The eponym “Meige syndrome” and the term “cranio-cervical dystonia” have been used inter-changeably in the literature and recently the validity of this eponym has been debated.

To study the uniqueness of the term “Meige syndrome”, from a large cohort of patients with cranio-cervical dystonia.

We describe the 15 years follow-up of a large cohort of 50 patients with Meige syndrome, seen in a neurology movement disorder clinic and in a multidisciplinary neuro-ear-nose-throat (ENT) clinic. Following the original description of Henry Meige (1866–1940), we have included patients with blepharospasm (BSP) at onset and anatomical spread of dystonia to the jaw, mouth floor and neck, to describe a clinical phenotype of Meige syndrome among the cranio-cervical dystonias.

We report 2 groups: group 1, including 12 patients with cranial dystonia, with BSP spreading around the nose, the mouth, the masticatory muscles with opening or closing jaw dystonia; group 2, including 38 patients with cranio-cervical dystonia, with BSP spreading further to the mouth floor muscles and to the neck including cervical and laryngeal muscles. Among them, 20/38 patients had a clinical presentation dominated by ENT sphere symptoms. In 18/38 patients, the clinical picture is dominated by an anterocollis posture, progressing in the 7 most elderly patients to an extreme posture of chin on sternum.

The eponym Meige syndrome should be reserved for a particular group of dystonic patients, unique in its clinical midline spasm phenotype, and in its rostro-caudal progression, starting with a BSP and resulting in a clinical spectrum with a continuum from cranial to cranio-cervical dystonia. Stormy dystonic episodes, often precipitated by stress, are important to recognise and can be a therapeutic challenge.