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Neuromuscular and functional disorders of the pharynx
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Neuromuscular and Functional Disorders of the Pharynx* J. P. MURRAY, M.B., F.F.R., D.M.R. (D), D.M.R.D. Sefton General and Alder Hey Children's Hospitals, Liverpool T ~ object of this paper is to discuss the value of radiology in pharyngeal disorders of a general type as distinct from paralysis of localized groups of muscles. I propose to deal with deglutition in myasthenia gravis, hysterical dysphagia, and pharyngo-~esophageal
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recesses, and constriction rings at the level of the cricoid cartilage. T h e anteroposterior projection is of course of most use in paralysis of specific groups of m u s c l e s - as after p o l i o m y e l i t i s - - t o demonstrate asymmetrical
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Fig. I . - - G e n e r a l i z e d myasthenia gravis. Female, aged 55. Frames selected at 2-second intervals swallowing thick paste. Above: Delay in clearing m o u t h and oropharynx. Below: Well-marked improvement at a repeat examination 30 minutes after 2 mg. prostigmin subcutaneously.
incoordination--a rather wide t e r m including conditions of diverse aetiology. T h e s e are conditions in which, until recently, clinicians have seldom sought aid from the radiologist except to exclude a neoplasm or diverticulum as the cause of the patient's symptoms. But with the wider availability of the screen image intensifier and cineradiography, more detailed information about pharyngeal function can be given. N o r m a l deglutition has been extensively studied in recent years, notably by Ardran and K e m p (195 I, I952, 1954) and by Roberts (1957) , and our conception of normal deglutition is on a clearer and more certain basis. By means of cineradiography with an image intensifier, Ardran, K e m p , and Wegelius (1957) have demonstrated the swallowing defects after poliomyelitis, and Crichlow (1956) has made a detailed study of the function and appearance of the cricopharyngeus muscle.
TECHNIQUE As regards technique a few points require mention : i. Screening and films in the lateral projection provide adequate information in most cases but the anteroposterior projection is also useful, especially in myasthenia gravis, and will provide information concerning the tone of the pharynx, pooling in the * Based partly on a paper read to the Provincial Meeting of the Faculty of Radiologists at Liverpool, October, 1957.
contractions of the constrictors and deflexion to either side of the bolus as it passes through the pharynx. 2. A little barium instilled into the nose helps to show the soft palate m o r e clearly, but in the conditions under discussion it is befter to omit this lest it obscure a slight degree of nasal reflux. 3. All cases should be examined initially with barium cream, but in cases where aspiration into the trachea may be anticipated it is safer to use an iodine medium. Patients in w h o m there is an appreciable residue around or over the entrance to the larynx should not be examined with thick paste nor be asked to carry out continuous swallowing with thin barium, as in such cases there is difficulty in re-establishing the airway and danger of aspirating the barium. 4- Patients in w h o m the diagnosis of myasthenia gravis is known or suspected require a modification of the usual technique. Repeated swallows may be necessary to demonstrate an abnormality of muscular dysfunction because a single swallow may appear to be normal. In the milder cases without obvious bulbar symptoms thick paste in the r e c u m b e n t position will accentuate the abnormalities. Alternatively, continuous swallowing of thin barium taken through a straw will show easy fatigue of deglutition w h e n it is present. Such cases are best examined in the afternoon and preferably after a meal. 5- Suitable factors for cineradiography in the lateral projection are 70-75 kV., 5-6 mA., and 18 frames per second. An increase in kilovoltage to
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a b o u t 85 kV. is necessary for t h e a n t e r o p o s t e r i o r projection. MYASTHENIA
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and in some cases a r o u n d t h e sides of the t o n g u e with each swallowing a t t e m p t . d. T h e s e defects result in a m e a s u r a b l e delay in clearing the m o u t h a n d o r o p h a r y n x , a n d there is increasing difficulty in m a k i n g repeated swallowing acts (Fig. i). 2. T h e abnormalities involving the pharyngeal stage consis~ of: a. Stasis in the p h a r y n x and pooling in the p h a r y n g e a l recesses, due to weakness of the afterc o m i n g c o n t r a c t i o n wave.
GRAVIS
S c h w a b a n d Viets in 1941 first drew a t t e n t i o n to the application of the p r o s t i g m i n test w i t h b a r i u m swallow in cases w i t h d y s p h a g i a as the p r e d o m i n a n t or only s y m p t o m . Viets in 1947 f u r t h e r elaborated on this, a n d c o n t i n e n t a l workers have u s e d k y m o g r a p h y in s t u d y i n g the tonicity of t h e p h a r y n g e a l musculature. Edwards a n d M u r r a y (1957) have
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Fig. a.--Bulbar myasthenia gravis. Female, aged 46. Swallowing thin barium. A, Stasis in the pharynx. B, Ballooning of pharynx after repeated swallowing acts. C, Nasal reflux in the erect position.
Fig. 3.--Bulbar myasthenia gravis. Female, aged 25. Dionosil swallow. Films at 5-second intervals with repeated swallowing to show the progressive loss of tone of the pharynx. Pooling in the recesses.
recently r e p o r t e d t h e i r findings in 8 cases, 6 of w h i c h h a d p r o n o u n c e d a b n o r m a l i t i e s of t h e oral stage of deglutition, b u t only m i n o r d i s t u r b a n c e of the p h a r y n g e a l stage. T h e abnormalities of d e g l u t i t i o n fall into two g r o u p s : (I) T h e oral stage, a n d (2) t h e p h a r y n g e a l stage. i. T h e a b n o r m a l i t i e s i n v o l v i n g t h e oral stage are : - a. H e s i t a n c y in initiating deglutition. b. Slow a n d inefficient m o v e m e n t s of t h e t o n g u e w h i c h b e c o m e progressively weaker a n d slower as c o n t i n u e d a t t e m p t s at swallowing are made. T h e rotational propulsive m o v e m e n t of t h e t o n g u e becomes weaker a n d less effective. c. Inefficiency in f o r m i n g a bolus on t h e back of the tongue, especially w i t h paste so t h a t t h e paste is b r o k e n u p a n d p u s h e d piecemeal t h r o u g h the fauces into t h e m e s o p h a r y n x , p a r t of it escaping forwards
b. Loss of tone a n d ballooning of the p h a r y n x after r e p e a t e d swallowing acts w i t h inability to raise the larynx a n d to close t h e u p p e r p h a r y n x d u r i n g the first stage of d e g l u t i t i o n (Figs. 2-4). c. E v e n t u a l l y cessation of swallowing usually associated w i t h sagging of t h e jaw. d. Nasal r e f l u x - - I have seen this in only 2 cases o u t of 15, b u t it w o u l d p r o b a b l y occur m o r e freq u e n t l y if t h e p a t i e n t s were e x a m i n e d s u p i n e with c r e a m (Fig. 2 C). D i s t u r b a n c e of t h e oral stage tends to be associated w i t h generalized m y a s t h e n i a a n d m a y occur alone or w i t h only m i n o r d i s t u r b a n c e of the pharyngeal function. I n b u l b a r cases, t h e p h a r y n g e a l dysfunction is the m a i n cause of disability, b u t ther~ is also i n t e r f e r e n c e w i t h the oral phase of deglutition. Because of t h e p a t i e n t ' s inability to close the' u p p e r p h a r y n x a n d fauces a n d to appose the posterior p h a r y n g e a l wall a n d palate effectively to t h e base of
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the tongue, there is difficulty in forming a bolus ready for transmission through the pharynx. It is obviously dangerous to use paste in these bulbar cases as there may be difficulty in re-establishing an airway because of the stasis in the pharynx. Deglutition in bulbar cases is very similar to that in progressive bulbar palsy and has m a n y points
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T h e r e is never spasm or hypertrophy of the crico-pharyngeus as may be seen in Plummer-Vinson syndrome, pharyngo-cesophageal incoordination, and some cases of globus hystericus. S o m e cases of hysterical dysphagia may simulate the oral abnormality of myasthenia gravis, but the lack of response to prostigmin or tensilon will
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Fig. 4 . - - S e v e r e bulbar myasthenia gravis. Male, aged 51. A, Ballooned pharynx•
B B, Almost normal tone after 2"5 mg.
