Immune-Mediated Autonomic Neuropathies following Allogeneic Stem Cell Transplantation in Acute Myeloid Leukemia
Table 1
Table summarizing results of clinical/laboratory testing, treatment response, and outcomes in the two cases.
Test
Case 1
Case 2
Autonomic reflex screen
(i) Moderate cardiovagal, widespread postganglionic sudomotor and severe adrenergic impairment on this study. (ii) Decreased heart rate responses to deep breathing and Valsalva maneuver. (iii) QSWEAT responses reduced at all sites except the forearm.
(i) Significant adrenergic failure with mild cardiovagal and probable distal postganglionic sudomotor impairment. (ii) Heart rate responses to deep breathing and Valsalva maneuver were reduced. (iii) The quantitative sudomotor axon reflex tests were normal for all sites except marked reduction at foot.
Antibody tests (all were undetectable) tested in addition for case 2
ANNA-1, striated muscle, acetylcholine receptor (AChR muscle binding and neuronal ganglionic), neuronal K+ channel, GAD-65, and N- and P/Q type calcium channel, ganglioside, , Purkinje Cell .
Treatment
IVIG 0.4 gm/kg for 5 days.
IVIG at 0.4 gm/kg for 5 days, daily plasmapheresis, prednisone at 1 mg/kg.
Response
Significant symptomatic improvement. Posttreatment autonomic reflex testing not performed.
Significant symptomatic improvement in two weeks. Posttreatment autonomic reflex screen showed normal heart rate responses to deep breathing and Valsalva maneuver and normal quantitative sudomotor axon reflex tests at all sites except the foot.