multiple sclerosis in black africans zimbabweans(ineurolneurosurgpsychiatry 1994;57:1064-1069)...

16ournal of Neurology, iNeurosurgery, and Psychiatry 1994;57:1064-1069

Multiple sclerosis in black South Africans andZimbabweans

G Dean, A I G Bhigjee, P L A Bill, V Fritz, I C Chikanza, J E P Thomas, L F Levy,D Saffer

AbstractMultiple sclerosis is rare among theindigenous black people of Africa. Thefirst account of a black patient with multi-ple sclerosis in South Africa was pub-lished as late as 1987. Since then a searchto find black patients with multiple scle-rosis in Southern Africa has continued.Seven black patients have now beentraced in South Africa and five inZimbabwe in whom a diagnosis of multi-ple sclerosis can be accepted. Six of the12 patients became blind, or nearly so,from severe optic neuritis. Multiple scle-rosis in these few black patients moreoften resembled the disorder as it occursin oriental people than among white peo-ple in southern Africa or the black peopleofNorth America or the Caribbean.

Medico-SocialResearch Board,Dublin, IrelandG DeanDepartment ofNeurology, Faculty ofMedicine, WentworthHospital, Durban,South AfricaA I G BhigjeeP LA BillMedical School,University ofWitwatersrand,Johannesburg, SouthAfricaV FritzRheumatology Unit,UMDS, London,EnglandC ChikanzaDepartment ofMedicine and ofNeurosurgery,University ofZimbabwe, Harare,ZimbabweJ E P ThomasL F LevyFaculty of Medicine,University ofWitwatersrand;Department ofNeurology,BaragwanathHospital, Soweto,South AfricaD SafferCorrespondence to:Dr Geoffrey Dean, PO Box1851, Ballsbridge,Dublin 4, Ireland.

Received 16 August 1993and in final revised form4 February 1994.Accepted 18 February 1994

(I Neurol Neurosurg Psychiatry 1994;57:1064-1069)

Multiple sclerosis is rare among the blackpopulation of Africa, although it has beenreported from some African countries includ-ing Kenya,'-3 Uganda,4 Cameroon,) Ethiopia,6and Senegal.7 Multiple sclerosis is uncommonin the coloured and Indian people of SouthAfrica.89 Although neuromyelitis optica(Devic's syndrome) has been described inSouth Africa,'101 there was no well docu-mented case of multiple sclerosis in the blackSouth African population until 1987.12 Levy,Chikanza, and others had noted a few blackpatients with multiple sclerosis in Zimbabwe(unpublished data). We therefore decided tocollect the histories and clinical findings of theblack patients we could trace in South Africaand Zimbabwe.

MethodIn South Africa, the neurologists at the mainhospitals in Johannesburg, Pretoria, Durban,Pietermaritzburg, and Capetown were askedif they knew of any black patients with multiplesclerosis. Since 1947 copies of death certifi-cates, in which multiple sclerosis has beenmentioned, had been obtained by one of us

(GD) from the South African Bureau ofStatistics. Among these death certificates,there were six black South Africans with mul-tiple sclerosis reported on the death certifi-cate. Histories have been obtained from fourof the six but, until now, it has not been possi-ble to obtain good histories for the remainingtwo. No black patients with multiple sclerosis

were known to the South African MultipleSclerosis Society.

In Zimbabwe, one of us (ICC) has col-lected information about some black patientswith multiple sclerosis and further informa-tion was obtained from the ZimbabweanMultiple Sclerosis Society and from thepatients' doctors. Some of the Zimbabweanpatients with multiple sclerosis had also beenexamined by visiting neurologists from abroadand they agreed with the diagnosis. The deathcertificates in Zimbabwe were not searchedfor deaths reported as due to multiple sclerosis.

Case historiesSOUTH AFRICACase 1(Wentworth Hospital, Natal) In 1987Bhigjee'2 reported a 46 year old Zulu manwith a seven year history of progressive weak-ness of the legs and clumsiness of the righthand, with no remission. There was no familyhistory of a similar illness or history of alcoholabuse or exposure to toxins or trauma.On examination he had a right internuclear

ophthalmoplegia and nystagmus on upwardgaze. The optic fundi and pupillary responseswere normal. The tendon reflexes were exag-gerated, the abdominal responses wereabsent, and the plantar responses were exten-sor. There was pronounced ataxia of all fourlimbs so that he was unable to walk.Oligoclonal bands were found in his CSF.The treponemal antibody absorption test waspositive in the blood but negative in the spinalfluid. Tests for cysticercosis were negative.The visual evoked response was delayed inboth eyes (P100 = 121 and 116 ms). Hishuman leucocyte antigen (HLA) groups wereA2, AW30, B7, BW42, DR2, DR3, DQ1,DQ3. MRI showed multiple high intensitylesions in a periventricular distribution. He isconsidered to have laboratory supportedprobable multiple sclerosis by the Poser classi-fication.'3 He has been lost to follow up.

