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The Myotonic (Holmes Adie) pupil | ACNR

The Myotonic (Holmes Adie) pupil

Posted in History on 29th Might 2019

JMS Pearce MD, FRCP Emeritus Advisor Neurologist, Department of Neurology, Hull Royal Infirmary, UK.
Correspondence to: J.M.S. Pearce, 304 Beverley Street Anlaby, East Yorks, HU10 7BG, UK.
E mail: [email protected]

Battle of interest statement: None declared.

Date first submitted: 20/10/18

Acceptance date: 25/10/18

Revealed on-line first: 29/5/19

Featured image: WJ Adie
From: Alastair Compston; Idiopathic narcolepsy: a illness sui generis; with remarks on the mechanisms of sleep. By WJ Adie. Mind, 2008;131:2532–2535,

The harmless situation of a dilated pupil which reacts abnormally slowly (myotonic) to mild and convergence was absolutely described in 1931 by William John Adie (1886 – 1935),1 of the National Hospital, Queen Square:

I wish to draw attention to a benign symptomless disorder characterised by pupils which react on lodging but not to mild, and by absent tendon reflexes. 5 of the six instances I am about to explain got here underneath my discover in the middle of a number of weeks;… Though harmless in itself it deserves recognition as a result of it’s typically mistaken for a manifestation of syphilis of the nervous system, with unlucky penalties… Mr. Foster Moore has described seven instances beneath the title ‘Non-luetic Argyll Robertson pupil.2

Often seen in females, in 80% instances it’s unilateral. Adie was not the first to watch this uncommon curiosity and its variant manifestations. In 1818 the London ophthalmologist, James Ware (1756–1815), described a patient who might have had a myotonic pupil.three His affected person, whose pupillary abnormality had been recognized for twenty years, was:

A woman between thirty and forty years of age, the pupil of whose proper eye, when she is just not engaged in studying, or in working together with her needle, is all the time dilated very almost to the rim of the cornea; but each time she seems to be at a small object, nine inches from the eye, it contracts, within lower than a minute, to a measurement almost as small as the top of a pin. Her left pupil shouldn’t be affected like the appropriate; but in each diploma of sunshine and distance, it is contracted fairly greater than is usual in different individuals.… A number of situations have come beneath my discover, by which the pupil of 1 eye has grow to be dilated to an ideal diploma, and has been incapable of contracting on a rise of light,

Adie said: ‘The tonic or myotonic pupillary reaction was first described in 1902 by Saenger in the left pupil of a 34 year old woman)4 and by Strasburger in a 17 year old man5 independently.6  Later in the same year both Saenger and Nonne reported further cases for which Saenger proposed the name ‘myotonic pupillary movement’ to differentiate it from other forms of mydriasis or abnormal pupillary reflexes. Later, Adie related ‘atypical ‘forms, less clearly defined, but he included some forms of ‘internal ophthalmoplegia and complete light rigidity with atonic convergence reaction.’

Earlier than Adie, Hughlings Jackson in 1881 had also clearly described the identical dysfunction:7

A lady aged 26 was sent to see me simply because the fitting pupil was much larger than the left. It had been so three years…the suitable pupil was dilated and absolutely immobile to mild, and in addition during lodging, but the lodging itself on this aspect was good; this was severely tested by Mr Couper…this case at first puzzled me…It occurred to me to test the knees. Neither I nor Mr Couper discovered the smallest trace of the knee phenomenon [knee jerk]. Several occasions did I pertinaciously inquire for different signs of tabes; there have been no other symptoms of any variety.…Dr Buzzard …confirmed the above observations.8

In 1906 Markus had described an isolated case of partial iridoplegia,9 and Weill and Reys10 also described a tonic pupil reactions to convergence and accommodation with areflexia. Some studies bear their names as eponyms.

Adie’s extra detailed, basic paper in 1932,11 described 22 sufferers, with absent tendon reflexes, and famous 44 reported instances of tonic pupil in 9 of which there were absent tendon reflexes. In this account he outlined 4 incomplete types (the last wouldn’t now be accepted):

1. The complete type—typical tonic pupil and absence of reflexes.

2. Incomplete varieties : a) tonic pupil alone; b) atypical part of the tonic pupil alone (iridoplegia; inner opthalmoplegia); c) atypical phases of the tonic pupil with absent reflexes; d) absent reflexes alone.

At about the identical time, Gordon Holmes discovered 19 sufferers with the myotonic pupils, characterised in his Introduction to Medical Neurology:

By very sluggish contraction on convergence, and even slower rest. The reflex to mild is usually lost too. One or both eyes may be affected.

Holmes’s 1931 paper described it and its affiliation with symptoms of different illnesses of the nervous system:

Steadily no change in the measurement of the pupil was seen immediately on convergence, but when this was maintained for a couple of seconds the pupil slowly and regularly grew smaller, until its diameter equalled or was even narrower than that of the traditional eye. The price of contraction assorted very a lot…When contracted the pupil stays fixed and when convergence is relaxed it dilates slowly… Within the current state of our information a separation of those instances during which the tendon jerks are absent from these through which they persist is unjustifiable . . . the similarity of the signs in all these instances naturally suggests a standard aetiology.12

Edwin Bramwell linked Holmes’s identify with Adie’s in 1936.13 The sensible George Bruyn mischievously pointed to the ‘peculiarity’ that Morgan, Symonds, Holmes and Adie all revealed at about the identical time, in several journals with out referring to each other, but they knew one another nicely at Queen Square.1 Nevertheless Adie’s second paper (1932) did point out Holmes’s work .11

The credit have to be Adie’s for stressing its harmless nature and crucially by distinguishing it from neurosyphilis. Adie did not claim originality, recognizing a number of earlier accounts. He acknowledged that Morgan and Symonds had recorded:

IN Man’s Hospital Studies for 1927 Dr. Symonds and I drew attention to a small group of instances through which sure abnormalities of the pupil, together with inequality and faulty reaction to mild and convergence, and in addition some affection of accommodation, have been related to a pathological absence or diminution of the tendon- jerks.14

It was previously confused with the Argyll Robertson pupil related to luetic tabes dorsalis or Common Paralysis of the Insane, characterised by: a loss of each direct and consensual mild reflexes; pupillary inequality; irregularity and iris atrophy with out reaction to mild. Adie clearly distinguished this from his myotonic (Pseudo-Argyll Robertson) pupil.

