Volume 26, No. 5/2007(Sept/Oct)
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 Clinical Neuropathology
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Review
Pathology of post traumatic brainstem and hypothalamic <br />injuries
D. Shukla, A. Mahadevan, K.V.R. Sastry and S.K. Shankar
Abstract
D. Shukla, A. Mahadevan, K.V.R. Sastry and S.K. Shankar
Departments of 1Neurosurgery and 2Neuropathology, National Institute of Mental Health & Neurosciences, Bangalore, India
Objective: Several autopsy studies of head injury are available but pathology of brainstem and hypothalamic injuries are addressed in very few of them. We studied brains of 47 patients who succumbed to head injury, with special attention to topographical distribution of brainstem and hypothalamic injuries. Material and methods: Brains retrieved at autopsy of 47 patients who succumbed to head injury following road traffic accidents (32 cases) or fall from height (15 cases) were examined for brainstem and hypothalamic injuries. Brainstem lesions were grouped into Type I (in absence of raised intracranial pressure and downward herniation) and Type II (secondary to raised ICP and herniation). Lesions were mapped topographically and examined histologically for axonal and myelin changes. Anatomical location of lesions was correlated with mode of injury, site of impact, occurrence of associated lesions, prognosis and survival. Results: Brainstem injury was noted in 36 cases (76.5%) [Type I in 7 (14.9%) and Type II in 29 cases (61.7%)]; hypothalamic injuries in 20 (42.5%), and combined hypothalamic and brainstem injuries were seen in 17 (36.1%). 11 had no brainstem/hypothalamic injuries. Brainstem hemorrhages occurred in lower midbrain and upper pons, and hypothalamic injuries involved paraventricular nuclei. In cases with combined injury to brainstem and hypothalamus, lesions involved rostral ventral mesencephalon of brainstem and paraventricular region of hypothalamus. The site of impact had no definite correlation with occurrence of brainstem or hypothalamic injuries though basal fractures and road traffic accidents were more often associated with hypothalamic injuries. Survival of cases with Type I injury was shorter than Type II (14.2 vs 65.2 hours) due to involvement of raphe/reticular nuclei. Though cases with hypothalamic injuries did not result in early death, involvement of anterior hypothalamus resulted in shorter survival (< 6 hours) compared to posterior. Diffuse axonal injury and hypothalamic involvement were more often seen with Type II brainstem injuries. Cerebral contusions were a common accompaniment of hypothalamic injuries (80%). Conclusions: Brainstem and hypothalamic injuries are seen in significant proportion of traumatic brain injuries and may have an important role in determining the survival and prognosis. Insight into mechanisms of injury to hypothalamo-brainstem axis is essential for evolving better therapeutic strategies and prevention of post traumatic long term sequelae.Correspondence to:
Dr. S.K. Shankar, MD, FAMS, FNASc; Professor and Head, Department of Neuropathology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore 560 029, India
Email: shankar@nimhans.kar.nic.in
Tumor
Peroxiredoxins and antioxidant enzymes in pilocytic astrocytomas
K. Nordfors, J. Haapasalo, P. Helén, A. Paetau, L. Paljärvi, H. Kalimo, V.L. Kinnula, Y. Soini and H. Haapasalo
Abstract
K. Nordfors, J. Haapasalo, P. Helén, A. Paetau, L. Paljärvi, H. Kalimo, V.L. Kinnula, Y. Soini and H. Haapasalo
1Department of Pathology, Center for Laboratory Medicine, Tampere University Hospital, Tampere, 2Faculty of Medicine, University of Turku, 3Unit of Neurosurgery, Tampere University Hospital, Tampere, 4Department of Pathology, Helsinki University Hospital, Helsinki, Finland, 5Department of Neuropathology, University of Uppsala, Uppsala, Sweden, 6Department of Medicine, Division of Pulmonary Medicine, University of Helsinki, Finland and 7Department of Pathology, Oulu University Hospital, Oulu, Finland
