3 70 Op h t ha I mop1 eg i a in Childhood OPHTHALMOPLEGIA can be painless, or associated with pain usually along the distribution of the fifth cranial nerve, which can be an essential finding in establishing the cause of the condition. If the ocular palsy is of sudden onset, the child may well complain of blurred or double vision, but this is by no means always so, particularly among younger children. The child is likely to be dis- tressed and the squint may not be noted. Painless oph t halmoplegia, associaied with generalized disorders Sometimes the cause will be obvious, for example after head injury; in this instance spontaneous resolution often occurs'. Ocular signs associated with hyper- thyroidism will be accompanied by evidence. of increased metabolism. Ophthalmoplegia in children is more benign than that in adults'. Graves disease is an autoimmune disorder, and there may be a shared antigenicity between the thyroid and orbit3. When ocular palsies and ptosis occur, the possibility of myasthenia gravis must always be con- sidered, and the pathological fatiguability may be hard to demonstrate in young children. In cases of doubt it may be justifiable to carry out a tendon test. There are a number of different forms of congenital myasthenia, as well as classical myasthenia due to immunological damage to acetylcholine receptors. Some of these types do not respond to anticholinesterase agents, so other diagnostic tests and therapy will have to be used4. 1SOI.ATED PAINLESS OPHTHALMOPLEGIA Sometimes in childhood the muscle tissue of the external oculdr muscles can be replaced by fibrous tissue, for no apparent reason. General fibrosis syndrome can occur sporadically, but it is often of auto- soma1 dominant inheritance. The fibrosis can occur in all the extra-ocular muscles, and is often accompanied by refractive errors which may need urgent attention. Surgical correction of the strabismus is difficult, but should be attempted. Affected children often present early in life because of an unusual head position and the obvious ptosis'. Another condition of uncertain aetiology is recurrent lateral rectus palsy in childhood. Spontaneous recovery occurs within six months in most patients, recurrent episodes affect the same side as the initial palsy, and there is no associated pain. Theories to explain the condition include trauma, viral infections and neurovascular compression by an aberrant artery. No evidence of a demyelinating disorder has been found6. It is possible that some of these
Transcript
3 70
Op h t ha I mop1 eg i a in Childhood
OPHTHALMOPLEGIA can be painless, or associated with pain usually along the distribution of the fifth cranial nerve, which can be an essential finding in establishing the cause of the condition. If the ocular palsy is of sudden onset, the child may well complain of blurred or double vision, but this is by no means always so, particularly among younger children. The child is likely to be dis- tressed and the squint may not be noted.
Painless oph t halmoplegia, associaied with generalized disorders Sometimes the cause will be obvious, for example after head injury; in this instance spontaneous resolution often occurs'. Ocular signs associated with hyper- thyroidism will be accompanied by evidence. of increased metabolism. Ophthalmoplegia in children is more benign than that in adults'. Graves disease is an autoimmune disorder, and there may be a shared antigenicity between the thyroid and orbit3. When ocular palsies and ptosis occur, the possibility of myasthenia gravis must always be con- sidered, and the pathological fatiguability may be hard to demonstrate in young children. In cases of doubt it may be
justifiable to carry out a tendon test. There are a number of different forms of congenital myasthenia, as well as classical myasthenia due to immunological damage to acetylcholine receptors. Some of these types do not respond to anticholinesterase agents, so other diagnostic tests and therapy will have to be used4.
1SOI.ATED PAINLESS OPHTHALMOPLEGIA Sometimes in childhood the muscle tissue of the external oculdr muscles can be replaced by fibrous tissue, for no apparent reason. General fibrosis syndrome can occur sporadically, but it is often of auto- soma1 dominant inheritance. The fibrosis can occur in all the extra-ocular muscles, and is often accompanied by refractive errors which may need urgent attention. Surgical correction of the strabismus is difficult, but should be attempted. Affected children often present early in life because of an unusual head position and the obvious ptosis'.
Another condition of uncertain aetiology is recurrent lateral rectus palsy in childhood. Spontaneous recovery occurs within six months in most patients, recurrent episodes affect the same side as the initial palsy, and there is no associated pain. Theories to explain the condition include trauma, viral infections and neurovascular compression by an aberrant artery. No evidence of a demyelinating disorder has been found6.
