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LETTERS TO

THE EDITOR

A single focus of multiple sclerosis in

the cervical spinal cord mimicking a

radiculopathy

A 46 year old man was first examined in January 1996 for an impairment in the finemovements of his right hand. His historyshowed an optic retrobulbar neuritis, fouryears before, with a residual decrease of visualacuity to 6/10 and a pale disc on the left.Neurological examination disclosed only adisturbed stereognosis on his right hand.Visual evoked potentials (EPs) were veryabnormal in the left, normal in the right eye.Brainstem auditory EPs and somatosensoryEPs were normal. Both MRI of the brain andof the cervical spinal cord failed to show anyaspect suggesting plaques. Cervical MRIshowed extreme degenerative changes at theC5-C6 and C6-C7 level with a C6-C7 bulg-

ing disc. A cross section at the C5-C6 levelshowed a posterior ostheophyte impressingthe subarachnoid space but no areas of abnormal signal intensity within the spinalcord. Analysis of CSF showed a protein con-centration of 310 mg/l with gammaglobulinsat 21% and oligoclonal banding. Investiga-tion of blood and CSF for infectious andimmunological diseases was negative. Thediagnosis at discharge was cervical discopathyand possible multiple sclerosis. Two monthslater the patient complained of a sharp throb-bing, burning pain in the first and second fin-ger of his lefthandandon the outer sideof hisleft arm, with an intense itching character. Atexamination scratch lesions drawn on theskin showed the C6 dermatome in its fullextension. Thebicipitalreflexwas reduced on

the left. Sensory disturbance was limited tohyperalgesia in the C6 dermatome without aclear deficit of specific sensory modalities.Spinal MRI cross section at the C5-C6 levelshowed in T2 weighted images an area of abnormally high signal intensity within thecord, localised in the left posterolateral andposterior sections and extending to thecentral region (figure). The spondylosis wasunchanged and there was no evidence of spi-nal or radicular compression.

A left pseudoradicular C6 syndrome,secondary to a fresh plaque of multiplesclerosis in the dorsal root entry zone andposterior horn was diagnosed. Treatmentwith 1000 mg methylprednisolone/day forthree days led to partial improvement.Repetition of the treatment 20 days later led

to remission of symptoms.Pain in multiple sclerosis has been knownsince 1872, when Charcot referred to shoul-der and pelvic girdle pain as symptoms of thedisease.1 However, its incidence is variouslyquoted in the literature, from “uncommon”to 82%.2 Acute pain syndromes includetrigeminal neuralgia, paroxysmal burningextremities, painful tonic seizures; chronicpain syndromes include dysaesthetic extrem-ity pain, back pain, and painful leg spasms.3

To our knowledge only two reports areexpressly dedicated to radicular pain in mul-tiple sclerosis.Ramirez-Lassepas et al ,4 duringa 15 year span, found 11 patients (3% of thenewly diagnosed cases of multiple sclerosis in

patients admitted to hospital) who presentedwith radicular pain and in whom radicularcompression was ruled out by imagingtechniques; eventually, multiple sclerosis wasdiagnosed when new neurological symptomsoccurred, and was judged to be responsiblefor the acute radicular pain. In two casesdemyelinating plaques within the spinal cordat MRI were thought to be in the appropriatelocation to explain the radicular (or moreproperly, pseudoradicular) symptoms. Uldryand Regli5 reported four patients with aformerly diagnosed multiple sclerosis whocomplained of a radicular limb pain and inwhom a relation between the location of one

of the plaques shown at MRI and limb painwas postulated. In the present case therelation is unquestionable; in a patient with apossible multiple sclerosis but with previouslynon-contributory imaging, a single demyeli-nating plaque appeared within the spinalcord, in the appropriate location to explainthe concomitant pain syndrome.

A further note of interest in this case con-cerns the physiopathology of the pain. Thedistribution of pain was strictly dermato-meric; however, intense itching, unusual inradicular pain syndromes, suggested a pecu-liar mechanism. Indeed, a plaque in the dor-sal column may generate ectopic bidirec-tional sensory discharges with abnormalcentral and antidromic conduction to periph-eral endings.6 There is evidence that antidro-

mic impulses cause the release of substance Pat the peripheral terminals of primary aV er-ents; substance P in turn has been shown tocause release of histamine from mast cells.7

Antidromic conduction and release of hista-mine could explain the uncommon feature of pain in this case.

LUIGI TOSICARLO ALBERTO RIGHETTI

Servizio di Neurologia, Ospedale di Negrar,Verona,Italy

GIAMPIETRO ZANETTEClinica Neurologica,Università di Verona, Italy

ALBERTO BELTRAMELLOIstituto di Radiologia,Università di Verona, Italy

Correspondence to: Dr Luigi Tosi,Servizio di Neu-rologia, Ospedale “Sacro Cuore”, 37024 Negrar,Verona, Italy.

1 Charcot JM. Leçons sur les maladies du systèmenerveux faites à la Salpêtrière. Paris: Delahaye,1872:239–40.

2 Kassirer MR, Osterberg DH. Pain in chronicmultiple sclerosis. J Pain Symptom Manage1987;2:95–97

3 Moulin DE, Kathleen MF, Ebers GC. Pain syn-dromes in multiple sclerosis. Neurology 1988;38:1830–34.

4 Ramirez-Lassepas M, Tulloch JW, QuinonesMR, et al . Acute raducular pain as presentingsymptom in multiple sclerosis. Arch Neurol 1992;49:255–8.

5 Uldry PA, Regly F. Syndrome pseudo-radiculaire au cours de la la sclérose en

plaques: quatre cas avec imagerie par réso-nance magnétique. Rev Neurol (Paris) 1992;148:692–5.

6 Nordin M, Nystrom B, Wallin U, et al . Ectopicsensory discharges and paresthesiae in pa-thients with disorders of peripheral nerves,dorsal roots and dorsal column. Pain 1984;20:231–45.

7 Fields H.L. Pain syndromes in neurology. 1st ed.London: Butterworths, 1990:1–18.

Production of endogenous interferon-

and in patients with multiple sclerosis

Multiple sclerosis is considered to be anautoimmune disorder aV ecting the CNS, andseveral lines of evidence have supported therole of immunological mechanisms in itspathogenesis.1 2 The clinical finding that viral

infections are often associated with or fol-lowed by acute multiple sclerosis relapses alsosuggests that an activation of the peripheralimmune compartment may contribute to anacceleration of disease progression. Interfer-ons (IFNs)—known as cytokines with antivi-ral, antiproliferative and immunomodulatoryproperties—have become part of the treat-ment of the disease as IFN-1b has beenshown to alter thenatural course of relapsing-remitting multiple sclerosis.3 The interferonsystem forms an integral part of the defencesystem against infections. In response to viralstimulation, whole blood leucocytes of healthy people mainly produce IFN- and asmall proportion of IFN-. IFN- and are

MRI examination. The T2 weighted axial slice at the C5-C6 level shows an area of increased signal intensity in the spinal cord,mainly a V ecting the left posteriolateral and posterior quadrants.

