Clinical Neuroscience

[Association of temporal lobe inflammatory leukoencephalopathy with two B cell malignancies]

GARZULY Ferenc1, HAHN Katalin2, IVÁNYI János László3, KERESKAI László4, GÁBOR Valéria1, KOVÁCS G. Gábor5, BUDKA Herbert5, KÁLMÁN Bernadette6

MARCH 30, 2014

Clinical Neuroscience - 2014;67(03-04)

[Identification of etiological connections among virtually distinct diseases in a patient may be sometimes challenging. We report a unique case with two B cell malignancies and an inflammatory leukoencephalopathy. Three days prior to admission, the elderly male patient developed fatigue, headaches, recurrent vomiting, memory disturbances, depression and somnolence. Clinical, laboratory and imaging evaluations as well as post mortem histological studies were performed. Simultaneous presence of primary central nervous system B cell lymphoma, temporal lobe inflammatory leukoencephalopathy and multiple (smoldering) myeloma, was revealed by the detailed work up in the treatmentnaïve patient. Based on recent data from genomic studies, we propose that a sequential evolution of molecular pathology lead to the co-occurrence of multiple myeloma and primary central nervous system B cell lymphoma in this patient, and interpret the development of the temporal lobe leukoencephalopathy as a likely paraneoplastic complication of smoldering myeloma.]

AFFILIATIONS

  1. Markusovszky University Hospital, Department of Pathology; Szombathely
  2. Markusovszky University Hospital, Department of Neurology; Szombathely
  3. Markusovszky University Hospital, Department of Hematology; Szombathely
  4. University of Pécs, Institute of Pathology; Pécs
  5. Medical University of Vienna, Institute of Neurology; Austria
  6. Markusovszky University Hospital, Center for Molecular Medicine; Szombathely

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[Dilemma of further therapeutic step in RRMS in case of ineffectivity of first line treatment: fingolimod or natalizumab?]

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[Dilemma of further therapeutic step in RRMS in case of ineffectivity of first line treatment: fingolimod or natalizumab? 2014;67(03-04)]

Clinical Neuroscience

[Foreword]

SOMOGYI Árpád, SZABÓ Sándor, ROGER Guillemin, VOLKER Jahnke, MILAGROS Salas-Prato, YVETTE Taché, LÁZÁR György, GYIRES Klára, VÉCSEI László, HORVÁTH Zoltán, TUKA Bernadett, DUNAI Magdolna, ET al.

Clinical Neuroscience

[Validation of the Hungarian MDS-UPDRS: Why do we need a new Parkinson scale?]

HORVÁTH Krisztina, ASCHERMANN Zsuzsanna, ÁCS Péter, BOSNYÁK Edit, DELI Gabriella, PÁL Endre, KÉSMÁRKI Ildikó, HORVÁTH A. Réka, TAKÁCS Katalin, KOMOLY Sámuel, BOKOR Magdolna, RIGÓ Eszter, LAJTOS Júlia, KLIVÉNYI Péter, DIBÓ György, VÉCSEI László, TAKÁTS Annamária, TÓTH Adrián, IMRE Piroska, NAGY Ferenc, HERCEG Mihály, HIDASI Eszter, KOVÁCS Norbert

[Background - The Movement Disorder Society-sponsored revision of the Unified Parkinson’s Disease Rating Scale (MDS-UPDRS) has been published in 2008 as the successor of the original UPDRS. The MDS-UPDRS organizing team developed guidelines for the development of official non- English translations consisting of four steps: translation/back-translation, cognitive pretesting, large field testing, and clinimetric analysis. The aim of this paper was to introduce the new MDS-UPDRS and its validation process into Hungarian. Methods - Two independent groups of neurologists translated the text of the MDS-UPDRS into Hungarian and subsequently back-translated into English. After the review of the back-translated English version by the MDS-UPDRS translation administration team, cognitive pretesting was conducted with ten patients. Based on the results of the initial cognitive pretesting, another round was conducted. For the large field testing phase, the Hungarian official working draft version of MDS-UPDRS was tested with 357 patients with Parkinson’s disease (PD). Confirmatory factor analyses (CFA) determined whether the factor structure for the English-language MDS-UPDRS could be confirmed in data collected using the Hungarian Official Draft Version. To become an official translation, the Comparative Fit Index (CFI) had to be ≥0.90 compared to the English-language version. Results - For all four parts of the Hungarian MDS-UPDRS, the CFI was ≥0.94. Conclusion - The overall factor structure of the Hungarian version was consistent with that of the English version based on the high CFIs for all the four parts of the MDSUPDRS in the CFA; therefore, this version was designated as the ‘OFFICIAL HUNGARIAN VERSION OF THE MDSUPDRS.’]