p r o s t i g m i n subcutaneously.
of similarity to pharyngeal palsies resulting f r o m poliomyelitis and to senile pharyngo-cesophageal incoordination. T h e r e is persistent pooling in the vallecul~e and pyriform recesses despite repeated swallowing acts, and residual barium will remain around and over the entrance to the larynx. T h i s stasis in the pharynx appears to be due to the following factors : - I. T h e weak propulsive action of the tongue combined with the weak contraction of the constrictors. 2. T h e larynx is very often not raised to the level of the hyoid bone so that the action of a normal descent of a normally raised larynx in clearing the hypopharynx is lost. 3. T h e normal forward and u p w a r d m o v e m e n t of the posterior pharyngeal wall is either weak or lost completely, detracting from the efficiency in reducing the pharyngeal lumen. T h e r e is also inefficient closure of the nasopharynx allowing the barium to surge up into it even in the erect position (Fig. z c). T h e passage of the bolus through the pharynx is symmetrical and it is not deflected to either side as in many cases of poliomyelitis. T h e loss of tone and weakness of contraction affect both sides equally. II
differentiate, though in some cas~s an apparent response may be seen, w h e n it m a y be necessary to repeat the examination after an injection of normal saline. It is important to r e m e m b e r that pooling in the vallecul~e may also occur in normal persons, especially in the older age-groups. Repeat examinations to show the effects of various drugs may be carried out as follows : - i. One h o u r after prostigmin or mestinon orally. 2. T h i r t y minutes after prostigmin administered subcutaneously. 3. A b o u t one minute after tensilon, given intravenously. A suitable dose of prostigmin as a diagnostic test is 1"5 rag. subcutaneously with I / I o o gr. atropine to minimize any side-effects. T e n s i l o n Io mg. intravenously is a useful diagnostic agent if the examination i s being carried out with an image intensifier. It reduces the time of examination, but it is not very practicable with ordinary screening. It acts in less than a minute and its effects are very transient, lasting only for about 3o seconds to I 1 minutes, making it difficult to time the repeat examination. Barium swallow examination is, I feel, a worthwhile investigation in myasthenia gravis especially if an image intensifier and cine-camera are available.
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T h e e x a m i n a t i o n p r o v i d e s a p e r m a n e n t record for assessment of t h e p a t i e n t ' s progress a n d response to drugs a n d has t h e a d v a n t a g e of b e i n g to a large extent i n d e p e n d e n t of t h e p a t i e n t ' s v o l u n t a r y control and subjective feelings. A b n o r m a l i t i e s of deglutition may b e d e m o n s t r a t e d even in p a t i e n t s w h o have no corn. p l a i n t of dysphagia. O f 15 cases recently examined, I I h a d some form of dysphagia a n d in 7 of these it was a p r o m i n e n t s y m p t o m , b u t t h e o t h e r 4 also h a d a detectable a b n o r m a l i t y of the oral stage of deglutition. Distur. b a n c e of t h e oral stage varies w i t h t h e degree of general m u s c u l a r weakness, w h i c h is as one would expect, b e i n g entirely a f u n c t i o n of v o l u n t a r y muscle. P h a r y n g e a l d y s f u n c t i o n especially if severe is, I feel, of grave prognosis. T h r e e cases h a d severe p h a r y n g e a l a b n o r m a l i t i e s a n d of those one died w i t h i n t h r e e m o n t h s of t h e onset of t h e disease, one died w i t h i n eight m o n t h s , a n d one, n o w eighteen m o n t h s after the onset, is in a r a t h e r precarious state, h a v i n g b e e n close to d e a t h i n four m y a s t h e n i c crises, a n d it is d o u b t f u l if h e c a n ever be allowed to leave hospital. O f 4 cases w i t h m o d e r a t e l y severe pharyngeal d y s f u n c t i o n 2 are b e i n g controlled w i t h difficulty o n v e r y large doses of m e s t i n o n a n d p r o s t i g m i n and the o t h e r 2, one of w h i c h h a d a t h y m e c t o m y and one deep X - r a y t h e r a p y to a small t h y m i c t u m o u r , are r e a s o n a b l y well controlled.
HYSTERICAL DYSPHAGIA Fig; 5.--Prominence of the cricopharyngeus with a very small diverticulum. Male, aged 74- No dysphagia. No abnormality in the oesophagus.
T h i s label t e n d s to b e a t t a c h e d to any p a t i e n t with dysphagia for w h i c h n o cause can be found, especially if t h e r e is any i n d i c a t i o n of emotional instability or
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Fig. 6 . - - N o r m a l . A, Anterior convexity of the lumen, but B, N o indentation as the bolus passes t h r o u g h the post-cricoid region. Small anterior web.
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stress in t h e p a t i e n t ' s personality or history. M o s t patients state t h a t food sticks in the t h r o a t or at t h e level of the cricoid cartilage and m a n y have a c o n s t a n t feeling of s o m e t h i n g stuck t h e r e w h i c h t h e y are unable to swallow. F r o m the radiological p o i n t of view these cases fall into four groups : - I. T h o s e w i t h a p p a r e n t l y n o r m a l deglutition. 2. T h o s e w i t h delay i n initiating d e g l u t i t i o n a n d with hesitant, t r e m u l o u s m o v e m e n t s of t h e tongue.
the first stage of d e g l u t i t i o n w i t h paste. H e r response to p r o s t i g m i n I rag. s u b c u t a n e o u s l y was equivocal, b u t r e - e x a m i n a t i o n o n a n o t h e r occasion s h o w e d a definite i m p r o v e m e n t after p r o s t i g m i n 2 mg. s u b c u t a n e o u s l y (Fig. I). H e r s y m p t o m s were controlled b y oral p r o s t i g m i n a n d m e s t i n o n b u t relapsed w h e n , u n k n o w n to t h e patient, ascorbic acid tablets were substituted. Patients w i t h difficulty in relaxing the u p p e r constrictors m a y have a n irritative lesion in the
Fig. 7.--Normal. Indentation as the cricopharyngeus closes on the tail of the bolus.
