The other Babinski sign in hemifacial spasm.
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ABSTRACT: From the very first descriptions of dystonia, there has been a lack of agreement on the differentiation of organic from functional (psychogenic) dystonia. This lack of agreement has had a significant effect on patients over the years, most particularly in the lack of access to appropriate management, whether for those with organic dystonia diagnosed as having a functional cause or vice versa. However, clinico-genetic advances have led to greater certainty about the phenomenology of organic dystonia and therefore recognition of atypical forms. The diagnosis of functional dystonia rests on recognition of its phenomenology and should not be, as far as possible, a diagnosis of exclusion. Here, we present an overview of the phenomenology of functional dystonia, concentrating on the three main phenotypic presentations: functional cranial dystonia; functional fixed dystonia; and functional paroxysmal dystonia. We hope that this review of phenomenology will aid in the positive diagnosis of functional dystonia and, through this, will lead to more rapid access to appropriate management.Movement Disorders Clinical Practice. 04/2014; 1(1).
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ABSTRACT: Hemifacial spasm (HFS) is characterized by involuntary unilateral contractions of the muscles innervated by the ipsilateral facial nerve, usually starting around the eyes before progressing inferiorly to the cheek, mouth, and neck. Its prevalence is 9.8 per 100,000 persons with an average age of onset of 44 years. The accepted pathophysiology of HFS suggests that it is a disease process of the nerve root entry zone of the facial nerve. HFS can be divided into two types: primary and secondary. Primary HFS is triggered by vascular compression whereas secondary HFS comprises all other causes of facial nerve damage. Clinical examination and imaging modalities such as electromyography (EMG) and magnetic resonance imaging (MRI) are useful to differentiate HFS from other facial movement disorders and for intraoperative planning. The standard medical management for HFS is botulinum neurotoxin (BoNT) injections, which provides low-risk but limited symptomatic relief. The only curative treatment for HFS is microvascular decompression (MVD), a surgical intervention that provides lasting symptomatic relief by reducing compression of the facial nerve root. With a low rate of complications such as hearing loss, MVD remains the treatment of choice for HFS patients as intraoperative technique and monitoring continue to improve.TheScientificWorldJournal. 01/2014; 2014:349319.
Article: Psychogenic Movement Disorders[Show abstract] [Hide abstract]
ABSTRACT: Psychogenic movement disorders (PMDs) can present with varied phenomenology that may resemble organic movement disorders. The diagnosis is based on clinical evaluation with a supporting history and classic features on neurologic examination. Ancillary testing, such as imaging and neurophysiologic studies, can provide supplementary information but is not necessary for diagnosis. There is no standard protocol for the treatment of PMDs, but a multidisciplinary approach has been recommended. This review discusses the clinical characteristics of various PMDs as well as ancillary testing, treatment, and research in the pathophysiology of this complex group of disorders. Copyright © 2015 Elsevier Inc. All rights reserved.Neurologic Clinics 11/2014; · 1.61 Impact Factor
The Other Babinski Sign in Hemifacial SpasmThe Other Babinski Sign in Hemifacial Spasm
William Stamey, MD, Joseph Jankovic, MD
Parkinson’s Disease Center and Movement Disorders Clinic,
Department of Neurology, Baylor College of Medicine, Houston, Texas
Rarely attributed to Babinski is a phenomenon observed in patients with hemifacial spasm
(HFS), which he first described in 1905.
This “other Babinski sign”1is manifested as follows: “when orbicularis oculi contracts and the
eye closes, the internal part of the frontalis contracts at the same time, the eyebrow rises
during eye occlusion,” “this set of occurrences is impossibleto reproduce by will ...”2
In this study we examined the utility of the “other Babinski sign” in differentiating HFS from
All patients who presented to the Baylor College of Medicine Movement Disorders
Clinic between July, 1989 and July, 2006 and were diagnosed with HFS or BSP by
a movement disorders expert were included in this study.
Mean age at the time of initial evaluation for 75 patients with HFS (45 women) was 58.4
± 12.2 years; and for the 73 patients with BSP (50 women) was 58.2 ± 12.5 years.