Clinical Neuroscience

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Clinical Neuroscience

[CONGRESS CALENDAR]

[Congress calendar 2014;67(03-04)]

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Life threatening rare lymphomas presenting as longitudinally extensive transverse myelitis: a diagnostic challenge

TOLVAJ Balázs, HAHN Katalin, NAGY Zsuzsanna, VADVÁRI Árpád, CSOMOR Judit, GELPI Ellen, ILLÉS Zsolt, GARZULY Ferenc

Background and aims – Description of two cases of rare intravascular large B-cell lymphoma and secondary T-cell lymphoma diagnosed postmortem, that manifested clinically as longitudinally extensive transverse myelitis (LETM). We discuss causes of diagnostic difficulties, deceptive radiological and histological investigations, and outline diagnostic procedures based on our and previously reported cases. Case reports – Our first case, a 48-year-old female was admitted to the neurological department due to paraparesis. MRI suggested LETM, but the treatments were ineffective. She died after four weeks because of pneumonia and untreatable polyserositis. Pathological examination revealed intravascular large B-cell lymphoma (IVL). Our second case, a 61-year-old man presented with headache and paraparesis. MRI showed small bitemporal lesions and lesions suggesting LETM. Diagnostic investigations were unsuccessful, including tests for possible lymphoma (CSF flow cytometry and muscle biopsy for suspected IVL). Chest CT showed focal inflammation in a small area of the lung, and adrenal adenoma. Brain biopsy sample from the affected temporal area suggested T-cell mediated lymphocytic (paraneoplastic or viral) meningoencephalitis and excluded diffuse large B-cell lymphoma. The symptoms worsened, and the patient died in the sixth week of disease. The pathological examination of the presumed adenoma in the adrenal gland, the pancreatic tail and the lung lesions revealed peripheral T-cell lymphoma, as did the brain and spinal cord lesions. Even at histological examination, the T-cell lymphoma had the misleading appearance of inflammatory condition as did the MRI. Conclusion – Lymphoma can manifest as LETM. In cases of etiologically unclear atypical LETM in patients older than 40 years, a random skin biopsy (with subcutaneous adipose tissue) from the thigh and from the abdomen is strongly recommended as soon as possible. This may detect IVL and provide the possibility of prompt chemotherapy. In case of suspicion of lymphoma, parallel examination of the CSF by flow cytometry is also recommended. If skin biopsy is negative but lymphoma suspicion remains high, biopsy from other sites (bone marrow, lymph nodes or adrenal gland lesion) or from a simultaneously existing cerebral lesion is suggested, to exclude or prove diffuse large B-cell lymphoma, IVL, or a rare T-cell lymphoma.

Clinical Neuroscience

Isocitrate dehydrogenase mutations in defining the biology of and supporting clinical decision making in glioblastoma

KÁLOVITS Ferenc, TOMPA Márton, NAGY Ádám, BERNADETTE Kalman

Background and purpose - Oncogenesis is related to a sequential accumulation of somatic mutations. Comprehensive characterizations of the genomic landscapes have been completed recently for several tumors, glioblastoma being among the first ones. Our own translational research studies have been focused on defining molecular subtypes of glioblastoma in the clinical setting because of an expected prognostic and therapeutic utility of the information. Somatic mutations in genes of the isocitrate dehydrogenase (IDH) enzyme family appear to be among the best-defined biomarkers that also influence tumor behavior and confer clinical utility. Methods - We have reviewed the literature including our own results to summarize basic science and clinical correlates of IDH mutations. Results - The surveyed data reveal genomic, transcriptomic, epigenomic and biochemical consequences of IDH mutations in the context of glioblastoma biology and phenotype. In addition, a few studies highlight the therapeutic potential of targeting IDH, although thus far all tests have only been conducted in the preclinical setting. Conclusions - Somatic mutations in isoforms of IDH genes represent important biomarkers that correlate with biochemical, biological and phenotypic features of glioblastoma, and may also facilitate the development of new therapeutic strategies complementing the currently available approved protocols.