It is possible that some of these
painless ophthalmoplegias are due to viral infections or immunological reactions to such infections, but this is obviously difficult to prove. I f the child is otherwise fit and well, a period of observation is justified before considering surgical treatment.
CHRONIC PROGRESSIVE EXTERNAL 0PHTHAI.MOPLEGIA This condition may occur alone or with the involvement of other organs, as in Kearns-Sayre syndrome. These can now be included in a group of mitochondrial disorders related to mtnNA mutations'. When the ophthalmoplegia is associated with other disorders such as myopathies, rctinopathies, ataxia and heart block, the diagnosis of Kearns-Sayre syndrome is likely to be obvious, and relevant tests- including a muscle biopsy to show ragged red fibres-will be carried out.
Miller-Fisher syndrome, a variant of Guillain-Barr syndrome with external ophthalmoplegia, areflexia and ataxia without gross weakness of the limb or trunk muscles, may cause some diagnostic difficulties'. Ptosis can occur on its own, but it is usually accompanied by weakness of other ocular muscles which is often severe and asymmetrical. DANTA et aL9 suggest that chronic progressive external ophthalmoplegia represents a number of different degenerative disorders. The treatment of mitochondrial disorders is still speculative.
Painful ophthalmoplegia There are a number of conditions in which ocular palsies are associated with pain. These tend to be more common than painless ophthalmoplegia. One of the most important, even in childhood, is the presence of a cerebral aneurysm. TOLOSA, in 1954", emphasised that the clinicril diagnosis of subclinoid carotid aneurysm is based on pain occurring in the first division of the trigeminal nerve and progressive paralysis of the IIIrd, IVth, Vth and VIth cranial nerves; but the diagnosis can be confirmed only by neuroradiological studies. A similar association of symptoms and signs could be caused by a tumour, for example a lymphoma at the base of the skull.
Cavernous sinus thrombosis can cause
ophthalmoplegia, but fever and signs of infection are usually marked, and there is proptosis and orbital congestion. The condition should respond to treatment with antibiotics. In Gradenigo syndrome there is palsy of the VI th cranial nerve, associated with pain, and loss of sensation in the distribution of the Vth cranial nerve. This is usually due to petrositis secondary to middle-ear infection, but can be due to other causes such as a neoplasm, particularly a neurofibroma". It is now a very rare syndrome owing to the use of antibiotics.
ORBlTAL PSEUDOTUMOURS These can cause painful ophthalmoplegia as well as proptosis and visual impair- mentI2, usually result in displacement of the globe downwards and outwards, and can be associated with oedema and inflammation of the eyelids. However, 'pseudoturnour' is purely a descriptive term: the lesion seems to be the result of some form of non-specific inflammation; it certainly includes instances of Tolosa- Hunt syndrome when the CT scan is positive13; and in most instances i t responds to treatment with s t e r o i d ~ ' ~ . I t may well be best to discard this term which hinders, rather than helps, diagnosisI5.
TOLOSA-HUNT SYNDROME This syndrome of painful ophthalmoplegia is rare in childhood, but does occur. The name was first given to the syndrome b LAWTON SMITH and TAXDAL in 1966' . The pain is situated behind the eye, is continuous and usually gnawing or boring in nature, and can precede or follow the ophthalmoplegia. The IIIrd, IVth, Vth and VIth cranial nerves can be involved; and sometimes also the optic nerve, VIlth cranial nerve1'* l a , and the peri-arterial sympathetic fibres. Decreased visual acuity from involvement of the optic nerve may be due to its compression by granulomatous tissue at the apex of the orbit. The symptoms can last for weeks or months and remissions can occur, although occasionally there may be evidence of persistent neurological lesions. The attacks of pain and oculomotor paresis can occur over months or years, but investigations show no abnormalities outside the
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3 71
C 0 cavernous sinus and the orbit. Most clinical accounts of the syndrome
have been of adults, but a child reported
3% years when he developed right retro- orbital headache, and later a IIIrd cranial nerve palsy. The severe headaches lasted for about 10 to 15 minutes at a time, and occurred four or five times a day. A few days after the headaches started, the mother noted ptosis of the right eyelid, with external deviation of the right eye and enlargement of the right pupil. There was also a slight loss of visual acuity. Over a period of six to eight months there was almost complete resolution of the IlIrd nerve palsy, and only occasional headaches. A year later there was a recurrence of the symptoms and signs. Investigations-including angiograms and a brain scan-were normal, except for a slight increase in macronuclear cells in the CFS. The child was started on treatment with 45mg prednisone daily, and a few days later both symptoms and signs had practically disappeared. The dosage of prednisone was gradually reduced over a number bf months.