Fig. 8. Senilepharyngo-cesophagealincoordination. Male, aged 86. Four years' dysphagia especiallychoking with fluids; mild paralysis agitans. Ballooned pharynx with stasis and laryngeal spill. Anterior convexity in p6st-cricoid region simulating neoplasm, but endoscopy and biopsy negative.
T h e s e p a t i e n t s appear to have difficulty in f o r m i n g a bolus o n t h e back of the tongue. 3. T h o s e w i t h inability to relax t h e u p p e r constrictors to receive the bolus f r o m t h e m o u t h . T h e s e p a t i e n t s can swallow only b y s q u e e z i n g t h e bolus t h r o u g h the u p p e r p h a r y n x b y forceful r e p e a t e d rotational m o v e m e n t s of t h e tongue. M a n y of t h e m will ' g a g ' a n d ' r e t c h ' w h e n a t t e m p t i n g to swallow b a r i u m paste. T h e i r m a i n difficulty is w i t h d r y solid foods, b u t they can often swallow liquids a n d moist semi-solids quite easily. 4- T h o s e w i t h h y p e r t o n i c i t y a n d p r o m i n e n c e of the t r a n s v e r s e p a r t of t h e cricopharyngeus. I t is, of course, very i m p o r t a n t n o t to dismiss a n y case of dysphagia as b e i n g hysterical or f u n c t i o n a l w i t h o u t v e r y exhaustive efforts--clinical, endoscopic, and r a d i o l o g i c a l - - t o discover a n organic basis for it. Some, t h o u g h p r o b a b l y only a small p r o p o r t i o n , of t h e cases in G r o u p s i a n d 2 m a y b e examples of early m y a s t h e n i a gravis. O n e of m y r e c e n t cases of m y a s t h e n i a gravis was originally diagnosed as n e u r a s t h e n i a w i t h functional dysphagia. She h a d easy fatiguableness, b e c o m i n g worse later in t h e day, and said she was u n a b l e to swallow h e r food properly. At b a r i u m swallow e x a m i n a t i o n she h a d only delay in
p h a r y n x or larynx, e.g., a small foreign b o d y in one of t h e p h a r y n g e a l recesses or ulceration of the glottis. T h e significance of p r o m i n e n c e of the cricop h a r y n g e u s will b e discussed m o r e fully in the following section. T h r e e r e c e n t cases w i t h s y m p t o m s suggestive of globus hystericus, w h i c h s h o w e d this finding only o n e x a m i n a t i o n in the erect position, h a d free oes0phageal reflux w i t h a sliding hiatal h e r n i a w h e n a complete e x a m i n a t i o n was carried out. T h e finding of tonic c o n t r a c t i o n of t h e c r i c o p h a r y n g e u s s h o u l d n o t b e accepted as t h e c o m p l e t e answer in a p a t i e n t p r e s e n t i n g w i t h globus hystericus. PHARYNGO-(ESOPHAGEAL INCOORDINATION
T h e passage of a b o l u s f r o m t h e m o u t h into the u p p e r oesophagus is controlled b y a series of m u s c u l a r actions w h i c h occur w i t h great r a p i d i t y a n d require precise reflex co-ordination. O n l y t h e initial f o r m a tion of t h e bolus a n d its p r o p u l s i o n into the p h a r y n x are u n d e r v o l u n t a r y control, a n d t h e c o - o r d i n a t i o n of the p h a r y n g e a l m o v e m e n t s a n d relaxation of t h e
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entrance of the oesophagus are dependent on a series of reflexes through the pharyngeal plexuses and the nerves which constitute them--branches from the glossopharyngeal, vagus, and accessory nerves and the sympathetic chain. T h e reflex mechanisms are intricate and poorly understood, but a number of points are of interest to the radiologist. T h e pharyngeal reflexes are closely associated with neuromuscular activity in the oesophagus, e.g., the peristaltic waves which begin in the oesophagus when the patient goes through the motions of swallowing. Afferent stimuli from the distribution of the vagus also influence them, e.g., the gagging and difficulty in swallowing which many people have on the 'morning after' is probably a vagal reflex to protect the sensitive gastric mucosa from further insult. Control is also exerted by the higher centres, e.g., the exaggerated pharyngeal reflexes in bilateral lesions of the pyramidal tracts above the medulla (Brain, 1955). T he various sections of the gastro-intestinal tract are separated from each other by sphincters, either anatomical or functional, and the pharynx is no exception. T h e transverse belly of the cricopharyngeus apparently acts as the sphincter between the pharynx and oesophagus, relaxing to allow the bolus to pass down and also serving to prevent reflux of the oesophageal contents. As with all other sphincters its function may be deranged and it is to this dysfunction that I would like to apply the term pharyngo-oesophageal incoordination. The radiographic manifestation of this is incomplete relaxation of the cricopharyngeus and an indentation on the posterior aspect of the column of barium as it passes through the post-cricoid region. Crichlow describes four stages of hypertrophy, varying from a small indentation to a deep blunt cleft, occasionally with a pouch or early diverticulum above it. From his detailed cineradiographic studies he is quite convinced that there is no normal radiographic visualization of the cricopharyngeus and that its demonstration is of definite pathological significance. This is at variance with the opinion of other writers (Duff Gray, 1932; Johnstone, 1946, I952), who state that an indentation may occur due to the transverse part of the muscle and is not necessarily of pathological significance. I think it is incorrect to say that there is no normal visualization of the cricopharyngeus, but the demonstration of a prominent indentation by it should prompt a thorough investigation of the upper gastrointestinal or respiratory tract. I recently looked specifically for a demonstrable cricopharyngeus in 200 consecutive barium meal examinations, excluding from the series any patient with any form of dysphagia. A slight anterior convexity of the lumen in the post-cricoid region is seen very frequently, especially after the bolus has passed and this convexity can be quite prominent in the older age-group associated with some stasis in the pharyngeal recesses (Fig. 5). This convexity which is undoubtedly due to the cricopharyngeus was seen in 3I cases (Fig. 6). Contraction of the cricopharyngeus as the tail of the bolus passes it can occasionally be appreciated and was noted in 7 cases (Fig. 7). An indentation on the posterior wall of the barium column was easily
