Bevilacqua J A, Dellarossa D, Sinning M
Departamento de Neurología, Hospital Clínico, Universidad de Chile, Santiago, Chile.
Rev Neurol. 2004;38(3):247-9.
Diagnosis of amyotrophic lateral sclerosis (ALS) requires of the exclusion of several pathologies of the cranio cervical junction that mimic ALS. The importance of such exclusion is in fact sustained by the prognostic implications that ALS has.
We report the case of a 58-year-old woman with bulbar onset sporadic ALS associated with Chiari type I malformation on the MRI of the craniocervical junction. At the time of consultation, ALS was clinically defined but some of the symptoms were contributed by cerebellar amygdala compression of the medulla.
To our knowledge this is the first time were both diseases are reported symptomatic on the same patient, and despite its rarity, it is important to be aware of this possible association for the diagnostic, therapeutic and prognostic implications it has.
肌萎缩侧索硬化症(ALS)的诊断需要排除几种模仿ALS的颅颈交界区病变。事实上,这种排除的重要性在于ALS所具有的预后意义。
我们报告一例58岁女性,其延髓起病的散发性ALS与颅颈交界区MRI上的I型Chiari畸形相关。会诊时,ALS在临床上已明确诊断,但部分症状是由延髓的小脑扁桃体压迫所致。
据我们所知,这是首次报道同一患者同时出现这两种有症状的疾病,尽管这种情况罕见,但鉴于其对诊断、治疗和预后的影响,了解这种可能的关联很重要。