The second child was an eight-year-old girl admitted to hospital with right-sided retro- and supra-orbital pain, accompanied by a progressive lllrd nerve palsy with pupillary involvement. Investigations, in- cluding a carotid arteriogram, were normal. The symptoms and signs resolved over a period of three weeks, but recurred at the age of 13. This time the ophthal- moplegia was followed a few days later by the retro-orbital pain. There was also nambness in the distribution of the first and second divisions of the right trigeminal nerve for two days before the onset of the headache, although sensory examination was normal. Investigations with right carotid and vertebral arterio- grams showed no significant abnor- malities. The treatment with prednisone, 60mg a day, soon relieved the headache and the IIIrd nerve palsy.
To make the diagnosis of Tolosa- Hunt syndrome, two criteria in particular have to be satisfied: neurological deficits must be confined to structures in the cavernous sinus, superior orbital fissure or orbital apex; and there must be peri- or
3 72 retro-orbital pain preceding or con-
.- - - CJ
< by TERRENCE and SAMAHAI9 was aged
comirant with the onset of the ophthalmo- plegia. Diagnosis of the syndrome is usually regarded as one of exclusion, since tumours, aneurysms and other lesions can also fulfill these criteria. However, KWAN er a1.*' consider that this is not necessarily so: MRI or high-resolution CT scans of the orbital apex and cavernous sinus region can reveal a lesion in this area which, if i t resolves on treatment with steroids, is probably of a granulomatous nature. Therefore, it may be justifiable to label the condition with the eponym Tolosa- Hunt only so long as it is remembered that the cause of the lesion still must be sought. Orbital venography may confirm occlusion of the superior ophthalmic vein and partial or complete obliteration of the ipsilateral cavernous sinus. These findings are not specific for Tolosa-Hunt syn- drome, but do suggest an inflammatory lesion within the cavernous sinus and in the adjacent superior orbital fissure2'. Superior orbital fissure syndrome appears to be synonomous with Tolosa-Hunt syndrome22. When there is a space- occupying lesion in the orbit, the use of ultrasound-as well as cl'-can be helpful23.
Evidence suggests that the most likely cause of Tolosa-Hunt syndrome is a non-specific granuloma of the retro- orbital region in the cavernous sinus or superior orbital fissure, which usually responds to treatment with steroids. The steroid of choice is prednisone, which can rapidly resolve symptoms and signs. Recurrence can occur on cessation of treatment, so that several courses may be needed. If there are doubts about the diagnosis of a lesion demonstrated by radiological examination surgical investi- gation may have to be considered. Other- wise treatment can only be symptomatic.
OPHTHALMOPLEGIC MIGRAINE Ophthalmoplegic migraine can cause headache and oculomotor weakness. The pain is in or above the eye, and is usually associated with vomiting. The attacks are shorter in duration than those of Tolosa- Hunt syndrome (in which both of these symptoms have been recorded2') and the Vth cranial nerve is not involved, but complete unilateral ophthalmoplegia can occurx. The pain rarely lasts more than a
couple of days, but the ocular palsy may persist for several weeks or longer. The attacks almost always start in childhood, and sometimes the headache may be absent or overlooked.
KANDT and GOLDSTElN2’ report a six- year-old girl who had several episodes of painful ophthalmoplegia, with a good response to treatment with oral prednisone. Admitting the difficulties of differentiating Tolosa-Hunt syndrome from ophthal- moplegic migraine, they recommend that children suspected to have this syndrome should receive a trial of steroid therapy.
Proptosis and blurring of the optic disc were features of the ophthalmoplegic migraine of a child described by KUZEMKO and YOUNG^^. They con- sidered that narrowing of the internal carotid artery in the cavernous sinus due to vasoconstriction of the vessel or oedema of the arterial wall was the probable cause of this type of migraine; and that if permanent signs such as ptosis of mydriasis develop, this may result from ischaemic changes becoming irreversible.