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appreciated in I3 cases with free oesophageal reflux out of 24 occurring in. the series and in 5 cases with no demonstrable abnormality in the oesophagus. In all cases there was no appreciable hold-up of either barium fluid or paste and the indentation formed a small segment of a large circle and not the deep blunt cleft due to a tonic hypertrophied transverse belly. During the same period I have noted a deep indentation due to incomplete relaxation of the muscle associated with retention of the barium in the pharyngeal recesses in the following conditions: one case of carcinoma of the lower end of the oesophagus, one case of cardiospasm, one case of corkscrew oesophagus, and two cases with compression and displacement of the lower oesophagus by a tortuous atheromatous aorta. These patients, however, with the exception of the case of cardiospasm, were all elderly and the findings in the pharynx may have been due to coincidental senile pharyngo-oesophageal incoordination. I have already mentioned the three cases with symptoms suggestive Of globus hystericus and hypertonicity of the cricopharyngeus, which had free oesophageal reflux: Their globus may have been due to the cricopharyngeal spasm, but this was in all probability a protective reflex to prevent the oesophageal contents flooding into the pharynx. Achalasia of the exit from the pharynx may be functional or idiopathic in so far as no cause may be found for it in patients with symptoms which can be explained by it. When it occurs in the younger age-groups it is usually labelled 'globus hystericus ', but a rather similar condition is quite frequent in old people and may be termed senile pharyngo-oesophageal incoordination. In this condition there is hypertrophy of the cricopharyngeus associated with hypotonJcity of the pharynx, pooling in the pharyngeal recesses, and quite often spill over of fluid into the trachea. There is a prominent anterior convexity of the lumen in the post-cricoid region which might easily be mistaken for neoplasm (Fig. 8). These patients often have more difficulty with fluids than with solids and the dysphagia is often associated with senile degenerative conditions in the central nervous system, e.g., paralysis agitans or cerebral arteriosclerosis (Figs. 8, 9). It may be found also in psychoses of later life (Fig. io). It is important to distinguish this senile condition from myasthenia gravis (Fig. 4), especially as in about 2o per cent of cases myasthenia gravis may begin after 60 years of age (Viets, 1947). In the myasthenie, there is no hypertrophy or spasm of the cricopharyngeus and the improvement after an adequate dose of prostigmin is diagnostic. Incomplete relaxation of the cricopharyngeus is invariably present in recurrent laryngeal nerve palsy due to any cause. In patients at or after middle age the most difficult and common problem is to differentiate it from an early post-cricoid neoplasm. Irregularity of the contour, rigidity, and disturbance of the mucosal pattern will indicate neoplasm. Careful endoscopic examination and biopsy may be necessary. Th e indentation produced by large osteophytes on the cervical vertebrae is easily differentiated; it is constantly opposite the osteophytes. Th e Plumrner-Vinson syndrome might also be included in this group, and it is likely that the indentation due to the cricopharyngeus in this
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condition is s e c o n d a r y to t h e changes in t h e p h a r y n geal mucosa. As it forms a definite clinical a n d radiological e n t i t y it is p r o b a b l y b e s t o m i t t e d f r o m the g r o u p u n d e r consideration. N e o n a t a l p h a r y n g e a l ineoordination, o f t e n associated w i t h evidence of intracranial b i r t h injury, is p r o b a b l y best o m i t t e d also. I have n o t seen s p a s m of the c r i c o p h a r y n g e u s in this c o n d i t i o n a n d t h e
w i t h o t h e r a b n o r m a l i t i e s of t h e p h a r y n x a n d cesop h a g u s a n d w i t h neurological conditions in later life.
Fig. 9.--Indentation by the cricopharyngeus in patient (male, aged 73) with stasis in the pharynx. Free msophageal reflux. Swallowingliquids frequently induces coughing. Mild senile incoordination.
Fig. io.--Confusional hypomania. Male, aged 61. Incomplete relaxation of the cricopharyngeus ~vith an incoordinate pharynx. Chronic duodenal ulcer.
i n c o o r d i n a t i o n appears to be in the u p p e r p h a r y n x , p r o d u c i n g nasal r e g u r g i t a t i o n a n d spill-over into t h e trachea;
a n d interest. I wish to t h a n k Miss B. H o d g k i n s o n for help w i t h t h e c i n e r a d i o g r a p h y , M r . E. N o r m a n for the r e p r o d u c t i o n s , a n d Miss V. Peters for the typing.
A c k n o w l e d g e m e n t s . - - 1 a m grateful to Dr. P. H. W h i t a k e r for his valuable advice in the p r e p a r a t i o n of this p a p e r a n d for p e r m i s s i o n to carry out t h e c i n e r a d i o g r a p h i c studies at L i v e r p o o l Royal Infirmary, a n d to Dr. J. A. Ross for his e n c o u r a g e m e n t
CONCLUSIONS AND SUMMARY Radiology, especially w i t h cineradiography, can play a useful role in the diagnosis a n d m a n a g e m e n t of disorders of deglutition, a n d t h e f u n c t i o n is somet h i n g m o r e t h a n the m e r e d e m o n s t r a t i o n of a n a t o m i cal abnormalities. I t provides t h e only m e t h o d w h e r e b y the m o v e m e n t s of the p h a r y n x a n d oesophagus can b e directly studied. T h e advantages of a p e r m a n e n t record on a cinefilm are self e v i d e n t in t h e diagnosis and the assessment of progress in m a n y conditions, m o r e particularly in m y a s t h e n i a gravis, a disease w i t h a f l u c t u a n t course a n d in w h i c h n e w drugs are constantly u n d e r trial. T h e abnormalities of deglutition in m y a s t h e n i a gravis, hysterical dysphagia, a n d pharyngo-oesophageal i n c o o r d i n a t i o n are discussed. T h e o p i n i o n is expressed t h a t radiological visualization of the c r i c o p h a r y n g e u s muscle m a y occur w i t h o u t significance t h o u g h it is f r e q u e n t l y associated
REFERENCES ARDRAN, O. M~., and KEMP, F. H. (I95I), Proc. R. Soc. Med., 44, lO38. (t952), Brit. J. Radiol., 25, 406. (t954), J. Physiol., 126, 23. and WEOELIUS, C. (I957), Brit. J. Radiol., 30, I69. BRAIN, R. (I955) , Diseases of the Nervous System, 5th ed. London: Oxford University Press. CRlCHLOW, T. V. L. (I956), Brit. J. Radiol., 29, 546. EDWARDS, J. W., and MURRAY, J. P. (1957), Ibid., 30, 263. GRAY, E. D. (I932), Ibid., S, 640. JOHNSTONE, A. S. (1946), Ibid., 19, IOI. - - - - (I952), "Radiology of the (Esophagus" in Modern Trends in Gastro-enterology. London: Butterworth. ROBERTS, R. I. (I957), Brit. J. Radiol., 30, 449. SCHWAB, R. S., and VIETS, H. R. (194I), Arner. J. Roentgenol., 4$, 357. VIETS, H. R. (I947), J- Amer. reed. Ass., 134, 987.
