[P1-137] Two cases of Acute autonomic and sensory neuropathy
Acute autonomic and sensory neuropathy (AASN) is rare disorder characterized by dysautonomia with sensory impairment. We report two cases of AASN with mild motor dysfunction. Case 1 was a 7-year-old girl who presented with muscle weakness, areflexia, loss of light reflex and neurogenic bladder following Kawasaki disease. Brain magnetic resonance imaging (MRI) had no abnormalities and spinal MRI showed Gadolinium enhancement of cauda equina. Nerve conduction studies revealed reduction of compound muscle action potentials (CMAP) and absence of sensory nerve action potentials (SNAP). She was negative to all autoantibodies. She received intravenous immunoglobulin and high-dose methylprednisolone therapy, but autonomic symptoms such as vomiting, abdominal pain and pupil dilation remained. Case 2 was a 9-year-old girl who entered our hospital because of consciousness disorder due to hyponatremia associated with inappropriate secretion of antidiuretic hormone hypersecretion (SIADH). She developed muscle weakness, areflexia, loss of light reflex and neurogenic bladder. Spinal MRI showed Gadolinium enhancement of cauda equina. SNAP was absent in nerve conduction studies. She was found to have positive ganglionic acetylcholine receptor (AChR) autoantibody. After intravenous immunoglobulin and high-dose methylprednisolone therapy, ganglionic AChR autoantibody level reverted to normal value and her symptoms including gastrointestinal dysfunction were improved gradually. Because the immunotherapy was more effective in case 2 than in case 1, the presence or absence of antibody affected the reactivity of the treatment. Although the detailed pathophysiology of AASN is unknown, the presence of antibodies is useful for estimating the effectiveness of immunotherapy and prognosis.