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Abstract

N. Sinis¹, A. Kraus¹, N. Papagiannoulis¹, F. Werdin¹, J. Schittenhelm², R. Meyermann², M. Haerle³, S. Geuna⁴ and H.-E. Schaller¹

¹Klinik für Hand-, Plastische, Rekonstruktive und Verbrennungschirurgie, BG-Unfallklinik, ²Institut für Hirnforschung, Eberhard-Karls-Universität Tübingen, ³Klinik für Hand- und Plastische Chirurgie, Orthopädische Klinik Markgröningen, Germany, and ⁴Instituto Cliniche e Biologiche, Universita de Turino, Italy
Nerve injuries may result in sensory and motor deficits when not treated appropriately. Especially the surgical management of nerve defects still represents a challenge for the surgeon. In these cases the grafting of autologous nerves represents the only reasonable approach. Due to the side effects associated with this method (sacrifice of donor nerves, neuroma formation in the harvesting area, limited availability of donor nerves, etc.), numerous alternatives were proposed in order to avoid the transplantation of autologous tissue. This review provides a general view on the state of the art of how to supply gaping injuries in the peripheral nerve. Furthermore new approaches emphasizing tubulization techniques for the reconstruction of lost nerve tissue are described with a special focus on various materials with their advantages and disadvantages.Correspondence to:
Dr. N. Sinis
Klinik für Hand-, Plastische, Rekonstruktive und Verbrennungschirurgie, BG-Unfallklinik
Eberhard-Karls-Universität Tübingen
Schnarrenbergerstraße 95
72076 Tübingen, Germany
Email: nsinis@bgu-tuebingen.de

Abstract

P. Marmiroli¹, V. Rodriguez-Menendez¹, L. Rigamonti¹, E. Tonoli², R. Rigolio¹, G. Cavaletti¹, G. Tredici¹ and A. Vercelli²

¹Department of Neurosciences and Biomedical Technologies, University of Milan Bicocca, Monza, and ²Department of Anatomy, Pharmacology and Forensic Medicine, University of Turin, Turin, Italy
Objective: Type A Niemann-Pick is a severe neurological disease, caused by a mutation of the gene of acid sphingomyelinase (ASM) and reduced enzyme activity. Some studies reported neuropathological changes occurring in the central nervous system of ASM deficient transgenic (ASMKO) mice, while a detailed study on the peripheral nervous system (PNS) at different ages is currently lacking. The aim of our study was to examine the pathological changes occurring in the PNS and in the spinal cord in an AMSKO model of Niemann-Pick disease (NPD) Type A. Material and method: Dorsal root ganglia (DRG), peripheral nerves and spinal cord specimens were obtained from ASMKO mice and age-matched wild type animals (age range = 1 – 7 months). They were observed at the light and electron microscope. Behavioral testing was performed to assess motor coordination and reactivity. Fluoro-Jade B was used as a high affinity fluorescent marker for degenerating neurons. Results: Typical NPD cytoplasmic inclusions were observed in DRG neurons and satellite cells, in peripheral nerve Schwann cells, in spinal cord neurons and in endothelial cells. All these inclusions were present from the age of 1 month and increased with aging. By Fluoro-Jade B staining we demonstrated the occurrence of neuronal degeneration starting from 5 months of age. Conclusion: Despite the fact that a definite diagnosis of NPD Type A depends on enzymatic assays and/or molecular analysis, morphological investigation remains an important diagnostic procedure. Well-defined and complete neuropathological information about the ASMKO mouse model, inclusive of PNS examination, may be crucial in the pre-clinical evaluation of new therapies.Correspondence to:
Dr. P. Marmiroli
DNTB, Università di Milano Bicocca
Via Cadore 48
20052 Monza (MI), Italy
Email: paola.marmiroli@unimib.it