The “other Babinski sign” is an under-recognized and useful physical sign which may be present in HFS, and is therefore helpful in
distinguishing this disorder from BSP, wherein the sign is not reported. “The other Babinski sign” is not seen in BSP, presumably due to
the fact that the motor neuron pools for orbicularis oculi and frontalis muscles have different patterns of suprasegmental innervation. In
the case of HFS, however, the co-contraction of these muscles presumably reflects a peripheral co-activation. This “other Babinski sign”
should be differentiated from the compensatory frontalis muscle contraction that may occur in patients with ptosis, such as seen in
HFS is a peripherally-induced movement disorder characterized by irregular, clonic, or tonic contraction of the muscles of the face
innervated by the ipsilateral seventh cranial nerve.3
In HFS, twitching commonly begins in the orbicularis oculi, but usually spreads to involve muscles of the upper and lower face,
including the platysma muscle and may rarely become bilateral.
HFS is typically idiopathic or sporadic, usually attributed to compression of the facial nerve at the root entry zone by an aberrant artery.
BSP, a form of focal dystonia of central origin, is a forceful, involuntary, spasmodic contraction of the orbicularis oculi which may occur
in isolation or in combination with other dystonic contractions, including other facial muscles.
The combination of BSP and dystonia of other facial or jaw muscles is referred to as craniocervical dystonia (formerly known as
Patients with hemifacail spasm demonstrating the “other Babinski sign” manifested by elevation of the eyebrow caused
by contraction of the frontalis muscle ipsilateral to the facial spasm.
1. Devoize J L. “The other” Babinski's sign: paradoxical raising of the eyebrow in hemifacial spasm. J Neurol Neurosurg Psychiatry 2001;70:516.
2. Babinski J. Hémispasme facial périphérique. Nouvelle iconographie de la Salpétrière. 1905;18:418-423.
3. Wang A, Jankovic J. Hemifacial spasm: clinical findings and treatment. Muscle Nerve. 1998;21:1740-1747.
4. Jankovic J. Dystonic disorders. In: Jankovic J, Tolosa E, editors. Parkinson's disease and movement disorders, 5th edition, Philadelphia, PA: Lippincott
Williams and Wilkins 2007:321-347.
5. Jankovic J, Ford J. Blepharospasm and orofacial-cervical dystonia: clinical and pharmacological findings in 100 patients. Ann Neurol. 1983;4:402-411.
6. Toyka KV. Ptosis in myasthenia gravis: Extended fatigue and recovery bedside test. Neurol. 2006;67;1524.
Objective: To assess the frequency of the other Babinski sign among patients with hemifacial
spasm (HFS), and determine its use in differentiating HFS and blepharospasm (BSP).
Background: The other Babinski sign, described by Joseph Babinksi in 1905, is manifested by
simultaneous eye closure and eyebrow elevation. The utility of the sign in differentiating HFS and
BSP has not been systematically studied. We sought to characterize the frequency, sensitivity or
specificity of this finding.
Design/Methods: Patients included were diagnosed with HFS (n=75; 45 women) or BSP (n=73;
50 women), and videotaped. HFS was clinically diagnosed with the presence of unilateral
involuntary facial muscle contractions affecting one or more muscle groups innervated by the
ipsilateral facial nerve. Patients with BSP had bilateral contraction of periorbital muscles without
other associated facial or oromandibular dystonia. A patient was considered positive for the
presence of the other Babinski sign if the videotape showed unilateral contraction of the frontalis
muscle, causing eyebrow elevation, with concurrent contraction of the ipsilateral orbicularis oculi
muscle, causing eyelid closure. Patients with unilateral frontalis contraction but not concurrent eye
closure were excluded, as such contraction may have been compensatory (voluntary) as in
patients with BSP, rather than involuntary as typically observed in patients with HFS.
Results: The other Babinski sign was present in none of the BSP patients but was evident in 19
(25.3%) of the HFS patients (10 women). The sensitivity of the other Babinski sign as a test of the
presence of HFS was 25.3%; whereas specificity for the presence of the sign, compared with
BSP, was 100%.
Conclusions/Relevance: The other Babinski sign, found in 25% of our cases of HFS, but not in
any of the cases of BSP, is an under-recognized physical sign which can be used to distinguish
HFS from BSP.