Volume 27, No. 2/2008(March/April)
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 Clinical Neuropathology
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Epilepsy
Expression of monocyte chemoattractant protein-1 in brain tissue of patients with intractable epilepsy
Y. Wu, X. Wang, X. Mo, Z. Xi, F. Xiao, J. Li, X. Zhu, G. Luan, Y. Wang, Y. Li and J. Zhang
Abstract
Y. Wu, X. Wang, X. Mo, Z. Xi1, F. Xiao, J. Li, X. Zhu, G. Luan, Y. Wang, Y. Li and J. Zhang
1Department of Neurology, The First Affiliated Hospital, Chongqing Medical University, Chongqing, 2Neurology Department of the First Affiliated Hospital, Guangxi Medical University, Nanning, 3Beijing Tiantan Hospital of the Capital University of Medical Sciences, 4Beijing Xuanwu Hospital of the Capital University of Medical Sciences, Beijing, China
Objective: To investigate the expression of monocyte chemoattractant protein-1 (MCP-1) in the brain tissue of patients with intractable epilepsy (IE) and to explore its role in the pathogenesis of IE. Material: Patients with IE were collected from the Department of Neurosurgery of several Chinese hospitals between 2002 and 2005. The average age of these 40 patients was 23.65 ± 10.14 (X ± SD) years (11 – 58 years), with 19 males and 21 females. In addition to the typical symptoms and distinct EEG features of epilepsy, all recruited patients met the requirements of intractable epilepsy. Methods: On the basis of positive results obtained from gene chip analysis, the expression of MCP-1 was first investigated in the brain tissue of IE patients (40 cases) using Western Blotting and immunohistochemistry and then compared with the controls. Results: Gene chip scanning demonstrated that expression of MCP-1 mRNA was upregulated in the brain tissue of patients with IE. Western Blotting and immunohistochemical experiments further confirmed that, as compared with that in the control group, expression of MCP-1 protein was significantly increased in the IE patients (p < 0.05). Conclusion: These results suggest that MCP-1 are involved in the pathogenesis of IE.Correspondence to:
Dr. X. Wang; Department of Neurology, The First Affiliated Hospital, Chongqing Medical University, Chongqing 400016, China
Email: wuyuan90@126.com
Childhood Neurodegeneration
Early onset Alexander disease: a case report with evidence for manifestation of the disorder in neurohypophyseal pituicytes
R. Matej, L. Dvoráková, L. Mrázová, H. Houst’ková and M. Elleder
Abstract
R. Matej, L. Dvoráková, L. Mrázová, H. Houst’ková and M. Elleder
1Department of Pathology, Thomayer Teaching Hospital, 2Department of Pediatrics, 1st Faculty of Medicine and Thomayer Teaching Hospital, 3Institute of Inherited Metabolic Disorders, Charles University, 1st Faculty of Medicine and University Hospital, Prague, Czech Republic
We report the first case of Alexander disease diagnosed and published in the region of former Czechoslovakia. The case was characterized by early (late infantile) onset, the absence of megacephaly but with extensive internal hydrocephaly, despite a patent aqueduct. Neuropathology revealed severe depletion of oligodendroglia and myelin, loss of axons, prominent astrocytosis with massive intracellular, dense globular GFAP aggregates which differed from typical Rosenthal fibers. Additionally, many large aggregates of GFAP were located extracellularly. Globular GFAP aggregates were also identified in neurohypophyseal pituicytes. DNA analysis disclosed a heterozygous mutation c.1117G>A in the GFAP, which is predicted to lead to the amino acid exchange p.Glu373Lys (E373K) in the C-terminal tail of the GFAP protein. The parents and a healthy sister did not show any variation in GFAP in somatic cells.Correspondence to:
M. Elleder, MD, PhD; Institute of Inherited Metabolic Disorders, Bldg. D, Division B, Ke Karlovu 2, 128 08 Prague 2, Czech Republic
Email: melleder@cesnet.cz
Tumor
Papillary glioneuronal tumor – contribution to a new tumor entity and literature review
S.P. Guo, F. Zhang, Q.L. Li, Q. Li, W.L. Wang and F.F. Li
Abstract
S.P. Guo, F. Zhang, Q.L. Li, Q. Li, W.L. Wang and F.F. Li
Department of Pathology, The Fourth Military Medical University, Xi an,
Shan Xi province, China