Abstract

L.A. Nowak¹, G.G. Smith² and P.F. Reyes¹

¹Barrow Neurological Institute, St. Joseph’s Hospital and Medical Center, Phoenix, AZ, and ²University of Pennsylvania, Philadelphia, PA and DVAMC, Coatesville, PA, USA
Objective: Dementia in retired boxers, also referred to as “dementia pugilistica” (DP), is usually attributed to repeated concussive and subconcussive blows to the head. We report the case of a former world boxing champion whose progressive cognitive decline could be ascribed to DP, cerebral infarcts and Wernicke-Korsakoff syndrome. This case demonstrates that dementia in retired boxers may be caused and/or exacerbated by etiologic factors other than DP. Materials and methods: We correlated the clinical features with the histochemical and immunohistochemical changes observed on autopsy brain material from a retired boxer, reviewed the literature on boxing-related dementia, and compared our findings with previous reports on DP. Results: Neuropathologic examination revealed numerous neurofibrillary tangles (NFTs), rare neuritic plaques (NPs), multiple cerebral infarcts, fenestrated septum pellucidum, atrophic and gliotic mamillary bodies, and pale substantia nigra and locus ceruleus. Conclusions: Our neuropathologic data confirmed the notion that dementia in retired boxers could be due to several factors such as DP, multiple cerebral infarcts and Wernicke-Korsakoff syndrome. Our findings illustrate the need to comprehensively examine former boxers with dementia as well as carefully evaluate the neuropathologic changes that may cause or contribute to the patient’s cognitive and behavioral symptoms. Such an approach is crucial in order to provide prompt and more definitive therapies.Correspondence to:
P.F. Reyes, MD
Barrow Neurological Institute
NRC 4th Floor 428
350 West Thomas Road
Phoenix, AZ 85013, USA
Email: Patricio.Reyes@chw.edu

Abstract

M.M Rousset-Caron¹*, D. Wolowiec²*, B. Czapiga³, C.A. Maurage⁴, T. Trentesaux¹ and L. Nawrocki⁵

¹Laboratory for Cranial Development and Prevention, Department of Paediatric Dentistry, Faculty of Dentistry, University of Lille, Lille, France, ²Department of Hematology, ³Department of Neurosurgery, Wroclaw Medical University, Wroclaw, Poland, ⁴Department of Pathological Anatomy and Cytology, Regional University Hospital Center, Roger Salengro Hospital, and ⁵Department of Oral Surgery, Faculty of Dentistry, University of Lille, France
Meningiomas infrequently develop in children, and their clinical picture is somewhat different than in adults. We describe here a case of a meningioma in a 9-year-old girl unusual in two aspects. Firstly, it arose from the cavernous sinus what is exceptional in children. Secondly, despite the big tumor mass the child was almost asymptomatic. The only symptoms at presentation were a slight facial asymmetry and minimal laterodeviation of her mandible. Those symptoms had not been noticed by her parents and were detected during careful routine dental examination. The clinical course was quite aggressive and several neurosurgical interventions were necessary. This case underlines the importance of careful medical and dental examination during routine checkup consultations and undertaking necessary diagnostic procedures aimed at elucidating of all detected, even minimal abnormalities.Correspondence to:
Prof. D. Wolowiec, MD, PhD
Department of Hematology
Wroclaw Medical University
ul. Pasteura 4
50-367 Wroclaw, Poland
Email: wolowiec@hemat.am.wroc.pl

Abstract

J. Pimentel¹, C. Barroso¹, J. Miguéns², C. Firmo¹ and J.L. Antunes²

¹Laboratory of Neuropathology, Institute of Molecular Medicine, Lisbon Faculty of Medicine, and ²Department of Neurosurgery, Hospital de Santa Maria, Lisbon, Portugal
Objective: Papillary glioneuronal tumors (PGNT) is well-recognized in the literature, although reports usually have not attempted a critical analysis of their characteristics. We report two PGNT and perform a comprehensive review of the published cases, aiming to clarify their clinical, imaging and histopathological features. Material and methods: We have reviewed all glioneuronal tumors diagnosed in our laboratory over the last 10 years and found 2 cases PGNTs along with their clinical, imaging and surgical data. We have processed material for light microscopy, and for immunohistochemistry study, we have used antisera against glial fibrillary acidic protein, Olig-2C, neurofilament protein, synaptophysin and Ki-67. We searched Medline (1966 through October 2007) for original articles or previous reviews. Results: Case 1, a 19-year-old girl with a left, partially cystic, occipital tumor, totally removed, with no signs of recurrence 32 months after surgery, Case 2, a 9-year-old girl with a right, cystic with a solid nodule, temporal tumor, totally removed, with no signs of recurrence 19 months after surgery. Histopathology and immunohistochemistry studies favored a diagnosis of PGNT. A survey of 38 reported PGNT cases together with our two disclosed the following typical profile: young adulthood predominance, temporal lobe location, presence of cystic components: a close association with the lateral ventricles, a few anaplastic tumors, and gross total resections were usually possible with no recurrences – the extent of surgical removal being the main prognostic factor. Conclusions: Although histopathology is usually characteristic, imaging features may also be important in the presurgical evaluation of PGNTs. Gross total resections are usually possible and seem to govern prognosis. However, longer follow-up data are required.Correspondence to:
J. Pimentel, MD, PhD
Laboratory of Neuropathology
Hospital de Santa Maria
Av. Prof. Egas Moniz
1649-035 Lisbon, Portugal
Email: josepimentel@fm.ul.pt