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structurally and functionally related and clas-sified as type I interferons.4 However, studieson the role of endogenous type I interferonsin the disease process of multiple sclerosis arerare and the results have been largelyinconclusive, so that theexactmechanisms bywhich IFN-1b lessens the frequency of attacks of multiple sclerosis still remain opento speculation.5

We investigated the ability of whole-bloodleucocytes of 15 patients with multiplesclerosis to produce endogenous IFN- and

in response to stimulation with Newcastledisease virus. The whole-blood assay is aneV ective method for analysing the productionof interferons and provides an appropriatemodel of the in vivo situation.6 Briefly, 100 µlheparinised blood were mixed with culturemediumat a ratio of 1:10 andstimulated withNewcastle disease virus in a final concentra-tion of 0.8 haemagglutinating units/ml. Un-stimulated assays served as controls. Concen-trations of IFN- and in the supernatants of cell cultures were determined after 48 hoursof stimulation by means of quantitativeenzyme linked immunosorbent assay(ELISA). All patients had clinically definitemultiple sclerosis of the relapsing-remittingsubtype and had been in remission for at leastthree months. None of the patients hadreceived immunosuppressive treatment orsteroids during the three months beforeblood sampling and plasma concentrations of C reactive protein were normal in all patients.As a control group, we examined 20 agematched, healthy blood donors.

We found that patients with multiplesclerosis produced significantly loweramounts of IFN- and than the controlgroup (figure). There was no spontaneousrelease of IFNs in either group. Previousstudies on cytokine production in patientswith multiple sclerosis have mainly focusedon alterations during acute attacks anddisclosed an upregulation of proinflamma-tory cytokines—for example, TNF- —andan impaired production of immunosuppres-sive cytokines—for example, TGF- —in ac-

tive disease.2 7 Our findings imply a deficit inthe ability to produce type I interferons inpatients with stable multiple sclerosis, whichmaybe a predisposingfactorfor susceptibilityto disease. The finding seems to be specific tomultiple sclerosis, as type I interferon respon-siveness has been shown to be normal inpatients with idiopathic Parkinson´s disease

and in schizophrenic patients by our labora-tory recently.8 9 The mechanisms by whichtreatment with IFN-1b exerts its positiveeV ects on the disease process might beexplained by substitution of type-I interferonin patients with multiple sclerosis. Certainly,many diV erent explanations may also befound.

KLAUS-PETER WANDINGER PETRA REISSLAND

HOLGER KIRCHNER

Institute of Immunology and Transfusion Medicine

KARL WESSEL MARIT OTTO

Department of Neurology,University of Lübeck School

of Medicine,Lübeck,Germany

Correspondence to: Dr Holger Kirchner, Instituteof Immunology and Transfusion Medicine, Univer-sity of Lübeck School of Medicine, RatzeburgerAllee 160, 23538 Lübeck, Germany. Telephone0049 451 5002840; fax 0049 451 5002857.

1 McFarland HF. The multiple sclerosis lesion. Ann Neurol 1995;37:419–20.

2 Rieckmann P, Albrecht M, Kitze B, et al . Tumornecrosis factor-alpha messenger RNA expres-sion in patients with relapsing-remitting multi-ple sclerosis is associated with disease activity. Ann Neurol 1995;37:82–8.

3 The IFNB Multiple Sclerosis Study Group andthe University of British Columbia MS/MRIAnalysis Group. Interferon beta-1b in thetreatment of multiple sclerosis: final outcomeof the randomized controlled trial. Neurology1995;45:1277–85.

4 Belardelli F, Gresser I. The neglected role of type I interferon in the T-cell response:implication for its clinical use. Immunol Today1996;17:369–72.

5 Arnason BGW, Reder AT. Interferons and mul-tiple sclerosis. Clin Neuropharmacol 1994;17:495–47.

6 Kirchner H, Kleinicke C, Digel W. A whole-blood technique for testing production of human interferon by leukocytes. J Immunol Methods 1982;48:213–9.

7 Mokhtarian F, Shi Y, Shirazian D, Morgante L,Miller A, Grob D. Defective production of anti-inflammatory cytokine, TGF- by T cell

lines of patients with active multiple sclerosis. J Immunol 1994;152:6003–10.8 Klüter H, Vieregge P, Stolze H, Kirchner H.

Defective production of interleukin-2 in pa-tients with idiopathic Parkinson s disease. J Neurol Sci 1995;133:134–9.

9 Hornberg M, Arolt V, Wilke I, Kruse A, Kirch-ner H. Production of interferons and lym-phokines in leukocyte cultures of patients withschizophrenia. Schizophr Res 1995;15:237–42.

Repeated syncopes and extended

paediatric hydrosyringomyelia/Chiari I

malformation: relation or coincidence ?

Syringomyelia is defined as a condition of tubular cavitations within the spinal cord,lined by glial tissue. In theory it diV ers fromhydromyelia, a dilatation of the central canalof the spinal cord, which is lined withependyma. However, in practice the distinc-tion between the conditions is often diYcult

to make; thus the term hydrosyringomyeliafor all intraspinal cavities of a non-tumorousnature has been proposed. Hydrosyringomy-elia is often associated with anomalies of theposterior fossa, the most common of which isChiari type I malformation. In a few cases, anassociation of hydrosyringomyelia with iso-lated syncopal events has been described.These syncopes occurred especially in adultpatients with associated Chiari type I malfor-mation andwere typically, butnot in all cases,preceded by an increase in intrathoracic pres-sure caused by coughing, sneezing, or otherexertion such as a Valsalva manoeuvre.1–3

Here, we report the case of a child withrepeated syncopes, associated withhydrosyringomyelia/Chiari I malformation.

This 10 year old boy from Zaire was

admitted for evaluation of syncope. This wasthe first episode; the syncope occurred whileat rest at school. About one minute before thesyncope, the patient experienced numbnessof the left arm, occipital headache, and dou-ble vision, but no coughing, straining, orsneezing. Pallor of mucous membranes ac-companying the syncope was not seen. Lossof consciousness lasted about five seconds,after which numbness of the arm as well asthedoublevision haddisappeared. Except forthe occipital headache, the patient then feltwell.

Thus far psychomotoric development hadbeen normal with excellent school perform-ance in primary school. Family historyshowed no cases of epilepsy or otherneurological aV ections.

On examination the child was alert andoriented and had fluent speech and intactcomprehension. General physical examin-ation was unremarkable with normal findingsespecially for the cardiovascular system. Onneurological examination his mental as wellas cranial nerve status was normal. Muscletendon reflexes, muscle tone, and strengthwere normal. Testing of the sensory systemdisclosed no abnormalities; thermal, vibra-tory, and pinprick sensations were symmet-ric. Fine and gross motor proficiency werenormal. No pyramidal signs, spasticity, orrigidity were evident.

During the follow up of 24 months, fourfurther syncopes occurred with the samesymptomatology as described for the firstepisode, but without the preceding numbness

of the left arm or headache. Again there wasno preceding coughing or sneezing. Three of these five syncopal events were preceded bymovements and mild changes of bodyposition such as bending to his schoolbag.Subsequent to all these syncopal events thepatient’s neurological status was normal.

The patient’s cardiological investigation,including repeated Schellong tests, Valsalvamanoeuvres, ECG, echocardiographies and24 hour ECGs, was normal. Under clinicalobservation changes of body position imitat-ing everyday movements (such as bending tothe floor) were repeatedly performed, but didnot lead to syncope. Several resting EEGrecords with photostimulation/hyperventila-

Secretion of endogenous IFN- and in response to viral stimulation in patients with multiplesclerosis and controls. Each open figure represents the average of duplicate determination of IFN in thesupernatants of cell cultures from (A) patients with multiple sclerosis and (B) controls. The horizontal bars represent mean IFN concentrations in each group.Values are given in Units/ml. * p<0.001; **

p<0.0001 (Mann-Whitney U test). Interferon production was stimulated by Newcastle disease virus(Kumarov strain, inactivated by UV irradiation at 366 nm for 90 minutes) in a final concentrationof 0.8 haemagglutinating units/ml. Cell cultures consisted of heparinised blood mixed at a ratio of 1:10 with RPMI 1640 culture medium supplemented with 100 U penicillin and 100 µl streptomycin/ml.

