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Abstract

S. McClelland III, J.P. Vonsattel, R.E. Garcia, M.D. Amaya, L.M. Winfield, S.L. Pullman, Q. Yu, S. Fahn, B. Ford and R.R. Goodman

¹Department of Neurosurgery, University of Minnesota Medical School, Minneapolis, MN, Departments of ²Pathology, ³Neurological Surgery and ⁴Neurology, Columbia College of Physicians and Surgeons, New York, NY, USA
Objective/Background: Patients with medically refractory Parkinson’s disease (PD) obtain significant clinical benefit from subthalamic nucleus (STN) stimulation. The degree to which a successful outcome relates to the anatomic location of the stimulating electrode has not yet been clearly established. Many studies have attempted to correlate the clinical result with the electrode location using postoperative magnetic resonance imaging (MRI) and there have been a few that used autopsy-determined locations. In this report, we describe long-term clinical follow-up in a patient with autopsy-determined electrode tip anatomic location. Methods: A 67-year-old patient with a 27-year history of idiopathic PD complicated by disabling motor fluctuations and dopaminergic dyskinesias underwent bilateral STN deep brain stimulation (DBS). He was prospectively followed in a long-term clinical protocol until his death 40 months after electrode placement. Postoperative magnetic resonance (MR) imaging and postmortem studies of this patient’s brain were performed to localize DBS tip locations. Results: STN stimulation produced improvement of the patient’s motor fluctuations, dyskinesias and clinical motor performance, especially appendicular tremors, rigidity and bradykinesia. MRI showed the electrode tips to be within 2 mm of the intended target. Postmortem brain analysis identified the right DBS tip location at the dorsomedial edge of the STN, with the left electrode in the vicinity (but not within) the STN. Chronic DBS elicited minor reactive changes were confined to the immediate vicinity of the electrode tracks. The pathological analysis demonstrated numerous cortical Lewy bodies and degenerative encephalopathy, establishing the diagnosis of transitional type diffuse Lewy body disease (DLBD) rather than simple PD. Conclusion: This patient obtained clinical benefit from STN stimulation typical of that seen for most PD patients. Both the MR analysis and the autopsy demonstrated electrode placement at or outside the boundaries of the STN, suggesting that that clinical efficacy may not depend on electrode location within the central region of the STN.Correspondence to:
R.R. Goodman MD, PhD; Department of Neurological Surgery, Neurological Institute of New York, Columbia College of Physicians and Surgeons, 710 West 168th Street, Box 99, New York, NY 10032, USA
Email: rrg2@columbia.edu

Abstract

L. Parkkinen, P. Hartikainen and I. Alafuzoff

¹Department of Medicine, Neurology, ²Department of Neurology and

³Department of Pathology, Kuopio University Hospital, Kuopio, Finland
Screening of 1,800 brains with α-synuclein (αS) immunohistochemistry revealed five cases with abundant glial cytoplasmic inclusions (GCIs) within the white matter of the brainstem. Surprisingly, retrospective clinical assessment showed that one of these subjects did not fulfil the currently recommended clinical consensus criteria for the multiple system atrophy (MSA). One of the hallmark lesions of MSA, αS-positive GCIs, were widespread and abundant in this atypical case that nonetheless lacked any significant neuronal loss. If the patient had met the clinical criteria for MSA, the neuropathological phenotype would have undeniably confirmed the clinically suggested diagnosis. However, lacking overt clinical signs of MSA, the neuropathological phenotype in this subject is prone to be variably denoted or overlooked. We would therefore like to advise neuropathologists to acknowledge these cases with “occult” α-synucleinopathy and to inform the clinicians of such a finding. Whether these cases represent a preclinical stage of MSA or simply a biological coincidence, is yet unknown. The observation of abundant GCIs in an asymptomatic subject is, however, important, because even if these cases are rare in number, their occurrence challenge the current presumption, whereby simply the number of αS-positive GCIs mediates the neuronal dysfunction responsible for the clinical symptoms of MSA.Correspondence to:
Dr. I. Alafuzoff; Department of Medicine, Neurology, Kuopio University, PO Box 1627, 70211 Kuopio, Finland
Email: Irina.Alafuzoff@uku.fi

