Purkinje cell cytoplasmic autoantibody type 1 accompaniments

The cerebellum and beyond

Research output: Contribution to journalArticle

31 Citations (Scopus)

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. Design: Case series. Setting: Mayo Clinics, 3 sites (Minnesota, Arizona, and Florida). Patients: 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. Main Outcome Measures: 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%), primaryperitoneal 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-gatedcalcium 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.

Original languageEnglish (US)
Pages (from-to)1282-1289
Number of pages8
JournalArchives of Neurology
Volume68
Issue number10
DOIs
StatePublished - Oct 2011

Fingerprint

Purkinje Cells
Autoantibodies
Cerebellum
Cerebellar Diseases
Neoplasms
Autoimmunity
Brain Stem
Accompaniment
Cells
Paraneoplastic Cerebellar Degeneration
Anterior Horn Cells
Breast Neoplasms
Cerebellar Ataxia
Pyramidal Tracts
Spinal Ganglia
Peripheral Nerves
Upper Extremity
Signs and Symptoms
Cancer
Immunoglobulin G

ASJC Scopus subject areas

  • Clinical Neurology
  • Arts and Humanities (miscellaneous)

Cite this

Purkinje cell cytoplasmic autoantibody type 1 accompaniments : The cerebellum and beyond. / McKeon, Andrew B; Tracy, Jennifer A.; Pittock, Sean J; Parisi, Joseph E; Klein, Christopher Jon; Lennon, Vanda A.

In: Archives of Neurology, Vol. 68, No. 10, 10.2011, p. 1282-1289.

Research output: Contribution to journalArticle

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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. Design: Case series. Setting: Mayo Clinics, 3 sites (Minnesota, Arizona, and Florida). Patients: 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. Main Outcome Measures: 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{\%}), primaryperitoneal 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-gatedcalcium 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.",
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AU - Klein, Christopher Jon

AU - Lennon, Vanda A

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