Short CommunicationYoung onset Hemifacial Spasm in patients with Chiari type I malformation
Introduction
Hemifacial spasm (HFS) is typically an acquired movement disorder characterized by involuntary, tonic or clonic contractions from muscles innervated by the ipsilateral facial nerve [1]. The main underlying pathology of HFS is neurovascular contact around the root exit zone of the facial nerve [1].
Chiari type I malformation (CIM), on the other hand, is a congenital disease defined by downward displacement of the cerebellar tonsils bellow the level of foramen magnum to the upper cervical canal [2]. HFS due to CIM is unusual and few case reports have been emphasizing this association [3], [4], [5], [6].
The aim of our study was to depict the frequency of CIM among a series of patients with HFS and to report the clinical correlates of those patients who had simultaneously CIM and HFS. A review of the literature is also provided.
Section snippets
Methods
From 1995 to 2005, 103 consecutive patients with HFS underwent magnetic resonance imaging (MRI) at our movement disorders unit (MDU). We selected from this sample five patients with radiological proven CIM. All patients fulfilled brain MRI criteria for CIM [7]. The final diagnosis of HFS was always made concurrent with a movement disorders specialist and the same standard protocol for diagnostic criteria and follow-up during these 10 years. Patients with other facial dyskinesias (blepharospasm,
Results
The frequency of HFS associated to CIM in our sample was of 4.85%. Table 1 summarizes the main clinical and therapeutic features of these patients and the others listed on the literature. There were three women and two men with a mean/median age of 39.8/34 years (SD 15 years; range 30–66 years). Three patients were Caucasian–Brazilian and two were African-Brazilian. A considerable difference in their disease duration was seen (mean/median=28.8/12 months; SD=23.4 months; range 12–60 months).
Discussion
In this article we described clinical, radiological and therapeutic features of five individuals with HFS linked to CIM. This association has been described in few case reports [3], [4], [5], [6] and the prevalence of 4.85% patients in our series confirms that this is an unusual association.
The likelihood of assessment bias in this study may not be excluded due to its retrospective nature and possibly the frequency of CIM in our sample might have been underestimated.
CIM may sometimes present as
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