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Aktuelle Neurologie 2013; 40(09): 515-517
DOI: 10.1055/s-0033-1349645
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© Georg Thieme Verlag KG Stuttgart · New York

Infratentorielle Progressive Multifokale Leukenzephalopathie

Infratentorial Progressive Multifocal Leukoencephalopathy
S. Brown
1   Klinik und Poliklinik für Neurologie, Universitätsklinik Dresden
,
K. Engellandt
2   Institut und Poliklinik für Radiologische Diagnostik, Abteilung Neuroradiologie, Universitätsklinik Dresden
,
P. Spornraft-Ragaller
3   Klinik und Poliklinik für Dermatologie, Universitätsklinik Dresden
,
J. Schaefer
1   Klinik und Poliklinik für Neurologie, Universitätsklinik Dresden
› Author Affiliations
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Publication History

Publication Date:
17 October 2013 (online)

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Zusammenfassung

Die Progressive Multifokale Leukenzephalopathie (PML), Ausdruck einer JC-Virus-Reaktivierung, ist vorrangig als supratentorielle Entzündung bei kompromittiertem Immunstatus bekannt. Als Folge der AIDS-Pandemie, jedoch auch neuer immunsuppressiver Therapien, haben Bedeutung und Verständnis dieser oft fatal verlaufenden Erkrankung erheblich zugenommen. Da eine etablierte spezifische Therapie fehlt, richtet sich die Behandlung in erster Linie auf eine Verbesserung des Immunstatus. Ein 49-jähriger HIV-positiver Patient entwickelte eine rasch progrediente Hemiataxie und Dysarthrie. Die Diagnose einer PML konnte mithilfe von MRT und JC-Virus-PCR im Liquor cerebrospinalis gesichert werden, interessanterweise mit anfangs ausschließlich zerebellärer Manifestation. Trotz kombinierter antiretroviraler Therapie verlief die Erkrankung innerhalb weniger Wochen letal. Dieser Fallbericht soll die Möglichkeit einer selektiv infratentoriellen Manifestation der PML unterstreichen.

Abstract

Progressive multifocal leukoencephalopathy (PML), manifestation of a JC virus reactivation, is mainly known as supratentorial inflammation due to immunosuppression. The importance and comprehension of this disease, that is terminal in many cases, has increased over the past years as a consequence of new possibilities of medical immunosuppression and as a consequence of the AIDS pandemia. Treatment mainly aims at improving the patient’s immunostatus as there is no established specific therapy to date. A 49-year-old HIV-positive man presented with rapid progressive hemiataxia and dysarthria. The diagnosis of PML could be made using magnetic resonance imaging and JC virus PCR testing of cerebrospinal fluid, interestingly first concerning the cerebellum only. In spite of combined antiretroviral medication the disease was lethal within a few weeks. This case report is intended to underline the possibility of a selectively infratentorial manifestation of PML.

Schlüsselwörter

Progressive Multifokale Leukenzephalopathie - infratentoriell - AIDS - Ataxie

Keywords

progressive multifocal leukoencephalopathy - infratentorial - AIDS - ataxia
 

  • Literatur

  • 1 Weissert R. Progressive Multifocal Leukoencephalopathy. Journal of Neuroimmunology 2011; 231: 73-77
    CrossrefPubMedGoogle Scholar
  • 2 Berger JR. The clinical features of PML. Cleve Clin J Med 2011; 78 (Suppl. 02) S8-12
    CrossrefPubMedGoogle Scholar
  • 3 Arendt G, Nolting T. Psychiatric diseases in HIV-positive patients. Arzneimitteltherapie 2010; 28: 269-276
    PubMedGoogle Scholar
  • 4 Landgren O, Goedert JJ, Rabkin CS et al. Circulating serum free light chains as predictive markers or AIDS-related lymphoma. J Clin Oncol 2010; 28: 773-779
    CrossrefPubMedGoogle Scholar
  • 5 Tan CS, Koralnik IJ. Progressive multifocal leukoencephalopathy and other disorders caused by JC virus: clinical features and pathogenesis. Lancet Neurol 2010; 9: 425-437
    CrossrefPubMedGoogle Scholar
  • 6 Hernández B, Dronda F, Moreno S. Treatment options for AIDS patients with progressive multifocal leukoencephalopathy. Expert Opin Pharmacother 2009; 10: 403-416
    CrossrefPubMedGoogle Scholar
  • 7 Lanzafame M, Ferrari S, Lattuada E et al. Mirtazapine in an HIV-1 infected patient with progressive multifocal leukoencephalopathy. Infez Med 2009; 17: 35-37
    PubMedGoogle Scholar
  • 8 Mikita K, Maeda T, Fujikura Y et al. Does anti-JCV therapy improve the prognosis of AIDS-related PML?. Clin Neurol Neurosurg 2013; Mar 4. doi: pii: DOI: S0303-8467(13)00040-1.
    PubMedGoogle Scholar
  • 9 Fox R. Advances in the management of PML: focus on natalizumab. Cleve Clin J Med 2011; 78 (Suppl. 02) S33-37 doi: DOI: 10.3949/ccjm.78.s2.08.
    CrossrefPubMedGoogle Scholar
  • 10 Warnatz K, Peter H, Schumacher M et al. Infectious CNS disease as a differential diagnosis in systemic rheumatic diseases: three case reports and a review of the literature. Ann Rheum Dis 2003; 62: 50-57
    CrossrefPubMedGoogle Scholar
  • 11 Lach B, Connolly B, Wüthrich C et al. Inflammatory infratentorial progressive multifocal leukoencephalopathy in a patient with rheumatoid arthritis. Neuropathology 2013; May 20. doi: DOI: 10.1111/neup.12045.
    PubMedGoogle Scholar
  • 12 Svensson PÅ, Larsson EM. Infratentorial progressive multifocal leukoencephalopathy (PML) in a patient with SLE. Eur Radiol 2008; 18: 1526-1528
    CrossrefPubMedGoogle Scholar
  • 13 Bernal-Cano F, Joseph JT, Koralnik IJ. Spinal cord lesions of progressive multifocal leukoencephalopathy in an acquired immunodeficiency syndrome patient. J Neurovirol 2007; 13: 474-476
    CrossrefPubMedGoogle Scholar
  • 14 Lipton RB, Krupp L, Horoupian D et al. Progressive multifocal leukoencephalopathy of the posterior fossa in an AIDS patient: clinical, radiographic and evoked potential findings. Eur Neurol 1988; 28: 258-261
    CrossrefPubMedGoogle Scholar
  • 15 Gagne F, Bouchard JP, Bernier JP. Progressive multifocal leucoencephalopathy. Observation with predominant pontocerebellar lesions and association with congenital immune deficiency. Acta Neuropathol 1977; 38: 167-169
    CrossrefPubMedGoogle Scholar