Subject(s)
Osteochondrodysplasias , Humans , Osteochondrodysplasias/complications , Osteochondrodysplasias/diagnostic imaging , Osteochondrodysplasias/diagnosis , Congenital Bone Marrow Failure Syndromes/drug therapy , Congenital Bone Marrow Failure Syndromes/complications , Male , Female , Treatment Outcome , Pancytopenia/etiology , Pancytopenia/drug therapy , Cytopenia , Anemia, RefractoryABSTRACT
OBJECTIVE: We detailed the electrophysiological patterns of peripheral nerve temporal dispersion across spectrum of POEMS syndrome and Castleman disease (CD). METHODS: Compound muscle action potentials (CMAP) duration of 3 patients with POEMS syndrome and 2 with hyaline vascular type CD without clonal plasma cell dyscrasia were retrospectively analysed. RESULTS: Median and ulnar nerves distal CMAP duration were prolonged in all patients irrespective of plasma cell dyscrasia or M protein. All lower limbs distal CMAP responses were absent. Greatest distal CMAP duration prolongation was observed in median nerves for POEMS syndrome (17.0â¯ms, 158% upper limit normal) and in ulnar nerves for CD (9.8â¯ms, 47% upper limit normal). Distal/proximal CMAP duration ratio of <0.7 were seen in 33% of median and ulnar nerves studied among POEMS syndrome. Among nerves with ratio >0.7, all had distal CMAP duration prolongation (Range 7%-158% of upper limit normal). CONCLUSIONS: Abnormal distal CMAP dispersion is not uncommon in POEMS syndrome and CD without clonal plasma cell dyscrasia or M protein. POEMS syndrome has greater distal CMAP duration in median and ulnar nerves, particularly in median nerve that can reach up to 150% of upper limit normal, compared to <50% in CD. SIGNIFICANCE: Detailed electrophysiological analysis of distal CMAP duration may help in distinguishing POEMS syndrome and CD.
ABSTRACT
Two previously healthy Malaysian women presented abruptly with severe diabetic ketoacidosis during pregnancy and immediate postpartum period. Their clinical courses, biochemical and immunological profiles were consistent with fulminant type 1 diabetes first described in the Japanese. Fulminant type 1 diabetes may not be as rare as currently reported outside Japan.