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Abstract

Clinical Neuropathology, Vol. 30 – No. 1/2011

J.A. Hainfellner

Abstract

Clinical Neuropathology, Vol. 30 – No. 1/2011 (3-10)

Unclassifiable tauopathy associated with an A152T variation in MAPT exon 7

G.G. Kovacs¹, A. Wöhrer¹, T. Ströbel¹, G. Botond¹, J. Attems²,³ and H. Budka¹

¹Institute of Neurology, Medical University of Vienna, Vienna, Austria, ²Institute for Ageing and Health, Newcastle University, Newcastle upon Tyne, UK, ³Previous address: Department of Pathology, Otto Wagner Spital, Vienna, Austria
Mutations in the microtubule-associated tau (MAPT) gene are associated clinically with frontotemporal dementia with or without supranuclear palsy, corticobasal syndrome or parkinsonism. Here we present clinical, neuropathological, genetic and biochemical data on a patient with an A152T variation in exon 7 of MAPT. A 63-year-old man presented with memory disturbance and later speech disorder, followed by progressive dementia and terminally myoclonus together with periodic sharp waves in EEG. Duration of illness was 5 years. Similar neuropsychiatric symptoms were reported in the patient’s father. Neuropathological evaluation revealed neuronal loss mainly in the frontal and temporal cortices and substantia nigra. Abundant phospho-tau immunoreactive thread-like structures and diffuse staining of neuronal cytoplasm predominated in the frontal and temporal cortex, and hippocampus. There was a lack of astrocytic plaques and tufted astrocytes, and only a moderate number of oligodendroglial coiled bodies were seen. Tau pathology was characterized by the 4R tau isoform; immunoblot revealed bands at 64 and 68 kDa, and ultrastructure of filaments was compatible with twisted ribbons. Pathogenic mutations have not been reported in exon 7. Our observation of an apparently familial disorder with a novel neuropathological phenotype suggests a possible pathogenic role of thisMAPT gene variation, which might be different from mutations affecting the microtubule binding.Correspondence to:
G.G. Kovacs, MD, PhD
Institute of Neurology, AKH 4J
Währinger Gürtel 18 – 20
1097 Vienna, Austria
Email: gabor.kovacs@meduniwien.ac.at

Abstract

Clinical Neuropathology, Vol. 30 – No. 1/2011 (11-17)

Churg-Strauss syndrome complicated by neuropathy: a clinicopathological study of nine cases

E. Kararizou, P. Davaki, K. Spengos, N. Karandreas, A. Dimitracopoulos and D. Vassilopoulos

Section of Neuropathology, Neurological Clinic of University of Athens, Aeginition Hospital, Athens, Greece
Objective: The purpose of this study was to investigate the clinical, electrophysiological and pathological features of Churg Strauss syndrome (CSS) neuropathy. Methods: Biopsies were selected from over 700 sural nerve biopsies. The diagnosis of vasculitis was based on established clinicopathological criteria. Complete laboratory, clinical, electrophysiological and pathological studies were performed in all cases. Results: Nerve biopsies of 9 patients were diagnosed as Churg-Strauss syndrome. The pathological features were vasculitis with predominant axonal degeneration and a varying pattern of myelinated fiber loss. The vasculitic changes were found mainly in small epineural blood vessels. Mononeuritis multiplex and distal symmetrical and asymmetrical sensorimotor neuropathy, were equally frequent. Conclusion: We conclude that, Churg-Strauss syndrome complicated frequently with polyneuropathy, and as remission depends on immunosuppressive therapy, it is important to recognize it in the early stage. The diagnosis of polyneuropathy is based on clinical and electrophysiologic studies, but precise histology, immunolohistochemistry and morphometric study of the peripheral nerve biopsy may be decisive in establishing the diagnosis.Correspondence to:
E. Kararizou, MD, Ass. Professor
Section of Neuropathology
Neurological Clinic
Aeginition Hospital
72-74 Vas.Sofias av., 11528, Athens, Greece
Email: ekarariz@med.uoa.gr

Abstract

Clinical Neuropathology, Vol. 30 – No. 1/2011 (18-24)

