• J. Neurol. Sci. · Apr 2010

    Case Reports

    Progressive encephalomyelitis with rigidity and myoclonus preceding otherwise asymptomatic Hodgkin's lymphoma.

    • Caroline Schmidt, Tobias Freilinger, M Lieb, J Rémi, M Klein, A Straube, and T Pfefferkorn.
    • Department of Neurology, Klinikum Grosshadern der Ludwig-Maximilians-Universität München, Munich, Germany.
    • J. Neurol. Sci. 2010 Apr 15;291(1-2):118-20.

    AbstractStiff-person syndrome (SPS), a rare neuroimmunological disorder, is characterized by symmetrical rigidity and muscle stiffness, particularly of axial and proximal limb muscles. Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a variant of SPS which includes additional clinical features (e.g. sensory symptoms, brain stem signs and pathological CSF findings). An association of both SPS and PERM with (solid) malignancies has been previously reported. Beyond this, there have been single reports of SPS in the setting of Hodgkin's lymphoma (HL). Here, we present a case of PERM associated with HL, with PERM preceding occurrence of lymphoma by more than seven months. Our observation has obvious implications for the management and, in particular, diagnostic evaluation of patients with PERM.Copyright 2010 Elsevier B.V. All rights reserved.

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