Abstract

G. Torres, F. Petit, V. Vilchez, Z. Romero, S. Dorfman, D. Cardozo and J. Cardozo

¹Pathology Department, Hospital General del Sur and ²Surgical Oncology Service, Hospital Noriega Trigo, Maracaibo, Zulia, Venezuela
Objective: To describe the case of a primary cerebral fibrosarcoma in a child. Clinical case: A 6-year-old boy presented with a history of headache, drowsiness, vomiting and seizures. He was referred to our institution and died shortly upon arrival to the ER. The autopsy revealed a large left frontoparietal mass with histopathologic features characteristic of fibrosarcoma. Immunohistochemical and ultrastructural studies allowed the exclusion of other mimickers such as meningiomas, and neoplasms of glial or neuronal origin. No other growths were found in the CNS or in other parts of the body. Conclusions: Brain fibrosarcoma is an exceedingly uncommon tumor. Only 41 cases have been reported to date. The diagnosis of fibrosarcoma must be based on the identification of a predominant herringbone architectural pattern, and immunohistochemical features, although the latter are of limited diagnostic value, its importance being that they facilitate the exclusion of other entities considered in the differential diagnosis.Correspondence to:
Dr. J. Cardozo; Apartado 519, Maracaibo, Zulia, Venezuela
Email: cardozo1@telcel.net.ve

Abstract

J. Juco, E. Horvath, H. Smyth, F. Rotondo and K. Kovacs

¹Department of Laboratory Medicine, ²Department of Neurosurgery, St. Michael’s Hospital, University of Toronto, Ontario, Canada
Objective: Hemangiopericytoma (HPC) is a potentially malignant vascular neoplasm that in rare cases presents as a primary intracranial lesion, where most often it is meningeal in origin. Hemangiopericytoma arising within the sella turcica is an even more sporadic event. To our knowledge, only 9 cases of HPC presenting as a sellar or suprasellar mass have been reported in the literature. Often, these cases can mimic and be mistaken for a pituitary adenoma. Material and methods: We report a case of an 18-year-old woman presenting with a sellar mass which was thought both clinically and radiologically to be a pituitary adenoma. Results: Based on histologic, immunohistochemical and electron-microscopic studies, the diagnosis of sellar HPC was made. Conclusion: Hemangiopericytoma should be considered in the differential diagnosis of sellar or suprasellar masses.Correspondence to:
J. Juco, MD; Department of Laboratory Medicine, St. Michael’s Hospital, University of Toronto, 30 Bond Street, Toronto, ON, Canada
Email: jucoj@smh.toronto.on.ca

Abstract

D. Arsene, C. Ardeleanu, C. Balescu and A. Nistorescu

¹Department of Neuropathology, “Vlad Voiculescu” Institute of Cerebrovascular Diseases, ²Department of Histopathology, “Victor Babes” Institute of Pathology, ³Intensive Care Unit, “Vlad Voiculescu”, Institute of Cerebrovascular Diseases, ⁴Department of Neurology, “Vlad Voiculescu” Institute of Cerebrovascular Diseases, Bucharest, Romania
Primary diffuse leptomeningeal melanocytosis is a very rare form of brain tumor. We report on a rapidly fatal case in an 18-year-old man presenting with symptoms and imaging features suggestive for subarachnoid hemorrhage or meningitis. The laboratory findings and imaging examination were still confusing and the diagnosis remained unclear during the patient’s life. Autopsy was the cornerstone in disclosing the lesion, confirming its usefulness in the assessment of such unusual cases. The complete profile of the tumor was obtained only by histology and immunohistochemistry. Clinicians and pathologists must be aware of diagnosis difficulties in this rare disease which can represent a serious challenge in clinical practice.Correspondence to:
Dr. D. Arsene; Department of Neuropathology and Anatomic Pathology, “Vlad Voiculescu” Institute of Cerebrovascular Diseases, Sos. Berceni Nr 10-12, Sect 4, Bucharest, Romania
Email: dorelarsene@yahoo.com

