J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.50.4.498 on 1 April 1987. Downloaded from

498 Letters This is particularly true with rapid tumour 4 lnsel TR, Kalin NH, Risch SC, Cohen RM, The exophthalmometric readings in the 13 enlargement, as may occur with haem- Murphy DL. Abducens palsy after lumar patients with oculosympathetic dysfunction orrhage into the tumour.7 Other possible puncture. N Engl J Med 1980;303:703. gave an average of 16-2 mm on the side of 5 Miller EA, Savino PJ, Schatz NJ. Bilateral causes of a sixth nerve palsy related to pitu- oculosympathetic dysfunction and 15 8 mm sixth-nerve palsy. Arch Ophihalmol correction for the itary adenoma are hydrocephalus due to 1982;100:603-4. at the normal side (after obstruction of the foramen of Monro by 6 Black PMcL, Chapman PH. Transient error of calibration). In five cases there was tumour, and a coexistent cerebral abducens paresis after shunting for hydro- no difference at all, three cases showed aneurysm.8.9 cephalus. J Neurosurg 1981 ;55:467-9. enophthalmos (0 5 mm or 1 mOmni) and five Bromocriptine, a dopamine agonist, is a 7 Goodrich I, Lee KJ. The differential diagnosis showed exophthalmos (1 mm or 2 mm). potent inhibitor of the synthesis and release ofsellar and parasellar diseases. Clinical and Four patients had Horner's syndrome for of prolactin. It may dramatically reduce the Radiographic Features Otolaryngol. Clin more than ten years, one for at least five and there have North Am 1981;14:355-78. years, the rest for two years or less. size of large prolactinomas Fukushima T, Furihata T, Sano K. been several reports of improvement of 8 Wakai S, Enophthalmos should no longer be Association of cerebral aneurysm with pitu- syndrome. In visual fields and extraocular movements itary adenoma. Surg Neurol 1979;12:503-7. regarded as a part of Horner's after its administration.'0'" The tumour 9 Mangiardi JR, Aleksic SN, Lifshitz M, Pinto his original description Horner mentioned shrinkage is most likely related to reduction R, Budzilovich GN, Pearson J. Coincidental only in passing that the position of the eye- in cell size. There is no evidence of wide- pituitary adenoma and cerebral aneurysm ball seemed slightly inward ("sehr spread tumour necrosis, vascular injury, with pathologic findings. Surg Neurol unbedeutend zuruckgesunken").3 The casu- platelet aggregation or thrombosis after 1983;19:38-41. alness of this remark contrasts with the com- treatment with bromocriptin.'011 10 Bassetti M, Spada A, Pezzo G, Giannattasio pleteness of his description of miosis and G. Bromocriptine treatment reduces the cell Our patient developed a sixth nerve palsy ptosis and with his measurements of the size in human macroprolactinomas: a mor- of the face. Later writers, how- in association with a rapid reduction in pro- phometric study. J Clin Endocrinol Metab temperature lactinoma size, including its extrasellar com- 1984;58:268-73. ever, have included enophthalmos among ponents. There was no evidence of localised 11 Barrow DL, Tndall GT, Kovacs K, Thorner the main features of the syndrome, and tumour expansion or haemorrage, hydro- MO, Horvath E, Hoffman JC. Clinical and Horner's chance remark has been per- cephalus, cerebral aneurysm, recent viral pathological effects of bromocriptine on petuated into the textbooks of the present infection or other identifiable causes for the prolactin-secreting and other pituitary day.46 Our measurements fail to show nerve simultaneous inde- tumors. J Neurosurg 1984;60:1-7. even the slightest relationship between palsy. Although Protected by copyright. pendent disease is a possibility, we consider enophthalmos and oculosympathetic dys- Accepted 15 June 1986 that sixth nerve damage was more likely to function, not even in patients with miosis have been related to rapid decompression or and ptosis of more than 10 year's standing. shift in position of the nerve caused by Similar findings have recently been reported tumour shrinkage. Hence, the appearance of by Lepore7 and Nielsen.8 a sixth nerve palsy during treatment of a HL VAN DER WIEL large prolactinoma with bromocriptine may No enophthalmos in Horner's syndrome J VAN GIJN signify either rapid tumour expansion or, Departments ofNeurology, more rarely, a sudden decrease in tumour Sir: Enophthalmos is a controversial feature University Hospital Rotterdam, size. in Homer's syndrome. Some regard it as Protestant Hospital Nij Smellinghe, mere illusion created by narrowing of the Drachten, and JOHN W DUNNE palpebral fissure.' We investigated the pres- University Hospital Utrecht, Royal Perth Hospital ence of this sign in a prospective series of The Netherlands. EDWARD G STEWART-WYNNE patients with oculosympathetic dysfunction. Royal Perth Hospital Thirteen patients with unilateral ocu- Address for correspondence: Dr HL van der Wiel, losympathetic dysfunction were examined Department of Neurology, Nij Smellinghe, Protes- PETER T PULLAN 9200 DA Sir Charles Gairdner Hospital, with a "Krahn" exophthalmometer. This tant Hospital, I Compagnonsplein,

