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Images in Neurology
April 2006

Pure Autonomic Failure With Altered Dopamine Transporter Imaging

Arch Neurol. 2006;63(4):604-605. doi:10.1001/archneur.63.4.604

A woman aged 72 years was diagnosed with primary orthostatic hypotension because of a 2-year history of recurrent syncope while standing. She also had constipation, decreased sweating, and urinary disturbance. Despite treatment with ephedrine and fludrocortisone acetate, her symptoms worsened dramatically at age 78 years while she was bedridden because of a vertebral fracture. At that time, she was referred to our institution, and her general neurological examination results were strictly normal. Her blood pressure dropped from 130/90 mm Hg to 70/40 mm Hg at 70° tilting, with decreased heart rate variability during phase IV of the Valsalva maneuver. The plasma norepinephrine level was low in the supine position and failed to increase on standing. Electromyography ruled out peripheral neuropathy. Cardiac iodine 123–metaiodobenzylguanidine uptake was markedly reduced, making the diagnosis of multisystem atrophy unlikely. Cranial magnetic resonance imaging showed no abnormalities. A diagnosis of pure autonomic failure (PAF) was made. To investigate nigrostriatal neuronal integrity, dopamine transporter imaging using single-photon emission computed tomography with iodine 123 ioflupane was performed, showing a reduced uptake in the left caudate and both putamen (Figure, B). Postural rehabilitation and increased water intake enabled the patient to stand up and walk again. At present, 11 years after the onset of her symptoms, dysautonomy persists and is isolated.

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