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Young onset Hemifacial Spasm in patients with Chiari type I malformation

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Abstract

Hemifacial spasm (HFS) resulting from Chiari type I malformation (CIM) is rare. We retrospectively evaluated five patients with CIM and HFS among a series of 103 subjects. The frequency of HFS associated to CIM was of 4.85%. The clinical profile did not differ from the classical primary cases except for young-onset development of facial spasms in patients with CIM. Three patients were treated with BTX-A injections with favorable outcome. Although rare HFS may be associated with CIM especially in young subjects with peculiar phenotypic characteristics (short neck). Moreover, BTX may be an alternative to posterior fossa decompression in selected cases.

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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Cited by (13)

  • Hemifacial spasm secondary to Chiari malformation type I: Systematic review with case illustration

    2022, Journal of Clinical Neuroscience
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    Because of the rarity of the disease, there are limited data on the optimal treatment for patients with CM1 who present with HFS. The current literature regarding this topic consists of case reports and small, single-centered case series with variable recommendations [4–11]. The authors of the largest case series to date concluded that MVD should be considered for initial surgical treatment [9], whereas multiple case reports have advocated for suboccipital decompression as the initial treatment in these patients [4,5,7,8,11].

  • Surgical Management of Hemifacial Spasm Associated with Chiari I Malformation: Analysis of 28 Cases

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    HFS is related most commonly to vascular compression of the facial nerve. The association between HFS and CIM is extremely rare, and to our knowledge, there are only 23 reported cases in the literature.12-15,17-19 The clinical and therapeutic features of these reported cases with HFS and CIM are summarized in Table 2.

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