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Neuromuscular and Functional Disorders of the Pharynx* J. P. MURRAY, M.B., F.F.R., D.M.R. (D), D.M.R.D. Sefton General and Alder Hey Children's Hospitals, Liverpool T ~ object of this paper is to discuss the value of radiology in pharyngeal disorders of a general type as distinct from paralysis of localized groups of muscles. I propose to deal with deglutition in myasthenia gravis, hysterical dysphagia, and pharyngo-~esophageal
c
recesses, and constriction rings at the level of the cricoid cartilage. T h e anteroposterior projection is of course of most use in paralysis of specific groups of m u s c l e s - as after p o l i o m y e l i t i s - - t o demonstrate asymmetrical
4 ~
a:
Fig. I . - - G e n e r a l i z e d myasthenia gravis. Female, aged 55. Frames selected at 2-second intervals swallowing thick paste. Above: Delay in clearing m o u t h and oropharynx. Below: Well-marked improvement at a repeat examination 30 minutes after 2 mg. prostigmin subcutaneously.
incoordination--a rather wide t e r m including conditions of diverse aetiology. T h e s e are conditions in which, until recently, clinicians have seldom sought aid from the radiologist except to exclude a neoplasm or diverticulum as the cause of the patient's symptoms. But with the wider availability of the screen image intensifier and cineradiography, more detailed information about pharyngeal function can be given. N o r m a l deglutition has been extensively studied in recent years, notably by Ardran and K e m p (195 I, I952, 1954) and by Roberts (1957) , and our conception of normal deglutition is on a clearer and more certain basis. By means of cineradiography with an image intensifier, Ardran, K e m p , and Wegelius (1957) have demonstrated the swallowing defects after poliomyelitis, and Crichlow (1956) has made a detailed study of the function and appearance of the cricopharyngeus muscle.
TECHNIQUE As regards technique a few points require mention : i. Screening and films in the lateral projection provide adequate information in most cases but the anteroposterior projection is also useful, especially in myasthenia gravis, and will provide information concerning the tone of the pharynx, pooling in the * Based partly on a paper read to the Provincial Meeting of the Faculty of Radiologists at Liverpool, October, 1957.
contractions of the constrictors and deflexion to either side of the bolus as it passes through the pharynx. 2. A little barium instilled into the nose helps to show the soft palate m o r e clearly, but in the conditions under discussion it is befter to omit this lest it obscure a slight degree of nasal reflux. 3. All cases should be examined initially with barium cream, but in cases where aspiration into the trachea may be anticipated it is safer to use an iodine medium. Patients in w h o m there is an appreciable residue around or over the entrance to the larynx should not be examined with thick paste nor be asked to carry out continuous swallowing with thin barium, as in such cases there is difficulty in re-establishing the airway and danger of aspirating the barium. 4- Patients in w h o m the diagnosis of myasthenia gravis is known or suspected require a modification of the usual technique. Repeated swallows may be necessary to demonstrate an abnormality of muscular dysfunction because a single swallow may appear to be normal. In the milder cases without obvious bulbar symptoms thick paste in the r e c u m b e n t position will accentuate the abnormalities. Alternatively, continuous swallowing of thin barium taken through a straw will show easy fatigue of deglutition w h e n it is present. Such cases are best examined in the afternoon and preferably after a meal. 5- Suitable factors for cineradiography in the lateral projection are 70-75 kV., 5-6 mA., and 18 frames per second. An increase in kilovoltage to
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a b o u t 85 kV. is necessary for t h e a n t e r o p o s t e r i o r projection. MYASTHENIA
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and in some cases a r o u n d t h e sides of the t o n g u e with each swallowing a t t e m p t . d. T h e s e defects result in a m e a s u r a b l e delay in clearing the m o u t h a n d o r o p h a r y n x , a n d there is increasing difficulty in m a k i n g repeated swallowing acts (Fig. i). 2. T h e abnormalities involving the pharyngeal stage consis~ of: a. Stasis in the p h a r y n x and pooling in the p h a r y n g e a l recesses, due to weakness of the afterc o m i n g c o n t r a c t i o n wave.
GRAVIS
S c h w a b a n d Viets in 1941 first drew a t t e n t i o n to the application of the p r o s t i g m i n test w i t h b a r i u m swallow in cases w i t h d y s p h a g i a as the p r e d o m i n a n t or only s y m p t o m . Viets in 1947 f u r t h e r elaborated on this, a n d c o n t i n e n t a l workers have u s e d k y m o g r a p h y in s t u d y i n g the tonicity of t h e p h a r y n g e a l musculature. Edwards a n d M u r r a y (1957) have
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Fig. a.--Bulbar myasthenia gravis. Female, aged 46. Swallowing thin barium. A, Stasis in the pharynx. B, Ballooning of pharynx after repeated swallowing acts. C, Nasal reflux in the erect position.
Fig. 3.--Bulbar myasthenia gravis. Female, aged 25. Dionosil swallow. Films at 5-second intervals with repeated swallowing to show the progressive loss of tone of the pharynx. Pooling in the recesses.
recently r e p o r t e d t h e i r findings in 8 cases, 6 of w h i c h h a d p r o n o u n c e d a b n o r m a l i t i e s of t h e oral stage of deglutition, b u t only m i n o r d i s t u r b a n c e of the p h a r y n g e a l stage. T h e abnormalities of d e g l u t i t i o n fall into two g r o u p s : (I) T h e oral stage, a n d (2) t h e p h a r y n g e a l stage. i. T h e a b n o r m a l i t i e s i n v o l v i n g t h e oral stage are : - a. H e s i t a n c y in initiating deglutition. b. Slow a n d inefficient m o v e m e n t s of t h e t o n g u e w h i c h b e c o m e progressively weaker a n d slower as c o n t i n u e d a t t e m p t s at swallowing are made. T h e rotational propulsive m o v e m e n t of t h e t o n g u e becomes weaker a n d less effective. c. Inefficiency in f o r m i n g a bolus on t h e back of the tongue, especially w i t h paste so t h a t t h e paste is b r o k e n u p a n d p u s h e d piecemeal t h r o u g h the fauces into t h e m e s o p h a r y n x , p a r t of it escaping forwards
b. Loss of tone a n d ballooning of the p h a r y n x after r e p e a t e d swallowing acts w i t h inability to raise the larynx a n d to close t h e u p p e r p h a r y n x d u r i n g the first stage of d e g l u t i t i o n (Figs. 2-4). c. E v e n t u a l l y cessation of swallowing usually associated w i t h sagging of t h e jaw. d. Nasal r e f l u x - - I have seen this in only 2 cases o u t of 15, b u t it w o u l d p r o b a b l y occur m o r e freq u e n t l y if t h e p a t i e n t s were e x a m i n e d s u p i n e with c r e a m (Fig. 2 C). D i s t u r b a n c e of t h e oral stage tends to be associated w i t h generalized m y a s t h e n i a a n d m a y occur alone or w i t h only m i n o r d i s t u r b a n c e of the pharyngeal function. I n b u l b a r cases, t h e p h a r y n g e a l dysfunction is the m a i n cause of disability, b u t ther~ is also i n t e r f e r e n c e w i t h the oral phase of deglutition. Because of t h e p a t i e n t ' s inability to close the' u p p e r p h a r y n x a n d fauces a n d to appose the posterior p h a r y n g e a l wall a n d palate effectively to t h e base of
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the tongue, there is difficulty in forming a bolus ready for transmission through the pharynx. It is obviously dangerous to use paste in these bulbar cases as there may be difficulty in re-establishing an airway because of the stasis in the pharynx. Deglutition in bulbar cases is very similar to that in progressive bulbar palsy and has m a n y points
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T h e r e is never spasm or hypertrophy of the crico-pharyngeus as may be seen in Plummer-Vinson syndrome, pharyngo-cesophageal incoordination, and some cases of globus hystericus. S o m e cases of hysterical dysphagia may simulate the oral abnormality of myasthenia gravis, but the lack of response to prostigmin or tensilon will
A
Fig. 4 . - - S e v e r e bulbar myasthenia gravis. Male, aged 51. A, Ballooned pharynx•
B B, Almost normal tone after 2"5 mg.
