J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.37.6.745 on 1 June 1974. Downloaded from

Journal ofNeurology, Neurosurgery, and Psychiatry, 1974, 37, 745-749

Facial : a clinicopathological study

P. K. SETHI1, BERNARD H. SMITH, AND K. KALYANARAMAN From the Department of , Edward J. Meyer Memorial Hospital anid School of Medicine, State University of New York at Buffalo, N. Y., U.S.A.

SYNOPSIS Clinicopathological correlations are presented in a case of facial myokymia with facial palsy. The causative lesions were considered to be metastatic tumours to the pons and it was con- cluded that both the facial palsy and the myokymia were due to interruption of supranuclear path- ways impinging on the facial nucleus.

Oppenheim (1916) described a patient with con- CASE REPORT tinuous undulation and in the right A 57 year old white man was admitted to hospital on facial muscles. The movements had started in the 30 December 1971, suffering from productive cough, infraorbital region and progressed to involve the haemoptysis, and weight loss of some months' dura- entire territory of the facial nerve. He called the tion. He had been a heavy smoker for many years. Protected by copyright. condition facial myokymia, commented on its There was no history of fever or of pains around the association with sustained facial contraction, face. and expressed the view that, like facial palsy, it He was oriented as to time, place, and person but might be an early sign of . Kino confused and lethargic and unable to describe his (1928) reported three patients with undulating symptoms well. There was a palsy of the left face, fascicular twitching of the face which he inde- involving both upper and lower portions, with lagophthalmos and Bell's phenomenon. There were pendently termed facial myokymia. All three involuntary movements of the left facial muscles, patients were diagnosed as suffering from mul- most marked around the mouth and in the platysma, tiple sclerosis. Andermann et al. (1961) reviewed but also seen in the zygomatic region around the the literature, discussed seven previously reported lower lid. The movements were continuous, worm- cases, and added four of their own. They postu- like, and undulating; varying in location and not lated that the myokymia resulted from a central affecting all areas at the same time; undisturbing to excitatory focus of demyelination within or near the patient; and unaffected by sleep or emotional or the facial nucleus. However, they had no histo- volitional movements of the face, jaw, or eyes, or by http://jnnp.bmj.com/ pathological studies to substantiate their postula- patting or pulling of the skin of the face. There were tion. no percussion myotonia or myoedema, and no ab- normal synkineses or associated movements. There In earlier literature there are rare references to was no tenderness around the external ear or mastoid neoplastic and vascular lesions within the pons and no hearing loss, and the external acoustic being responsible for what was probably facial meatuses were normal. myokymia but referred to as continuous rhyth- There was also a left partial homonymous hemi- mic fascicular contraction of the facial muscles, anopia, affecting the lower visual fields more than on September 27, 2021 by guest. but in these cases histopathological data are the upper and sparing the macular area. Otherwise meagre. cranial nerve functions were intact and the power, The present case is reported because of the tone, coordination, and sensory functions in the rarity of previous reports, because the facial limbs were good and equal on the two sides. The myokymia was associated with facial palsy, and deep reflexes in the limbs were present and equal and because postmortem studies made clinicopatho- the plantar responses normal. The blood pressure was 120/80 mmHg, the pulse logical correlations possible. 100 per minute and regular. His right upper chest 1 Present address: Command Hospital, Lucknow, India. moved poorly on respiration and auscultation re- 745a e J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.37.6.745 on 1 June 1974. Downloaded from