Abstract

M.C. Sharma¹, D. Jain¹, C. Sarkar¹, V. Suri¹, A. Garg², B.S. Sharma³ and V.S. Mehta³

Departments of ¹Pathology, ²Radiology and ³Neurosurgery, All India Institute of Medical Sciences, New Delhi, India
Background: The papillary tumor of the pineal region (PTPR) is a distinct clinicopathologic entity, the exact biological behavior of which is not known. Finding: In the present report we describe 3 additional cases of PTPR because of its rarity. During a study period of 4 years (between January 2003 and December 2006), we diagnosed three cases of papillary tumor of the pineal region (PTPR). Clinico-radio-pathologic examination was done and follow-up was assessed. Microscopically, all 3 cases showed uniform morphology and consisted of papillary and solid areas. Immunohistochemistry showed strong and diffuse positivity for synaptophysin, NSE, chromogranin A, S-100 protein, MAP-2 and cytokeratin. Conclusion: PTPR is a distinct entity and needs to be differentiated from other tumors of the pineal region as the biological behavior of this tumor is not fully understood. Radiologically this tumor can sometimes be misdiagnosed as tectal glioma.Correspondence to:
Dr. M.C. Sharma
Associate Professor, Department of Pathology
All India Institute of Medical Sciences
New Delhi -110029, India
Email: sharmamehar@yahoo.co.in

Abstract

C.I. Coiré¹, E. Horvath³, H.S. Smyth² and K. Kovacs³

Departments of ¹Laboratory Medicine and ²Neurosurgery, Trillium Health Center, Mississauga, and ³Department of Laboratory Medicine and Pathology, St. Michael’s Hospital, University of Toronto, Toronto, ON, Canada
We report a rapidly recurring folliculostellate cell tumor of the adenohypophysis in a 63-year-old woman. Morphologically the tumor had the typical appearance of a spindle cell oncocytoma of the adenohypophysis. It recurred within 5 months of selective transsphenoidal resection, requiring a second transsphenoidal operation followed by radiation therapy. The spindle cell oncocytoma (SCO) of the adenohypophysis is a relatively recently described entity and a new addition to the fourth edition of the WHO Classification of Tumors of the Central Nervous System. In our case, the ultrastructural features were significantly different from those so far described in SCO, in that tumor cells formed a network of structures indistinguishable from pituitary follicles. In addition, a minority of tumor cells exhibited endocrine differentiation.Correspondence to:
C.I. Coiré, MD
Department of Pathology
Trillium Health Center
100 Queensway West
Mississauga, ON, L5B 1B8, Canada
Email: ccoire@thc.on.ca

Abstract

S. Weiss¹, F.A. Siebzehnrübl¹, J. Kreutzer², I. Blümcke¹ and R. Buslei¹

¹Department of Neuropathology and ²Neurosurgery, University of Erlangen-Nuremberg, Erlangen, Germany
The ability to isolate and propagate adult stem/progenitor cells from the human brain opens novel avenues for cell replacement therapy. This will also apply to the pituitary gland, i.e. following tumor induced endocrine deficiency. Herein, we examine autopsy derived pituitaries to unravel a putative stem/progenitor cell population in humans. In tissue sections of the anterior lobe nestin immunoreactive cells co-expressing smooth muscle actin (SMA) were identified in the perivascular space, indicating a pericytic differentiation. Under clonal conditions, this particular cell population generated primary and secondary cell aggregates (spheres). Pituitary cell cultures maintained a stable cell cycle length with a doubling time of 10 days for over eight months. Forskolin treatment induced a prolactin-expressing phenotype in the majority of cell progenies as well as few bIII-tubulin (Tuj1) expressing cells of putative neuronal lineage. The presence of sphere-forming, nestin-immunoreactive cells and their ability to generate differentiated cell lineages indicates the existence of a progenitor cell population persisting in the adult human pituitary. Further studies are needed to characterize this cell population in more detail and to clarify their potential to initiate neoplastic transformation for example in the cellular pathogenesis of pituitary adenoma.Correspondence to:
R. Buslei, MD
Department of Neuropathology
University of Erlangen, Medical School
Schwabachanlage 6
91054 Erlangen, Germany
Email: rolf.buslei@uk-erlangen.de

Abstract

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Volume 28, No. 4/2009(July/August)