Late-Onset PANDAS Syndrome with Abdominal Muscle Involvement

Abstract

Case Report We observed a 20-year-old woman, with a negative medical and family history, referred to our Movement Disorder Clinic for belly twitching, restlessness, and insomnia. All symptoms had an abrupt onset 6 months before admission, some weeks after an antibioticresponsive sore throat. On admission, she presented motor hyperactivity, a variety of subtle choreic movements, involving neck and upper and lower limbs, and bilateral synchronous or asynchronous abdominal muscle contractions causing the umbilicus to move around the abdomen, without stimulus sensitiveness, uninfluenced by posture or voluntary movements. Brain and spine magnetic resonance imaging, EEG, and somatosensory evoked potentials were normal. Laboratory tests revealed an elevated antistreptolysin O titer (379 U, normal !200 U). Polygraphic evaluation showed intermittent involuntary bursts of marked variability in duration (800– 1,200 ms) with a semirhythmic low repetition (0.1 Hz) rate in the rectus abdominis muscles, synchronous on both sides, with synchronous or asynchronous bursts lasting 50–500 ms in both oblique muscles (fig. 1). Diazepam at an oral dose of 5 mg/day was followed by a slight clinical improvement. One year later, she became anxious and hyperkinetic; she started dreading poisoning and developed compulsive behaviors (changing her underclothes and washing her hands frequently) some days after a febrile illness. Physical examination disclosed variable motor and vocal tics like throat clearing, sniffing, shoulder shrugging, head and body swinging, piano-playing movements of her fingers, moving toes. The tics showed voluntary suppressibility with a subsequent rebound. Abdominal wall contractions were still present. Laboratory tests revealed elevated antideoxyribonuclease B (319 U, normal !200 U) and antistreptolysin O (277 U, normal !200 U) titers. Electrocardiographic findings were normal. Polygraphic recording showed pseudoperiodic (near 0.5 Hz) isolated or short trains of twitches in the oblique muscles (data not shown), sparse twitches, and isolated jerks or groups of clonic bursts of 250–1,000 ms duration with a repetition rate of 1–0.5 Hz on the paraspinal muscles of T8 level; asymmetric bursts with a duration of 800–1,200 ms, grouped in trains of 8–10 discharges, were recorded from electrodes placed at the intercostal level T6 (fig. 2). The involuntary movements disappeared during sleep.