Feb 5, 1988 - dysraphic anomalies like diastematomyelia, dermal sinus and thickened filum terminale. Patients born with a lipomyelomenin- gocele are ...
546 swellings were noted in the thoracolumbar and lumbosacral regions. The upper swelling was covered by scar tissue and measured 5 cm x 3 cm. The lower swelling was covered by normal skin and measured 8 cm by 5 cm (fig). A sacral dermal sinus was seen below the lower swelling. Movements in both lower limbs were spontaneous but weak and the right lower limb moved better than the left. Anal sphincter tone was good. No other abnormality was noted. Radiographs of the chest and skull showed no abnormality. Thoracolumbar spine radiographs showed involvement of thoracic vertebrae T8-T,, and L3-S, in the lumbosacral defects. On 5 February 1988 at operation a ruptured thoracic myelomeningocele and a lumbosacral lipomyelomeningocele were found and repaired. The dermal sinus was not continuous with the spinal canal. 3 5 gm of fat was excised from the lipomyelomeningocele. The postoperative period was complicated by cerebrospinal fluid (CSF) leakage into the distal lesion which later broke down and required repeated aspiration and secondary wound closure. Movements in both lower limbs have not improved. Very few cases of more than one lesion have been observed in the same patient. Tryfonas,2 reported three spinal bifida lesions in the same patient. The patient presented in this report had a thoracic myelomeningocele and lumbosacral lipomyelomeningocele (fig). A sacral dermal sinus was also present but it did not extend into the conus medularis. Johnson3 in 1857 was the first to describe a lipomyelomeningocele as a subcutaneous lipoma usually in the lumbosacral region. The lipoma usually passes through a defect in the muscles, vertebrae and dura into a low-lying spinal cord contiguous with the fat, which may be large or finger like. According to Schut,4 who reported an incidence of 25% lipomyelomeningocele to myelomeningocele, lipomyelomeningocele far outstrip other dysraphic anomalies like diastematomyelia, dermal sinus and thickened filum terminale. Patients born with a lipomyelomeningocele are typically neurologically intact and present with a developing neurological deficit with increasing age. Many have a significant neurological deficit, by the time they attain the age of 4 years.3 Surgical repair not only aims at rectifying the cosmetic appearance but also at preventing neurological deterioration. The two spina bifida defects seen in this patient could each account for the decreased muscle power in both lower limbs. The good anal sphincteric tone without a patulous anus was noted. There was no talipes equinovarus deformity
Letters of the foot. The patient did not develop A 50 year old right handed Pakistani hydrocephalus post operatively and has not railway storeman was first seen in 1965 at the shown neurological deterioration during age of 28 years, with a long history of skull outpatient follow-up. swelling and recent onset of double vision on OP BINITIE looking to the right. The diagnosis of fibrous Department of Surgery, dysplasia was confirmed on skull University of Jos, radiographs. In 1966 he had two operations, Jos, Plateau State, in the first excess bone was removed from the Nigeria region of the left orbit with some improvement in his visual symptoms, but References when these recurred a large quantity of dysplastic bone was removed from the skull 1 Reigel DH. Spina bifida. In: Mclaurin RL. ed. vault, leaving a full thickness bone defect, Paediatric Neurosurgery. Surgery of the which was later filled with an acrylic prosDeveloping Nervous System. New York: thesis. He did not receive radiotherapy and Grune and Stratton 1982:23-47. 2 Tryfonas G. Three spina bifida defects in one appears to have been lost to follow up. The patient presented again in 1987 comchild. J Pediatr Surg 1973;8:75-6. 3 Johnson A. Fatty tumour from the sacrum of a plaining that for the past 2 years there had child connected with the spinal membranes. been skull enlargement in the region of the Trans Pathol Soc London 1857;8:16-8. left forehead and vertex, as well as episodes 4 Schut L, Bruce DA, Sutton LN. The man- of visual blurring in the left eye, but no agement of the child with lipomyelomenin- change in the longstanding diplopia. On gocele. Clin Neurosurg 1983;30:446-76. 5 Hoffman HJ, Taecholarn C, Hendrick EB, examination there was obvious left frontal Humphreys RP. Management of bossing. Although the visual accuities were lipomyelomeningoceles. Experiences at the normal, the visual fields revealed bilateral Hospital for sick children, Toronto. J constriction and the fundi showed early Neurosurg 1985;62:1-8. papilloedema. The left eye movements were limited with restricted elevation and abduction. There were no other abnormal findings. Routine biochemical indices were Accepted 4 October 1988 unremarkable, in particular serum calcium, phosphate and alkaline phosphatase levels were normal. A skull radiograph showed a large bone defect from his previous craniectomy. The frontal bones were thickened, Meningioma-an unrecognised complication particularly on the left, the left anterior of fibrous dysplasia of the skull? clinoid was expanded and sclerotic with the appearances of fibrous dysplasia. CT of the Sir: Fibrous dysplasia is a congenital, non- head showed extensive fibrous dysplasia familial, benign anomaly of bone develop- involving the frontal, parietal and sphenment occurring in single or several bones, characterised by the replacement of normal bone by fibro-osseous tissue,' with normal serum calcium, phosphate and alkaline phosphatase levels. The condition may be associated with skin pigmentation and endocrine abnormalities (Albright's syndrome).2 Approximately one-third of patients with fibrous dysplasia have involvement of the cranial or facial bones,3 complications of which include facial pain, headache and cranial nerve palsies due to bony compression, optic nerve damage being the most serious. Although rare. malignant change occurring within dysplasic bone is well recognised. Various types of sarcoma have been reported including osteosarcoma, fibrosarcoma, chondrosarcoma and neurosarcoma.4" The present case report describes the development of a meningioma in association with Fig CThead scan showing contrast fibrous dysplasia of the skull. enchancing mass beneath dysplastic bone.
