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Year : 2015  |  Volume : 18  |  Issue : 2  |  Page : 246-248

Mirror movements in progressive hemifacial atrophy

Department of Neurology, King George Medical University, Lucknow, Uttar Pradesh, India

Date of Submission18-Aug-2014
Date of Decision26-Oct-2014
Date of Acceptance24-Nov-2014
Date of Web Publication8-May-2015

Correspondence Address:
Rajesh Verma
Department of Neurology, King George Medical University, Lucknow - 226 001, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0972-2327.150606

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Mirror movements are simultaneous, involuntary, identical movements occurring during contralateral voluntary movements. These movements are considered as soft neurologic signs seen uncommonly in clinical practice. The mirror movements are described in various neurological disorders which include parkinsonism, cranio veretebral junction anamolies, and hemiplegic cerebral palsy. These movements are intriguing and can pose significant disability. However, no such observation regarding mirror movements in progressive hemifacial atrophy have been reported previously. We are reporting a teenage girl suffering from progressive hemifacial atrophy and epilepsy with demonstrable mirror movements in hand.

Keywords: Hemifacial atrophy, mirror movements, parry romberg syndrome

How to cite this article:
Verma R, Dixit PK, Lalla R, Singh B. Mirror movements in progressive hemifacial atrophy. Ann Indian Acad Neurol 2015;18:246-8

How to cite this URL:
Verma R, Dixit PK, Lalla R, Singh B. Mirror movements in progressive hemifacial atrophy. Ann Indian Acad Neurol [serial online] 2015 [cited 2021 Dec 4];18:246-8. Available from:

   Introduction Top

The mirror movements (MMs) are simultaneously occurring unintended movements in homologous muscles during contralateral voluntary movements. It was first described by Erlenmeyer in 1857. It was truly defined by Cohen et al. in 1991. [1] The MMs are rarely observed in clinical practice. These are tricky, unintentional movements, sometimes causing injury to the patient. These MMs are exhibited in cerebral palsy, craniovertebral junction anamoly,  Parkinsonism More Details, post stroke phase, and myelomeningocoele. [2] In this report, we intended to present an unsual association of MMs in a young girl suffering from progressive facial atrophy along with cerebral hemiatrophy. The literature is silent about presence of MMs in progressive facial atrophy.

   Case Report Top

A 17-year-old girl has generalized tonic clonic seizures followed by loss of consciousness since last 12 years. Initially, these movements are well-controlled with drugs with 4-5 episodes per year but since last 3 years, these movements increases in frequency; and now since last 2 months, patient have daily one to two episodes of these movements. There was no history of headache, vomiting, rash, arthralgia, delayed menarche, and peripheral limb weakness. There is no short neck, low hair line, or decreased neck movement.

On examination, she has atrophy of her left face with slight deviation of mouth and nose towards left side [Figure 1]. During nervous system examination, we found that when she held the examiner finger by her left hand she perform the similar kind of movement (MM) in her right hand [Video 1]. There was no movement noted proximally in upper limb and lower limb. Rest of her neurological examination is normal.
Figure 1: Photograph of the patient showing atrophy of her left face with slight deviation of mouth and nose toward left side

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Routine hematological and biochemical parameters of the patient were within normal limits. Electroencephalography (EEG) shows generalized slowing. Magnetic resonance imaging (MRI) brain shows left cerebral atrophy [Figure 2]. The patient was treated with antiepileptic drugs with leviteracetam 500 mg and oxcarbazepine 300 mg twice daily. Her seizures are well-controlled at follow-up after 5 months.
Figure 2: Magnetic resonance imaging of the cranium (a) T2-weighted image (b) T2 fluid attenuated inversion recovery image depicting left sided cerebral hemiatrophy

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   Discussion Top

The MMs can be normally observed till 10 years at the point of complete myelination of corpus callosum. [3] The persistence of MMs beyond this age is usually pathological [4] and represent abnormality of central motor drive to relaxed limb. The MMs have been described in cerebral palsy, cranio-vertebral junction anomaly, Klippel feil anamoly, Kallaman syndrome, Parkinson's disease, corticobasal degeneration, post stroke, essential tremors, focal hand dystonia, Creutzfeldt-jakob's disease, and Huntigton's disease. However, rarely, it is seen in normal individuals and mostly while performing complex unimanual tasks. [5],[6] The MMs can often impede the bimanual tasks and at times can be very disabling. As found in most cases, in our patient, MMs were limited to distal upper limb (wrists and finger movements) [6] and our patient did not have any disability due to MMs.