4000

3000

2000

1000

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278 Letters, Book reviews











tion as well as two sleeping EEG records and24 hour EEGrecords showed normal activity.Due to the unexplained nature of therepeated syncopes, a cerebral MRI wasperformed. This showed type I Chiarimalformation with herniated cerebellar ton-sils and compression of the foramen mag-num. A subsequent MRI of the spine showed

hydrosyringomyelia from C5 to T4, with fur-ther smaller cavitations to T7, with noevidence of cavitation above C4. No associ-ated tumour could be found (figure). Afterconsultation with our neurosurgeons wedecided against neurosurgical interventionfor the time being in view of the completelynormal neurological status of the patient. Acerebral and spinal control MRI one and twoyears later showed no progression of the syr-inx.

After diagnosing the extended hydrosyrin-gomyelia and Chiari type I malformation, thequestion arose whether there is a relationbetween this and the repeated syncopalevents. Considering the normal results of repeated cardiological investigations andEEG records (includingresting, sleeping, and

24 hour EEGs) a cardiogenic or epilep-togenic aetiology of the described syncopesseems unlikely.

There are various pathophysiological mod-els of syncopes in patients with hydrosyringo-myelia. According to the model of Williams,an increase of intrathoracic pressure caused,for example, by coughing, leads to a shift of CSF from the spinal to the cranial compart-ment. During the subsequent relaxationperiod a flow back to the spinal compartmentdue to a “sucking eV ect” may lead tohindbrain herniation with impaction of thecerebellar tonsils and to interference with themedullary baroreceptor reflex or dysfunctionof the midbrain reticular activating system,

thus leading to syncope.4 In our patient, withsyncopes occurring with no evidence of preceding symptoms such as coughing tocause consecutively cerebral or spinal pres-sure changes, this explanation seems unlikely.The fact that autonomic disturbances occureven in subjects without foramen magnumanomaly indicates that anomalies of the pos-terior fossa may be not the only factor in thepathogenesis of autonomic disturbances as-sociated with syringomyelia. In this contextNogues et al found some subclinical auto-nomic disturbances in patients with syringo-myelia, especially in those with brainsteminvolvement. However, in some patients withno signs of such involvement, a fall of morethan 2 SD in mean arterial pressure inresponse to standing was still found. There-fore for some patients the authors assume anunderlying sympathetic defect (for example,produced by destruction of the lateral hornsof grey matter), which is incomplete and notextensive enough to cause permanent orthos-tatic hypotension.5 This would suggest thatcavitation in the medulla with involvement of sympathetic structures could be another fac-tor responsible for consecutive autonomicdisturbances in patients with hydrosyringo-myelia. This defect could result in temporaryinterruption of the spinal vegetative reflex arcwith temporary postural hypotension. Con-sidering the pattern of syringomyelic areasaV ected in our patient, damage to sympa-thetic structures neighbouring the syrinx mayhave led to temporary dysfunction of thesympathetic system with subsequent syn-cope. The assumption of a merely temporaryinterruption of the spinal vegetative reflexmight explain the repeated normal results of clinical examination and Schellong tests, andthat several changes of body position did notlead to a syncopal event in our patient.Furthermore it may be that pathophysiologyof syncopes in patients with hydrosyringo-myelia/Chiari I malformations is morecomplex—for example, due to synergisticneuropsychological influences such as in-creased attention while studying at school.

Despite the theoretical models outlined,the relation between isolated syncopal eventsin patients with hydrosyringomyelia andanomalies of the posterior fossa generallyremains speculative. Furthermore the associ-ation of isolated syncopes and hydrosyringo-myelia represents a very small fraction of allpatients presenting with syncopes. Thus thedecision to perform cerebral MRI in patientswith unexplained syncopes must be consid-eredon a caseby casebasis,although our casereport shows that some patients may profit byperformance of cerebral and cervical MRI torule out hydrosyringomyelia and anomalies of the posterior fossa.

JOACHIM WOELFLEFRITZ HAVERKAMPDepartment of Pediatrics

BURKARD KREFTDepartment of Radiology, University of Bonn,

Germany

Correspondence to: Dr J Woelfle, Zentrum f Kinderheilkunde, Rhein Friedrich-Wilhelms-Universität, Adenauerallee 119, D-53113 Bonn,Germany. Telephone 0049 228 2873200; fax 0049228 2873314.

1 Hampton F, Williams B,Loizou LA. Syncope asa presenting feature of hindbrain herniationwith syringomyelia. J Neurol Neurosurg Psy chia-try 1982;45:919–22.

2 Larson SJ, Sanges A, Baker JB, Reigel DH. Her-niated cerebellar tonsils and cough syncope. J Neurosurg 1974;40:524–8.

3 AminoV MJ, Wilcox CS. Autonomic dysfunc-tion in syringomyelia. Postgrad Med J 1972;48:113–5.

4 Williams B. Simultaneous cerebral and spinalfluid pressure recordings. 2. Cerebrospinal dis-sociation with lesions at the foramen magnum. Acta Neurochir (Wien) 1981;59:123–42.

5 Nogues MA, Newman PK, Male VJ, Foster JB.Cardiovascular reflexes in syringomyelia. Brain1982;105:835–49.

Magnetic resonance imaging of acute

infarction of the anterior spinal cord

Infarction in the territory supplied by thecervical anterior spinal artery occurs infre-quently, especially in young people. Theanterior spinal artery supplies the ventral twothirds of the spinal cord and provides itsmajor blood supply. In the cervical cord, theanterior spinal artery is supplied by anteriorradicular arteries arising from the cervicalbranches of the vertebral arteries and theascending cervical arteries.1 There have beenfew reports of MRI in the first hours after thestart of an anterior spinal artery syndrome.We describe a case of an acute anterior spinalcord syndrome appearing after two sup-pressed sneezes that was studied with MRIonly four hours after the onset of symptoms.

A 37 year old previously healthy womansuddenly experienced severe anterior tho-racic pain after two consecutive suppressedsneezes. Five minutes later, the pain irradi-ated to the arms and was followed byparesthesiae and weakness in both upperlimbs. One hour later the weakness andparesthesiae had extended to the legs. Onadmission neurological examination showedtetraparesis, with a predominantly distalmotor deficit in the arms (0/5 power distaland 3/5 proximal). There was an overallreduction in power to 3/5 in the legs. Thedeep tendon reflexes were diminished in thearms andabsentin thelegs.Plantarresponseswere indiV erent.There was a loss of sensationfor pain and temperature below the T2dermatome. Light touch and vibratory andposition sensitivity were preserved. Thepatient also developed a neurogenic bladder.

With these the clinical features, an infarc-tion in the territory of the anterior spinalartery was suspected and an emergency MRIof the cervical spine was performed fourhours after the onset of symptoms. Thisinitial MRI, using fast spin echo sequences,failed to show any spinal cord signal abnor-mality (figure, A); immediately afterwards, aconventional sagittal dual echo long TR spinecho sequence showed a linear high signalintensity lesion aV ecting the anterior part of the cervical spinal cord between C3 and C7(figure, B and C). No cord swelling was iden-tified. All the findings were consistent withthe clinical diagnosis of infarction of theanterior spinal artery.