p r o s t i g m i n subcutaneously.
of similarity to pharyngeal palsies resulting f r o m poliomyelitis and to senile pharyngo-cesophageal incoordination. T h e r e is persistent pooling in the vallecul~e and pyriform recesses despite repeated swallowing acts, and residual barium will remain around and over the entrance to the larynx. T h i s stasis in the pharynx appears to be due to the following factors : - I. T h e weak propulsive action of the tongue combined with the weak contraction of the constrictors. 2. T h e larynx is very often not raised to the level of the hyoid bone so that the action of a normal descent of a normally raised larynx in clearing the hypopharynx is lost. 3. T h e normal forward and u p w a r d m o v e m e n t of the posterior pharyngeal wall is either weak or lost completely, detracting from the efficiency in reducing the pharyngeal lumen. T h e r e is also inefficient closure of the nasopharynx allowing the barium to surge up into it even in the erect position (Fig. z c). T h e passage of the bolus through the pharynx is symmetrical and it is not deflected to either side as in many cases of poliomyelitis. T h e loss of tone and weakness of contraction affect both sides equally. II
differentiate, though in some cas~s an apparent response may be seen, w h e n it m a y be necessary to repeat the examination after an injection of normal saline. It is important to r e m e m b e r that pooling in the vallecul~e may also occur in normal persons, especially in the older age-groups. Repeat examinations to show the effects of various drugs may be carried out as follows : - i. One h o u r after prostigmin or mestinon orally. 2. T h i r t y minutes after prostigmin administered subcutaneously. 3. A b o u t one minute after tensilon, given intravenously. A suitable dose of prostigmin as a diagnostic test is 1"5 rag. subcutaneously with I / I o o gr. atropine to minimize any side-effects. T e n s i l o n Io mg. intravenously is a useful diagnostic agent if the examination i s being carried out with an image intensifier. It reduces the time of examination, but it is not very practicable with ordinary screening. It acts in less than a minute and its effects are very transient, lasting only for about 3o seconds to I 1 minutes, making it difficult to time the repeat examination. Barium swallow examination is, I feel, a worthwhile investigation in myasthenia gravis especially if an image intensifier and cine-camera are available.
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T h e e x a m i n a t i o n p r o v i d e s a p e r m a n e n t record for assessment of t h e p a t i e n t ' s progress a n d response to drugs a n d has t h e a d v a n t a g e of b e i n g to a large extent i n d e p e n d e n t of t h e p a t i e n t ' s v o l u n t a r y control and subjective feelings. A b n o r m a l i t i e s of deglutition may b e d e m o n s t r a t e d even in p a t i e n t s w h o have no corn. p l a i n t of dysphagia. O f 15 cases recently examined, I I h a d some form of dysphagia a n d in 7 of these it was a p r o m i n e n t s y m p t o m , b u t t h e o t h e r 4 also h a d a detectable a b n o r m a l i t y of the oral stage of deglutition. Distur. b a n c e of t h e oral stage varies w i t h t h e degree of general m u s c u l a r weakness, w h i c h is as one would expect, b e i n g entirely a f u n c t i o n of v o l u n t a r y muscle. P h a r y n g e a l d y s f u n c t i o n especially if severe is, I feel, of grave prognosis. T h r e e cases h a d severe p h a r y n g e a l a b n o r m a l i t i e s a n d of those one died w i t h i n t h r e e m o n t h s of t h e onset of t h e disease, one died w i t h i n eight m o n t h s , a n d one, n o w eighteen m o n t h s after the onset, is in a r a t h e r precarious state, h a v i n g b e e n close to d e a t h i n four m y a s t h e n i c crises, a n d it is d o u b t f u l if h e c a n ever be allowed to leave hospital. O f 4 cases w i t h m o d e r a t e l y severe pharyngeal d y s f u n c t i o n 2 are b e i n g controlled w i t h difficulty o n v e r y large doses of m e s t i n o n a n d p r o s t i g m i n and the o t h e r 2, one of w h i c h h a d a t h y m e c t o m y and one deep X - r a y t h e r a p y to a small t h y m i c t u m o u r , are r e a s o n a b l y well controlled.
HYSTERICAL DYSPHAGIA Fig; 5.--Prominence of the cricopharyngeus with a very small diverticulum. Male, aged 74- No dysphagia. No abnormality in the oesophagus.
T h i s label t e n d s to b e a t t a c h e d to any p a t i e n t with dysphagia for w h i c h n o cause can be found, especially if t h e r e is any i n d i c a t i o n of emotional instability or
A
B
Fig. 6 . - - N o r m a l . A, Anterior convexity of the lumen, but B, N o indentation as the bolus passes t h r o u g h the post-cricoid region. Small anterior web.
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stress in t h e p a t i e n t ' s personality or history. M o s t patients state t h a t food sticks in the t h r o a t or at t h e level of the cricoid cartilage and m a n y have a c o n s t a n t feeling of s o m e t h i n g stuck t h e r e w h i c h t h e y are unable to swallow. F r o m the radiological p o i n t of view these cases fall into four groups : - I. T h o s e w i t h a p p a r e n t l y n o r m a l deglutition. 2. T h o s e w i t h delay i n initiating d e g l u t i t i o n a n d with hesitant, t r e m u l o u s m o v e m e n t s of t h e tongue.
the first stage of d e g l u t i t i o n w i t h paste. H e r response to p r o s t i g m i n I rag. s u b c u t a n e o u s l y was equivocal, b u t r e - e x a m i n a t i o n o n a n o t h e r occasion s h o w e d a definite i m p r o v e m e n t after p r o s t i g m i n 2 mg. s u b c u t a n e o u s l y (Fig. I). H e r s y m p t o m s were controlled b y oral p r o s t i g m i n a n d m e s t i n o n b u t relapsed w h e n , u n k n o w n to t h e patient, ascorbic acid tablets were substituted. Patients w i t h difficulty in relaxing the u p p e r constrictors m a y have a n irritative lesion in the
Fig. 7.--Normal. Indentation as the cricopharyngeus closes on the tail of the bolus.