Objective: Peroxiredoxins are antioxidant enzymes (AOEs), which are redox-regulated thiol proteins with potential effects on the growth, invasion and drug resistance of neoplastic cells. In this study, their biology and clinical significance were examined in pilocytic astrocytomas (PAs). Material and methods: The expression of peroxiredoxins (Prx I-VI) was investigated in 105 PAs by the means of immunohistochemistry and compared with the expression of selected other antioxidant enzymes, cell proliferation, angiogenesis, apoptosis, p53, histopathology and patient survival. Results: Peroxiredoxins were strongly expressed in general suggesting that oxidative damage and consequent defense takes place during the progression of pilocytic astrocytomas. In agreement with this hypothesis, several other AOEs correlated with the degenerative features and angiogenesis possibly associated with reactive oxygen species-derived cellular damage. Moreover, the expression of the AOEs was associated with each other indicating a concurrent activation of the enzymes. With the exception of manganese superoxide dismutase (MnSOD), a strong expression of AOEs was generally associated with higher cell proliferation. Prx VI seemed to have a positive association with a longer recurrence-free interval while other AOEs had no association with patient survival. Many AOEs, such as MnSOD, induce chemo- and radioresistance and are highly elevated in aggressive malignancies. PAs lack this confounding factor, and these tumors are treated only by surgery. Conclusions: Taken together, the results of this study on pilocytic astrocytomas suggest that the levels of Prxs and other AOEs and their related thiol proteins are generally strongly expressed in these tumors. At least Prx VI can contribute to tumor behavior which can make it a potential prognostic factor.Correspondence to:
K. Nordfors, MB; Department of Pathology, Center for Laboratory Medicine, Tampere
University Hospital, P.O. Box 2000, 33521 Tampere, Finland
Email: kmenor@utu.fi
Tumor
Simultaneously occurring vestibular schwannoma and meningioma in the cerebellopontine angle: case report and literature review
Y. Izci, H.I. Secer, E. Gönül and Ö. Öngürü
Abstract
Y. Izci, H.I. Secer, E. Gönül and Ö. Öngürü
1Department of Neurosurgery, 2Department of Pathology, Gulhane Military Medical Academy, Ankara, Turkey
Simultaneously occurring multiple primary brain tumors of different histological types are rare, and the coexistence of schwannoma and meningioma in the same cerebellopontine angle (CPA) without neurofibromatosis is extremely rare. A 57-year-old female patient presented with headache, speech disturbance, left facial numbness and deafness in the left ear. Magnetic resonance imaging demonstrated two different tumors in the left CPA. These tumors were not in continuity. The tumors were totally removed through the left suboccipital approach. Histopathological examination revealed that the large tumor was a vestibular schwannoma and the smaller was a meningioma. Neurofibromatosis was not diagnosed in the patient. No recurrence was observed at the end of 9 years after the operation. The simultaneous occurrence of vestibular schwannoma and meningioma in the CPA appears coincidental. This association must be kept in mind if two different tumors are detected radiologically in the same CPA.Correspondence to:
Y. Izci, MD; Gulhane Askeri Tip Akademisi, Beyin ve Sinir Cerrahisi AD, 06018 Etlik-Ankara, Turkey
Email: yusufizci@yahoo.com
Tumor
Concurrent <em>EGFR</em> amplification and TP53 mutation in glioblastomas
R. Gil-Benso, C. Lopez-Gines, R. Benito, J.A. López-Guerrero, R.C. Callaghan, A. Pellín, P. Roldán and M. Cerdá-Nicolás
Abstract
R. Gil-Benso, C. Lopez-Gines, R. Benito, J.A. López-Guerrero, R.C. Callaghan, A. Pellín, P. Roldán and M. Cerdá-Nicolás
1Department of Pathology, Medical School, University of Valencia,
2Molecular Biology, Fundación Instituto Valenciano de Oncología, and
3Department of Neurosurgery, University of Valencia, Valencia, Spain
Glioblastoma multiforme is the most common and most aggressive of the primary brain tumors. The mean survival of patients is 10 – 12 months. Conventional therapy of surgery, radiation and chemotherapy is largely palliative. Cytogenetically, karyotypes of glioblastomas are very complex with trisomy 7 and monosomy 10 as the most frequent abnormalities. A genetic alteration that is significantly more frequent in primary than in secondary glioblastomas, the latter arising from preceding low-grade gliomas, is epidermal growth factor receptor gene (EGFR) amplification, whereas TP-53 mutations are significantly more frequent in low-grade gliomas and secondary glioblastomas derived therefrom. We report the histological and genetic study of two glioblastomas, one case