Journal of Neurology

, Volume 250, Issue 12, pp 1488–1491

Multiple vascular abnormalities and a paradoxical combination of vitamin B12 deficiency and thrombocytosis in a case with POEMS syndrome

Authors

    • Research Group Protective Signaling, Zentrum für Molekulare Neurobiologie and Department of NeurologyUniversity Hospital Hamburg-Eppendorf
  • A. Gocht
    • Department of PathologyUniversity Hospital
  • P. H. Hoeger
    • Department of DermatologyUniversity Hospital
  • P. von den Driesch
    • Department of DermatologyUniversity Hospital
  • B. Eckert
    • Department of NeuroradiologyUniversity Hospital
  • K. Lamszus
    • Department of NeurosurgeryUniversity Hospital
  • H.-J. Stuerenburg
    • Department of NeurologyUniversity Hospital
  • A. Methner
    • Research Group Protective Signaling, Zentrum für Molekulare Neurobiologie and Department of NeurologyUniversity Hospital Hamburg-Eppendorf
ORIGINAL COMMUNICATION

DOI: 10.1007/s00415-003-0261-7

Cite this article as:
Lewerenz, J., Gocht, A., Hoeger, P.H. et al. J Neurol (2003) 250: 1488. doi:10.1007/s00415-003-0261-7

Abstract.

POEMS/Crow-Fukase syndrome is a rare multisystem disorder associated with elevated vascular endothelial growth factor (VEGF), which clinically presents with polyneuropathy, organomegaly, endocrinopathy, Mprotein, and skin changes. We report a case of POEMS syndrome due to a gammopathy of undetermined significance with thrombocytosis, vitamin B12 deficiency, highly elevated VEGF and in addition to glomeruloid angiomas two previously undescribed proliferative vascular lesions: a spinal arteriovenous fistula and a plexogenic pulmonary arteriopathy, which ultimately resulted in lethal pulmonary hypertension. We assume that the high VEGF levels caused the vascular abnormalities observed in our patient.

Key words

POEMS syndromeVEGFthrombocytosisarteriovenous malformationspulmonary hypertension

Copyright information

© Steinkopff Verlag 2003