rare brain tumor that poses diagnostic difficulty. Described herein is a case of atypical EVN arising in a 54-year-old woman. A well-circumscribed lesion (3.0 x 3.0 x 3.0 cm) in the right parietal lobe showed diffuse proliferation of monotonous tumor cells with perinuclear clearing within a delicate fibrillary matrix similar to neuropil. Tumor also showed vascular proliferation and high mitotic activity. Immunohistochemically, these tumor cells were strongly positive for synaptophysin both in the neuropil and in the perinuclear cytoplasm, and were negative for glial fibrillary acidic protein and Olig2. Ki-67 labeling index was 13.0% in the most stained areas, but accumulation of p53 was not observed. These findings were compatible with those of EVN with histological atypia. EVN should be considered as a candidate in the differential diagnosis of parenchymal brain tumor, especially oligodendroglioma. The important features are the delicate fibrillary matrix similar to neuropil, diffuse and strong immunoreactivity for synaptophysin, and negative immunoreactivity for Olig2. High proliferative activity without accumulation of p53 suggests that other factors are involved in oncogenesis of atypical EVN.     

A case surviving for over a year of renal tubular dysgenesis with compound heterozygous angiotensinogen gene mutations

Renal tubular dysgenesis (RTD) is a developmental abnormality of the renal proximal tubules found in patients with Potter syndrome. We report a female newborn with RTD who has survived for more than 18 months. Infusions of fresh frozen plasma (FFP) in the early neonatal period were effective in raising and maintaining her blood pressure. Peritoneal dialysis was required until the appearance of spontaneous urination at 29 days after birth. Histopathological examinations of the kidney revealed dilated renal tubular lumina and foamy columnar epithelial cells in the renal tubules. Endocrinological studies showed a discrepancy between low plasma renin activity (<0.1 ng/ml/hr) and high active renin concentration (135,000 pg/ml), suggesting an aberration in the renin substrate, angiotensinogen. Direct sequencing analysis revealed two novel mutations in the coding region of the angiotensinogen gene (AGT): a nonsense mutation in exon 2 (c.604C > T) and a frameshift deletion at nucleotide 1290 in exon 5 (c.1290delT). The mutations were in the compound heterozygous state, because each parent had each mutation. These findings suggest that angiotensinogen deficiency is one of the causes of RTD. A treatment of the condition with FFP may help to promote long survival.     

Field potential recording in the ventral tegmental area: pharmacological and toxicological evaluations of postsynaptic dopaminergic neuron activity

Addictive drugs and psychologic stress influence the input strength of ventral tegmental area (VTA) neurons, which implies the involvement of synaptic plasticity in dopaminergic neurons. Properties of excitatory synaptic transmission to the dopaminergic neurons have been analyzed using intracellular and patch-clamp recording methods. In the present study, we attempted to establish the field recording procedure in VTA slice preparations to monitor excitatory synaptic transmission. We evaluated this procedure using slice preparations from 6-hydroxydopamine (6-OHDA)-treated animals. In horizontal slices containing the VTA, electrical stimulation of anterior afferent fibers produced two distinct negative field potentials, presumably a fiber volley component and a transsynaptic component. Pharmacological analysis revealed that the transsynaptic component was composed of bicuculline-sensitive and CNQX-sensitive components. Neonatal 6-OHDA administration reduced approximately 90% of tyrosine hydroxylase expression in the VTA and eliminated more than 50% of the transsynaptic components. This result suggests that at least 50% of the observed transsynaptic component reflected the postsynaptic responses of the dopaminergic neurons.     

Reduced serum levels of brain-derived neurotrophic factor in adult male patients with autism

BACKGROUND: The precise mechanisms underlying the pathophysiology of autism are currently unknown. Given the key role of brain-derived neurotrophic factor (BDNF) in brain development, we hypothesized that BDNF may play a role in the pathophysiology of autism. In this study, we studied whether serum levels of BDNF are altered in patients with autism. METHODS: We measured serum levels of BDNF in 18 adult male patients with autism and 18 age-matched healthy male control subjects. RESULTS: The serum levels of BDNF in patients with autism (25.6+/-2.15 ng/ml (mean+/-S.D.)) were significantly (z = -4.42, p < 0.001) lower than those of normal controls (61.6+/-10.9 ng/ml (mean+/-S.D.)). Nevertheless, we found no correlations between BDNF levels and clinical variables in autistic patients. CONCLUSIONS: This study suggests that reduced BDNF levels may play a role in the pathophysiology of autism.     

