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 »  Abstract
 »  Introduction
 »  Case report
 »  Discussion
 »  Acknowledgement
 »  References

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Year : 2002  |  Volume : 50  |  Issue : 3  |  Page : 369-70

Facial myokymia as a presenting symptom of vestibular schwannoma.


Department of Neurological Sciences, Christian Medical College and Hospital, Vellore - 632 004, Tamil Nadu, India.

Correspondence Address:
Department of Neurological Sciences, Christian Medical College and Hospital, Vellore - 632 004, Tamil Nadu, India.

  »  Abstract

Facial myokymia is a rare presenting feature of a vestibular schwannoma. We present a 48 year old woman with a large right vestibular schwannoma, who presented with facial myokymia. It is postulated that facial myokymia might be due to a defect in the motor axons of the 7th nerve or due to brain stem compression by the tumor.

How to cite this article:
Joseph B V, Rajshekhar V. Facial myokymia as a presenting symptom of vestibular schwannoma. Neurol India 2002;50:369


How to cite this URL:
Joseph B V, Rajshekhar V. Facial myokymia as a presenting symptom of vestibular schwannoma. Neurol India [serial online] 2002 [cited 2023 Feb 5];50:369. Available from: https://www.neurologyindia.com/text.asp?2002/50/3/369/1420




   »   Introduction Top

Facial myokymia (FM) consists of continuous fine rippling activity of all the muscles on one side of the face, due to simultaneous or sequential spontaneous contractions of multiple motor units.[1] Electromyogram shows spontaneous asynchronous discharge of adjacent motor units, appearing singly or in doublets or triplets at a rate varying from 30-70 cycles per second.[1] It is usually associated with multiple sclerosis[2],[3] or with intrinsic brainstem tumors.[3],[4] While hemifacial spasm can be caused by cerebellopontine (CP) angle tumors,[5] FM is extremely rare.


   »   Case report Top

A 48 year old housewife presented with decreased hearing of the right ear and involuntary continuous twitching movements of the right side of face for the past 4 years. Over the past two years she developed gait ataxia and headache. On examination she had right sided coarse gaze evoked nystagmus, wasting of temporalis muscle, decreased sensation in V2 and V3 dermatomes, lower motor neurone type of facial nerve involvement, profound sensorineural hearing loss, and decreased palatal sensations. There was continuous abnormal fine rippling movements of the entire right half of face and neck. There were cerebellar signs on the right side. No other deficits were noted. MRI scan showed a large right CP angle tumour measuring5x3x4cms [Figure - 1], enhancing uniformly with contrast, and extending into the internal auditory meatus. There was compression of the brain stem and mild ventricular dilatation. Electromyography showed myokimic discharges at a rate of 30-40/sec, with pseudomyotonic discharges in right frontalis and orbicularis oculi. Facial conductions showed prolonged latencies and decreased amplitude on the right.
A right retromastoid suboccipital craniectomy and near total excision of the tumor was done without preservation of the facial nerve. A small 7 mmx7mm tumor was left at the VII/VIII nerves root entry zone, as it was adherent to a vessel supplying the brain stem. Postoperatively she had a right lower motor neurone facial palsy. There were no further episodes of myokymia.
Postoperative EMG showed fibrillations in the right facial muscles. It was not possible to activate the right facial muscles. No myokymic discharges were seen. Follow-up at 14 months, showed no change in the right lower motor neurone facial palsy. Myokymia was not present. MRI scan showed no residual tumor.


   »   Discussion Top

FM is a continuous involuntary rippling movement of unilateral facial muscles. There is continuous flickering from frontalis to platysma, recurring rapidly thereby appearing to pass over the face in rapid undulating waves. Bernhardt reported the earliest case of FM in 1902. Only speculations have been made as to the pathophysiology of FM. Within the brain stem, at supranuclear level, it has been attributed to disruption of inhibitory supranuclear pathways, or isolation of the facial motoneurones. At nuclear level, irritation of the facial motor nucleus has been implicated. At peripheral nerve level, changes in the microenviroment of the intraxial and extraxial course of the 7th nerve have been implicated. Ephaptic transmission, antidromic stimulation with resulant chronic hyperexcitability, and axonal compression and subsequent edema are other suspected contributing factors.
A number of conditions can cause FM. It has been described most often with multiple sclerosis[2],[3] and brain stem masses.[3],[5] It has also been described in association with Guillain Barre syndrome, unilateral basilar invagination, lymphocytic meningoradiculitis, lymphangitis carcinomatosa, chronic inflammatory polyradiculoneuropathy, following cardiopulmonary arrest, and as a false localizing sign in obstructive hydrocephalus. It has also rarely been described in association with posterior fossa extra axial tumors. To our knowledge, there have been 6 such cases reported so far.[3],[6],[7],[8] A recent review of 1000 cases of vestibular schwannomas revealed that while 0.6% patients had facial spasm, no mention of facial myokymia was made.[5]


   »   Acknowledgement Top

We would like to thank Dr. Nathaniel Sase for his help with the electrophysiological studies.

 

  »   References Top

1.Adams RD, Victor M, Ropper AH : Principles of neurology, 6th edition. McGraw Hill International edition. 1997; 45 : 1379.   Back to cited text no. 1    
2.Andermann F, Cosgrove JBR, Lloyd DL et al : Facial myokymia in multiple sclerosis. Brain 1961; 84 : 31-44.   Back to cited text no. 2    
3.Espinosa RE, Lambert EH, Klass DW : Facial myokymia affecting the electroencephalogram. Mayo Clinic Proceedings 1967; 42 : 258-270.   Back to cited text no. 3    
4.Selvapandian S, Rajshekhar V : Facial myokymia as the presenting symptom of a pontine glioma. Neurol India 1999; 47 : 241-242.   Back to cited text no. 4    
5.Matthies C, Samii M : Management of 1000 vestibular schwannomas (acoustic neuromas): clinical presentation. Neurosurgery 1997; 40 : 1-10.   Back to cited text no. 5    
6.Tharakan J, Bhatia R : Continuous hemifacial spasm in cerebello-pontine angle tumors. Neurol India 1981; 29 : 112-115.   Back to cited text no. 6    
7.Kiriyanthan G, Krauss JK, Glocker FX et al : Facial myokymia due to acoustic neurinoma. Surg Neurol 1994; 41 : 498-501.   Back to cited text no. 7    
8.Radu EW, Skorpil V, Kaeser HE : Facial myokymia. European Neurology 1975; 13 : 499-512.   Back to cited text no. 8    

 

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