Chest radiography, ECG, and routinelaboratory examinations were normal. ChestCT performed to rule out aortic dissectionwas also normal. The patient was treated ini-tially with intravenous methylprednisolone(1 g daily for three days).

Over the next 12 hours she developed flac-cid paralysis of the lower and distal upperlimbs, with continued diminished musclepower of the deltoid and biceps muscles.Deep tendon reflexes were abolished exceptfor the biceps reflexes. The sensorial level wasunchanged. An ECG failed to disclosethrombi. Somatosensory evoked potentials inthe arms and legs showed no conductionblocks. Blood coagulation tests were normal.

Figure 1 T1 weighted spin echo midsagittal MRI (TR: 404 ms, TE: 15 ms) showing Chiari I malformation with herniated cerebellar tonsils(arrow) and spindle shaped hydrosyringomyelia

from C5 to T4 (arrow), with further smaller cavitations to T7 (arrow).

Letters, Book reviews 279











Antinuclear and antiphospholipid antibodiesanalysis were negative. Serological tests forHIV, Epstein-Barr virus, lues, Borrelia bur-dogferi, varicella zoster virus, and herpessimplex virus were negative. Four days later afollow up cervical spine MRI, including angi-ographic sequences of the vertebrobasilarsystem, was performed. Vertebral arterydissection could be ruled out, but the spinalcord showed pronounced swelling and on thelong TR conventional spin echo sequences adiV use high intensity signal covering almostthe entire diameter of the cervical spine wasidentified that spared only its posterolateralborders (figure, D). The inferior extension of the signal abnormality reached the T1 level.Spinal angiography was not performed, as itwas not considered to be clinically justified. Athird MRI performed two months latershowed an extensive area of atrophic myelo-malacia of the cervical cord between C3 andT1.

The patient made a slow clinical recovery,and four months later appreciable tetrapare-sis persisted.

Infarction of the cervical spine cord is rare,especially in young people. We think that thepatient presented with infarction of the ante-rior spinal cord because of the sudden onsetand rapid development of typical clinical fea-tures after two violent sneezes, with no poste-rior column involvement (dissociated sensoryimpairment), absence of cord compression,and exclusion of other known neurologicaldiseases, all of which point to anterior spinalartery syndrome as a result of infarction inthe territory supplied by this artery.

DiV erent causes of anterior spinal cord inf-arction have been described in youngpeople—namely, arteriovenous fistula, spinalsurgery, cardiac surgery, arteriography, fibro-catilagenous embolism, polyarteritis nodosa,and carotid or vertebral artery dissection.However, the final cause is not identified in

half of the cases.2 3 Spinal cord or brainsteminfarctions have been reported in associationwith chiropractic manipulation1 and hyperex-tension of the neck. Dissection of thevertebral artery was found in all these cases.In our patient this second diagnosis was ruledout with the combination of conventionalMRI and MR angiography. Gutowski et al 4

reported another case of cervical posteriorspinal artery infarction after sneezing. In ourpatient, the spinal cord infarction could havebeen caused by abnormal neck movements inassociation with the suppressed sneezes. Therelation of extreme flexions of the neck (vio-lent sneeze) with the abrupt onset of symptoms suggests vascular compression orobstruction of the vertebrobasilar systemwithout arterial dissection.

Most cases of spinal cord infarction areassociated with arteriovenous fistulas5 thatare not always identified on MRI, and it hasbeen suggested that spinal angiograms should

(A) Sagittal fast spin echo T2 weighted image (5000/112) performed four hours after the clinical event showing no remarkable abnormalities. (B) Sagittal conventional spin echo proton density weighted image (2200/20) performed immediately after the sequence shown in (A) clearly shows a linear high signal intensity lesion a V ecting the anterior portion of the cer vical spinal cord (arrows). (C) Sagittal conventional spin echo T2 weighted image (2200/80)confirms the high signal intensity lesion a V ecting the anterior portion of the cervical spinal cord (arrows). (D) A follow up MRI study performed four dayslater, using a conventional spin echo T2 weighted image (2200/80) shows a swollen and hyperintense cervical spinal cord.












be performed, at least in young patients,when no obvious cause is known. However, amore recent report shows a higher sensitivityof MRI in detecting this kind of malforma-tion.In thepresented case vertebralangiogra-phy was not considered clinically justified.The clinical contribution of angiography inthose cases of anterior spinal infarction withan obvious traumatic event and in which ahigh quality cervical spine MRI has ruled outspinal vascular malformations and vasculardissection, may be limited and should not beconsidered mandatory.

MRI is a sensitive modality in the evalua-tion of the spinal cord for infarction. It rulesout extradural compression, vascular malfor-mations, and space occupying lesions. ThediV erential diagnosis includes infectious orparainfectious myelitis, multiple sclerosis,and vasculitis. In inflamatory or demyelinat-ing lesions, it is well known that T2 weightedimages show lesions some time before theclinical onset, whereas with ischaemic lesionsa normal or almost normal MRI is usuallyseen in the first few hours. The sudden onsetof clinical symptoms after a rapid movementof the neck is very suggestive of infarction,but not of myelitis. In the acute phase afterinfarction the diameter of the spinal cordremains normal and diagnosis is based onsignal abnormalities on the long TR se-quences, which reflect the presence of cytotoxic oedema. However in the subacutephase, with the appearance of extensivevasogenic oedema, the high signal abnormali-ties are more evident and associated withcord swelling. In our case the anteriorlocation and the temporal MRI changes,together with the sudden onset and rapiddevelopment of typical clinical symptoms,should help in diV erentiating spine infarctionfrom other spinal cord lesions such as multi-ple sclerosis and neoplastic conditions.

We have not found any reported case of spinal cord infarction in which MRI was per-formed in the first four hours after theclinicalevent. In this acute phase only a subtle ante-rior linear hyperintensity was identified on

the T2 weighted images. This abnormalintensity was not clearly seen on the fast spinecho sequence; however, we were able toidentify it with a conventional spin echosequence. Despite the fact that fast spin echosequences have been accepted for routine usein the examination of spinal cord lesions,replacing conventional spin echo sequences,the second are more sensitive and should beused in selected cases, when obvious clinicallesions have not not been clearly shown onthe fast spin echo sequences. Fast spin echoimaging of the spine is in most ways similar toconventional spin echo imaging. However,there may be diYculties in detecting verysmall intramedullary lesions.6

A ROVIRAS PEDRAZA

Magnetic Resonance Unit

M COMABELLA J ALVAREZ

Department of Neurology

A SALGADOIntensive Care Unit , Hospital General Universitari

Vall d’Hebron, Barcelona

Dr A Rovira, Unitat de Ressonància Magnètica,Hospital General Universitari Vall d’Hebron, PsgVall D’Hebron 119–129, 08035 Barcelona, Spain.Telephone 0034 3 4286034; fax 0034 3 4286059.

1 Sherman DG, Hart RG, Easton JD. Abruptchange in head position and cerebral infarc-tion. Stroke 1981;12:2–6.

2 Foo D, Rossier AB. Anterior spinal arterysyndrome and its natural history. Paraplegia1983;21:1–10

3 Yuh WTC, Marsh EE, Wang AK, Rusell JW,Chiang F, Koci TM, et al. MR Imaging of spi-nal cord and vertebral body infarction. AJNR Am J Neuroradiol 1992;13:145–54.

4 Gutowski NJ, Murphy RP, Beale DJ. Unilateralupper cervical posterior spinal artery syndromefollowing sneezing. J Neurol Neurosurg Psychia-try 1992;55:841–3.

