Marano Massimo, Motolese Francesco, Lanzone Jacopo, Di Santo Alessandro, Rossi Mariagrazia, Bevacqua Maria Gabriela, Ranieri Federico, Capone Fioravante, Gatto Emilia Mabel, Di Lazzaro Vincenzo
Neurology, Neurophysiology and Neurobiology Unit, Department of Medicine Università Campus Bio-Medico di Roma Rome Italy.
Psychiatry, "Psyche" Neurosciences clinic Rome Italy.
Mov Disord Clin Pract. 2020 Mar 5;7(3):313-317. doi: 10.1002/mdc3.12911. eCollection 2020 Apr.
Stiff-limb syndrome is part of stiff person spectrum, presenting with fluctuating gait disorders attributed to leg stiffness, spasms, and posturing. It could also manifest with anxiety and specific phobias such as pseudoagoraphobia. We aimed to describe the importance of specific gait phobia as a diagnostic clue to anti-glutamic acid decarboxylase stiff-limb syndrome.
We reported on 2 cases of stiff-limb syndrome sharing a similar diagnostic path and phenomenology. Both were featured by pseudoagoraphobia, which has documented to typically cover organic conditions, and a remarkable diagnostic delay attributed to misdiagnoses. Presence of pseudoagoraphobia should not point to the diagnosis of a functional disorder-although a negative instrumental workup is documented.
Both cases are emblematic of the high misdiagnosis rate affecting stiff person syndrome patients. A proper diagnostic process, including the identification of a pseudoagoraphobia, should help in reaching a diagnosis and providing an early and effective treatment.
僵腿综合征是僵人谱系障碍的一部分,表现为因腿部僵硬、痉挛和姿势异常导致的步态障碍波动。它也可能伴有焦虑和特定恐惧症,如假性广场恐惧症。我们旨在描述特定步态恐惧症作为抗谷氨酸脱羧酶僵腿综合征诊断线索的重要性。
我们报告了2例僵腿综合征病例,它们具有相似的诊断路径和临床表现。两者均以假性广场恐惧症为特征,该症状通常与器质性疾病相关,且由于误诊导致诊断显著延迟。尽管有阴性的辅助检查结果,但假性广场恐惧症的存在不应指向功能性障碍的诊断。
这两个病例均体现了僵人综合征患者误诊率高的问题。一个恰当的诊断过程,包括识别假性广场恐惧症,应有助于做出诊断并提供早期有效的治疗。