Conclusion External ophthalmoplegia, both painless and painful, can be due to a variety of causes-some common, some rare. Associated symptoms and signs may suggest the diagnosis and the tests needed to establish it. I f the paresis occurs on its own without pain, some form of myopathy is likely, including myasthenia gravis, and the investigations for any such disorders are indicated. I t is commoner for there to be ophthalmoplegia and pain when the diagnosis is of greater urgency. Conditions such as ophthalmoplegic migraine and Tolosa-Hunt syndrome are amenable to treatment. Although space- occupying lesions can occur without pain, it is more probable that pain and paresis will occur together, and in such cases it is important to decide on the need for surgery. I f no remediable cause is found, surgical treatment of a squint may be necessary.
NEIL GORDON
Hun f ly wood, 3 Styal Road, Wilmslo w, Cheshire SK9 4AE.
References 1. Sydnor. C..F., Seaber. J. H.. Buckley, E. G .
(1982) ‘Traumatic superior oblique palsies.’ Journal of Ophthalmolo~y, 89, 134-138.
2. Uretsky. S. H., Kennerdell. J . S. . Gutai. J . P . (1980) ‘Graves’ ODhthalmoeathv in childhood and adolescence.”A rchives of Ophthalmology, 98. 1963-1964.
3. Franklyn, J. A. (1993) ‘Graves’ ophthalmopathy and the TSH receptor.’ Lancet, 342, 318-319.
4. Gordon, N. (1986) ‘Congenital myasthenia.’ Developmental Medicine and Child Neurology, 28, 810-813. (Annotation.)
5. Hiatt. R. I... Halle. A. A. (1983) ‘General fibrosis syndrome.’ Annals of Ophthalmology.
6. Afifi. A. K., Bell, W. E.. Bale, J. F., Thompson, H. S. (1990) ‘Recurrent lateral rectus palsy in childhood.’ Pediutric Neurology, 6, 3 15-3 18.
7. Poulton. J. (1993) ‘Mitochondria1 DNA and genetic disease.’ Developmental Medicine and Child Neurology, 35, 833-840. (Annotation.) .
8. Tripp, J . H. (1975) ‘,Miller-Fisher polyneuritis.’ Proceedings oj the Royul Society of Medicine.
9. Danta, G., Hilton, R. C., Lynch, P. G . (1975) ‘Chronic progressive external ophthalmoplegia.’ Brain, 98, 473492.
10. Tolosa. E. (1954) ‘Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm.’ Journal of Neurology, Neurosurgery. and Psychiatry. 17, 300-302.
1 I. Norwood, V. F., Hailer, J. S. (1989) ‘Gradenigo syndrome as presenting sign of T-cell lymphoma.’ Pediatric Neurology, 5, 377-380.
12. Abramovitz. J. N., Kasdon, D. L.. Sutula, F.. Post, K. D., Chong, F. K. (1983) ‘Sclerosing orbital pseudotumor.’ Neurosurgery, 12, 463-468.
13. Goto, Y., Goto, I.. Hosokawa,. S. (1989) ‘Neurological and radiological studies in painful ophthalmoplegia: Tolosa-Hunt syndrome and orbital pseudotumours.’ Journal of Neurology, 236, 448-45 I.
14. Flanders, A. E.. Mafee, M. F., Rao. V. M., Choi, K. H. (1989) ‘CT characteristics of orbital pseudotumors and other orbital in- flammatory processes.’ Journal of Computer Assisted Tomography, 13, 40-47.
IS. Kennerdell, J. S.. Dresner, S. C. (1984) ‘The nonspecific orbital inflammatory syndromes.’ Survey of Ophthalmology, 29,93-103.
16. Lawton Smith, J., Taxdal. D. S. R. (1966) ‘Painful ophthalmoplegia. The Tolosa-Hunt syndrome.’ American Journal of Ophthalmology, 61, 1466-1472.
17. Swerdlow, B. (1980) ‘Tolosa-Hunt syndrome; a case with associated facial nerve palsy. Annals of Neurology, 8, 542-543.
18. Vallat, J. M., Vallat, M., Julien, J., Dumas, M.. Dany, A. (1980) ‘Painful ophthalrnoplegia (Tolosa-Hunt) accompanied by peri heral facial paralysis.’ Annak of Neurology. { 645.
19. Terrence, C. F., Samaha. F. J. (1973) ‘The Tolosa-Hunt syndrome (painful ophthalm- oplegia) in children.’ Developmental Medicine and Child Neurology, 15, 506-509.
