title: Progressive Ataxia and Palatal Tremor: Think about POLG Mutations.
abstract: BACKGROUND
  Progressive ataxia and palatal tremor (PAPT) can be observed in both acquired brainstem or cerebellar lesions and genetic disorders.
  PAPT due to mutation in POLG, the gene encoding the mitochondrial DNA polymerase.
  POLG mutation should be considered in patients with PAPT, particularly when additional clues such as a sensory neuronopathy or an ophthalmoplegia are present.
authors.affiliation: CHRU de Tours, Hôpital Bretonneau, Service de Neurologie, Tours, France; Université François Rabelais de Tours, Tours, France.
authors.forename: Marie
authors.initials: M
authors.lastname: Mongin
authors.affiliation: Département de Neurologie, Assistance Publique - Hôpitaux de Paris, Hôpital de la Pitié Salpêtrière, Paris, France; Inserm U 1127, Sorbonne Universités, Institut du Cerveau et de la Moelle épinière, ICM, Paris, France.
authors.forename: Cécile
authors.initials: C
authors.lastname: Delorme
authors.affiliation: Département de Neurophysiologie, Assistance Publique - Hôpitaux de Paris, Hôpital de la Pitié Salpêtrière, Paris, France.
authors.forename: Timothée
authors.initials: T
authors.lastname: Lenglet
authors.affiliation: AP-HP, Hôpital de la Pitié Salpêtrière, Service de Biochimie Métabolique et Centre de Génétique moléculaire et chromosomique, Paris, France; Inserm U 1016, Institut Cochin, Paris, France.
authors.forename: Claude
authors.initials: C
authors.lastname: Jardel
authors.affiliation: Neuroophthalmology Department, Rothschild Ophthalmologic Foundation, Paris, France.
authors.forename: Catherine
authors.initials: C
authors.lastname: Vignal
authors.affiliation: Département de Neurologie, Assistance Publique - Hôpitaux de Paris, Hôpital de la Pitié Salpêtrière, Paris, France; Inserm U 1127, Sorbonne Universités, Institut du Cerveau et de la Moelle épinière, ICM, Paris, France.
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Roze
keywords: Polymerase gamma mutation; ganglionopathy; ophthalmoplegia; progressive ataxia and palatal tremor