Lege Artis Medicinae

[Neurological symptoms in a patient with treated multiple myeloma]

ZOMBORI Tamás, PIUKOVICS Klára

[INTRODUCTION - Meningeal infiltration by multiple myeloma is rare. Its incidence among cases of multiple myeloma is 1%. CASE REPORT - Multiple myeloma was diagnosed in a 53-year-old woman in December 2014. After chemotherapy, the disease was treated with autologous bone marrow transplantation in June 2015. Remission was observed through two months, but in August the patient was hospitalized due to severe headache with neck stiffness. Meningitis or viral encephalitis were suspected following her investigation. She was taken to the Intensive Care Unit because of a progression to status epilepticus. The EEG-examination revealed generalized slow wave activity and a right temporal epileptiform focus manifesting rarely. Clinical brain death developed on the 17th day in hospital. DISCUSSION - Although meningeal infiltration is infrequent in multiple myeloma, the present case report draws attention to this possibility. ]

Lege Artis Medicinae

[THE RESULTS OF MOLECULAR BIOLOGY IN THE MANAGEMENT OF MYELOMA MULTIPLEX]

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[Among heamatological diseases, myeloma multiplex is the disease demanding the greatest attention. This statement could refer to the difficulties in diagnosis through the success or failure of a medical treatment and to the unavoidable deterioration of condition caused by complications. The illness, as it is known, is incurable but can be treated for a long period of time. The results of molecular biology offer new possibilities to secure the collection of instruments facilitating future recovery. These new modalities are targeted radiotherapy, the abundance of new drugs attacking the subcellular organellums of affected cells (thalidomid, Neovastat, oblimersen, bortezomib, etc.) and immunotherapy against myeloma cells. The introduction of new therapies, however, is to be permitted only with the greatest caution because the crossing of roads cannot be calculated and this could bring about deterioration too. The delay in theoretical considerations and experience however, allows the possibility to do a critical analysis. The application would become reality after collecting numerous experiences.]

Lege Artis Medicinae

[Concomitant occurrence of multiple myeloma and diffuse large B-cell lymphoma]

RADVÁNYI Mónika, ILLÉS Árpád, MÉHES Gábor, KAJTÁR Béla, FAZAKAS Ferenc, GARAI Ildikó, GERGELY Lajos

[We report a case of a 50-year-old male patient, in whom the coexistence of multiple myeloma and diffuse large B cell lymphoma was confirmed. In December 2007, the patient was admitted to the department of internal medicine with fatigue resulting from mild anemia. A tumor of the left testis was discovered, and after semicastration diffuse large B cell lymphoma was diagnosed by histopathological analysis (clinical stage: II/EB). Examination of the bone marrow revealed a plasmocytic infiltrate of 60%, while 36.1 g/l IgG-kappa paraprotein was found in the peripheric blood, fulfilling the diagnostic criteria of multiple myeloma (Durie-Salmon stage: I). The patient received six cycles of rituximab- CHOP-21 chemotherapy for diffuse large B cell lymphoma. Following a transient improvement, the multiple myeloma showed progression, therefore we switched to VTD protocol (bortezomib, thalidomid, dexamethason). The patient underwent high dose chemotherapy and autologous hematopoietic stem cell transplantation. Both hematologic diseases showed complete remission. Both tumour samples were tested for immunoglobulin heavy-chain rearrangement by polymerase chain reaction and DNA sequence analysis, according to which the possibility for clonal relationship between multiple myeloma and diffuse large B cell lymphoma could not be confirmed.]