Annexin-1 is no useful surrogate marker of Multiple Sclerosis – an immunocytochemical study of the cerebrospinal fluid

S. Probst-Cousin¹, D. Heuß² and M. Bergmann³

¹Department of Neurology, Klinikum Bremen-Ost, Bremen, ²Department of Neurology, Friedrich-Alexander-University Erlangen-Nuremberg, Erlangen, and ³Department of Clinical Neuropathology, Klinikum Bremen-Mitte, Bremen, Germany
Objective: Annexin-1 is a calcium-binding protein with anti-inflammatory properties, which has previously been described in MS plaque tissue. We investigated the feasibility and specificity of annexin-1-immuncytochemistry of CSF cells to test its potential as a surrogate marker for MS. Materials and methods: CSF-specimens of 49 MS cases with different courses and 94 control cases were immunocytochemically studied with a monoclonal antibody to annexin-1. Results: The highest level of cytoplasmic immunoreaction was seen in the most acute inflammatory disorders, such as bacterial meningitis and neuroborreliosis. CIS-, RR-MS-, and viral meningoencephalitis cases came next. The lowest annexin-1 expression was observed in neurosyphilis and SP-MS. In PP-MS and non-inflammatory control cases, annexin-1 expression was entirely lacking. Conclusion: Immunocytochemical staining of CSF cells with an antibody to annexin-1 is feasible. This may be helpful in further study of its role in the pathophysiology of inflammatory CNS diseases. The expression pattern seems to rather reflect the acuteness of the inflammatory process than specifying a certain underlying pathology. Although differences were observed between diverse disease groups, because of considerable overlap, a certain diagnosis of an individual case cannot be achieved. Thus, at present, we cannot recommend annexin-1 as a reliable surrogate marker of MS.Correspondence to:
PD Dr. S. Probst-Cousin
Department of Neurology
Klinikum Bremen-Ost
Züricher Straße 40
28325 Bremen, Germany
Email: stefan.probst@klinikum-bremen-ost.de

Abstract

Clinical Neuropathology, Vol. 30 – No. 1/2011 (25-27)

Unusual localization of an unusual tumor: calcifying pseudoneoplasm of the foramen magnum

M. Ozdemir¹, M. Bozkurt², O. Ozgural², E. Erden³, H. Tuna² and Y.S. Caglar²

¹Department of Neurosurgery, Ergani State Hospital, Diyarbakir, ²Department of Neurosurgery, School of Medicine, Ankara University, Ankara, ³Department of Pathology, School of Medicine, Ankara University, Ankara, Turkey
Objective: We report a rare case of calcifying pseudoneoplasm in the foramen magnum. A large variety of tumors can be found in the foramen magnum; meningiomas, neurofibromas, chordomas, chondrosarcomas and metastases are among those that have been reported. Based on the histopathological structure of the tumor, radical excision or, in the case of tumors with good behavioral pattern, only decompressive debulking can be applied, in an effort to avoid unnecessary morbidities. Thus, it is important to know the nature of the tumor before planning the surgery. Case report: A 53-year-old man with a 1-year history of pain on the left side of his face was admitted to our service. Magnetic resonance imaging revealed a calcific mass at the left side of the spinal cord at the level of the foramen magnum. Median suboccipital craniectomy and total tumor resection were performed and there was no additional neurological deficit postoperatively. Conclusion: We report, to our knowledge, the second case of a calcifying pseudoneoplasm of the foramen magnum. We emphasize that these tumors are pathologically benign and do not require aggressive surgical treatment and suggest that asymptomatic cases can be followed radiologically.Correspondence to:
Dr. M. Ozdemir
Department of Neurosurgery
Ergani State Hospital
21950 Diyarbakir, Turkey
Email: drmevci@hotmail.com

Abstract

Clinical Neuropathology, Vol. 30 – No. 1/2011 (28-32)

Cerebral coenurosis mimicking hydatid disease – report of two cases from South India

A. Mahadevan¹, S. Dwarakanath², S. Pai⁴, J.M.E. Kovoor³, S. Radhesh⁵, H.V. Srinivas⁶, B.A. Chandramouli² and S.K. Shankar¹

