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HTLV-1-assoziierte Myelopathie/tropische spastische Paraparese

Eine Differenzialdiagnose der Multiplen Sklerose

HTLV-1-associated myelopathy/tropical spastic paraparesis

A differential diagnosis in multiple sclerosis

Zusammenfassung

Die HTLV-1 („human T-cell lymphotropic virus 1”)-assoziierte Myelopathie/tropische spastische Paraparese (HAM/TSP) ist eine durch HTLV-1 verursachte – in Europa seltene, in weiten Teilen der Welt jedoch endemische – neurologische Erkrankung. Die Immunpathogenese ist bisher nicht eindeutig charakterisiert, zugrunde liegt aber eine möglicherweise immunvermittelte Schädigung vorwiegend des zervikothorakalen Myelons. Klinisch imponiert die HAM/TSP als eine langsam progrediente spastische Paraparese, die mit Blasenfunktionsstörungen assoziiert ist. Dabei kann die HAM/TSP häufig den Krankheitsverlauf autoimmuner und neurodegenerativer Erkrankungen imitieren. Die Diagnostik erfolgt anhand typischer Klinik sowie über die Bestimmung von HTLV-1-Antikörpern und proviraler HTLV-1-DNA bzw. HTLV-1-RNA. Die Therapie beschränkt sich auf eine symptomatische Behandlung. Die Übertragung kann vertikal über Muttermilch, durch Sexualkontakt und über infizierte Blutprodukte erfolgen. Vor dem Hintergrund eines klinischen Falles aus unserer Klinik stellen wir in diesem Artikel eine aktuelle Übersicht zu Pathophysiologie, Epidemiologie, Klinik, Diagnostik und Behandlung der HAM/TSP vor.

Summary

Human T-cell lymphotropic virus 1 (HTLV-1) associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a neurological disease caused by infection with HTLV-1. The disorder is very rare in Europe but endemic in many parts of the world. The pathogenesis is not clearly characterized but is based on a possibly immune-mediated injury of the cervicothoracic spinal cord. Clinically, HAM/TSP constitutes a slowly progressive spastic paraparesis associated with bladder dysfunction and often mimics the course of autoimmune and neurodegenerative diseases. The diagnosis is based on typical symptoms as well as detection of HTLV-1 specific antibodies and proviral HTLV-1 DNA or HTLV-1 RNA. The therapy is limited to symptomatic treatment. Transmission of HTLV-1 can occur vertically by breast feeding, through sexual contact or via infected blood products. Based on a clinical case report, we present here a current review on the pathophysiology, epidemiology, clinical manifestations, diagnosis and treatment of HAM/TSP.

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Abbreviations

ATL:

adulte T-Zell-Leukämie

CIDP:

chronisch inflammatorische demyelinisierende Polyneuropathie

EDTA:

ethylene diamine tetraacetic acid

ELISA:

enzyme linked immunosorbent assay

HTLV-1:

humanes T-lymphotropes Virus 1

HAM:

HTLV-1-assoziierte Myelopathie

Ig:

Immunglobulin

MRT:

Magnetresonanztomographie

NMO:

Neuromyelitis optica

OKB:

oligoklonale Banden

PDE-5:

Phosphodiesterase 5

PNP:

Polyneuropathie

TSP:

tropische spastische Paraparese

ZNS:

Zentralnervensystem

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Correspondence to Prof. Dr. B. Wildemann.

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Liesz, A., Hähnel, S., Korn, K. et al. HTLV-1-assoziierte Myelopathie/tropische spastische Paraparese. Nervenarzt 83, 1028–1034 (2012) doi:10.1007/s00115-012-3565-y

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Schlüsselwörter

  • HTLV-1
  • HAM/TSP
  • Multiple Sklerose
  • Myelopathie
  • Retrovirus

Keywords

  • Human T-cell lymphotropic virus 1
  • Tropical spastic paraparesis
  • Multiple sclerosis
  • Myelopathy
  • Retrovirus