RT Journal Article
SR Electronic
T1 THUR 219 Myopathic manifestations in haematological conditions
JF Journal of Neurology, Neurosurgery & Psychiatry
JO J Neurol Neurosurg Psychiatry
FD BMJ Publishing Group Ltd
SP A31
OP A31
DO 10.1136/jnnp-2018-ABN.109
VO 89
IS 10
A1 Vinojini, Vivekanandam
A1 H, Devine
A1 J, Holton
A1 Z, Jaunmuktane
A1 C, Turner
A1 P, Machado
A1 H, Manji
A1 C, McNamara
A1 S, D’Sa
A1 A, Carr
YR 2018
UL http://jnnp.bmj.com/content/89/10/A31.3.abstract
AB Neurological complications of haematological malignancies are wide ranging but isolated myopathy as a presenting complaint is rare.Cases: 63 year-old lady with progressive proximal upper then lower limb weakness over 7 years to wheelchair-dependence and hoist-transfers. Muscle biopsy revealed nemalin rods, HIV negative, IgG kappa paraprotein=6 g/L. Late-onset nemalin rod myopathy was diagnosed; function is stable 6/12 post-autologous bone marrow transplant.56 year-old lady with limb-girdle weakness leading to immobility over 2 years and IgG lambda MGUS. Re-examination of muscle biopsy revealed amyloid deposition, SAP scan negative for systemic AL amyloid. Treated with velcade and dexamethasone but died from amyloid cardiomyopathy 6/12 later.73 year-old man with painless deltoid weakness. Muscle biopsy revealed plasma cells infiltrate and lightchain deposition with matched lightchain restriction in bone marrow, IgG kappa paraprotein=8 g/L. Cyclosporin and dexamethasone were recommended for infiltrative multifocal, myopathic plasmacytoma.28 year-old female with pain, fatigue and pelvic-girdle weakness was diagnosed with haemophagocytic lymphohistiocytosis without systemic lymphoma or connective tissue disease. Muscle biopsy demonstrated T-cell and macrophage infiltration. Muscle symptoms are responding to steroids, mycophenolate, cyclosporine and etopiside. An allogenic bone marrow transplant is planned.We describe four cases of progressive myopathic weakness as the primary manifestation of an underlying haematological disorder.