Department of ¹Neuropathology, ²Neurosurgery, ³Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurosciences, Department of ⁴Neurosurgery and ⁶Neurology, Agadi Hospital, and ⁵Clumax Diagnostics, Bangalore, India
Coenurosis, a rare zoonotic disease caused by the larval form of Taenia multiceps (bladderworm) is common in sheep rearing countries, but human infections are rare. Central nervous system involvement produces large giant sized cysts that radiologically closely mimic hydatid cysts. Most human infections resulting in cerebral coenuri have been reported from Europe and Africa. We report two cases of cerebral coenurosis from India, the first in a 55-year-old male presenting with a large cystic lesion in the right parietooccipital region and the second occurring in a 36-year-old male involving the left temporal trigonal region, that radiologically closely mimicked hydatid cyst. Histopathologic examination revealed characteristic features of coenuri with multiple protoscolices invaginating into a large cyst lined by outer cuticular layer. Awareness of this rare parasitic infestation is important to discriminate from the more common hydatid and giant cysticercal cysts.Correspondence to:
Dr. A. Mahadevan, Assistant Professor
Department of Neuropathology
National institute of Mental Health and Neurosciences
Bangalore 560 029, India
Email: anita_mahadevan@yahoo.com

Abstract

Clinical Neuropathology, Vol. 30 – No. 1/2011 (33-40)

Erythropoietin receptor expression in normal and neoplastic choroid plexus

R. Beschorner¹, T. Psaras², R. Meyermann¹, J. Bremer³, T. Schmidt⁴, M. Mittelbronn⁵ and J. Schittenhelm¹

¹Institute for Brain Research, ²Department of Neurosurgery, University of Tübingen, Tübingen, Germany, ³Institute of Neuropathology, University Hospital of Zürich, Zürich, Switzerland, ⁴Department of Medical Genetics, University of Tübingen, Tübingen, and ⁵Institute of Neurology (Edinger Institute), University of Frankfurt, Frankfurt a. M., Germany
Background: The erythropoietin receptor (EpoR) is expressed widely throughout the human CNS, including the choroid plexus. Recent studies have shown that EpoR is also expressed in various human tumors, including carcinomas, meningiomas and gliomas. Thereby, the Epo-EpoR pathway plays a role in inhibition of apoptosis and tumor growth, infiltration, angiogenesis and metastasis as well as treatment resistance and is a potential target in oncological treatment. Lower levels of EpoR have been associated with shorter survival in high grade gliomas and higher risk of tumor recurrence in meningiomas. Methods: Since the EpoR status in human choroid plexus tumors (CPT) is not known, we investigated 57 CPT from 43 cases including 14 recurrent tumors and compared them with 23 samples of normal choroid plexus (CP). CPT samples consisted of choroid plexus papillomas/CPP (n = 41), atypical CPP (n = 15) and choroid plexus carcinoma/CPC (n = 1). EpoR expression was determined by immunohistochemistry using semi-quantitative scoring for staining intensity and was validated in exemplary cases using western blot and RT-PCR. Results: EpoR expression was observed in all samples of normal and neoplastic CP with significantly lower expression levels in CPT (p < 0.001). Conclusion: No significant correlation was found between EpoR expression and age, gender, WHO grade, number of mitosis or tumor recurrence. EpoR expression in CPT is in line with its expression in normal CP and with previous reports on EpoR expression in other glial neoplasms. Association of EpoR levels in CPT with survival, as known in astrocytic gliomas, remains to be determined.Correspondence to:
J. Schittenhelm, MD
Institute for Brain Research
Calwerstr. 3
72076 Tübingen, Germany
Email: jens.schittenhelm@med.uni-tuebingen.de

Abstract

Unusual fibrous meningioma in a patient with sarcoidosis: central necrosis associated with multinucleated giant cells

J. Manfield, L. Thorne, T. Wilhelm and M. Galloway

Abstract

Society News of the European Confederation of Neuropathological Societies

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Volume 30, No. 1/2011(Jan/Feb)