Abstract

G. Levidou, P. Korkolopoulou, A. Papetta, E. Patsouris and E. Agapitos

Department of Pathology, National and Kapodistrian University of Athens, Greece
Primary melanoma of the central nervous system accounts for only 1% of the cases of melanoma, having a relatively rare frequency of being reported in the literature. We report two cases of leptomeningeal melanoma of unknown primary site diagnosed after post mortem examination. In the first case, the patient presented with resisting epilepsy, whereas in the second with persisting fever and mental slowness. Cranial CT in the first patient showed postgadolinium enhancement of the ependyma and the infundibulum, while in the second there was diffuse enhancement of the leptomeninges. Analyses of the CSF in both cases did not establish the presence of malignant cells but revealed altered CSF glucose and increased CSF protein levels. There were no extracranial abnormalities. Both patients were treated for infectious meningitis and died a few days afterwards. At autopsy, all body cavities including oral cavity and the entire integument were examined. In both cases the leptomeninges were diffusely covered with brownish material. Histological examination of the brain specimens revealed the presence of a malignant neoplasm of low differentiation. Diagnosis was established with the results of immunohistochemistry, tumor cells were positive for HMB-45 and S-100 whereas they were negative for cytokeratins, CD45 and GFAP. In conclusion, both patients, although presenting with symptoms and signs highly suggestive of meningitis, suffered from leptomeningeal melanomas of unknown primary site. Clinical, radiological and histological findings are discussed with a review of the literature.Correspondence to:
G. Levidou, MD; 75 Mikras Asias Street, 11527 Athens, Greece
Email: glevidou@yahoo.gr

Abstract

K. Burkhardt, F. Heuberger and J. Delavelle

¹Department of Surgical Pathology, ²Hôpital de Loëx, ³Department of Neuroradiology, University Hospitals of Geneva, Geneva, Switzerland
Pilocytic astrocytoma (WHO Grade I) is a low-grade glioma with a favorable prognosis most commonly diagnosed in patients aged below 20. It is the most common glioma in children, and cases discovered in elderly patients are rare. We report the highly unusual case of an 85-year-old man whose neurological signs included Parkinsonism, and in whom post mortem examination revealed a pilocytic astrocytoma of the brainstem. We also discuss the clinical, neuroradiological and neuropathological differential diagnosis.Correspondence to:
Dr. K. Burkhardt; Department of Surgical Pathology, University Hospitals of Geneva (HUG), rue Micheli-du-Crest 24, 1211 Genève 14, Switzerland
Email: Karim.Burkhardt@hcuge.ch

Abstract

M. Majores, P. Niehusmann, M. von Lehe, I. Blümcke and H. Urbach

Departments of ¹Neuropathology, ²Epileptology, ³Neurosurgery, ⁴Neuroradiology, University of Bonn Medical Center, ⁵Department of Neuropathology and Neuropathological Reference Center for Epilepsy Surgery, University of Erlangen, Germany
Case report: We report on a 46-year-old male patient with pharmacoresistant temporal lobe epilepsy (TLE). Based on ictal EEG patterns and MRI scans, Ammon’s horn sclerosis (AHS) or an epilepsy-associated tumor was included in the differential diagnosis. Results: Histopathological examination of the surgical specimen revealed the unusual finding of a monomorphous angiocentric neuroepithelial tumor composed of small round cells and bipolar processes with perivascular aggregation. Immunohistochemistry detected perivascular-oriented expression of GFAP and cytoplasmic immunoreactivity of EMA and vimentin. Mitotic or other signs of proliferative activity were lacking. During a 2-year follow-up, the patient was seizure-free. Conclusions: Albeit AHS is the most frequent finding in TLE specimens, uncommon neuroepithelial tumors with hippocampal growth pattern have to be considered in the differential diagnosis of mesial TLE. The present case meets the criteria of an angiocentric neuroepithelial tumor recently proposed as a new clinicopathological entity. These tumors may be compatible with a maldevelopmental origin during early brain development.Correspondence to:
Dr. M. Majores; Department of Neuropathology, University of Bonn Medical Center, Sigmund-Freud-Straße 25, 53105 Bonn, Germany
Email: majores@uni-bonn.de

Abstract

Euro-CNS

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Volume 26, No. 6/2007(Nov/Dec)