Papillary glioneuronal tumor (PGNT) is a recently identified low-grade mixed glial-neuronal neoplasm of juvenile and young adult patients. The WHO classification does not categorize this tumor as a separate entity, but rather considers it as a variant of ganglioglioma. We present a new case of this rare entity, representing the 3rd case of this lesion in Chinese patients and review the findings in 34 patients from different case reports found in the international literature. This report describes a histologically similar-appearing lesion arising in the left frontoparietal lobe of a 23-year-old man. Its salient morphological characteristics are the presence of pseudopapillary structures composed of blood vessels, often hyalinized, lined by uniform small astrocytes and a proliferation of neurocytic cells which eventually admixed with ganglioid and ganglion cells. Sporadic Rosenthal fibers, foci of calcification, areas of hemosiderin deposition were identified. The mean Ki67 labeling index remained below 1%. Signs of anaplasia, in particular mitotic figures, endothelial proliferation or necrosis were consistently lacking. It is important that every new case of PGNT is reported to allow its recognition and classification. We perceive PGNT as a clinically and morphologically well-delineated subgroup of extraventricular low malignant potential neoplasm, whose presentation may allow for consideration as an entity.Correspondence to:
Prof. Q. Li; Department of Pathology, the Fourth Military Medical niversity, 710032 Xian, Shan Xi Province, China
Email: liqing@fmmu.edu.cn
Tumor
Congenital ependymoblastoma arising in the sacrococcygeal soft tissue: a case study
M. Santi, D. Bulas, R. Fasano, T. Ponsky, A. Sandler E. Richter and E.J. Rushing
Abstract
M. Santi, D. Bulas, R. Fasano, T. Ponsky, A. Sandler E. Richter and E.J. Rushing
Divisions of 1Pathology, 2Radiology, 3Hematology Oncology, 4General Surgery, Children’s National Medical Center, Washington, DC and 5Department of Neuropathology and Ophthalmic Pathology, Armed Forces Institute of Pathology, Washington, DC, USA
Ependymoblastomas are distinct embryonal tumors of the central nervous system reported only rarely in the literature. Most examples arise in young children under the age of 2 years, in the supratentorial compartment, and may or may not be related to the ventricular system. We report the case of a one-day-old infant who presented with a 6.4 × 5.6 × 3.5 cm ruptured buttock mass. Ultrasound demonstrated a solid mass at the base of the spine that displaced the bladder anteriorly with resultant hydronephrosis. Magnetic resonance images confirmed the presence of a solid mass surrounding the lower sacrum with an internal component partially encircling and deviating the rectum. Histopathological evaluation confirmed the diagnosis of ependymoblastoma. Of note, immunohistochemical analysis revealed diffuse staining with vimentin and patchy expression of synaptophysin, glial fibrillary acidic protein, neurofilament proteins, neuron-specific enolase, CD99 and nestin. On the 42nd day of life, chemotherapy was initiated with a modified Children’s Oncology Group (COG) AGCT01P1 (cyclophosphamide, cisplatin, 70% etoposide, no bleomycin) regimen. The authors describe their experience and review the literature, emphasizing that ependymoblastomas should be considered in the differential diagnosis of sacral masses in the newborn.Correspondence to:
E.J. Rushing, MD; Department of Neuropathology, Armed Forces Institute of Pathology Washington, DC, 20306-6000, USA
Email: rushinge@afip.osd.mil
Tumor
DNA topoisomerase II-α as a proliferation marker in human gliomas: correlation with PCNA expression and patient survival
H. Zhao, H. Yu, Y. Liu, Y. Wang and W. Cai
Abstract
H. Zhao, H. Yu, Y. Liu, Y. Wang and W. Cai
1Department of Neurosurgery, 2Congenital Malformation Laboratory, Department of Pediatric Surgery, The Second Affiliated Hospital (Shengjing Hospital), China Medical University, 3Department of Neurosurgery, The First Affiliated Hospital, China Medical University, Shenyang, China
Objective: DNA topoisomerase II-α (Topo-IIα) is the inducible form of the enzyme responsible for the first step in the modification of DNA topology. Topo-IIα upregulation has been demonstrated in different tumors. Topo-IIα products may modulate tumoral growth, metastasis and immunosuppression, inhibit apoptosis and cause resistance to chemotherapy. Moreover, the antitumoral effect of Topo-IIα inhibitors has been documented. Material and methods: We studied the immunohistochemical expression and the prognostic value of Topo-IIα on 57 surgical specimens of glioma. Furthermore, we evaluated the correlation between the immunohistochemical expression of Topo-IIa and proliferating cell nuclear antigen (PCNA). Results: A statistically significant correlation with survival time was found, there was no statistically significant difference in survival between patients receiving or not receiving carmustine-based combined chemotherapy (p > 0.05), regardless of histological type. A significant correlation between Topo-IIα and PCNA was documented (r = 0.9245, p < 0.001). Conclusions: Our findings show that gliomas immunohistochemically express Topo-IIa that is correlated with PCNA expression, and which is significantly less frequent in long survivors. The presence of a statistical correlation with survival time and tumor histological grade encourages further studies on a larger series to verify the prognostic value of Topo-IIα expression in gliomas.
