WOBBLE, WOBBLE – A PARKINSON’S DISEASE MIMICKER A. Reine MD, R. Thibodaux MD Department of Medicine, LSU Health Sciences Center, Baton Rouge, LA
INTRODUCTION: Paraneoplastic neurologic syndromes (PNSs) are rare, usually autoimmune disorders associated with malignancy and not caused by direct tumor invasion, metastasis or consequences of therapy. PNSs typically precede the diagnosis of the malignancy making the diagnosis quite difficult. Almost 30 autoantibodies have been identified and are usually directed against intracellular neuronal proteins or neuronal cell surface proteins. PNSs can affect the central, peripheral, and autonomic nervous systems. Neuroimaging is often unrevealing, and the diagnosis is made by demonstrating a specific paraneoplastic autoantibody. CASE: A 61-year-old woman with hypertension, hypothyroidism, type 2 diabetes, hyperlipidemia, and endometrial carcinoma (Stage IIIc with metastatic intraabdominal nodes) presented for right hand tremors. She had undergone hysterectomy, chemotherapy and radiation 1 year prior with significant improvement in her lymphadenopathy and stable disease noted on most recent abdominal imaging three months prior. She presented 2 months later with worsening tremor affecting writing, “wobbly” gait, and recurrent falls. MRI of the brain revealed no acute intracranial abnormality and toxic/metabolic evaluation was unrevealing. She was diagnosed and treated for Parkinson disease however, her symptoms continued to progress. A paraneoplastic antibody panel was obtained and was markedly positive (1:491,520 titer) for PCA-1(purkinje cell cytoplasmic antibody type 1 or Anti-Yo) which is associatedwith paraneoplastic cerebellar degeneration (PCD). Subsequent surveillance CT scans revealed worsening intraabdominal lymphadenopathy as well as bilateral pleural effusions for which a thoracentesis was performed revealing metastatic endometrial carcinoma. Systemic chemotherapy was unsuccessful due to intolerable side effects and she was discharged to home hospice. DISCUSSION: PCA-1 is a paraneoplastic autoantibody associated with PCD and occurs primarily in patients with breast or gynecological malignancies. The target for these antibodies are the cerebellar degeneration related proteins expressed by purkinje, breast cancer, and gynecological cancer cells. PCA-1 associated PNS occurs in less than 1% of associated malignancies and usually presents with symptoms of acute to subacute truncal and/or limb ataxia, dizziness, diplopia, and dysarthria. Severe disability is common with greater than 90% of affected patients. Brain imaging at the time of presentation of symptoms does not commonly reveal cerebellar atrophy. Treatment of the tumor is critical for any neurologic recovery, although response rates are typically poor. Nevertheless, PNSs are important to recognize as they may allowmore prompt diagnosis and treatment of an otherwise occult malignancy.
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