Since Adie’s descriptions,2,6,11 this conception of an atypical tonic pupil has been widened,15 which unnecessarily complicates a analysis which is safe if medical observations are exactly noticed.


Stanley Graveson (1915-1976) studied 15 sufferers, three men, 12 ladies, aged 12 to 55.16 He set out to illuminate: (1) the location of the anatomical lesion and (2) the character and specificity, or in any other case, of the underlying pathological course of. Two kinds of tonic pupil have been distinguished, (a) the fastened sort, and (b) the peculiar sort of tonic pupil. The solely widespread features of this latter variety are (1) their regularity of shape or place of the pupil and (2) the slowness of pupillary dilatation after convergence. Graveson pointed out that the lesion had to be on the efferent limb of the sunshine reflex arc, to account for the absence of a light-weight response in a unilateral tonic pupil with a simultaneous brisk consensual response in the normal pupil. The prompt response of the pupil to pilocarpine meant that the muscle of the iris couldn’t be at fault.

The pathogenesis remained unsure. Adie had reported: ‘all we can say is that the ocular and reflex signs we have considered seem to be the expression of a kind of perversion of nervous activity of which, at present, we can form no conception.’ Harriman and Garland nevertheless, clarified issues in a minutely examined post-mortem case in 1968 that confirmed the causal neuronal degeneration within the ciliary ganglion, denervation of ciliary body, partial atrophy of pupilloconstrictor muscle—all on the appropriate, affected aspect. The website of the lesion answerable for areflexia was much less conclusive, however they famous a selective degeneration of neurones in dorsal root ganglia, probably these supplying muscle spindles. This was in line with a neurophysiological discount in measurement of the H waves, evidence of melancholy of the monosynaptic spinal reflex arc, beforehand observed.17 It confirmed the same full loss of ciliary ganglion cells in the one other autopsied case at the time,18 and matches with the recognized denervation cholinergic hypersensitivity of the pupil to methacholine and pilocarpine.


  1. Bruyn GW, Gooddy W. Adie’s syndrome. In: Neurological Eponyms. edited Peter J. Koehler PJ, Bruyn GW, Pearce JMS. Oxford, OUP 2000;181-185.
  2. Adie W. J. Pseudo-Argyll Robertson pupils with absent tendon reflexes. Br Med J 1931;1:1091.
  3. Ware J. Observations relative to the near and distant sight of different individuals. Phil Tr Roy Soc London 1813;103:36-38.
  4. Saenger A. Myotonic Pupil Motion .Neurol Centralbl 1902;21:837 and 1137.
  5. StrasburgerJ. Sluggishness of the Pupil to Accommodation and Convergence. Neurol.ZbI 1902;21,738 and 1052.
  6. Adie WJ. Tonic Pupils and absent tendon reflexes: A benign disorder sui generis; its full and incomplete varieties. Brain. 1932;55,98-113.
  7. Pearce JMS. Hughlings Jackson and the Holmes-Adie tonic pupil. J Neurol Neurosurg Psychiatry 1995; 58 (1): 87
  8. Jackson JH. Paralytic affections. 1. On eye symptoms in locomotor ataxy. Tr ophthal Soc. UK. 1881; 1 :139-54.
  9. Markus Ch. Notes on a peculiar pupil-phenomenon in instances of partial iridoplegia. Trans Ophthal Soc UK. 1906;26:50-58.
  10. Weill G, Reys L. Rev de l’accomodation avec areflexie a la lumiere chez un sujet de crises tetanoides et d’areflexie. Revue d’Oto-Neuro-Ophtalmologie. 1926;4: 433–441.
  11. Adie WJ. Complete and incomplete types of the benign dysfunction characterised by tonic pupils and absent tendon reflexes. Br J Ophthalmol. 1932;449-60.
  12. Holmes G. Partial iridoplegia related to symptoms of different illnesses of the nervous system. Trans ophthal Soc. UK. 1931; 51 : 209-28.
  13. Bramwell E. The Holmes-Adie Syndrome. A Benign Medical Entity which Simulates Syphilis of the Nervous System. Edinburgh Med. Jour., New Collection. 43:1936;83-91
  14. Morgan, OG. and Symonds, CP. Inner Ophthalmoplegia with Absent Tendon-jerks. Proceedings of the Royal Society of Drugs 1931; pp.41-43.
  15. Lowenstein, O., and Friedman, E. D. (1942). Arch. Ophthal., Chicago, 28, 1042.
  16. Graveson G. S. The Tonic Pupil. J. Neurology Neurosurgery &#zero38; Psychiatry. 1949;12:219-30.
  17. Harriman DGF, Garland H. The pathology of Adie’s syndrome. Mind 1968; 91 ;401-18.
  18. Ruttner, F. Die tonische Pupillenreaktion. Mschr Psychiat Neurol. 1947;114:265–286.