Fig. 8. Senilepharyngo-cesophagealincoordination. Male, aged 86. Four years' dysphagia especiallychoking with fluids; mild paralysis agitans. Ballooned pharynx with stasis and laryngeal spill. Anterior convexity in p6st-cricoid region simulating neoplasm, but endoscopy and biopsy negative.
T h e s e p a t i e n t s appear to have difficulty in f o r m i n g a bolus o n t h e back of the tongue. 3. T h o s e w i t h inability to relax t h e u p p e r constrictors to receive the bolus f r o m t h e m o u t h . T h e s e p a t i e n t s can swallow only b y s q u e e z i n g t h e bolus t h r o u g h the u p p e r p h a r y n x b y forceful r e p e a t e d rotational m o v e m e n t s of t h e tongue. M a n y of t h e m will ' g a g ' a n d ' r e t c h ' w h e n a t t e m p t i n g to swallow b a r i u m paste. T h e i r m a i n difficulty is w i t h d r y solid foods, b u t they can often swallow liquids a n d moist semi-solids quite easily. 4- T h o s e w i t h h y p e r t o n i c i t y a n d p r o m i n e n c e of the t r a n s v e r s e p a r t of t h e cricopharyngeus. I t is, of course, very i m p o r t a n t n o t to dismiss a n y case of dysphagia as b e i n g hysterical or f u n c t i o n a l w i t h o u t v e r y exhaustive efforts--clinical, endoscopic, and r a d i o l o g i c a l - - t o discover a n organic basis for it. Some, t h o u g h p r o b a b l y only a small p r o p o r t i o n , of t h e cases in G r o u p s i a n d 2 m a y b e examples of early m y a s t h e n i a gravis. O n e of m y r e c e n t cases of m y a s t h e n i a gravis was originally diagnosed as n e u r a s t h e n i a w i t h functional dysphagia. She h a d easy fatiguableness, b e c o m i n g worse later in t h e day, and said she was u n a b l e to swallow h e r food properly. At b a r i u m swallow e x a m i n a t i o n she h a d only delay in
p h a r y n x or larynx, e.g., a small foreign b o d y in one of t h e p h a r y n g e a l recesses or ulceration of the glottis. T h e significance of p r o m i n e n c e of the cricop h a r y n g e u s will b e discussed m o r e fully in the following section. T h r e e r e c e n t cases w i t h s y m p t o m s suggestive of globus hystericus, w h i c h s h o w e d this finding only o n e x a m i n a t i o n in the erect position, h a d free oes0phageal reflux w i t h a sliding hiatal h e r n i a w h e n a complete e x a m i n a t i o n was carried out. T h e finding of tonic c o n t r a c t i o n of t h e c r i c o p h a r y n g e u s s h o u l d n o t b e accepted as t h e c o m p l e t e answer in a p a t i e n t p r e s e n t i n g w i t h globus hystericus. PHARYNGO-(ESOPHAGEAL INCOORDINATION
T h e passage of a b o l u s f r o m t h e m o u t h into the u p p e r oesophagus is controlled b y a series of m u s c u l a r actions w h i c h occur w i t h great r a p i d i t y a n d require precise reflex co-ordination. O n l y t h e initial f o r m a tion of t h e bolus a n d its p r o p u l s i o n into the p h a r y n x are u n d e r v o l u n t a r y control, a n d t h e c o - o r d i n a t i o n of the p h a r y n g e a l m o v e m e n t s a n d relaxation of t h e
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entrance of the oesophagus are dependent on a series of reflexes through the pharyngeal plexuses and the nerves which constitute them--branches from the glossopharyngeal, vagus, and accessory nerves and the sympathetic chain. T h e reflex mechanisms are intricate and poorly understood, but a number of points are of interest to the radiologist. T h e pharyngeal reflexes are closely associated with neuromuscular activity in the oesophagus, e.g., the peristaltic waves which begin in the oesophagus when the patient goes through the motions of swallowing. Afferent stimuli from the distribution of the vagus also influence them, e.g., the gagging and difficulty in swallowing which many people have on the 'morning after' is probably a vagal reflex to protect the sensitive gastric mucosa from further insult. Control is also exerted by the higher centres, e.g., the exaggerated pharyngeal reflexes in bilateral lesions of the pyramidal tracts above the medulla (Brain, 1955). T he various sections of the gastro-intestinal tract are separated from each other by sphincters, either anatomical or functional, and the pharynx is no exception. T h e transverse belly of the cricopharyngeus apparently acts as the sphincter between the pharynx and oesophagus, relaxing to allow the bolus to pass down and also serving to prevent reflux of the oesophageal contents. As with all other sphincters its function may be deranged and it is to this dysfunction that I would like to apply the term pharyngo-oesophageal incoordination. The radiographic manifestation of this is incomplete relaxation of the cricopharyngeus and an indentation on the posterior aspect of the column of barium as it passes through the post-cricoid region. Crichlow describes four stages of hypertrophy, varying from a small indentation to a deep blunt cleft, occasionally with a pouch or early diverticulum above it. From his detailed cineradiographic studies he is quite convinced that there is no normal radiographic visualization of the cricopharyngeus and that its demonstration is of definite pathological significance. This is at variance with the opinion of other writers (Duff Gray, 1932; Johnstone, 1946, I952), who state that an indentation may occur due to the transverse part of the muscle and is not necessarily of pathological significance. I think it is incorrect to say that there is no normal visualization of the cricopharyngeus, but the demonstration of a prominent indentation by it should prompt a thorough investigation of the upper gastrointestinal or respiratory tract. I recently looked specifically for a demonstrable cricopharyngeus in 200 consecutive barium meal examinations, excluding from the series any patient with any form of dysphagia. A slight anterior convexity of the lumen in the post-cricoid region is seen very frequently, especially after the bolus has passed and this convexity can be quite prominent in the older age-group associated with some stasis in the pharyngeal recesses (Fig. 5). This convexity which is undoubtedly due to the cricopharyngeus was seen in 3I cases (Fig. 6). Contraction of the cricopharyngeus as the tail of the bolus passes it can occasionally be appreciated and was noted in 7 cases (Fig. 7). An indentation on the posterior wall of the barium column was easily
