Argente-Escrig Herminia, Bataller Luis, Krstulovic Roa Claudio, Pérez Guillén Vanesa, Perez Garrigues Herminio, Casanova Estruch Bonaventura
Department of Neurology, Hospital Universitari i Politècnic La Fe, 106 Fernando Abril Martorell Ave, 46026, Valencia, Spain.
Department of Otolaryngology, Hospital Universitari i Politècnic La Fe, Valencia, Spain.
J Neuroinflammation. 2017 Mar 31;14(1):71. doi: 10.1186/s12974-017-0846-1.
Acquired periodic alternating nystagmus (PAN) is a rare but well-defined syndrome that consists of a horizontal nystagmus that cyclically reverses its direction. PAN can be caused by degenerative, neoplastic, or toxic diseases of the cerebellum and, in a few cases, by subacute cerebellar ataxia of immune origin.
A 44-year-old man came to our attention because of rapidly progressive gait instability and blurred vision. Clinical examination showed PAN and a mild pancerebellar syndrome. Eye movement recordings disclosed a short cycle PAN with significant slow-phase velocity only in darkness. Under the effect of a γ-aminobutyric acid type B (GABA) agonist, PAN was not modified. Right after treatment with intravenous immunoglobulin (IVIg) was started, PAN was essentially eliminated. Three months after last dose of IVIg, this nystagmus reappeared.
IVIg resolved PAN in this patient. This finding may point to an autoimmune mechanism underlying this patient's nystagmus. This case suggests that the usefulness of IVIg at treating PAN might be worth a consideration in similar clinical settings.
获得性周期性交替性眼球震颤（PAN）是一种罕见但定义明确的综合征，其特征为水平眼球震颤周期性地改变方向。PAN可由小脑的退行性、肿瘤性或中毒性疾病引起，少数情况下由免疫源性亚急性小脑共济失调所致。
一名44岁男性因快速进展的步态不稳和视力模糊前来就诊。临床检查发现PAN及轻度全小脑综合征。眼动记录显示为短周期PAN，仅在黑暗中具有显著的慢相速度。在γ-氨基丁酸B型（GABA）激动剂作用下，PAN未发生改变。静脉注射免疫球蛋白（IVIg）治疗开始后，PAN基本消失。最后一剂IVIg治疗三个月后，这种眼球震颤再次出现。
IVIg使该患者的PAN得到缓解。这一发现可能提示该患者眼球震颤的自身免疫机制。该病例表明，在类似临床情况下，IVIg治疗PAN的有效性值得考虑。