arising de novo and the other case arising 3 years after a previously diagnosed anaplastic astrocytoma, with concurrent EGFR amplification and TP-53 mutation. These anomalies were initially deemed as mutually exclusive. However, a small percentage of cases have been found with both anomalies although at a significantly lower level than could be expected. We have analyzed these two cases cytogenetically and by molecular studies in order to detect additional alterations associated with this phenotype. Cytogenetically, both cases showed in common the monosomy of chromosomes 10 and 17. At the molecular level, a rare mutation of TP-53 was found in the secondary glioblastoma and hypermethylation of the promoter region of p16INK4a and p14ARF genes were observed in the primary and secondary glioblastoma, respectively.Correspondence to:
R. Gil-Benso, PhD; Department of Pathology, Medical School, University of Valencia, Av. Blasco Ibanez, 15, 46010 Valencia, Spain
Email: Rosario.Gil-Benso@uv.es
Dementia
Brain imaging and neuropsychology in late-onset dementia due to a novel mutation (R93C) of valosin-containing protein
S. Krause, T. Göhringer, M.C. Walter, B.G.H. Schoser, P. Reilich, J. Linn, G.E. Pöpperl, L. Frölich, F. Hentschel, H. Lochmüller and A. Danek
Abstract
S. Krause, T. Göhringer, M.C. Walter, B.G.H. Schoser, P. Reilich, J. Linn, G.E. Pöpperl, L. Frölich, F. Hentschel, H. Lochmüller and A. Danek
1Neurologische Klinik and 2Friedrich-Baur-Institut, 3Abteilung für Neuroradiologie am Institut für Diagnostische Radiologie, and 4Klinik für Nuklearmedizin, Ludwig-Maximilians-Universität München, 5Abteilung für Gerontopsychiatrie and 6Abteilung für Neuroradiologie, Zentralinstitut für Seelische Gesundheit Mannheim, Ruprecht-Karls-Universität Heidelberg, Germany
Inclusion body myopathy with Paget disease of bone and frontotemporal dementia (IBMPFD, MIM 167320) is a recently identified autosomal dominant disorder due to mutations in the valosin-containing protein (VCP) that affects muscle, bone and brain. Brain involvement and neuropsychological findings of IBMPFD have not been described in detail. A patient carried a novel heterozygous base pair change, 47832C>T, in the VCP gene that resulted in substitution of an arginine residue by cysteine at position 93 (R93C). He presented first with myopathy while bone involvement remained subclinical. The patient developed behavioral abnormalities in his 60s and showed frank personality change with fluent empty speech at the age of 74 years. This syndrome was best classified as semantic dementia. Magnetic resonance imaging disclosed slight but progressive cerebral atrophy with prominent callosal and frontal white matter loss. Positron emission tomography demonstrated glucose hypometabolism of the frontal and temporal lobes disproportionate to their structural involvement. This first comprehensive clinical and neuroimaging study in IBMPFD may raise the awareness among clinicians as well as basic scientists for this exemplary genetic model of dementia.Correspondence to:
Prof. A. Danek; Neurologische Klinik, LMU, PF 701260,
81377 München,Germany
Email: danek@lmu.de
Obituary
In memoriam Jürgen Peiffer (1922 – 2006)
W. Paulus
Abstract
W. Paulus
Institute of Neuropathology, University Hospital Münster, Münster, Germany
Correspondence to:
Werner Paulus; Institute of Neuropathology, University Hospital Münster, Domagkstr. 19, 48129 Münster, Germany
Email: werner.paulus@uni-muenster.de
Abstracts
Joint Meeting XLIII Congress of the Italian Association of Neuropathology (AINP) XXXIII Congress of the Italian Association of Research on Brain Aging (AIRIC) Verona, Italy, September 30, 2007 – October 3, 2007
Local Organizing Committee: A. Simonati, T. Cavallaro, F. Fenzi, G.M. Fabrizi, S. Ferrari, S. Monaco, A. Salviati, G. Tomelleri, P. Tonin, G. Vattemi and G.L. Zanusso
Abstract
Local Organizing Committee: A. Simonati, T. Cavallaro, F. Fenzi, G.M. Fabrizi, S. Ferrari, S. Monaco, A. Salviati, G. Tomelleri, P. Tonin, G. Vattemi and G.L. Zanusso
Book reviews
Forensic Neuropathology and Associated Neurology /
Clinical Neuropathology. Text and Color Atlas
R.O. Welle and P.G. Ince
Abstract
R.O. Welle and P.G. Ince
Announcement
A European Board
Examination in Neuropathology
D. Troost and M. Graeber
Abstract
D. Troost and M. Graeber
Euro-CNS News
Society News of the European Confederation of Neuropathological Societies
Euro-CNS
