title: Autoimmune atypical parkinsonism - A group of treatable parkinsonism.
abstract: BACKGROUND
  Immunological causes of atypical parkinsonism/Parkinson plus syndromes are rare.
  
  
  OBJECTIVE
  To study the clinical and laboratory features and treatment outcome of autoimmune atypical parkinsonism.
  
  
  METHODS
  Retrospective case series. Patients with atypical parkinsonism and positive antibodies were identified retrospectively. Those who received immunotherapy (intravenous methyl prednisolone 1g daily for five days followed by mycophenylate mofetil 2g daily or azathioprine 2-3mg/kg/day) and consented for publication of non-anonymized videos were included.
  
  
  RESULTS
  There were ten cases (nine males, age range 49-75years, disease duration 2months to 13years, follow-up 1-7months) of atypical parkinsonism [probable multiple system atrophy (MSA)-2, possible progressive supranuclear palsy (PSP)-1, probable PSP-3]. Eight had new uncharacterized neuronal antibodies, leucine rich glioma associated protein 1 (LGI1) antibody in one, and the other had another uncharacterized neuronal antibody along with LGI1 antibody. Four had abnormal CSF. There was a prompt, dramatic improvement in terms of Unified Parkinson Disease Rating Scale motor scale and or modified Rankin Scale as well as improvement in eye movement, postural instability, cerebellar, autonomic and non-motor symptoms. Two had reappearance of symptoms on discontinuing steroids and improvement on restarting. One died of infection despite good recovery of encephalopathy and parkinsonism.
  
  
  CONCLUSIONS
  Autoimmune atypical parkinsonism is characterized by atypical parkinsonism with neuronal specific antibodies, sometimes associated with abnormal CSF and significant response to immunotherapy.
authors.affiliation: Department of Neurology, Amrita Institute of Medical Sciences, Ponekkara PO, Kochi 682041, Kerala, India; Neuroimmunology Laboratory, Amrita Institute of Medical Sciences, Ponekkara PO, Kochi 682041, Kerala, India. Electronic address: sudheerank@aims.amrita.edu.
authors.forename: Sudheeran
authors.initials: S
authors.lastname: Kannoth
authors.affiliation: Department of Neurology, Amrita Institute of Medical Sciences, Ponekkara PO, Kochi 682041, Kerala, India.
authors.forename: Anandkumar
authors.initials: A
authors.lastname: Anandakkuttan
authors.affiliation: Department of Neurology, Amrita Institute of Medical Sciences, Ponekkara PO, Kochi 682041, Kerala, India; Neuroimmunology Laboratory, Amrita Institute of Medical Sciences, Ponekkara PO, Kochi 682041, Kerala, India.
authors.forename: Annamma
authors.initials: A
authors.lastname: Mathai
authors.affiliation: Department of Neurology, Amrita Institute of Medical Sciences, Ponekkara PO, Kochi 682041, Kerala, India; Neuroimmunology Laboratory, Amrita Institute of Medical Sciences, Ponekkara PO, Kochi 682041, Kerala, India.
authors.forename: Anuja Nirmala
authors.initials: AN
authors.lastname: Sasikumar
authors.affiliation: Department of Neurology, Amrita Institute of Medical Sciences, Ponekkara PO, Kochi 682041, Kerala, India.
authors.forename: Vivek
authors.initials: V
authors.lastname: Nambiar
mesh_terms: D000368:Aged; D001327:Autoimmune Diseases / Q000150:complications* / Q000628:therapy*; D001379:Azathioprine / Q000627:therapeutic use; D005260:Female; D005968:Glutamate Decarboxylase / Q000097:blood; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007167:Immunotherapy / Q000379:methods*; D047908:Intracellular Signaling Peptides and Proteins; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000627:therapeutic use; D016202:N-Methylaspartate / Q000276:immunology; D010300:Parkinson Disease / Q000150:complications* / Q000628:therapy*; D011506:Proteins / Q000276:immunology; D012189:Retrospective Studies
chemical_list: D007166:Immunosuppressive Agents; D047908:Intracellular Signaling Peptides and Proteins; C116499:LGI1 protein, human; D011506:Proteins; D016202:N-Methylaspartate; D005968:Glutamate Decarboxylase; C401141:glutamate decarboxylase 2; D009173:Mycophenolic Acid; D001379:Azathioprine
keywords: Atypical parkinsonism; Autoimmune atypical parkinsonism; Multiple system atrophy; Parkinson plus syndrome; Progressive supranuclear palsy