Revista de neuroinmunología clínica y experimental

Abstracto

Parsonage: Turner Syndrome or Amyotrophic Neuralgia

M. Fournier Mehouas

Parsonage and Turner syndrome or amyotrophic neuralgia manifests itself by the sudden onset of violent pain in the shoulder area, followed by paralysis and amyotrophy of the muscles of the scapular belt, frequently accompanied by sensory disturbances. It is essentially an attack on the brachial plexus, most commonly on the upper trunk, but it can also be located outside the brachial plexus (cranial nerves, phrenic nerve, or even lumbosacral plexus). The diagnosis is based above all on the clinical history and physical examination, confirmed by additional tests: Electromyogram, high-resolution ultrasound, magnetic resonance imaging, which will make it possible to specify the topography of the damage and eliminate differential diagnoses, mainly shoulder tendon damage and cervical radiculopathies. In addition to idiopathic forms, there are more severe and recurrent familial forms with autosomal dominant transmission, often associated with dysmorphia. Their genetic diagnosis is based on the mutation of the Septin Gene (SEPT9). The etiology remains unknown at present, a dysimmunitary hypothesis associated with certain favourable factors, essentially infectious and vaccinal, is suspected. The treatment is symptomatic, based in the acute phase on analgesic treatments or even corticotherapy and on re-education adapted according to the phase. The evolution is favourable in the majority of cases, with recovery over a few months and in 70% of cases a total recovery at 3 years. However, motor after-effects and recurrences are possible.