5 Dillon WP, Norman D, Newton TH, Bolla K,Mark A. Intradural spinal cord lesions: Gd-DTPA-enhanced MR imaging. Radiology1989;170:229–37

6 Thorpe JW, Kendall BE, MacManus DG,McDonald WI, Miller DH. Dynamicgadolinium-enhanced MRI in the detection of spinal arteriovenous malformations. Neuroradi-ology 1994;36:522–9.

Myoclonus induced by tacrine

Several adverse eV ects of tetrahydroami-noacridine, or tacrine, have been reportedsince 1986, when Summers and colleaguesdescribed that its use could lead to animprovement in cognitive defects in patientswith Alzheimer’s disease. The most commonof them are hepatic toxicity, which makesliver function monitoring necessary, andcholinergic eV ects. Convulsions have alsobeen described.1 We report on a patient with

moderate Alzheimer’s disease who presentedwith non-epileptic myoclonus during treat-ment with tacrine.

The patient was a 68 year old woman whohad been diagnosed with dementia of prob-able Alzheirmer’s type four years previously.A mini mental test examination score was 12points. She had no other disease and wastreated with clometiazole. She had no historyof epilepsy or myoclonus related to dementia.Four days before admission she had startedtreatment with 40 mg tacrine daily and 24hours later she progressively developed gen-eralised uncontrolled abnormal movements,aV ecting all her limbs and her mouth. Theseabnormal movements were non-rhythmic,with a spontaneous presentation, althoughthey could be brought on by touch and

sound. They were suggestive of myoclonusand were reduced after treatment withclonazepam. Blood analyses were normal.Brain MRI showed a moderate degree of cor-tical and subcortical atrophy and EEGshowed mild and diV use neuronal disfunc-tion with an absence of spikes. Myoclonusdisappeared 24 hours after withdrawal of tacrine. A few months later, tacrine wasrestarted to confirm the causative relation.She developed generalised myoclonus duringthe next 48 hours.

To our knowledge, non-epileptic myo-clonus may appear in 20%–30% of patientswith very advanced Alzheimer’s disease, butnon-epileptic myoclonus in association withtacrine in humans has not been previouslyreported. Tacrine is a centrally active non-

competitive reversible acetylcholinesteraseinhibitor and its action results in a prolonga-tion of cholinergic activity.2 Svejdova et al reported that non-epileptic myoclonus wasinduced in baboons (Papio papio) by7-MEOTA (7-methoxytacrine, a tacrinederivative).3 They speculated about a possibleanticholinergic eV ect of 7-MEOTA by anantagonistic action on the muscarinic acetyl-choline receptors. This eV ect has also beensuggested by other authors.

S ABILLEIRAML VIGUERA

F MIQUEL Department of Neurology,Hospital General

Universitari de la Vall d´Hebron, Barcelona, Spain

Correspondence to: Dr Sònia Abilleira, Depart-ment of Neurology, Hospital General Universitaride la Vall d’Hebrón, Pg Vall d’Hebrón 119–129,08035 Barcelona, Spain.

1 Lebert F, Hasenbroekx C, Pasquier F, et al .Convulsive eV ects of tacrine. Lancet 1996;347:1339–40.

2 Davis KL,Powchik P. Tacrine. Lancet 1995;345:625–30.

3 Svejdova M, Rektor I, Silva-Barrat C, et al .Unexpected potentializing eV ect of a tacrinederivative upon the non-epileptic-myoclonus inbaboons Papio papio. Prog Neuropsychopharma-col Biol Psychiatry 1990;6:961–66.

Primary HIV-1 infection presenting

with transient neurological deficit

An association between cerebrovascular dis-ease (causing transient neurological deficit,transient ischaemic attacks, or cerebral inf-arction) and advanced HIV disease or AIDSis recognised.1–3 There is also one report of ischaemic stroke as the first manifestation of HIV infection.4 We report a patient with pri-mary HIV-1 infection who presented with atransient neurological deficit. A previouslyhealthy 33 year old right handed male homo-sexual presented as an emergency withsudden onset of right sided weakness, dysar-

thria, and dysphasia. He had been unwell for10 days with fever, pharyngitis, malaise,myalgia, transient non-pruritic macular rashon the upper chest, and transient paraesthe-siae aV ecting the hands. On admission tohospital the neurological features were im-proving but mild weakness of the right lowerlimb and expressive dysphasia were present.The neurological deficit resolved completelywithin 24 hours. Fever (>39°C), a petechialenanthem on the hard palate, and cervicallymphadenopathy were also noted. Therewere no features of endocarditis or meningealirritation. Atypical lymphocytes were presentin a blood film with normal full blood count.The erythrocyte sedimentation rate wasraised at 45 mm/h; serum C reactive proteinconcentration was 14 mg/l (normal <10

mg/l). A chest radiograph,, echocardiogram,cerebral MRI, urinalysis, blood and urinebacterial cultures, and serological tests forsyphilis were negative. An examination of CSF showed a lymphocytic pleocytosis (70lymphocytes/mm3) and increased CSF pro-tein (1.62 g/l; normal <0.45 g/l); HIV RNAgag and pol sequences were detected in theCSF by polymerase chain reaction. Antibodystudies indicated recent HIV-1 seroconver-sion, with rising anti-HIV IgG (enzymelinked immunosorbent assay (ELISA)) andfalling anti-HIV IgM (ELISA). Serology forEpstein-Barr virus, cytomegalovirus, andtoxoplasma was negative. Serum anticardioli-pin antibodies were detected at a low concen-tration (18 GPL U/ml, normal <10 U/ml);lupus anticoagulant was not detected. Fever

and leg pains persisted for three weeks. Hewas treated with zidovudine, lamivudine,saquinavir, and low dose aspirin, and made acomplete symptomatic recovery within onemonth. No explanation other than HIVinfection was identified to account for theneurological features, which were assumed tohave an ischaemic cause. Antiviral tripletherapy was continued for six months.Themost common neurological manifestations of primary HIV infection are lymphocytic men-ingitis, reversible acute encephalitis, andperipheral mononeuritis; these have previ-ously been found to be associated with accel-erated progression of HIV disease. Focalneurological events would seem to occur












rarely during primary HIV illness but theymay be underreported; we were unable toidentify other reports of transientneurological deficit in this setting. In patientswith advanced HIV disease, transientneurological deficit, and cerebrovascularevents including cerebral infarction may beassociated with non-bacterial endocarditis,CNS infections (including Cryptococcus andtoxoplasma), and CNS tumours, but they mayalso occur in the absence of an identifiableunderlying cause.1–3 In these patients themechanism for transient neurological deficitand cerebrovascular events in HIV disease isunknown; anticardiolipin antibodies, whichmay predispose to cerebrovascular disorders,5

were detected in 70% of patients withHIV-related transient neurological deficits ina controlled study.2 In our patient, the causeof the transient neurological deficit is un-known; the possibilities include localisedischaemia caused by vascular obstruction orspasm, or a focal inflammatory lesion devel-oping at the time of seroconversion. The pre-ponderance of transient neurological deficitin late stage HIV disease and its occurrenceduring primary infection in our patientsuggest a possible association with high HIVviral load. Primary HIV infection should be

considered in at risk patients presenting withunexplained focal neurological symptoms.

