1. Purkinje cell cytoplasmic autoantibody type 1 accompaniments: the cerebellum and beyond.
- Author
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McKeon A, Tracy JA, Pittock SJ, Parisi JE, Klein CJ, and Lennon VA
- Subjects
- Adult, Aged, Aged, 80 and over, Arizona epidemiology, Breast Neoplasms complications, Breast Neoplasms immunology, Carcinoma complications, Carcinoma immunology, Central Nervous System Diseases cerebrospinal fluid, Female, Florida epidemiology, Humans, Male, Middle Aged, Minnesota epidemiology, Neurologic Examination, Peripheral Nervous System Diseases cerebrospinal fluid, Phosphopyruvate Hydratase metabolism, Primary Dysautonomias cerebrospinal fluid, Central Nervous System Diseases pathology, Cerebellum metabolism, Guanine Nucleotide Exchange Factors immunology, Immunoglobulin G metabolism, Peripheral Nervous System Diseases pathology, Primary Dysautonomias pathology, Spectrin immunology
- 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%), 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.
- Published
- 2011
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