Case 2(Wentworth Hospital, Natal) This 26 year oldman was first seen in 1984 with a three dayhistory of pain, numbness, and weakness inboth legs, followed by urinary retention. Onexamination at that time, his mental state wasnormal. His fundi were normal and he hadnystagmus on looking to the left. He wasdysarthric and had incoordination of bothupper limbs. All tendon reflexes were brisk,the abdominal reflexes were absent, and both

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Multiple sclerosis in black South Africans and Zimbabweans

plantar responses were extensor. There wasimpairment to pinprick and touch sensationin the lower limbs but vibration and jointposition sense were normal. The rapid plasmareagin test for syphilis was positive to a dilu-tion 1:2 but the corresponding test in the CSFwas negative. A myelogram and CT of thebrain were normal. An EEC showed occa-sional bursts of frontal intermittent rhythmicactivity. Although the evidence for neuro-syphilis was limited he was given a course ofpenicillin. He improved gradually over aperiod of five weeks and, at the time of dis-charge, was able to walk with only mild dis-ability. In 1988 he developed the rapid onsetof hoarseness, weakness of the legs and theright arm, and incontinence of urine and fae-ces. Examination at that time showed a nor-mal mental state, pallor of the left optic disc,mild weakness of abduction of the right eye,horizontal nystagmus, and asymmetric tongueand palatal movements. Tone was increasedin all four limbs. The tendon reflexes werebrisk bilaterally and both plantar responseswere extensor. Touch and pinprick sensationwere impaired up to the T12 dermatome.Joint position sense was intact but vibrationsense was impaired in the legs. There wasincoordination of the arms. A repeat myelo-gram and CT of the head were normal as werethe serum B12 and folic acid. The rapidplasma reagin test in blood was positive forsyphilis, but the test was negative in CSF.Antibodies to HTLV1 were not detected inthe blood or CSF. A diagnosis of clinicallydefinite multiple sclerosis was made and thepatient was treated with prednisone. Heimproved to the extent that he could walkbetween parallel bars. He failed to return forfollow up.

Case 3(Wentworth Hospital, Natal) This 40 year oldwoman was first seen in May 1987. She gave a10 year history of progressive difficulty inwalking, disturbance of balance, shaking ofthe right arm and right leg, and slurredspeech. There was no history of exposure totoxins. There was no family history of a similarillness. Examination showed bilateral horizon-

Figure 1 MRI ofpatient 3. There are extensive white matter lesions in the periventricularand subcortical areas.

tal nystagmus and dysarthria. Her visual acu-ity, the fundi, and cranial nerves were normal.Tone was increased in all four limbs. Power inthe arms was normal but it was decreased inthe legs. Deep tendon reflexes were brisk,more so on the right than on the left. Theabdominal reflexes were absent and bothplantar responses were extensor. Touch andpinprick sensation were normal but vibrationsense and position sense was absent in thefeet. There was bilateral cerebellar ataxia,more pronounced on the right side. A myelo-gram showed no abnormality. The CSF wasacellular and had a protein concentration of0-22 g/l. The blood rapid plasma reagin testfor syphilis was negative.

She was readmitted in May 1989 with arapid decrease in visual acuity in the left eyeand a similar, but more gradual, failure ofvision in the right eye. She also complained ofurgency and incontinence of urine. Exam-ination confirmed progression of cerebellarand pyramidal dysfunction and she also hadbilateral internuclear ophthalmoplegia. Shehad optic atrophy of the left eye and mild discpallor of the right. The visual acuity of the lefteye was reduced to an ability to count fingers,and was 6/36 in the right eye. She was con-fined to a wheelchair. A repeat CT scan of thebrain, after double dose contrast, was unre-markable except for generalised atrophy. Theright visual evoked response was delayed(P100 = 135 ms). No discernible waveformwas noticeable from the left eye. The latewaves of the brainstem auditory evokedresponses were absent on the right anddelayed on the left. The median somatosen-sory evoked response was normal on the rightand delayed on the left (N20 = 25-6 ms). TheCSF was, again, acellular but the IgG indexwas raised (1 1; normal < 0 07). The HLAprofile was A26, A28, B7, B14, DR2, DR53,and DQ1. Antibody tests for HIV andHTLV1 in the serum gave negative results.MRI (fig 1) showed extensive white matterlesions in the periventricular and subcorticalareas, as well as in the brainstem and cerebel-lum. She was considered to have clinicallydefinite multiple sclerosis. She improved withtreatment with azathioprine. In subsequentadmissions there was considerable neurologi-cal deterioration and she had repeated urinarytract infections. She died in September 1990and there was no postmortem examination.