T CARDC G WATHEN

Department of General Medicine

G A LUZZIDepartment of Genitourinary/HIV Medicine, South

Buckinghamshire NHS Trust,Wycombe Hospital,High Wycombe, HP11 2TT, UK

Correspondence to: Dr G A Luzzi, Department of Genitourinary/HIV Medicine, Wycombe Hospital,High Wycombe HP11 2TT, UK.

1 Engstrom JW, Lowenstein DH, Bredesen DE.Cerebral infarctions and transient neurologicaldeficits associated with acquired immunodefi-ciency syndrome. Am J Med 1989;86:528–32.

2 Brew BJ, Miller J. Human immunodeficiency

virus type 1-related transient neurological defi-cits. Am J Med 1996;101:257–61.3 Pinto AN. AIDS and cerebrovascular disease.

Stroke 1996;27:538–43.4 Naranjo IC, Santos JAT, Rodriguez MAA.

Ischemic stroke as the sole manifestation of human immunodeficiency virus infection.Stroke 1992;23:117–8.

5 Gallo P, Sivieri S, Ferrarini AM, et al . Cerebrov-ascular and neurological disorders associatedwith antiphospholipid antibodies in CSF andserum. J Neurol Sci 1994;122:97–101.

The pallidoreticular pattern of brain

damage on MRI in a patient with carbon

monoxide poisoning

Neuropathological changes of carbon mon-oxide (CO) poisoning include the pallidore-ticular pattern of brain damage1 consisting of bilateral necrosis of the globus pallidus andsubstantia nigra.Wehere report brain MRI of a CO poisoning victim that showed bilaterallesions of the globus pallidus and substantia

nigra. This is the first neuroimaging demon-stration of the pallidoreticular pattern of brain damage in a patient with CO poisoning.

A 17 year old previously healthy man wasfound unconscious in a bathroom. He wastransferred to an emergency room anddiagnosed as having CO poisoning due tofaulty ventilation. Hyperbaric oxygen therapyimproved his consiousness from coma tobeing fully alert. However, he had parkinson-ism. Severe akinesia was noted although therewas no tremor or rigidity of the limbs. Addi-tional hyperbaric oxygen therapy for sixmonths did not improve his neurologicaldeficit. Brain MRI, a year after CO exposure,disclosed bilateral pallidal lesions: the globuspallidus appeared streaked with a decreasedsignal intensity on T1 weighted images and

an increased signal intensity on T2 weightedimages (figure A). Also noted was anincreased signal intensity of the substantianigra on T2 weighted images (figure B).There was otherwise no alteration on brainMRI. A CSF examination showed a decreasein homovanillic acid concentration to 5.9ng/ml (normal range 28.83–54.28 ng/ml)with normal values of CSF protein and sugarconcentrations and normal cell counts.

Auer and Benveniste1 described the pal-lidoreticular pattern of brain damage innecropsied cases of CO poisoning. Theyspeculated that the iron rich regions could beaV ected by binding of CO to iron molecules.In our patient, bilateral pallidallesions on MRIwere consistent with necrosis because of MR signal features. A decreased concentration of

homovanillic acid in the CSF2 and an in-creased signal intensity of the substantia nigraon T2 weighted imagesseem to be due to neu-ronal loss and gliosis in the substantia nigra.

The pallidoreticular pattern of braindamage1 to our knowledge has not beenreported on MRI in patients with COpoisoning. We emphasise the possible in-volvement of the substantia nigra in some

victims of CO poisoning. In those patients,parkinsonism would be explained partly byneuronal loss in the substantia nigra in addi-tion to the bilateral pallidal necrosis.3

TORU KAWANAMIDivision of Internal Medicine, Yamagata Prefectural

Shinjo Hospital, Yamagata, Japan

TAKEO KATOKEIJI KURITA

HIDEO SASAKIThird Department of Internal Medicine, Yamagata

University School of Medicine, Yamagata, Japan

Correspondence to: Dr T Kato, Third Departmentof Internal Medicine, Yamagata University Schoolof Medicine, 2–2–2 Iida-Nishi, Yamagata 990–23,

Japan. Telephone 0081 236 28 5316; fax 0081 23628 5318.

1 Auer RN, Benveniste H. Carbon monoxide poi-soning. In: Graham DI, Lantos PL, eds.Greenfield’s neuropathology, 6th ed. London:Arnold, 1996;1:275–6.

2 Gibson CJ, Logue M, Growdon JH. CSFmonoamine metabolise levels in Alzheimer’sand Parkinson’s disease. Arch Neurol 1985;42:489–92.

3 Bucher SF, Seelos KC, Dodel RC, et al . Pallidallesions. Structural and functional magneticresonance imaging. Arch Neurol 1996;53:682–6.

Information searching for multiple

sclerosis

The acquisition of information is an impor-tant part of scientific work. In recent years,available information sources have multipliedexponentially, and as a result, we have cometo find ourselves immersed in an “infor-mation overload”. Although we feel commit-ted to keeping up to date with the advances inneurology by using our information seekingskills, paradoxically the new, widely expandedinformation technologies have made it moreand more diYcult to gain knowledge aboutrelevant literature sources and to retrieveinformation. Under the pressure of this situa-tion, many of us have resorted to the passive-

ness of searching only the most commonlyused databases such as Medline and CurrentContents to acquire the information we need.

In the frequent practice of retrieving infor-mation needed for patient assistance, it isaccepted that the use of Medline gives goodresults for European and North Americancountries. However, in searches directedtowards investigation, we suspected thatother, perhaps less accessible databases,could provide valuable additional infor-mation. To determine to what extent thismight be true, we performed a literaturesearch in the field of multiple sclerosis.

The analysis began by connecting to the179 Science and Technology databases in-cluded in the host Dialog (Knight-RidderInc), using the commands for multiple

database searching. To avoid classificationproblems due to variations in the internalstructure of the databases, we requested thatthe search term “multiple sclerosis”appear inany part of the document. As the databasescover diV erent periods and have diV erentindexing policies, we restricted the search tothe complete year of 1995 and, at the begin-ning, to the title field. However, we realisedthat using only the title would lead to poorresults (for example, 613 titles in Biosisinstead of the 1100 we used later), so we usedthe entire reference fields instead. Finally, weexcluded datatabases devoted to medicalIndustries and ones that are contained inmore general information sources (for exam-

(A) Axial T2 weighted MRI at the level of the basal ganglia. The globus pallidus shows an increased signal intensity. (B) Axial T2 weighted MRI of the midbrain. The substantia nigra shows anabonrmally high signal intensity.












ple, the New England Journal Full TextDatabase). The first search yielded 24 usabledatabases with a total of 5350 references.

It was found that most references retrieved(4615) were concentrated within the follow-ing databases: Current Contents (1239),Biosis (1100), Embase (892), Medline (739),Pascal (406 ), and IAC Health and Wellness(378). The titles of the articles recoveredwere extracted and two main points weredeveloped: duplication of references amongthe databases and the suitability of thedatabases for each information requirement.To determine suitability, the documents wereclassified according to their titles into fourgroups, representing the major headings in

McAlpines’ multiple sclerosis handbook1 : (1)epidemiology, (2) clinical aspects (signs andsymptoms, course, and prognosis, naturalhistory, neuropsychology, diagnosis, labora-tory diagnosis,and therapy),(3) pathogenesis(including genetics, immunology, and animalmodels), and (4) pathology. After carryingout the classification, we found that animportant subset of documents did notcorrespond to any of the groups; these werethen categorised into: (5) health promotion(including quality of life and social aspects),(6) general aspects (particularly review arti-cles dealing with several of theformer topics),and, (7) noise (documents with no apparentrelation with multiple sclerosis). All thedocuments retrieved were classified by mem-bers of the multiple sclerosis unit (cliniciansand basic researchers) after training sessionsto establish the classification criteria, and thefinal review was performed by a neurologistexpert in multiple sclerosis.

