Progressive encephalomyelitis with rigidity and myoclonus preceding otherwise asymptomatic Hodgkin's lymphoma.
Stiff-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.
Schmidt
Caroline
C
Department of Neurology, Klinikum Grosshadern der Ludwig-Maximilians-Universität München, Munich, Germany.
Freilinger
Tobias
T
Lieb
M
M
Rémi
J
J
Klein
M
M
Straube
A
A
Pfefferkorn
T
T
D018450:Disease Progression; D004679:Encephalomyelitis / Q000150:complications* / Q000000981:diagnostic imaging; D005500:Follow-Up Studies; D006689:Hodgkin Disease / Q000150:complications* / Q000000981:diagnostic imaging; D006801:Humans; D008297:Male; D009127:Muscle Rigidity / Q000150:complications* / Q000000981:diagnostic imaging; D009207:Myoclonus / Q000150:complications* / Q000000981:diagnostic imaging; D049268:Positron-Emission Tomography; D016750:Stiff-Person Syndrome / Q000150:complications / Q000000981:diagnostic imaging; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D055815:Young Adult