746 P. K. Sethi, Bernard H. Smith, and K. Kalyanaraman vealed rhonchi in the underlying lung and radio- left than on the right and there was on the left an ill- graphy showed a right upper lobe cavity. sustained ankle and a Babinski plantar response. Pin-prick appreciation in the face and LABORATORY DATA The sputum was repeatedly corneal sensitivity were depressed on the left. The negative for acid-fast bacilli and malignant cells, and visual field defect had not changed. no definite pathogenic organisms were grown. Other Bronchoscopy showed, coming mainly from the routine laboratory tests on urine and blood, includ- right bronchial trees, abundant purulent sero- ing those for syphilis, were normal. sanguineous secretion, specimens of which failed to Nerve conduction studies showed normal conduc- reveal tumour tissue. tion times in the facial nerve (left orbicularis oculi- On the electroencephalogram there was depression 3 ms, conduction distance being 8 cm; left orbicu- of background rhythm on the right hemisphere and laris oculi-2 ms, conduction distance being 6 cm. mild to moderately severe slowing over the central using fine monopolar needle and parietal areas on the right. No epileptiform dis- electrodes showed a suggestion of rhythmic spon- charges were seen. taneous discharge in the platysma, and in all other areas only spontaneous continuous low amplitude Brain scan revealed independent areas of increased potentials, hardly distinguishable from normal uptake in the right posterior frontal and right motor unit action potentials except for their low parietal region; and a right carotid arteriogram amplitude. showed avascularity in the posterior frontal region and a small 'blush' in the right parietal area. There HOSPITAL COURSE The patient became more and was no evidence of displacement of midline vessels or more lethargic and confused. Two weeks after of uncal herniation. admission he had a further bout of haemoptysis and On 16 January 1972, a large frontoparietal crani- about the same time an episode of dizziness with fall- otomy revealed evidence of what to the naked eye Protected by copyright. ing. He was disoriented as to time and place and appeared to be two tumours, one on the right inferior exhibited motor impersistence in that he had to be frontal area, and the other in the right lateral repeatedly reminded to keep his eyes closed during parietal area. Biopsy from these areas showed frag- sensory testing. The left facial weakness persisted but ments of cerebral cortex, extensive areas of necrosis, the myokymia was now limited to the platysma and 'nests' of tumour cells which the pathologist muscle. There was also mild weakness of the left thought to be of metastatic origin and probably limbs and some suggestion of his neglecting the left adenocarcinomatous. Postoperatively the patient half of the body on bilateral simultaneous stimula- remained disoriented and lethargic and over the tion. The knee and ankle jerks were brisker on the ensuing week deteriorated and died. http://jnnp.bmj.com/

FIG. 1. Section ofpons showing the two metastases,

one median, the other in the on September 27, 2021 by guest. left tegmentum. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.37.6.745 on 1 June 1974. Downloaded from

Facial myokymia: a clinicopathological study 747

PATHOLOGICAL FINDINGS At necropsy, broncho- In the upper and mid pons, two well-circumscribed genic carcinoma in the upper lobe of the right lung metastases were noted, corresponding to the areas of was confirmed, with metastases to lymph nodes, greyish discolouration in the gross specimen (Fig. 1). liver, kidneys, and adrenal glands. The brain was One of the metastases was in the left tegmentum, somewhat swollen and its convolutions flattened and and the other in the basis pontis with greater exten- the pia-arachnoid membranes over the right lateral sion to the right than the left side. These metastases frontal convexity were opaque. The arteries at the appeared to be rostral to and not to involve the base showed spotty atheromatosis without luminal facial nucleus or its intramedullary fibres-the level occlusion. of the lesion is indicated in Fig. 2 where the section is On coronal sections, a slight general brain swelling just above the facial colliculus. was confirmed. In the white matter of the right posterior frontal and inferior parietal areas there were several small fine cystic areas of encephalomalacia DISCUSSION and there was softening in the right parietal sub- The facial musculature may be involved in cortical area and in the right superior temporal A gyrus. various types of involuntary movements. care- Fairly sharply delimited areas of pale grey dis- ful clinical examination combined with relevant colouration were found in the tegmentum of the left ancillary investigations can give much help in mid pons and in the upper and mid pons on the right differentiating them. in the region of the medial lemniscus. No lesions Facial myokymia is one of the rare facial were noted in the midbrain, cerebellum, or medulla, but stands out as a distinct clinical and no lesions of the facial nerve or its nucleus were entity. It is usually easily distinguished from detected. , which appear as brief, irregularly Protected by copyright. Histologically no cancer cells were obvious in the occurring twitches in different parts of the facial necrotic tissue in the right hemisphere, but several of muscles; whereas myokymia is a slower contrac- the pial arteries were packed with cancer cells. No gross atheromatous lesions were encountered, and tion involving small bands or strips of muscles the large arteries at the base were unoccluded. The and occurs more continuously, transmitting a cancer cell stasis in the local leptomeningeal arterioles ripple (like a 'bag of worms') to the overlying was thought to have contributed to the underlying skin. encephalomalacia. Benign myokymia of normal people is limited The face area in the motor and sensory cortex was to the eyelids and tends to occur during states of microscopically intact and the internal capsule . showed no lesion at any level. The corticospinal and The facial myokymia under discussion has corticobulbar fibres of the midbrain were also intact. been reported so far in only 45 patients. Note- worthy among previous reports is that of Ander- mann and his colleagues (1961) who described own culled seven four cases of their and from http://jnnp.bmj.com/ the literature. Matthews (1966) described a fur- ther six personal cases, and Gutmann et al. (1969) described two more and gathered 40 from the literature. Most of these cases had been found to be due to multiple sclerosis; but Espinosa et al. (1967) found evidence to incriminate tumours in (______Level of the and around the pons. Of their patients with P \l section facial myokymia, 10 had clinical evidence of on September 27, 2021 by guest. intramedullary tumour of the brain-stem, and Facial Colliculus two had extramedullary tumours of the posterior fossa. Of the 10 patients considered to have had intramedullary tumours, the clinical diagnosis was strengthened in four cases by the findings on pneumoencephalography; of the other six FIG. 2. Diagrammatic representation of the level of patients, one had glioma of the brain-stem and pons at which the section was taken. cerebellum, and the others had biopsy evidence J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.37.6.745 on 1 June 1974. Downloaded from

