Case Report

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.

TestCase  1Case  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 .

TreatmentIVIG 0.4 gm/kg for 5 days.IVIG at 0.4 gm/kg for 5 days, daily plasmapheresis, prednisone at 1 mg/kg.

ResponseSignificant 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.