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Late Onset Globoid Cell Leukodystrophy Patient's Late Onset (At Age 14

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Late Onset Globoid Cell Leukodystrophy Patient's Late Onset (At Age 14 980 Matters arising can not only show "some abnormality that 6 Mas JL, Henin D, Bousser MG, Chain F, Hauw 1 Hinse P, Thie A, L. Dissection of Lachenmayer J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.55.10.980-b on 1 October 1992. Downloaded from encourages angiographic examination" but JJ. Dissecting aneurysm ofthe vertebral artery the extracranial vertebral artery: report of and cervical manipulation: a case report with four cases and review of the literature. Jf can also diagnose dissections involving the autopsy. Neurology 1989;39:512-5. Neurol Neurosurg Psychiatry 199 1;54:863-9. pretransverse, C6-C5 and C5-C4 intertrans- 7 D'Anglejan-Chatillon J, Ribeiro V, Mas JL, Youl 2 Mokri B, Houser OW, Sandok BA, Piepgras verse segments of the VA.5 The diagnosis is BD, Bousser MG. Migraine-A risk factor for DG. Spontaneous dissections of the vertebral dissections of cervical arteries. Headache arteries. Neurology 1988;38:880-5. based on the association of a localised 1989;29:560-1. 3 Mas JL, Bousser MG, Hasboun D, Laplane D. increase in arterial diameter with haemody- Extracranial vertebral artery dissections: a namic signs of stenosis or occlusion and/or review of 13 cases. Stroke 1987;18:1037-47. decreased pulsatility and intravascular echoes Hinse and Thie reply: 4 Mokri B. Traumatic and spontaneous extrac- ranial internal carotid artery dissections. J at the same level. Furthermore, ultrasonic We thank Dr Mas and colleagues for their Neurol 1990;237:356-61. examination is an excellent tool for the follow interest in our recent paper,' and we appre- 5 Touboul PJ, Mas JL, Bousser MG, Laplane D. up of dissection. ciate the opportunity to comment on a few of Duplex scanning in extracranial vertebral 6) Among other diagnostic procedures, the the issues raised by them. artery dissection. Stroke 1987;18: 116-21. authors did not mention thin-section con- Our paper did not deal with the incidence trast-enhanced dynamic CT scan and MRI. of vertebral artery (VA) dissection which By virtue of its sensitivity to both blood flow remains unknown. Better diagnosis and sys- and thrombus formation, its multiplanar tematic study will hopefully shed more light Late onset globoid cell leukodystrophy imaging capability, and its noninvasiveness, on incidence of this condition in the future. MRI (and soon MR angiography) is becom- In our analysis, we have included only case I read with great interest the paper by Grewal ing the imaging modality of choice for the reports providing sufficient detail of the et al.' I would like to add a few comments. evaluation of suspected carotid or vertebral individual patient, but not summarised First, the authors suggest that their dissection (fig). At present, however, MRI series, thus the results of Mokri et al2 were patient's late onset (at age 14) distinguishes does not assist in distinguishing between not considered. We apologise for not includ- his disease from globoid cell leukodystrophy intraluminal and intramural thrombus and ing the well documented patients by Mas et (GLD) distinct from the infantile and late therefore does not allow the diagnosis of a1.3 infantile onset types. This may be true for the occlusive forms of vertebral dissection. The question of internal carotid artery first type, but the latter can occur within one 7) The relation of trauma to dissection is a (ICA) dissection was not the subject of our family together with a later onset type.2 complex issue. Hinse et al' considered their paper. We agree that angiographic visual- Second, it would be interesting to know patient 4 as an example of traumatic (chiro- isation of all four brain-supplying vessels whether the white matter hyperintensities on practic manipulation) dissection. We recently should be attempted in acuteVA dissection in the MRI were diffuse or rather restricted to reported6 the case of a woman with a 3 week order not to miss concomitant asymptomatic the occipito-parietal white matter, as des- history of cervical pain who developed iscae- ICA dissection. This point is of particular cribed in other late onset GLD.2 3 If so, this mia in the basilar artery territory following importance in spontaneous VA dissection: in posterior white matter involvement on MRI cervical manipulation. Necropsy revealed 2 5 of 29 reviewed patients concomitant ICA would seem to be very useful to distinguish VA dissections, a recent one probably due to dissection was documented, but not in 28 GLD from other cerebral white matter dis- cervical manipulation and a second one, a patients with traumatic VA dissection. eases. few weeks old, accounting for the initial Mas et al correctly state that dissection as a PETER VERDRU cervical pain. This case demonstrates that cause of arterial occlusion Neurologie, may be hard to Universitaire Ziekenhuizen, cervical pain that precedes and motivates diagnose. However, the angiographic appear- Herestraat 49, chiropractic manipulation may be the first ance of tapering occlusion is highly sugges- 3000 Leuven, Belgium symptom of a hitherto unrecognised sponta- tive of dissection.4 In our patients, complete neous (or traumatic) dissection and illus- recanalisation of a formerly occluded VA 1 Grewal RP, Petronas N, Barton NW. Late onset trates the difficulty in classifying with (cases 2 and 3) and visualisation of a small globoid cell leukodystrophy. J Neurol Neu- certainty whether dissection is spontaneous pseudoaneurysm (case 2) made the diagnosis rosurg Psychiatry 1991;54:1011-2. or traumatic. ofVA dissection probable. 