20. Kwan, E. S. K.. Wolpert, S. M., Hedges, T. R., Laucella, M. (1987) ‘Tolosa-Hunt syndrome revisited: not necessarily a diagnosis of exclusion.’ American Journal of Neuro- radiology, 8, 1067-1072.
22. Lakke, J . P. W. F. (1962) ‘Superior orbital fissure syndrome.’ Archive9 of Neurology, 7,
23. Harr, D. L.. Quencer, R. M.. Abrams. G. W. (1982) ‘Computed tomography and ultrasound in tile evalurftion of orbital infection and pseudotumor. Radiology, 142, 395-401.
24. Bickerstaff, E. R. (1964) ‘Ophthalmoplegic
289-300.
migraine.’ Revue Neurologique, 110,
25. Kandt, R. S., Goldstein, G. W. (1985) ‘Steroid- responsive ophthalmoplegia in a child.’ Archives of Neurology. 42, 589-591.
26. Kuzemko, J. A., Young, W. (1967) ‘Ophthalm- oplegic migraine: a case report.’ Develop- mental Medicine and Child Neurology, 9, 427-429.
582-588.
21st International Epilepsy Congress Sydney, 3rd to 8th September 1995
Major topics will include: genetics and molecular biology of epilepsy, surgical treatment of epilepsy in childhood, functional neuro-imaging, choice of drugs in childhood and adult epilepsy, epilepsy and the law. intellectual disabilities and epilepsy. and the role of psychiatry in epilepsy. For further information, please contact: Congress Secretariat, P.O. Box 1231, North Sydney, NSW 2059. Australia. Tel: 02 956 8333; Fax: 02 956 5154.
6th European Regional Conference of Rehabilitation International Budapest. 4th to 9th September 1994
For further details. please contact Ian Byfield, International Rehabilitation Secretariat, The Old Vicarage, Haley Hill, Halifax HX3 6DR, UK. Tel: 422 359161; Fax: 422 355604.
National Conference on Lesch-Nyhan Disease New Jersey, May 10th and 11th 1994
This conference, sponsored by Matheny School and Hospital and The Institute of Applied Research, will be held at the Headquarters Plaza Hotef in Morristown. New Jersey. Dr William Nyhan will be the featured speaker. Topics will include current research, as well as practical issues dealing with the management of Lesch-Nyhan disease. For more information, please contact: Martha Bostick. Matheny School and Hospital, Main Street, Peapack, NJ 07977. Tel: 908-234-0618.
Research in Neuroradiology; Recent Advances in Neuroradiology; Functional Neuro-imaging Marseilles. 12th and 13th July 1994
This meeting is organised by the European Association of Radiology and the University of ,Marseilles, with the help of thc French Institute of Health and Medical Research. I t will be held at the Hospital La Timone in Marseilles, and all seminars will be in English. For further information, contact Mrs M. Obadia or Mrs D. Labato, Department of Neuroradiology. HBpital de La Timone, 13005 Marseilles, France. Tel: 33 91 49 40 41/91 38 65 26; Fax: 33 91 85 83 17.
The Eighth International Conference on Biomedical Engineering: Call for Papers Singapore. 7th to 10th December 1994
The conference will focus on the following areas: ( I ) orthopaedic biomechanics, (2) biomaterials, (3) bionuid dynamics and (4) computers in medicine. Abstracts should be submitted by 1st April 1994; authors will be notified of acceptance on 1st June 1994; full papers should be submitted by 15th August 1994. Further information may be obtained from The Secretary. 8th ICBME 1994. Department of Orthopaedic Surgery, National University Hospital, Lower Kent Ridge Road, Singapore 05 1 I . Tel: 65 772 4424; Fax: 65 778 0720.
Research in Neuroradiology; Recent Advances in Neuroradiology; Functional Neuro-imaging Marseilles. 12th and 13th July 1994
This meeting is organised by the European Association of Radiology and the University of Marseilles, with the help of the French Institute of Health and Medical Research. I t will be held at the Hospital La Timone in Marseilles, and all seminars will be in English. For further information, contact Mrs M. Obadia or Mrs D. Labato, Department of Neuroradiology, Hopital de La Timone, 13005 Marseilles. France. Tel: 33 91 49 40 41/91 38 65 26; Fax: 33 91 85 83 17.
