title: Purkinje cell cytoplasmic autoantibody type 1 accompaniments: the cerebellum and beyond.
abstract: OBJECTIVE
  To investigate the full extent of Purkinje cell cytoplasmic autoantibody type 1 autoimmunity (classically associated with paraneoplastic cerebellar degeneration) from clinical, immunohistochemical, and neuropathological perspectives.
  
  
  METHODS
  Case series.
  
  
  METHODS
  Mayo Clinics, 3 sites (Minnesota, Arizona, and Florida).
  
  
  METHODS
  Of 133,138 patients tested over a 21-year period, 83 (0.06%) were identified as seropositive for Purkinje cell cytoplasmic autoantibody type 1 IgG.
  
  
  METHODS
  The frequency of cerebellar and noncerebellar disorders and the clinical outcomes (neurological and oncological) of the patients.
  
  
  RESULTS
  All patients were women. At initial presentation, 64 patients (77%) had a cerebellar disorder, and 19 patients (23%) had an extracerebellar disorder. Over the clinical course, neurological symptoms and signs were multifocal in 50 patients (60%), and they involved the cerebellum (89% of patients), the pyramidal tract (30%), the brainstem (13%), and the spinal anterior horn cells or peripheral nerve (10%; frequently upper limb predominant); 11% of patients did not develop cerebellar ataxia. Serological and neuropathological findings were observed in the cerebellum, the brainstem, the spinal cord, the anterior horn, and the dorsal root ganglion that paralleled the diversity of clinical signs. After a median follow-up of 18 months, 1 or more carcinomas had been detected in 88% of patients: ovarian epithelial cancer (53%), breast cancer (22%), fallopian tubal cancer (11%), primary peritoneal cancer (5%), metastases of unknown primary cancer (4%), and other cancers (4%). Sustained improvement was reported in 15% of patients following oncological or immunological therapies. Voltage-gated calcium channel antibodies coexisted in 23 patients (28%).
  
  
  CONCLUSIONS
  Purkinje cell cytoplasmic autoantibody type 1 autoimmunity most commonly affects the cerebellum, but the spectrum of neurological symptoms and presentations is broad. Neurological outcomes are usually poor, even when cancer remission is achieved.
authors.affiliation: Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA. mckeon.andrew@mayo.edu
authors.forename: Andrew
authors.initials: A
authors.lastname: McKeon
authors.forename: Jennifer A
authors.initials: JA
authors.lastname: Tracy
authors.forename: Sean J
authors.initials: SJ
authors.lastname: Pittock
authors.forename: Joseph E
authors.initials: JE
authors.lastname: Parisi
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: Klein
authors.forename: Vanda A
authors.initials: VA
authors.lastname: Lennon
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001130:Arizona / Q000453:epidemiology; D001943:Breast Neoplasms / Q000150:complications / Q000276:immunology; D002277:Carcinoma / Q000150:complications / Q000276:immunology; D002493:Central Nervous System Diseases / Q000134:cerebrospinal fluid / Q000473:pathology*; D002531:Cerebellum / Q000378:metabolism*; D005260:Female; D005431:Florida / Q000453:epidemiology; D020662:Guanine Nucleotide Exchange Factors / Q000276:immunology*; D006801:Humans; D007074:Immunoglobulin G / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D008910:Minnesota / Q000453:epidemiology; D009460:Neurologic Examination; D010523:Peripheral Nervous System Diseases / Q000134:cerebrospinal fluid / Q000473:pathology*; D010751:Phosphopyruvate Hydratase / Q000378:metabolism; D054969:Primary Dysautonomias / Q000134:cerebrospinal fluid / Q000473:pathology*; D013049:Spectrin / Q000276:immunology*
chemical_list: D020662:Guanine Nucleotide Exchange Factors; D007074:Immunoglobulin G; C503982:PLEKHG4 protein, human; D013049:Spectrin; D010751:Phosphopyruvate Hydratase