2 Verdru P, Lammens M, Dom R, Van Elsen A, highly Carton H. Globoid cell leukodystrophy: a Apart from trauma and fibromuscular dys- The value of ultrasound method in the family with both late-infantile and adult type. plasia, other conditions implicated as risk diagnosis of VA dissection remains to be Neurol 1991;41:1382-4. factors for dissection include migraine, oral determined. Our own experience' and the 3 Shapiro EG, Lockman LA, Krivit W. Correla- tion of Magnetic Resonance Imaging and contraceptives, and chronic high blood pres- work of Touboul et al' on three patients Neuropsychological Abnormalities in Late- sure. In a case control study,7 we found a examined by duplex scan are too preliminary onset Leukodystrophies. Ann Neurol 1991; significant positive association of dissection to allow any firm conclusions. As we have 30:466-7. with and migraine current oral contraceptive pointed out in our paper, ultrasound meth- http://jnnp.bmj.com/ use but not with hypertension. However, the ods may be suitable for diagnosis of recanal- mechanisms leading to this association isation, possibly obviating the need for remains speculative. repeated angiography in some patients. The 8) Finally, we agree that anticoagulants are same holds true for modern neuroimaging Aseptic meningitis associated with high not harmful in extracranial VA dissection and methods. Contrast-enhanced CT scan and dose intravenous immunoglobulin may even be of benefit although no conclu- MRI may corroborate the diagnosis of VA therapy sion can be drawn from the comparison of dissection, and are also increasingly recom- nonrandomised treatment groups. mended for follow up studies. However, both We read with great interest the report by JEAN-LOUIS MAS methods do not allow on October 1, 2021 by guest. Protected copyright. Hpital Ste-Anne, examination of the Watson et al' and we would like to draw the 75674 Paris Cedex 14, affected vessels in their entire length. These authors attention to a similar case we pub- France neuroimaging techniques will have to prove lished last year.2 M G BOUSSER their value in a systematic study against the Our patient was a seven year old boy with P J TOUBOUL present "gold standard" (angiography). idiopathic thrombocytopenic purpura who Hopital Saint-Antoine, The role of many presumably predisposing had 75571 Paris Cedex 12, well-documented episodes of acute asep- France conditions, in particular migraine, use of oral tic meningitis on two occasions after the contraceptives or hypertension, remains second intravenous dose ofimmune globulin. totally obscure. It is speculative whether On these two occasions, the patient devel- 1 Hinse P, Thie A, Lachenmayer L. Dissection of some of these factors might act by merely oped aseptic meningitis on day three; quite the extracranial vertebral artery: report of facilitating the occurrence of stroke after identical to the two four cases and review of the literature. _7 patients reported by Neurol Neurosurg Psychiatry 1991;54:863-9. dissection, but not dissection itself. It is also Watson, whereas Kato's patient developed 2 Mas JL, Bousser MG, Hasboun D, Laplane D. unknown why minor trauma may induce the aseptic meningitis two days after a five Extracranial vertebral artery dissections: a cervical dissections in some patients at any day course of intravenous immune review of 13 cases. Stroke 1987;18:1037-47. globulin 3 Mokri B, Houser OW, Sandok BA, Piepgras particular time, but why recurrences in these therapy.3 DG. Spontaneous dissections of the vertebral patients are rare. In our patient the immune globulin prep- arteries. Neurology 1988;38:880-5. P HINSE aration used was Sandoglobulin IV (Sandoz), 4 Hart RG. Vertebral artery dissection. Neurology A THIE which is a formulation prepared by cold 1988;38:987-9. Department ofNeurology, 5 Touboul PJ, Mas JL, Bousser MG, Laplane D. University of Hamburg, ethanol fractionation. It was given at a dose Duplex scanning in extracranial vertebral Martinistr 52, of 0 4 g per kilogram of body weight infused artery dissection. Stroke 1987;18: 116-21. 2000 Hamburg 20, Germany over a 11 hour period. Matters arising 981 From these three reports, we conclude: during five consecutive days, a total of 370 At present I would therefore feel comfort- J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.55.10.980-b on 1 October 1992.
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