748 P. K. Sethi, Bernard H. Smith, and K. Kalyanaraman of astrocytomas of the pons. Detailed histo- myokymia and the dense affecting the pathological studies with a view to localization upper and lower left face; the lesions in the right are not available. posterior frontal, temporal, and parietal regions Two facts emerge from the reports of facial were thought to explain the progressive left myokymia in multiple sclerosis: the myokymia, , homonymous hemianopia, and although it may recur in the same or the opposite left-sided sensory inattention. side of the face, is almost invariably transient No lesion was found that might involve the (persisting from two days to three months, with peripheral part of the facial nerve; and the con- the exception of one case in which it lasted six tinuing normal conduction velocities in that months); and appreciable facial has not nerve also helped to exclude such a lesion. Nor been a feature. In contrast, in the cases associated was there macroscopic or microscopic evidence with tumours (Espinosa et al., 1967) significant that the facial nucleus was involved, so a and progressive facial paralysis was often present nuclear lesion can be excluded as the cause of as well as progressive involvement of other the myokymia. Likewise, the lesions were too cranial nerves and of the pyramidal and cerebel- rostral to involve the intramedullary portion of lar systems. the seventh nerve; nor was there clinical evidence There have been no previous reports incrimina- of sixth nerve palsy or of a defect of conjugate ting metastatic lesions. In our case the pontine lateral gaze such as often accompanies such an metastases were thought to be the cause of the intramedullary facial nerve paralysis. In brief, the clinical and pathological findings favoured a

supranuclear lesion of the left facial neurones. Protected by copyright. There appear to be no universally agreed data as to the course of the corticobulbar fibres below the level of the internal capsule. Dejerine's view (1906), based on human material, was that the corticobulbar fibres were subdivided into groups, some running directly to their termination in close association with the corticospinal (pyra- midal) fibres; other (so-called aberrant) fibres leaving the corticospinal tract at various levels in the midbrain and pons to pass to their respective cranial motor nuclei. Decussation of the cortico- bulbar fibres is partial and occurs near the level of the cranial motor nuclei for which they are destined. Kuypers (1958) has described direct and in- http://jnnp.bmj.com/ direct corticobulbar fibres in man. The facial (and hypoglossal) nuclei receive fibres from both corticobulbar systems, direct and aberrant. The corticobulbar fibres arise in the precentral gyrus and in the adjoining middle and inferior pre- frontal gyri, traverse the internal capsule near its genu, and pass through the basis pedunculi in its on September 27, 2021 by guest. middle three-fifths in association with the corticospinal fibres to enter the pons. In the FIG. 3. Diagrammatic representation of crossed and pons, near the level of the seventh motor uncrossed corticobulbar fibres as they approach and nucleus, a group of fibres breaks away and enter facial nucleus. (Modified from Bing's Local Diagnosis in Neurological Diseases.) Approximate passes dorsally into the tegmentum where some location of metastases is also diagrammatically decussate to terminate in the contralateral facial shown. * = To upper part offace. a = To lower part motor nucleus, especially in the part of the offace. nucleus which innervates the lower facial muscles. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.37.6.745 on 1 June 1974. Downloaded from

