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Peripheral neuropathy as clinical onset of monoclonal IgM/k-related amyloidosis.
Briani, Chiara; Ferrari, Sergio; Berno, Tamara; Visentin, Andrea; Cacciavillani, Mario; Cavallaro, Tiziana; Fedrigo, Marny; Rizzo, Stefania; Salvalaggio, Alessandro; Trentin, Livio; Piazza, Francesco.
Afiliación
  • Briani C; Department of Neurosciences, University of Padova, Padova, Italy.
  • Ferrari S; Neurology Unit, Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona, Padova, Italy.
  • Berno T; Hematology and Clinical Immunology Unit, Department of Medicine, University of Padova, Padova, Italy.
  • Visentin A; Hematology and Clinical Immunology Unit, Department of Medicine, University of Padova, Padova, Italy.
  • Cacciavillani M; Datamedica Group, CEMES SynLab, Padova, Italy.
  • Cavallaro T; Neurology Unit, Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona, Padova, Italy.
  • Fedrigo M; Cardiovascular Pathology, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, Padova, Italy.
  • Rizzo S; Cardiovascular Pathology, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, Padova, Italy.
  • Salvalaggio A; Department of Neurosciences, University of Padova, Padova, Italy.
  • Trentin L; Hematology and Clinical Immunology Unit, Department of Medicine, University of Padova, Padova, Italy.
  • Piazza F; Hematology and Clinical Immunology Unit, Department of Medicine, University of Padova, Padova, Italy.
J Peripher Nerv Syst ; 28(2): 262-265, 2023 06.
Article en En | MEDLINE | ID: mdl-36859783
AIM: Neuropathy is a frequent complication of Waldenström's macroglobulinemia (WM), the most common being a demyelinating polyneuropathy with anti-myelin associated glycoprotein (MAG) antibodies, but also cryoglobulins, vasculitis, neurolymphomatosis, and amyloidosis. We describe a patient with IgM/kappa WM who presented with a severe, not length-dependent, peripheral neuropathy as clinical onset of IgM/kappa-related amyloidosis. METHODS: A 69-year-old woman came to our attention for weight loss, gait imbalance and sensory loss at upper limbs. In her medical history, she was in hematological follow-up for WM, and had undergone left carpal tunnel release. At neurological evaluation she had weakness and loss of sensation at upper limbs up to the elbows, more at the left side, gait was unsteady with right foot drop. Hypotrophy and areflexia were present at four limbs. Sensory loss and vibration sense were dramatically reduced. She underwent extensive diagnostic workup. RESULTS: Laboratory workup revealed an IgM/kappa monoclonal paraprotein of 16 g/L and increased NT-proBNP; anti-MAG antibodies were absent. Bone marrow biopsy demonstrated a population of neoplastic B-lymphocytes. Total-body CT scan and echocardiogram were negative. Neurophysiology revealed a symmetric, no length dependent sensory-motor polyneuropathy Periumbilical fat biopsy was positive for amyloid. Sural nerve biopsy detected amyloid in the wall of an epineurial vein. CONCLUSIONS: This case report describes a rare and unusual manifestation of IgM-related AL amyloidosis in WM. The patient presented with a subacute clinically asymmetric neuropathy with no pain or dysautonomic features as clinical onset of IgM/kappa-related amyloidosis. Sural nerve biopsy was crucial for the diagnosis.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Polineuropatías / Macroglobulinemia de Waldenström / Enfermedades del Sistema Nervioso Periférico / Amiloidosis Tipo de estudio: Diagnostic_studies / Etiology_studies Límite: Aged / Female / Humans Idioma: En Revista: J Peripher Nerv Syst Asunto de la revista: NEUROLOGIA Año: 2023 Tipo del documento: Article País de afiliación: Italia Pais de publicación: Estados Unidos
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Polineuropatías / Macroglobulinemia de Waldenström / Enfermedades del Sistema Nervioso Periférico / Amiloidosis Tipo de estudio: Diagnostic_studies / Etiology_studies Límite: Aged / Female / Humans Idioma: En Revista: J Peripher Nerv Syst Asunto de la revista: NEUROLOGIA Año: 2023 Tipo del documento: Article País de afiliación: Italia Pais de publicación: Estados Unidos