A high percentage of duplication of refer-ences was found in five of the databases stud-ied (average overlap 22.4%). Health andWellness showed no overlapping and, moreo-ver, indexed most of the articles on SocialAspects. Considering only Medline, Embase,and Current Contents, average overlap was26.6%.

The table shows the number of referencesretrieved and the percentage of references in

relation to the total for each aspect of multi-ple sclerosis on each database.

For our purposes, quantity was more valu-able than percentage, as the larger thenumber of references obtained the more theinformation that was gathered.

Thus according to the categories estab-lished, we found that the most suitable data-base for epidemiology, clinical aspects, andpathogenesis was Biosis, whereas the mostappropriate for health promotion was Healthand Wellness; pathology was best covered byCurrent Contents, and general aspects byEmbase. The highest rate of noise was foundin Current Contents.

These results evidence that use of Medlinealone for multiple sclerosis informationsearches will not provide optimum returns

and can have economic implications resultingfrom duplication of scientific eV orts.

However, Medline is the best known andmost available database aroundthe world andit is unusual to find other information sourcesintegrated within institutional informationsystems.

A way out of this problem is the use of On-line Search Services, a common elementof library services. They can provide access toa wide range of databases and are managedby specialised information professionals.

M JORDÀ

Unidad de Información, Agencia de Investigación yDocencia,Spain

C NOSI DURÁN

J RÍOM TINTORÉ

X MONTALBANUnitat de Neuroimmunología Clínica-Esclerosi

Múltiple, Hospitals Vall d’Hebron, Barcelona, Spain

Dr C Nos, Unitat de Neuroimmunología Clínica-Esclerosi Múltiple, Escola d’Infermeria 5ª P, Hospi-tal General Universitari Vall d’Hebron, Pg Valld’Hebron 119–129, 08035 Barcelona, Spain. Tel-ephone 0034 3 274 62 02; fax 0034 3 274 60 84;email: [email protected]

1 Matthews WB, ed. McAlpine’s multiple sclerosis,2nd ed. New York: Churchill Livingstone,

1991.

Lack of association between hepatitis G

virus and multiple sclerosis

The aetiology of multiple sclerosis is still notfully understood. Infectious agents have beenpostulated as causes of the disease for over acentury.A theory proposes that an exogenousstimulus initiates an immune responseagainst endogenous CNS proteins. Support-ing this hypothesis, some epidemiologicalstudies strongly implicate an environmentalfactor in the development of multiplesclerosis.1 Several common human viruseshave been implicated in the pathogenesis of multiple sclerosis. However, despite data

obtained from epidemiological, serological,and animal studies, no virus has beenconsistently isolated, or viral materialuniquely identified, from patients with multi-ple sclerosis.2 Hepatitis G virus (HGV), anovel potentially hepatotrophic flavilike virus,hasrecentlybeen identifiedbut littleis knownabout the relation of this virus to chronic viralhepatitis and other chronic diseases.3–4

To investigate the relation between multi-ple sclerosis and HGV, we have studied thepresence of HGV RNA, a marker of ongoinginfection, and anti-E2 HGV antibodies, amarker of exposure and recovery of infection,in serum of patients with multiple sclerosis.5

We tested serum from 99 consecutivepatients (68 females, mean age 35.2 (SD11.9) years) with definite multiple sclerosis

seen at our hospital. Fifty five patients had arelapsing-remitting, 17 a secondary progres-

sive, and 27 a primary progressive disease. Ascontrols, we included 1000 consecutive blooddonors who had tested negative for HCV,HBV, and HIV markers. HGV RNA wasdetermined by reverse transcription/polymerase chain reaction with specific prim-ers of the 5’ and NS5 regions (BoehringerMannheim) and anti-E2 antibodies weredetected from 10µl serum by µPLATEanti-HGenv (Boehringer Mannheim).

Results in patients with multiple sclerosisdid not diV er significantly from those inhealthy blood donors (table). Two patients

with multiple sclerosis had ongoing HGVinfection, normal liver tests, and were nega-tive for anti-E2 antibodies. None of thepatients with HGV exposure (RNA oranti-E2 positive) had received blood transfu-sions and were not intravenous drug users orhealthcare workers. No diV erences in age,sex, duration of disease, and clinical formswere found among patients with multiplesclerosis. Although the only two patientspositive for HGV RNA were primary pro-gressive patients, this finding must be inter-preted with caution.

In conclusion, the prevalence of HGVinfection is not higher in our population of patients with multiple sclerosis than in ourcontrols. Our results do not therefore supportany causative role for HGV in the pathogen-

esis of multiple sclerosis.S SAULEDA

Liver Unit, Department of Medicine

J RIOX MONTALBAN

E MARTINEZ-CACERESUnit of Clinical Neuroimmunology, Department of

Neurology

JI ESTEBÁN J GUARDIA

Liver Unit,Depar tment of Medicine, Hospital General,Universitari Vall d’Hebron,Barcelona, Spain

Correspondence to: Dr J Río, Unitat de Neuroim-munologia Clinica, Escola d’Enfermeria 5ª planta,Hospital Universitario Vall d’Hebron, Psg. Valld´Hebron 119–129, 08035 Barcelona, Spain. Fax0034 3 4274700; email: [email protected]

1 Kurtzke JF. Epidemiologic contributions tomultiple sclerosis: an overview. Neurology1980;30:61–79.

2 Cook SD, Rohowsky-Kochan, Bansil S, et al .Evidence for a viral etiology of multiple sclero-sis. In: Cook SD, ed. Handbook of multiple scle-rosis. New York: Marcel Dekker, 1996:97–118.

3 Linnen J, Wages J, Zhang-Keck Z, e t al .Molecular cloning and disease association of hepatitis G virus: a transfusion-transmissibleagent. Science 1996;271:505–8.

4 Tacke M, Kiyosawa K, Stark K, et al . Detectionof antibodies to a putative hepatitis G envelopeprotein. Lancet 1997;349:318–20.

5 Schlueter V, Schmolke S, Stark K, et al . Reversetranscription-PCR detection of hepatitis Gvirus. J Clin Microbiol 1996;34:2660–4.

Number (%) of references for each group and database

Biosis Embase Pascal Health and Wellness Medline

Current Contents Total

Epidemiology 52 (29.2) 31 (17.4) 19 (10.6) 4 (2.2) 36 (20.2) 36 (20.2) 178Clinical aspects 399 (22.9) 372 (21.3) 192 (11) 116 (6.6) 321 (18.4) 341 (19.5) 1741Pathogenesis 509 (31.1) 319 (19.5) 116 (7) 26 (1.5) 265 (16.2) 400 (24.4) 1635Patholo gy 35 (23.8) 26 (17.6) 17 (11.5) 2 (1.3) 31 (2. 1) 36 (24.4) 147Health promotion 20 (11.2) 24 (13.4) 11 (6.1) 82 (46) 21 (11.7) 20 (1.2) 178General aspects 16 (5.8) 23 (22.7) 8 (7.9) 19 (18.8) 15 (14.8) 20 (19.8) 101Noi se 92 (14.4) 126 (19.8) 49 (7.7) 104 (16.3) 74 (11.6) 190 (29.9) 635Total 1123 921 412 353 763 1043 4615

Number of patients and controls positive for HGV RNA and anti-E2 antibodies

GroupHGV RNA(%)

anti-E2(%)

RRMS (n=55) 0 8SPMS (n=17) 0 2PPMS (n=27) 2 8Total MS (n=99) 2(2) 18(18)Blood donors (n=1000) 19(1.9) 140(14)

RRMS=relapsing-remitting multiple sclerosis;

SPMS=secondary progressive multiple sclerosis;PPMS=primary progressive multiple sclerosis.