Correspondence to:
W. Cai, MD; Congenital Malformation Laboratory, Department of Pediatric Surgery, The Second Affiliated Hospital (Shengjing Hospital), China
Medical University, 36 Sanhao Street, Heping District, Shenyang, China, 110004
Email: cailab9@hotmail.com
Tumor
Primary anaplastic ganglioglioma with a small-cell glioblastoma component
J. Schittenhelm, G. Reifenberger, R. Ritz, T. Nägele, M. Weller, G. Pantazis, D. Capper, R. Beschorner, R. Meyermann and M. Mittelbronn
Abstract
J. Schittenhelm, G. Reifenberger, R. Ritz, T. Nägele, M. Weller, G. Pantazis, D. Capper, R. Beschorner, R. Meyermann and M. Mittelbronn
1Institute of Brain Research, Eberhard Karls University Tübingen, 2Brain Tumor Reference Center, Department of Neuropathology, Heinrich Heine University Düsseldorf, 3Department of Neurosurgery, 4Department of Neuroradiology and 5Department of Neurology, Eberhard Karls University Tübingen, Germany
Gangliogliomas usually present as benign tumors corresponding to World Health Organization (WHO) Grade I. Very rarely, gangliogliomas show histological features of malignancy and are then classified as anaplastic gangliogliomas of WHO Grade III or IV. In most cases, anaplastic gangliogliomas developed after radiation therapy or progression from a pre-existing low-grade ganglioglioma. Here, we report the case of a 77-year-old male patient who was operated on a primary ganglioglioma with a highly anaplastic glial component corresponding to a small-cell glioblastoma. To our knowledge, this is the first reported case of a primary anaplastic ganglioglioma with a small-cell glioblastoma component.Correspondence to:
Dr. J. Schittenhelm; Institute of Brain Research, University Tübingen,
Calwerstr. 3, 72076 Tübingen, Germany
Email: jens.schittenhelm@med.uni-tuebingen.de
PNS Disorders
Sarcoid neuropathy: clinicopathological study of 4 new cases and review of the <br />literature
A. Vital, A. Lagueny, X. Ferrer, P. Louiset, M.-H. Canron and C. Vital
Abstract
A. Vital, A. Lagueny, X. Ferrer, P. Louiset, M.-H. Canron and C. Vital
1Neuropathology Department, 2Neurology Department, University Victor Segalen – Bordeaux 2, France
There are several reviews devoted to neurosarcoidosis and a few reports restricted to sarcoid neuropathy. Since 1989, we have investigated 4 new cases of sarcoid neuropathy, 1 with chronic sensory motor neuropathy (CSMN), another with painful neuropathy and 2 with atypical chronic inflammatory demyelinating polyneuropathy (CIDP). In each case, biopsy specimens from the superficial peroneal nerve and peroneus brevis muscle were taken by the same cutaneous incision and studied on paraffin sections, semi-thin sections and under electron microscope. We compared neuropathological findings from our 4 cases with those from 34 well-studied nerve biopsies previously reported in the literature, and which concerned 16 cases of CSMN, 13 cases of mononeuropathy multiplex, 2 cases of painful neuropathy and three cases of CIDP. In all of these 38 cases of sarcoid neuropathy, the characteristic noncaseiting granulomas (NCG) were observed on the nerve in 11 cases, on the muscle alone in 5, on both muscle and nerve in 10, and in the nerve and another parenchyma in 4. In the 8 remaining cases, NCG were observed in another parenchyma, mainly lung or lymph nodes. Moreover, necrotizing vasculitis was present in nerve biopsies from 8 cases and microvasculitis without obvious necrosis in 2 others. Nerve fiber lesions, which are mainly axonal, are probably related to mechanical compression by NCG and/or to an ischemic process due to vasculitis. Cytokines and immune factors may also play a role, especially in certain cases with a clinical presentation of CIDP.Correspondence to:
Dr. A. Vital; Laboratoire de Neuropathologie BP 42, Université Victor Segalen – Bordeaux 2, 146, rue Léo-Saignat, 33076 Bordeaux cedex, France
Email: anne.vital@chu-bordeaux.fr
Meeting Abstracts
Meeting, Barcelona, Spain, November 23 – 24, 2007
Scientific Committee: T. Tuñon, President and F. García-Bragado, Secretary
Abstract
Scientific Committee: T. Tuñon, President and F. García-Bragado, Secretary
Letters to the Editor
Genetic testing for all forms of myotubular/centronuclear myopathy
P.M. Foye
Letters to the Editor
Reply to P.M. Foye
G. Siciliano, M. Mancuso, G. Alì, S. Pistolesi and G. Fontanini
Abstract
G. Siciliano, M. Mancuso, G. Alì, S. Pistolesi and G. Fontanini
Euro-CNS News
Society News of the European Confederation of Neuropathological Societies
Euro-CNS