Netherlands. http://jnnp.bmj.com/ Perth, Australia instrument makes it possible to measure the Drachten, The exact distance between the anterior surface of the cornea and the lateral margin of the Correspondence to Dr EG Stewart-Wynne, References C/- Neurology Department, Royal Perth orbit. The patients were examined while sit- to an Hospital, Perth, Western Australia, 6001. ting. The readings were "blind", owing I Walshe FB, Hoyt WF. Clinical Neuro- error of calibration, which caused an ophthalmology. 3rd ed. Baltimore: Williams artificial difference between the right and left and Wilkins, 1969:517. eye, and of which the examiner was 2 Van der Wiel HL, Van Gijn J. Homer's syn- References unaware. We excluded all intracranial or drome: Criteria for oculosympathetic intraorbital diseases that could affect the denervation. J Neurol Sci 1982;56:293-8. on September 23, 2021 by guest. I Williams PL, Warwick R, eds. Gray's Anat- position of the eyeball other than by ocu- 3 Homer F. Ueber eine Form von Ptosis. Klin omy. 36th Ed. Edinburgh: Churchill Liv- losympathetic denervation. Horner's syn- Monatsbi Augenheilk 1869;7:193-8. ingstone, 1980:1068-70. drome had been present in these patients 4 Scarlet HW. Frequency of the Claude- BR. of cranial Bernard-Homer syndrome. Report of 16 2 Rush JA, Younge Paralysis from a few months up to more than ten nerves 1II, TV, and VI. Arch Ophthalmol cases. Trans Am Ophthalmol Soc 1981 ;99:76-9. years. The diagnosis of unilateral ocu- 1928;26:266-75. 3 Nathan H, Ouaknine G, Kosary IZ. The losympathetic dysfunction was based on 5 Schafer WD, Leinwand B. Die bedeutung des abducens nerve. Anatomical variations in its previously published criteria involving serial Cocain-Adrenalin-Testes beim Homerschen course. J Neurosurg 1974;41:561-6. photography of the pupil.2 Symptomenkomplex. In: Dodt E, Schrader J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.50.4.498 on 1 April 1987. Downloaded from