Case 4(Wentworth Hospital, Natal) This 36 year oldman presented in 1990 with a three year his-tory of progressive weakness of both legs, theright greater than left. He also developedurgency of micturition and poor vision andincoordination of his right arm. There was nohistory of exposure to toxins or substanceabuse, nor a family history of any similarproblem. On examination his mental stateseemed normal. There was pallor of bothoptic discs. There was bilateral and rotatorynystagmus. He had spastic paraparesis, gener-alised hyper-reflexia, and a bilateral extensorplantar response. There was ataxia of both

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Figure 2 MRI ofpatient 4. There are extensive lesions-both focal and confluent.

upper limbs. Vibration sense was absent at theankle and joint sense was impaired. Hewalked with a walking stick. The CSF IgGwas 0-2 g/l. MRI (fig 2) showed extensivelesions typical of multiple sclerosis in the cere-

bral hemispheres and brain stem, both focaland confluent in nature. The right mediansomatosensory response showed an N,0latency of 24-8 ms and the left a latency of23-2 ms. Other investigations, including testsfor syphilis, HIV, and HTLV1 were negative.He was considered to have laboratory sup-ported probable multiple sclerosis and was

treated with prednisone. He did not return forfollow up.

Case 5

(Hillbrow Hospital, Transvaal) This 47 yearold woman was admitted to hospital in April1987 with rapidly diminishing vision and painin the left eye. After three days she becameblind in this eye despite treatment with pred-nisone. In August 1987 she developed painand diminished vision in the right eye whichalso became blind after a few days. In August1988 she complained of a burning pain in thelegs, which slowly resolved. In August 1989she developed weakness in her left arm and inMarch 1991 she returned to hospital withpronounced weakness in the left arm andsome weakness in the right arm. She alsocomplained of urinary and faecal incontinenceand loss of sensation in both legs.On examination in July 1991, she was blind

and confined to a wheelchair. She had no

speech defects. There was bilateral optic atro-phy and no pupillary reaction to light. There

were no involuntary muscle movements.Tone was increased in the left arm and rightleg and decreased in the right arm and left leg.Both knee jerk responses were absent and theleft ankle jerk was also absent. The triceps andbiceps reflexes were normal. Plantar responsescould not be obtained. There was diminishedsensation to touch and pinprick on the leftside. She had loss of position and vibrationsense in both legs. It was not possible to testfor ataxia. The myelogram and CT were nor-

mal. An HIV test and a Wasserman reactionwere negative. There was increased IgG in the

CSF. Tests for cysticercosis were negative.No improvement was noted throughout thepatient's stay in hospital and she remainedtetraplegic. She was diagnosed as having clini-cally definite multiple sclerosis. She died inSeptember 1991 and there was no post-mortem examination.

Case 6(Johannesburg Hospital, Transvaal) This 42year old Shangaan woman first noticed weak-ness and unsteadiness in her legs in 1989. Onexamination, in January and July 1990, therewas nystagmus on upward gaze, she could notabduct the right eye, and there was left uppermotor neuron facial palsy. She had a spastictetraparesis, most pronounced in the legs. Allher reflexes were brisk and she had bilateralankle clonus and extensor Babinski'sresponses. There was truncal ataxia with anabnormal finger to nose test. Her myelogramwas normal, and a Wasserman reaction andHIV antibody tests were negative. There wasa prolonged latency in both her visual andauditory responses. Her HLA analysis wasA30, B13, CW2, W6, DR15(11), DRW52,DQW1, and DQW3. MRI showed the classicperiventricular lesions that are seen in multi-ple sclerosis and there were also lesions of thecerebellum, brainstem, and corpus callosum.She was given methyl prednisone with noimprovement in her condition. When she wasseen again, in May and October 1991, therehad been no remission. There was left sidedoptic atrophy. She had a right internuclearophthalmoplegia. There was left upper motorneuron facial weakness and spastic tetrapare-sis, mainly affecting the lower limbs, so thatshe could not walk. Touch sensation wasdiminished distally in the lower limbs. Shehad a truncal ataxia, with abnormal finger tonose tests and bilateral dysdiadochokinesia.Oligoclonal bands were present in the CSF.She was diagnosed as having clinically definitemultiple sclerosis. She died in January 1992and there was no postmortem examination.

