[POEMS Syndrome with central and peripheric nervous system demyelination].
POEMS syndrome is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes. This polyneuropathy belongs to the chronic inflammatory demyelinating polyradiculoneuropathies (CIDP) group. Sporadic reports have emphasized the clinical association between CIDP and central nervous system (CNS) demyelination, but as far as we know not in the context of POEMS syndrome. We report the case of a 67 years old patient who developed POEMS syndrome associated to Castleman's disease, characterized by demyelinating polyradiculoneuropathy, hepatosplenomegaly, mediastinal and supraclavicular adenopathies and monoclonal IgG lambda gammapathy. Cranial magnetic resonance imaging disclosed diffuse alteration of the cerebral white matter highly suggestive of demyelination. CNS demyelination can be another feature of POEMS syndrome. Cranial MRI should be performed in patients with POEMS syndrome in order to verify this new feature.
Masjuan Vallejo
J
J
Servicio de Neurología, Hospital Ramón y Cajal, Madrid. jmasjaun@saludalia.com
Herrero Valverde
A
A
Mera Campillo
J
J
López Jiménez
J
J
D000368:Aged; D005871:Castleman Disease / Q000473:pathology / Q000503:physiopathology; D002490:Central Nervous System / Q000473:pathology*; D015897:Comorbidity; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D016878:POEMS Syndrome / Q000175:diagnosis / Q000473:pathology* / Q000503:physiopathology; D017933:Peripheral Nervous System / Q000473:pathology*; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000175:diagnosis / Q000473:pathology* / Q000503:physiopathology