Letters 499 KE, eds. Die Normale und die Gestorte constant for a certain word or sound, but Abnormal speech with normal proposi- Pupillenbewegung. Miinchen: Bergman JF, changed during conversation. Inflection was tional language can be subdivided into three 1973:194-8. radically altered. She could not sing a scale 6 Wagener HP. Enophthalmos in Homer's syn- groups according to the kind of articulatory drome. Am J Ophthalmol 1934;17:209-14. or simple songs because of the dysprosody, disorder and the accompanying neurological 7 Lepore FL. Enophthalmos and Homer's syn- but humming was normal. The patient was symptoms and signs: dysarthria, aprosodia, drome. Arch Neurol 1983;40:460. definitely concerned about her symptoms. and aphemia. In dysarthria the articulation 8 Nielsen PJ. Upside down ptosis in patients with Extensive blood examination disclosed no disorder is basically a simplification of artic- Homer's syndrome. Acta Ophthalmol abnormality. There was no serological evi- ulation with articulatory distortions.6 In the 1 983;61 :952-7. dence of viral or treponemal infection. A aprosodies the affective components of contrast enhanced CT scan of the brain on speech are impaired after right hemisphere Accepted 17 January 1986 admission was normal. Cerebrospinal fluid lesions.7 The third group, called aphemia or (CSF) contained 73 erythrocytes, 16 lym- apraxia of speech comprises articulation phocytes, and 2 monocytes per mm3. Total disorders which have caused much debate. protein was 0 35 g/l with an IgG index of Different authors have stressed aphasic, 1-58 (normal less than 0 60) and a normal dysarthric, or apraxic aspects, and named Aphemia as a first symptom of multiple albumin ratio. Electrophoresis showed oli- this group of articulation disorders accord- sclerosis goclonal bands confirming intrathecal IgG ingly.8 Basically aphemia concerns in- synthesis EEG and evoked potential studies volvement of a purely expressive aspect of Sir: Speech disorders are common in the (visual, sensory and auditory) were normal. spoken language, and as such it is difficult to course of multiple sclerosis, but aphasia is Within a few days the weakness disappeared distinguish from motor aphasia.9 Proposi- rare. I - 3 We describe a patient with and in 2 weeks there was a resolution of the tional language however is entirely normal. aphemia, that is, an articulation disorder speech disorder. A second CT scan now There has been discussion about the inter- caused by a left hemisphere lesion, as a first showed a hypodense area with central con- pretation of phonematic errors as aphasic or symptom of multiple sclerosis. trast enhancement in the frontal white apraxic.'0 The inconsistency of the articu- A 29 year old right handed female medical matter of the left hemisphere (fig a). She was latory problems is used as a distinction from student complained of speech problems and discharged in good condition and resumed dysarthria and to stress the apraxic aspect.6 of a loss of strength in the right hand, which her studies without signs of recurrent neu- A frequent concomitant finding is oral or had developed insidiously in 5 days. The rological deficit during a follow-up of one facial apraxia. " The symptoms of Protected by copyright. our speech difficulties mainly consisted of an year. After 6 months magnetic resonance patient fit in with the picture of aphemia. A altered inflection. Writing was unimpaired, imaging (MRI) showed multiple para- left-sided lesion of the posterior part of the but less skilfully performed. She had no ventricular lesions (fig b). Spontaneous inferior frontal lobe and especially the deep other symptoms and her medical history recovery, CSF findings, and lack of any white matter of the anterior limb of the was uneventful. Apart from contraceptives other explanation were suggestive of multi- internal capsule is the most consistent no medication was taken. Neurological ple sclerosis. In combination with the MRI pathological finding. The aetiology is nearly examination disclosed a minimal weakness findings 6 months later the criteria for labo- always vascular or traumatic.8 In our of the right arm and leg. Cranial nerves, ratory supported definite multiple sclerosis patient a lesion became visible on the CT coordination, sensory testing, and reflexes were fulfilled.4 I scan which extended into the area of the were normal, with both plantar responses flexor. There were no signs of bucco-facial apraxia. There was no dysphagia, increase of facial reflexes or lability of emotional expression. Her speech was monotonous with a loss of interpunction and emotional expression. Neuropsychological examina-

tion did not show any language disorder. http://jnnp.bmj.com/ Conversational speech was fluent with a normal syntax and vocabulary, without paraphasias. Comprehension of spoken and written language was excellent. Con- frontation naming, series speech, reading aloud, writing, and repetition of spoken lan- guage were entirely normal. No ideational or ideomotor apraxia was present. Vis- uospatial skills, tactile perception and on September 23, 2021 by guest. memory functions were unimpaired. There was no attentional or planning disturbance. The speech difficulty was characterised by hesitation in starting a sentence and in pro- nouncing words starting with a consonant.

She did not simplify certain phonemes, but In 0 rather gave the impression of stuttering although there was no actual repetition of Fig (a) Contrast enhanced CTscan at the level ofthe cella media, (b) axial MRIscan, 6 phonemes. The speech difficulty was not months later, at the level ofthe cella media. (TR 1400 ms; TE 32ms; TI 400ms).