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appreciated in I3 cases with free oesophageal reflux out of 24 occurring in. the series and in 5 cases with no demonstrable abnormality in the oesophagus. In all cases there was no appreciable hold-up of either barium fluid or paste and the indentation formed a small segment of a large circle and not the deep blunt cleft due to a tonic hypertrophied transverse belly. During the same period I have noted a deep indentation due to incomplete relaxation of the muscle associated with retention of the barium in the pharyngeal recesses in the following conditions: one case of carcinoma of the lower end of the oesophagus, one case of cardiospasm, one case of corkscrew oesophagus, and two cases with compression and displacement of the lower oesophagus by a tortuous atheromatous aorta. These patients, however, with the exception of the case of cardiospasm, were all elderly and the findings in the pharynx may have been due to coincidental senile pharyngo-oesophageal incoordination. I have already mentioned the three cases with symptoms suggestive Of globus hystericus and hypertonicity of the cricopharyngeus, which had free oesophageal reflux: Their globus may have been due to the cricopharyngeal spasm, but this was in all probability a protective reflex to prevent the oesophageal contents flooding into the pharynx. Achalasia of the exit from the pharynx may be functional or idiopathic in so far as no cause may be found for it in patients with symptoms which can be explained by it. When it occurs in the younger age-groups it is usually labelled 'globus hystericus ', but a rather similar condition is quite frequent in old people and may be termed senile pharyngo-oesophageal incoordination. In this condition there is hypertrophy of the cricopharyngeus associated with hypotonJcity of the pharynx, pooling in the pharyngeal recesses, and quite often spill over of fluid into the trachea. There is a prominent anterior convexity of the lumen in the post-cricoid region which might easily be mistaken for neoplasm (Fig. 8). These patients often have more difficulty with fluids than with solids and the dysphagia is often associated with senile degenerative conditions in the central nervous system, e.g., paralysis agitans or cerebral arteriosclerosis (Figs. 8, 9). It may be found also in psychoses of later life (Fig. io). It is important to distinguish this senile condition from myasthenia gravis (Fig. 4), especially as in about 2o per cent of cases myasthenia gravis may begin after 60 years of age (Viets, 1947). In the myasthenie, there is no hypertrophy or spasm of the cricopharyngeus and the improvement after an adequate dose of prostigmin is diagnostic. Incomplete relaxation of the cricopharyngeus is invariably present in recurrent laryngeal nerve palsy due to any cause. In patients at or after middle age the most difficult and common problem is to differentiate it from an early post-cricoid neoplasm. Irregularity of the contour, rigidity, and disturbance of the mucosal pattern will indicate neoplasm. Careful endoscopic examination and biopsy may be necessary. Th e indentation produced by large osteophytes on the cervical vertebrae is easily differentiated; it is constantly opposite the osteophytes. Th e Plumrner-Vinson syndrome might also be included in this group, and it is likely that the indentation due to the cricopharyngeus in this
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I4I
condition is s e c o n d a r y to t h e changes in t h e p h a r y n geal mucosa. As it forms a definite clinical a n d radiological e n t i t y it is p r o b a b l y b e s t o m i t t e d f r o m the g r o u p u n d e r consideration. N e o n a t a l p h a r y n g e a l ineoordination, o f t e n associated w i t h evidence of intracranial b i r t h injury, is p r o b a b l y best o m i t t e d also. I have n o t seen s p a s m of the c r i c o p h a r y n g e u s in this c o n d i t i o n a n d t h e
w i t h o t h e r a b n o r m a l i t i e s of t h e p h a r y n x a n d cesop h a g u s a n d w i t h neurological conditions in later life.
Fig. 9.--Indentation by the cricopharyngeus in patient (male, aged 73) with stasis in the pharynx. Free msophageal reflux. Swallowingliquids frequently induces coughing. Mild senile incoordination.
Fig. io.--Confusional hypomania. Male, aged 61. Incomplete relaxation of the cricopharyngeus ~vith an incoordinate pharynx. Chronic duodenal ulcer.
i n c o o r d i n a t i o n appears to be in the u p p e r p h a r y n x , p r o d u c i n g nasal r e g u r g i t a t i o n a n d spill-over into t h e trachea;
a n d interest. I wish to t h a n k Miss B. H o d g k i n s o n for help w i t h t h e c i n e r a d i o g r a p h y , M r . E. N o r m a n for the r e p r o d u c t i o n s , a n d Miss V. Peters for the typing.
A c k n o w l e d g e m e n t s . - - 1 a m grateful to Dr. P. H. W h i t a k e r for his valuable advice in the p r e p a r a t i o n of this p a p e r a n d for p e r m i s s i o n to carry out t h e c i n e r a d i o g r a p h i c studies at L i v e r p o o l Royal Infirmary, a n d to Dr. J. A. Ross for his e n c o u r a g e m e n t
CONCLUSIONS AND SUMMARY Radiology, especially w i t h cineradiography, can play a useful role in the diagnosis a n d m a n a g e m e n t of disorders of deglutition, a n d t h e f u n c t i o n is somet h i n g m o r e t h a n the m e r e d e m o n s t r a t i o n of a n a t o m i cal abnormalities. I t provides t h e only m e t h o d w h e r e b y the m o v e m e n t s of the p h a r y n x a n d oesophagus can b e directly studied. T h e advantages of a p e r m a n e n t record on a cinefilm are self e v i d e n t in t h e diagnosis and the assessment of progress in m a n y conditions, m o r e particularly in m y a s t h e n i a gravis, a disease w i t h a f l u c t u a n t course a n d in w h i c h n e w drugs are constantly u n d e r trial. T h e abnormalities of deglutition in m y a s t h e n i a gravis, hysterical dysphagia, a n d pharyngo-oesophageal i n c o o r d i n a t i o n are discussed. T h e o p i n i o n is expressed t h a t radiological visualization of the c r i c o p h a r y n g e u s muscle m a y occur w i t h o u t significance t h o u g h it is f r e q u e n t l y associated
REFERENCES ARDRAN, O. M~., and KEMP, F. H. (I95I), Proc. R. Soc. Med., 44, lO38. (t952), Brit. J. Radiol., 25, 406. (t954), J. Physiol., 126, 23. and WEOELIUS, C. (I957), Brit. J. Radiol., 30, I69. BRAIN, R. (I955) , Diseases of the Nervous System, 5th ed. London: Oxford University Press. CRlCHLOW, T. V. L. (I956), Brit. J. Radiol., 29, 546. EDWARDS, J. W., and MURRAY, J. P. (1957), Ibid., 30, 263. GRAY, E. D. (I932), Ibid., S, 640. JOHNSTONE, A. S. (1946), Ibid., 19, IOI. - - - - (I952), "Radiology of the (Esophagus" in Modern Trends in Gastro-enterology. London: Butterworth. ROBERTS, R. I. (I957), Brit. J. Radiol., 30, 449. SCHWAB, R. S., and VIETS, H. R. (194I), Arner. J. Roentgenol., 4$, 357. VIETS, H. R. (I947), J- Amer. reed. Ass., 134, 987.