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Year : 1981  |  Volume : 29  |  Issue : 1  |  Page : 59-61

Raeder's paratrigeminal syndrome

Department of Ophthalmology, Jawaharlal Institute of Post-Graduate Medical Education & Research, Pondicherry, India

Correspondence Address:
Vasudev Anand Rao
Deptt. of Ophthalmology Jipmer, Pondichery-605006
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Source of Support: None, Conflict of Interest: None

PMID: 7287130

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How to cite this article:
Rao VA, Srinivasan R. Raeder's paratrigeminal syndrome. Indian J Ophthalmol 1981;29:59-61

How to cite this URL:
Rao VA, Srinivasan R. Raeder's paratrigeminal syndrome. Indian J Ophthalmol [serial online] 1981 [cited 2023 Dec 10];29:59-61. Available from: https://journals.lww.com/ijo/pages/default.aspx/text.asp?1981/29/1/59/30996

In 1924, Raeder[1] reported five cases of paralysis of the ocular sympathetic pathway. These cases differed from typical cases of Horner's syndrome in that his patients had no anhidrosis. The sympathetic paralysis was associated with involvement of one or more cranial nerves and Raeder felt that he could localize the lesions to an area adjacent to the trigeminal nerve. Pain and numbness in the distribution of the trigeminal nerve was present in Raeder's cases and was therefore called 'Paratrigeminal syndrome'.

The purpose of this communication is to report another case of this rare syndrome. The other features of interest in the present case are its occurrence in a female and para sellar cranial nerve involvement.

  Case report Top

A 38 year old woman was admitted with history of pain and paresthesia over the left side of the face and around the left eye, of four months duration. It was followed a month later by diplopia.

General examination revealed a moderately built lady, normotensive with no abnormal systemic findings.

Ophthalmological examination revealed ­visual acuity RE 6/6, LE 6/6; there was 3 mm ptosis on the left side [Figure - 1] ; pupillary size RE 3.5 mm, LE 2.5 mm; Hertels exophthalmo­meter reading RE 9 mm, LE 7 mm; intraocular pressure RE 17.5 mm Hg, LE 12 0 mm Hg (Schiotz). There was no heterochromia and accommodation amplitude was equal in both eyes. Corneal sensation was impaired in the left eye. There was left convergent squint of 15°. There was restriction of abduction on levo-version [Figure. 1]. The right lateral rectus was normal. Instillation of I : 1000 epinephrine in both eyes caused full dilatation of the left pupil with no action on the right. Starch­ iodine test produced equal staining on the face and forehead bilaterally. Fundus examination and visual fields revealed no abnormality. Neurological examination revealed diminished sensations on the left side of the face and forehead.

Blood examination showed Hb.12 g%, Leucocyte count 9400,/cm, ESR 14 mm; Neutrophils 65% Lymphocytes 24%, Eosinophils 11%. Blood sugar was normal and VDRL non reactive. Mantoux test was negative. X-rays chest, paranasal sinuses and skull were normal. A vertical mental view of the base of the skull revealed no pathology of the trigeminal foramen. Examination of CSF revealed no cells, protein 42 mg%, Sugar 37 ma%, Chloro­ride 114 mg%. A left carotid angiogram revealed no abnormality. The patient was given a trial of antitubercular treatment and systemic corticosteroids with no significant improve­ment.

  Discussion Top

The ocular manifestation of Raeder's syndrome is due to a lesion of the third order neuron of the ocular sympathetic pathway. A lesion{of the intracranial sympathetic pathway as occurs in this syndrome would spare the fibers that subsere sweating of the face. The site of the lesion can be distinguished by the cocaine and adrenaline tests. Instillation of 4% cocaine causes no dilatation of the pupil when the lesion is located in the second or third order neurons. This is probably because of lack of adrenaline, which is potentiated by cocaine. The dilator becomes sensitized to sympathomimetics in lesions involving the third order neuron. Hence instillation of 1 : 1000 epinephrine causes mydriasis of the affected eye.

Boniuk and Schlesinger[2] divided Raeder's syndrome into two groups. Group-I includes cases with neuralgia and parasellar cranial nerve involvement and Group II cases with neuralgia but without parasellar cranial nerve involvement. Of the 30 cases reported in the literature only five belonged to Group I. i.e. had cranial nerve involvement, other than that of the trigeminal. Our case had left abducens palsy. The presence of objective signs of trigeminal involvement in the form of diminished sensation over the face and impaired corneal sensitivity is also a rare feature of the case. In most of the earlier reports an intact corneal reflex has been found [2],[3],[4]

Another observation made in this case and also in most of the earlier reports[3],[5],[6]. was the presence of relative ocular hypotony of the affected eye. The exact mechanism of the autonomic disturbance on the intra ocular pressure is difficult to postulate.

Another unusual feature in this case is its occurrence in a female for only 3 cases have been reported in females[6],[7],[8]

This case of Raeder's syndrome is also the second of its kind to be reported from India. The earlier case[6] was also of a female belong­ing to Group I.

A variety of causes have been implicated in Raeder's syndrome-neoplasm[1] trauma[2], inflammations like chronic sinusitis, otitis media, herpes zoster, syphilitic osteitis, tuber­culosis[2],[5],[6] aneurysms of the internal carotid artery[4],[9]. In many cases no etiology was found[2],[3],[5] In our case too, no etiological factor could be found despite extensive investi­gations.

  Summary Top

A rare case of Group I, Raeder's syndrome in a female is reported. Rare features of the case are discussed.

  References Top

Raeder, J.G., 1924, Brain, 47 : 149.  Back to cited text no. 1
Boniuk, M. and Schlezinger, W.S. 1962, Amer. J. Ophthalmol. 54 : 1074.  Back to cited text no. 2
Smith, J.L., 1958, Amer. J. Ophthalmol. 46 194.  Back to cited text no. 3
Davis-Robert H. Daroff Robert. B. and Hoyt F. 1968, J. Neuro-Surg., 20 : 94.  Back to cited text no. 4
Minton, L.R. and Bounds G.W., 1964, Amer. J. Ophthalmol. 58 :271.  Back to cited text no. 5
Sood, G.C. and Rao, V.A., 1977, East. Arch. Ophthalmol 5 : 170.  Back to cited text no. 6
Klington G.H. and Smith, W.M., 1956, Neuro­logy, 6 : 750.  Back to cited text no. 7
Cohen, D.N., Zakov, Z.N., Salango, V.D. and Dohn, D.G., 1975, Amer. J. Ophthalmol. 79: 1044.  Back to cited text no. 8
Law, W.R. and Nelson, 1968, Neurology 18:43.  Back to cited text no. 9


  [Figure - 1]


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