Hemophagocytic syndrome associated with clostridial infection in a pancreatic carcinoma patient

This report describes the autopsy case of a 71-year-old man presenting with clostridial infection and hemophagocytic syndrome (HS). The patient underwent pancreatoduodenectomy for a pancreatic tumor, and a histological examination revealed an invasive ductal adenocarcinoma. Multiple peritoneal metastases were noted when laparotomy was performed because of postoperative ileus 2 months after the initial operation. Then, acutely progressive anemia associated with fever developed in the patient before death. The autopsy revealed advanced cancer dissemination and HS. In addition, systemic spread of clostridium, confirmed by the polymerase chain reaction method, had resulted in generalized bleb formation. The clostridial infection appeared to be responsible for the HS. This case indicates that HS may occur as a result of clostridial infection.     

Finite element analysis of the stresses around endosseous implants in various reconstructed mandibular models.

PURPOSE: To calculate the location and intensity of the maximum stresses occurring around implants in the reconstructed mandible. MATERIAL AND METHODS: Human preserved mandibles and fibulae were used to design 3-D mandibular models on a Desktop Computer. Various 3-D simulation models representing mandibles were made. Implants (fixtures) and superstructures were then embedded in each model and occlusal pressure was applied to the cantilever portion of the superstructure. Von Mises stress, the maximum and minimum principal stress on each model were calculated using finite element structural analysis software. Finally, evaluation of the stresses around each fixture hole was performed. RESULTS: It appeared that for each fixture hole, the direction and the magnitude of the stresses were influenced by complex factors such as the thickness of the cortical bone where the fixtures were embedded, the degree of vertical displacement under load, and the relationship between adjacent fixtures. CONCLUSION: Location and intensity of the stresses occurring around fixtures differs significantly between various types of mandibular reconstruction.Copyright 2002 European Association for Cranio-Maxillofacial Surgery. Published by Elsevier Science Ltd.     

Roles played by histamine in strenuous or prolonged masseter muscle activity in mice

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Copy-neutral loss of heterozygosity is prevalent and a late event in the pathogenesis of FLT3/ITD AML

Patients with high FLT3 internal tandem duplication allelic ratios (FLT3/ITD-ARs) have a poor prognosis. Single-nucleotide polymorphism/comparative genomic hybridization, single-cell PCR and colony-forming assays were used to evaluate genotypic evolution of high FLT3/ITD-ARs in 85 acute myeloid leukemia (AML) patients. Microarrays were used to examine molecular pathways disrupted in leukemic blasts with high FLT3/ITD-ARs. Copy-neutral loss of heterozygosity (CN-LOH) was identified at the FLT3 locus in diagnostic samples with high FLT3/ITD-ARs (N=11), but not in samples with low FLT3/ITD-ARs (N=24), FLT3-activating loop mutations (N=11) or wild-type FLT3 (N=39). Single-cell assays showed that homozygous FLT3/ITD genotype was present in subsets of leukemic blasts at diagnosis but became the dominant clone at relapse. Less differentiated CD34⁺/CD33⁻ progenitor colonies were heterozygous for FLT3/ITD, whereas more differentiated CD34⁺/CD33⁺ progenitor colonies were homozygous for FLT3/ITD. Expression profiling revealed that samples harboring high FLT3/ITD-ARs aberrantly expressed genes within the recombination/DNA repair pathway. Thus, the development of CN-LOH at the FLT3 locus, which results in high FLT3/ITD-ARs, likely represents a late genomic event that occurs after the acquisition of the FLT3/ITD. Although the etiology underlying the development of CN-LOH remains to be clarified, the disruption in recombination/DNA repair pathway, which is present before the development of LOH, may have a role.     

Ankle arthrodesis combined with tibial lengthening using the Ilizarov apparatus

 We report our experience using the Ilizarov method to perform combined ankle arthrodesis and tibial lengthening in six patients (mean age 47 years; range 25–66 years). The average distraction length was 4.1 cm (range 1.1–6.8 cm), and the mean period of follow-up was 36 months (range 26–44 months). Three patients had active infection of the ankle. Four patients had undergone previous surgery, two of which were primary ankle arthrodeses. We performed compression-distraction in three patients and bone transport in three. In the compression-distraction group, the mean length gained was 1.9 cm, the mean external fixation index (EFI) was 144 days/cm, and the mean external fixation time was 246 days. In the bone transport group, the mean length gained was 6.2 cm, the mean EFI was 35.4 days/cm, and the mean external fixation time was 233 days. All cases achieved a good clinical result with a solid ankle arthrodesis and no infection, deformity, or need for additional support. The Ilizarov method may be practically applied for ankle arthrodesis, especially in complicated cases. The EFI and external fixation time tended to increase for patients with a length gain of 3 cm or less. Received: January 16, 2002 / Accepted: August 1, 2002 Offprint requests to: H. Tsuchiya