MM either seems to arise due to activation of both hemispheres when only one of the hemispheres is intended to fire. [7] Another possible mechanism is presence of fast conducting ipsilateral pyramidal tract that does not decussate instead projects on the ipsilateral spinal motor neurons. Both mechanisms can occur in tandem with one another and contribute to clinical symptomatology. However, it has been observed that over-activation of ipsilateral pyramidal tract plays a major role in congenital disorders associated MM. [8]

MM also occur in several acquired and degenerative neurological disorders but the mechanism of mirroring in such conditions remains elusive. The theory of physiological cross talk was proposed because of formation of false synapses. Other mechanisms attributed to MMs were deficient corpus callosum, incomplete pyramidal decussation, loss of inhibition, and over-activation of ipsilateral pyramidal tract. [9]

Our patient was diagnosed as progressive facial atrophy, repetitive focal seizures, and cerebral hemiatrophy with demonstrable MMs. Progressive facial atrophy (Parry Romberg syndrome) is characterized by unilateral facial atrophy which involves muscles, subcutaneous tissues, and even underlying bones. [10] The neurological manifestations include migraine headache, trigeminal neuralgia, focal seizures, and sometimes associated with cerebral hemiatrophy. [11] En coupe De Sabir (localized Morphea) and Parry Romberg syndrome can also have similar appearance and almost 17% of such patients have shown to present with MRI brain abnormalities. However, the authors concluded that whether there is a causal relationship or true association cannot be ascertained. [12]

The MMs in our patient could be related to cerebral hemiatrophy. The plausible explanation for this association may be deficient transcallosal connections, increased activation of non-diseased cerebral cortex, or formation of aberrant corticopyramidal connections are postulated mechanisms. Our hypothesis is supported by previous observations made in a case of acquired MMs in a case of refractory epilepsy after focal cortical resection. [13] A similar case reported by Takajo et al. of MMs along with focal onset seizures was in a patient with open lip schizencephaly. The authors postulated neuronal reorganization and reallocation of motor control on the non-diseased cerebral hemisphere. The loss of transcallosal inhibitory control from affected hemisphere may lead to overflow of the stimulatory signals from the non-diseased hemisphere and thereby cause simultaneous homologous movement. [14]

   References Top

Cohen LG, Meer J, Tarkka I, Bierner S, Leiderman DB, Dubinsky RM, et al. Congenital Mirror Movements. Abnormal organization of motor pathway in two patients. Brain 1991;114:381-403.  Back to cited text no. 1
Ottaviani D, Tiple D, Suppa A, Colosimo C, Fabbrini G, Cincotta M, et al. Mirror movements in patients with Parkinson′s disease. Mov Disord 2008;23:253-8.  Back to cited text no. 2
Connolly K, Stratton P. Developmental changes in associated movements. Dev Med Child Neurol 1968;10:49-56.  Back to cited text no. 3
Rocca MA, Mezzapesa DM, Comola M, Leocani L, Falini A, Gatti R, et al. Persistence of congenital mirror movements after hemiplegic stroke. AJNR Am J Neuroradiol 2005;26:831-4.  Back to cited text no. 4
Arányi Z, Rösler KM. Effort-induced mirror movements. A study of transcallosal inhibition in humans. Exp Brain Res 2002;145:76-82.  Back to cited text no. 5
Schott GD, Wyke MA. Congenital mirror movements. J Neurol Neurosurg Psychiatry 1981;44:586-99.  Back to cited text no. 6
Kim YH, Jang SH, Chang Y, Byun WM, Son S, Ahn SH. Bilateral sensori-motor cortex activation of post-stroke mirror movements: An fMRI study. Neuroreport 2003;14:1329-32.  Back to cited text no. 7
Ueki Y, Mima T, Oga T, Ikeda A, Hitomi T, Fukuyama H, et al. Dominance of ipsilateral corticospinal pathway in congenital mirror movements. J Neurol Neurosurg Psychiatry 2005;76:276-9.  Back to cited text no. 8
Li JY, Espay AJ, Gunraj CA, Pal PK, Cunic DI, Lang AE, et al. Interhemispheric and ipsilateral connections in Parkinson′s disease: Relation to mirror movements. Mov Disord 2007;22:813-21.  Back to cited text no. 9
Verma R, Ram H, Gupta M, Vidhate MR. A case of extensive left-sided facial atrophy of Romberg. Natl J Maxillofac Surg 2013;4:77-80.  Back to cited text no. 10
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Restivo DA, Milone P. Teaching neuroimages: Progressive facial hemiatrophy (Parry-Romberg syndrome) with ipsilateral cerebral hemiatrophy. Neurology 2010;74:e11.  Back to cited text no. 11
Chiu YE, Vora S, Kwon EK, Maheshwari M. A significant proportion of children with morphea en coup de sabre and Parry-Romberg syndrome have neuroimaging findings. Pediatr Dermatol 2012;29:738-48.  Back to cited text no. 12
Ramachandran Nair R, Otsubo H, Ochi A, Rutka J, Donner EJ. Mirror movements following cortical resection of polymicrogyria in a child with intractable epilepsy. Pediatr Neurol 2006;34:135-8.  Back to cited text no. 13
Takajo I, Ohi T, Shiomi K, Sugimoto S, Matsukura S. A case with symptomatic epilepsy and mirror movement due to unilateral schizencephaly. No To Shinkei 2000;52:617-20.  Back to cited text no. 14


  [Figure 1], [Figure 2]

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