BOOK REVIEWS

A History of Neurosurgery. Edited bySAMUEL H GREENBLATT. (Pp 623; £72.00).Illinois:The American Association of Neurological Surgeons, 1997. ISBN

1-879284-17-0.

Reviewing this book has given me consider-able pleasure and as one who knew little of the historical development of neurosurgerythis has been a real voyage of discovery. Icannot help but admire the remarkableachievements of our predecessors which haveled to the evolution of a surgical discipline thescope and eV ectiveness of which could neverhave been contemplated even 50 years ago.

The long history of surgery of the head andbrain before the late 19th century is of someinterest, but it was really the major advancesmade in bacteriology, cerebral localisation,and anaesthesia at the end of the last centurywhich allowed the birth of neurosurgery. Theearly development of the specialty relied very

heavily on cross fertilisation of ideas fromdoctors and physiologists working in differentcentres in Europe and the United States. Theimportance and sheer excitement of the earlyscientific meetings is well described. Theheady mix of important clinical discoveriestogether with a dramatic personae of eminentand innovative people could not be repro-duced today.

In the early days it is amazing that anypatient survived an operation on their head.Picture an operating theatre in which aneurologist is directing the surgeon to lookelsewhere when the initial exposure has notuncovered the lesion. This is what Gowersdid for Horsley in 1887, computerised imageguidance—who needs it? Imagine controllingscalp haemorrhage without artery forceps,

clips, or diathermy and, although Horsleyintroduced bone wax at a relatively early date,once the surgeons entered the brain there wasno eV ective or safe means of achievinghaemostasis. They relied on the use of galvanic cautery, just a hotwire loop andbothbrain damage and reactive swelling were fre-quent complications. Attempts were made totie oV bleeding vessels in the brain with heavysilk or linen suture and the result was thatsatisfactory haemostasis was rarely achievedand operations would be abandoned as aresult of uncontrollable haemorrhage andmany patients had postoperative haemato-mas. Control of intracranial pressure duringsurgery was rudimentary to say the least.Coughing and straining associated with opendrop ether and an uncertain airway often led

to sudden deaths and there were no reliablemethods for monitoring the depth of anaes-

thesia. Many surgeons tried one or two braincases before deciding that there was little tobe gained in this field of surgery.

In this rather unpromising environment itis remarkable that Cushing announced hisintention to specialise in neurosurgery in1901 and although his name remains pre-eminent in the subject, it is perhaps WilliamMcKewan of Glasgow and Sir Victor Horsleyof Queen Square who should be recognisedas the fathers of modern neurosurgery. Cush-ing’s remarkable contribution to neurologicalsurgery was both to expand neurosurgicalknowledge and techniques and at the sametime to synthesise what knowledge wasalready available. He managed to do all thisdespite a very heavy clinical workload andwithout the benefit of modern research toolsand methods. After an address by Cushing tothe American College of Surgeons in 1919the chairman of the session Dr William Mayorose and solemnly announced “Gentlemen,we have this day witnessed the birth of a newspecialty neurological surgery.” However,Cushing was not admired by all and was inmany ways a diYcult colleague. Amongstothers Dandy thought that his approach toresearch was flawed,in that he was inclined tohave a theory and then use all of his eV orts

and ingenuity to prove the validity of it.Although this can be an eV ective approach toscientific advance it can also lead to seriouserrors. This book contains a very thoroughaccount of the historical development of thespecialty, much of it written by neurosur-geons who are able to appreciate theimportance of the individual contributionsand technical advances. The text also suc-ceeds in giving the reader a feel for the intel-lectual milieu in which these developmentstook place. The Editor, Dr Greenblatt,initially trained as a historian, but his openingchapter was disappointing. The reader shouldnot be discouraged by his rather ponderousand quasiscientific analysis of the historicaldevelopments of neurosurgery. Although hisuse of English may be oV putting, especially

to a British audience, his achievement inediting this splendid book should not gounrecognised. Overall this is an interestingand well written book and I am sure manyneurosurgeons would wish to have their ownpersonal copy. Among other reasons for buy-ing it is that the illustrations are a rich sourceof material for slides which may enliven eventhe most tedious lecture.

ROD LAING

Syr ingomyeli a and the Chiar i

Malformations. Neurosurgical Topics

Series. Edited by JOHN A ANSON, EDWARD C

BENZEL , AND ISSAM A AWAD. (Pp 202; $95).The Illinois:American Association of Neurological Surgeons, 1997. ISBN1-879284-42-1.

Syringomyelia is one of the many challengingconditions that neurosurgeons encounter andI was very pleased to be given this neurosur-gical topic from the American Association of Neurological Surgeons to review. I wasfurther pleased to see that the book has beendedicated to Bernard Williams whom I wasprivileged to know. He was kind enough toallow me to spend a day with him in his oper-ating theatre shortly before he died. He con-centrated his powerful and original intellect

on syringomyelia and made an outstandingcontribution to the understanding and man-agement of this condition. He began withsome well designed and conducted physi-ological studies on patients and then re-corded his clinical data both prospectivelyand with complete honesty. The best chapterin this volume has been written by Bernardand one needs to read no further than this togain a working understanding of the condi-tion and a pragmatic approach to its treat-ment. However,Bernard would be the first toadmit that he did not have all the answers andI enjoyed reading contributions from othereminent surgeons, many of whom have pub-lished extensively about syringomyelia. Thisbook reinforces my opinion that authors whocontribute chapters to books should have a

wide personal experience of the conditionsthat they write about, which goes far beyonda review of the literature.

There are two particularly challengingsituations which arise in the management of syringomyelia. One is patients with an associ-ated hind brain hernia who do not improveafter adequate craniovertebral decompres-sion in whom postoperative MRI showsadequate CSF at the cervicomedullary junc-tion and no hydrocephalus. Many of the con-tributors (including Bernard) advocateshunting the syrinx but it seems to me nomore logical to shunt the syrinx cavity in thissituation than to shunt it initially. There is norationale for the use of a shunt and the eV ectof shunting is unpredictable and may beassociated with deterioration. Equally, pa-

tients with an idiopathic syrinx are by nomeans uncommon and attempts to demon-strate meningeal fibrosis are often unsuccess-ful. Sadly the book has not contributed to myunderstanding of the pathophysiology of either of these problems;nor has it helped meto treat this group of patients.

Readers familiar with these neurosurgicaltopics will know that there is a list of CMEquestions at the end. These are a very usefulexercise asit is all too easy toread, and merelyremember those tracts of the text which rein-force one’s pre-existing prejudices.

Overall I thought that this was an excellentcontribution and I am sure all surgeons whotreat syringomyelia will wish to buy a copy forthemselves and all departmental librariesshould have one on their shelves.

ROD LAING












doi: 10.1136/jnnp.64.2.277

1998 64: 277J Neurol Neurosurg Psychiatry LUIGI TOSI, CARLO ALBERTO RIGHETTI, GIAMPIETRO ZANETTE, et al.

radiculopathycervical spinal cord mimicking aA single focus of multiple sclerosis in the

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