Paraneoplastic Cerebellar Degeneration Associated with Metastatic Neuroendocrine Tumor

Paraneoplastic Cerebellar Degeneration Associated with Metastatic Neuroendocrine Tumor

Patel A; Brooks AK; Itrat A

Journal of Medical Sciences at NEOMED

2024

Paraneoplastic cerebellar degeneration (PCD) is a rare disorder characterized by progressively worsening cerebellar dysfunction associated with onconeural antibodies. Commonly, these antibodies are anti-neuronal nuclear antibody type 1 (ANNA-1, also known as anti-Hu) and Purkinje cell cytoplasmic antibody (PCA-1, also known as anti-Yo). Here, we report a unique presentation of PCD. A 60-year-old man presented to the outpatient clinic with worsening ataxia. Over the span of three weeks, he progressed to walking with a cane and then required a wheelchair. Upon examination, the patient was found to have moderate dysmetria and substantial gait and truncal ataxia. Magnetic resonance imaging (MRI) did not show any cerebellar atrophy or acute intracranial process; however, CT chest/abdomen and pelvis identified a right hepatic lesion with biopsy demonstrating a metastatic small-cell neuroendocrine tumor (NET) of unknown primary origin. The serum paraneoplastic antibody panel revealed three positive antibodies: dipeptidyl-peptidase–like protein 6 (anti-DPPX) antibody, glutamic acid decarboxylase-65 (GAD-65) antibody, and P/Q-type voltage-gated calcium channel (VGCC) antibody simultaneously. This is the first case of PCD with simultaneous detection of multiple antibodies in the context of a metastatic neuroendocrine tumor (NET).

paraneoplastic cerebellar degeneration; neuroendocrine tumor; dipeptidyl-peptidase-like protein 6 antibody; glutamic acid decarboxylase -65; type voltage gated calcium channel antibody

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