Adult-Onset Opsoclonus-Myoclonus Syndrome Associated With Ganglionic Acetylcholine Receptor Autoantibody  

Oleh:

Galli, Jonathan R. ; Clardy, Stacey L. ; Paz Soldan, M. Mateo

Jenis: Article from Journal - ilmiah internasional
Dalam koleksi: The Neurologist vol. 21 no. 06 (Nov. 2016), page 99-100.
Topik: Opsoclonus; Myoclonus; Paraneoplastic Syndrome; Autoimmune Diseases

Ketersediaan

Perpustakaan FK Nomor Panggil: N06.K Non-tandon: 1 (dapat dipinjam: 0) Tandon: tidak ada

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Isi artikel

Introduction: Opsoclonus-myoclonus syndrome (OMS) may have a toxin induced, parainfectious, or paraneoplastic etiology. Several autoantibodies have been associated with adult-onset OMS, most commonly antineuronal nuclear antibody 2 (Ri), and it is most frequently associated with breast or small cell lung cancer. The nicotinic ganglionic acetylcholine receptor autoantibody (a3-AChR Ab) has not been described in association. Case Report: A 46-year-old woman was evaluated for symptoms of oscillopsia, tremor, gait imbalance, and mild cognitive deficits that began 6 weeks prior. Neurological examination demonstrated opsoclonus, myoclonus, and mild gait ataxia. Laboratory evaluation revealed an elevated a3-AChR Ab at 0.27 nmol/L (normal =0.02 nmol/L) with no other autoantibodies or infectious etiology detected. Thorough screening revealed no evidence of associated malignancy. Immunotherapy with weekly methylprednisolone led to significant improvement. Conclusions: This first reported case of a3-AChR Ab positivity in the setting of adult-onset OMS expands the spectrum of associated autoantibodies. The mechanism of disease may be linked to cholinergic nuclei within the brainstem. This case suggests including a3-AChR Ab in the evaluation of adult-onset OMS, and highlights the importance of further understanding a3-AChR within the brain.

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