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 myokymia: a clinicopathological study P. K. SETHI1, BERNARD H. SMITH, AND K. KALYANARAMAN From the Department of Neurology, 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 fasciculation 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 multiple sclerosis. 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 clonus 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 Electromyography 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, dyskinesias 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. fasciculations, 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.