CASE 1

A 66 year old woman suffered right jaw and multiple limb fractures when she was 45. Since then she had recurrent sudden onset episodes of right hemifacial flushing and sweating, occurring with physical exertion and heat, rarely spontaneously, and lasting 20 to 30 minutes. Approximately one year after the trauma, her sleep became restless, being interrupted by excessive motor activity, seemingly purposeful and sometimes harmful, associated with vocalisation and a report of dreaming corresponding to the motor manifestations. Hypnagogic hallucinations and sleep paralysis could also rarely occur. Both facial flushing and sleep disturbances worsened after another accident to her face when she was 64.

Examination at age 66 showed partial ptosis of the left eyelid, myosis, and iris heterochromia with subtle enophthalmos. Anisocoria increased in dim light, and 0.01% epinephrine eye drops did not modify the size of the left pupil. Brain magnetic resonance imaging (MRI), somatosensory evoked potentials, transcranial magnetic stimulation, blink reflexes and palmar sympathetic skin responses, baseline and tilt test cardiovascular reflexes, and circadian body core temperature rhythm were all normal. Sweating—assessed by the application of alizarin powder during a heating test¹—was absent on the left side of the face and reduced on the right upper limb. After heating, only the right side of the face showed flushing. These changes were replicated by physical exercise. There was no …