Letters oidal bones as well as irregular bone destruction superiorly in the vault. There was also considerable encroachment on the left hemisphere with oedema of the white matter, compression of the left lateral ventricle and displacement of the ventricles to the right. After contrast an enhancing mass was demonstrated in the area of dysplastic bone (fig). At surgery a grey gelatinous tumour was found beneath the left parietal bone compressing the cerebral cortex, which was shown histologically to be a meningothelial meningioma infiltrating bone. There were no features of a sarcoma. The association between fibrous dysplasia and meningioma does not seem to be recognised and this may be the first report. The direct juxtaposition of the dysplastic bone and the tumour make it most likely that the link is causal. Neither previous craniectomy nor acrylic prostheses are known to be associated with meningioma and the patient had never been treated with radiotherapy. Although bone histology was not available in this case, the radiological appearances, the normal serum biochemistry and the age of
Matters arising Seasonal variations in the incidence of photoparoxysmal response Sir: The phenomenon of seasonal variability in photosensitivity of epileptic patients, reported by Danesi' is at variance with the findings from two other British centres2 and is not corroborated by our data from the Netherlands. Since 1980 we have conducted a prospective study of photosensitivity and its clinical implications in 100 photosensitive patients (Kasteleijn-Nolst Trenite, thesis in preparation) and no such consistent seasonal variability has been found. Table 1 lists the Table 1 Relation between the incidence of newly identifiedphotosensitive patients and the number of new EEG referrals per season 1980-1983
Season
Spring (Mar, Apr, May) Summer (Jun, Jul, Aug) Autumn (Sep, Oct, Nov) Winter (Dec, Jan, Feb) Total
Total Newly number of identified photosensitive new EEG patients referrals 20 20
586 492
37 23 100
609 655 2342
547 make fibrous in childhood onset late dys- References plasia the only reasonable diagnosis. The bony changes could not be explained on the I Lello GE, Sparrow DC. Craniofacial polyostotic fibrous dysplasia. J Maxillofac Surg basis of the known hyperostosis which is 1985;13:267-72. associated with meningiomas both because F, Butler AM, Hampton AO, Smith many cranial bones were involved 2 Albright PH. Syndrome characterised by fibrosa disradiologically which were distant from the seminata, areas of pigmentation and endotumour and secondly no meningioma was crine dysfunction, with precocious puberty present at his original craniectomy 22 years in females: report of five cases. N Engl J Med previously. 1937;216:727-46. The authors thank Dr G M Stern for 3 Edgerton MT, Persing JA, Jane JA. The surgical treatment of fibrous dysplasia. Ann permission to report this case. JONATHAN FRANKEL FAUSTO IANOTTI MICHAEL POWELL FREDERICK SCHON*
Department ofNeurology and Neurosurgery, The Middlesex Hospital and Department of Neurology,* Atkinson Morley's Hospital, London, UK
Surg 1985;202:459-79.
4 Dabska M, Buraczewski J. On malignant transformation in fibrous dysplasia of bone.
Oncology 1972;26:369-83. 5 Halawa M, Aziz AA. Chondrosarcoma in fibrous dysplasia of pelvis. A case report and review of the literature. J Bone Joint Surg [B] 1984;66-8:760-4.
Address for correspondence: Dr J Frankel, Department of Neurology, The Middlesex Hospital, Mortimer Street, London W1N 8AA, UK.
Accepted 12 November 1988.
number of newly identified photosensitive patients against the total number of new EEG referrals for EEG investigation of epilepsy per season. Apart from a slight autumnal excess in incidence of photosensitivity (p < 01, chi-square), there is no seasonal difference. Moreover, unlike Danesi, we quantified the degree of sensitivity by measurement of the photosensitivity range.3 From Danesi's model one might expect not only the prevalence but also the degree of photosensitivity to be greater during winter time, yet when our 100 patients are divided into two equal groups according to their photosensitivity ranges at the time of the first examination, no such seasonal effect is present (table 2). Dr Danesi's hypothesis of increased photosensitivity during the winter season is thus not supported by the findings of three other centres. On the contrary, in our
material a weak seasonal effect appears to result from patients presenting with visuallyinduced seizures during the summer who then attend for EEG investigation in the autumn. This issue is not of purely academic interest as Dr Danesi's hypothesis leads to the prediction that chronic exposure to high levels of ambient lighting may have adverse effects on people with photosensitive epilepsy.
Table 2 Relation between degree of photosensitivity and season in 100 photosensitive patients Photosensitivity range Season
++
+
Total
Spring Summer
10 9 20
10 11 17 12 50
20 20 37 23 100
Autumn Winter Total
11
50
DGA KASTELEIJN-NOLST TRENITE CD BINNIE J OOSTING W VAN EMDE BOAS
Achterweg 5, Heemstede, The Netherlands References I Danesi MA. Seasonal variations in the incidence of paroxysmal response to stimulation among photosensitive epileptic patients. J Neurol Neurosurg Psychiatry
1988;51:875-7. 2 Scott DF, Thurlong PF, Moffat AM, Harding GFA. Is sunshine protective in photosensitive epilepsy? Electroencephalogr Clin
Neurophysiol 1985;61:S216-7. 3 Jeavons PM, Harding GFA. Photosensitive Epilepsy. Heinemann, London, 1975:21 pp.
Accepted 12 September 1988