Facial myokymia: a clinicopathological study 749

Some decussating fibres proceed to cells that both upper and lower facial muscles, we natur- innervate the upper facial muscles (frontalis, ally considered the likelihood of a lower motor orbicularis oculi and corrugator supercilii): neurone lesion. We finally concluded that the these muscles are also supplied by non-decussa- left facial palsy was of central type and that the ting fibres from the ipsilateral hemisphere. In explanation for the upper and lower facial palsy brief, the upper face has bilateral, and the lower lay in the fact that all the supranuclear connec- face mainly unilateral representation. This view tions to the left facial muscles, both contra- is also supported by electrical stimulation studies lateral and ipsilateral, had been interrupted. on the human cortex (Foerster, 1936; Penfield and Boldrey, 1937) and, of course, by clinical We are most grateful to Dr. Joseph C. Lee for his data. valuable suggestions and criticism. We also appreci- Figure 3 is a diagrammatic representation of ate the courtesy of Dr. Webb Haymaker and the the corticobulbar fibres as they approach and publishers, The C.U. Mosby Co, St. Louis, for enter the facial nucleus (directly or through inter- permission to modify Fig. 14-5 in the 15th edition of calated cells). The two pontine metastases are in Bing's Local Diagnosis in Neurological Diseases, ed. a position to involve these fibres, the more 15 (1969). central one interrupting the crossing fibres for the left lower and upper face, the other interrupt- REFERENCES fibres for the left face. In ing ipsilateral upper Andermann, F., Cosgrove, J. B. R., Lloyd-Smith, D. L,. other words, the two lesions in combination are Gloor, P., and McNaughton, F. L. (1961). Facial myo- interrupting all the main supranuclear pathways kymia in multiple sclerosis. Brain, 84, 31-44. Protected by copyright. for the control of the left upper and lower face. Dejerine, J. (1906). L'aphasie sensorielle: sa localisation et sa physiologie pathologique. Presse Medicale, 14, 437-439; Although the metastases do not involve the 453-457. facial nucleus directly, they are perhaps suffi- Espinosa, R. E., Lambert, E. H., and Klass, D. W. (1967). near it (1) to set up the steady depolarizing Facial myokymia affecting the electroencephalogram. ciently Mayo Clinic Proceedings, 42, 258-270. state postulated by Andermann and his col- Foerster, 0. (1936). Motorische Felder und Bahnen, in leagues as affecting the soma-dendritic mem- Handbuch der Neurologie, Vol. 6, pp. 1-357. Edited by 0. branes of the facial motor neurones and so Bumke and 0. Foerster. Gutmann, L., Thompson, H. G., Jr., and Martin, J. D. causing the facial myokymia; or, alternatively (1969). Transient facial myokymia. Journal ofthe American (2) to 'release' the facial nucleus from higher Medical Association, 209, 389-391. control, resulting in increased excitability of the Kino, F. (1928). Muskelwogen (Myokymie) als Friihsymptom der multiplen sklerose. Deutsche Zeitschriftfur Nervenheil- facial motor neurones. kunde, 104, 31-41. In further support of the causative lesion of Kuypers, H. G. J. M. (1958). Corticobulbar connexions to myokymia being rostral to the seventh nerve and the pons and lower brain-stem in man. Brain, 81, 364-388.

Andermann Matthews, W. B. (1966). Facial myokymia. Journal ofNeuro- http://jnnp.bmj.com/ its nucleus was the observation by logy, Neurosurgery, and Psychiatry, 29, 35-39. and his co-workers (1961) that two of their Oppenheim, H. (1916). Leistungen und Fortschritte aus dem patients had trigeminal nerve involvement, and Gebiete der Neurologie und Psychiatrie, 20, xiv (cited by the similar finding in two of Matthews's (1966) Andermann et al., 1961). Penfield, W., and Boldrey, E. (1937). Somatic motor and patients. sensory representation in the cerebral cortex of man as Because in our patient there was paralysis of studied by electrical stimulation. Brain, 60, 389-443. on September 27, 2021 by guest.