Codeluppi Luca, Bigliardi Guido, Chiari Annalisa, Meletti Stefano
Department of Biomedical Science, Metabolism and Neuroscience, University of Modena and Reggio Emilia, Modena, Italy.
BMJ Case Rep. 2013 Oct 16;2013:bcr2013200777. doi: 10.1136/bcr-2013-200777.
Paroxysmal dysarthria is an unusual condition characterised by brief episodes of dysarthria with the sudden onset and frequent recurrence. It has been mainly reported in multiple sclerosis and an association with midbrain lesions has been claimed; however, most of the reported patients had multiple brain alterations so it was difficult to associate this symptom with a specific lesion site. We illustrate the cases of two patients with an isolated demyelinating midbrain lesion presenting paroxysmal dysarthria as the only symptom; both participants had oligoclonal bands in the cerebrospinal fluid and an unremarkable follow-up. Both patients had benefit from carbamazepine treatment, similarly to previously reported cases. Our report confirms that a demyelinating midbrain lesion is sufficient to provoke paroxysmal dysarthria. It is noteworthy that an erroneous diagnosis of psychogenic disorders was initially made in both cases, highlighting the importance not to underestimate isolated paroxysmal symptoms in clinical practice.
发作性构音障碍是一种罕见病症,其特征为构音障碍的短暂发作,起病突然且频繁复发。主要在多发性硬化症中有所报道,有人声称它与中脑病变有关;然而,大多数报道的患者存在多处脑部改变,因此很难将这种症状与特定病变部位联系起来。我们举例说明了两名患有孤立性脱髓鞘中脑病变的患者,他们仅以发作性构音障碍为唯一症状;两名患者脑脊液中均有寡克隆带,且随访结果无异常。与先前报道的病例相似,两名患者均从卡马西平治疗中获益。我们的报告证实,脱髓鞘中脑病变足以引发发作性构音障碍。值得注意的是,这两例最初均被误诊为精神性障碍,凸显了在临床实践中不可低估孤立性发作性症状的重要性。