Case 7(Groote Schuur Hospital, Capetown, andJohannesburg Hospital, Transvaal) Thiswoman was born in 1943 in the Transkei. Hermother was a Swazi and her father a Zhosa.There was no known history of inter-racialmarriage. In 1974, at the age of 31, she devel-oped blurring of vision in the left eye thatresponded to treatment with steroids. In 1979she again had blurring of vision in the left eyeand the vision recovered once again on treat-ment with steroids. In 1981 she developedweakness in both legs, which improved ontreatment to the extent that she was ambula-tory with aid. In 1987 she had a furtherrelapse with severe weakness in both lowerlimbs that did not improve.

In April 1990, she had acute onset of spas-ticity and weakness of the right arm and wasadmitted to hospital in Johannesburg. Onexamination at that time, her mental func-tions and speech were normal. There was nonystagmus. Both lower limbs were totally

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paralysed and flaccid. There was weaknessand spasticity of both upper limbs, most pro-nounced in the right arm. She was incontinentof urine. There was loss of touch, pam, posi-tion, and vibration sense from the level of T2.It was difficult to assess coordination althoughshe had an element of intention tremor in herarms. The visually evoked response wasdelayed in both eyes. Brain MRI showedlesions scattered throughout the periventriculararea, typical of those seen in multiple sclero-sis. Her CSF was normal and Wasserman andfluorescent treponemal antibody tests werenegative. She was considered to have clinicallydefinite multiple sclerosis. She died in 1992and there was no postmortem examination.The neurology unit at Baragwanath

Hospital, Soweto, Johannesburg, has seen fivepatients during the past 20 years-two ofthem in the past five years-in whom a diag-nosis of multiple sclerosis was seriously con-sidered. The two most recent patients hadinternuclear ophthalmoplegia and unilateraloptic atrophy and one had evidence of pyra-midal tract and posterior column involvementand a delayed visual evoked response. In bothof the recent patients oligoclonal bands werepresent on analysis of CSF. All five patientshad, however, coexisting infections of the ner-vous system, in three the blood and CSFexamination showed antibodies for treponemainfection; in the fourth patient there wereboth antibodies for syphilis and HTLV1infection in the CSF and the fifth patient hadraised cysticercosis antibodies. The primaryinfections were treated with some clinicalimprovement. None of the five patientsreturned for follow up.Two other female black South Africans

were reported as dying from multiple sclerosison their death certificates, but good historiesfor these patients have not so far beenobtained.

ZIMBABWECase 8(Harare and Parirenyatwa Central Hospitals,Harare) In 1987, this 47 year old nursing sisterdeveloped weakness and loss of touch sensa-tion in her left arm. This recovered sponta-neously. In February 1988, she developedpain and stiffness in her neck, followed bynumbness of the right arm and on the rightside of the body and also, later, some weak-ness of the left arm and leg and loss of touchsensation. On examination at that time shehad nystagmus, scanning speech, dysdiado-chokinesis, and intention tremor and therewas profound proprioceptive loss in her leftarm. Improvement followed steroid treat-ment.

In July 1990, she developed weakness andnumbness of her arms and legs and was seenby a visiting neurologist, Dr D Leary, whoagreed that she had multiple sclerosis and shewas treated with methyl prednisolone infu-sion. This resulted in some improvement. InOctober 1990, she developed blurring ofvision in the right eye and pronounced opticatrophy was noted. In July 1991, she was

readmitted to hospital with a relapse of weak-ness in the left arm and leg, again sheimproved on treatment with prednisolone. Afurther relapse occurred in July 1992. She isconsidered to have clinically definite multiplesclerosis.

Case 9(Harare and Parirenyatwa Central Hospitals,Harare) In 1988, an 18 year old man devel-oped blurred vision in his right eye, numbnessin his legs and hands and, later, weakness ofthe hands. He was treated with prednisoloneand recovered. He was seen by one of us(LFL) in December 1988, who found thatvision had almost returned to normal in theright eye. At that time he was areflexic in thearms and he had poor temperature and vibra-tion sense in the lower limbs. There was slightataxia on the right finger to nose test andon alternating movements of the hand.Lhermitte's sign was positive. A CT was nor-mal and lumbar puncture showed normalprotein and a negative Pandy's test. Thepatient improved on treatment with pred-nisolone.

In December 1989, he developed spasms ofhis left hand; again he was given prednisolonewith some improvement. In June 1991 he hada further relapse with numbness in his legsand abdomen. In June 1992 he developedpain and severely impaired vision in his righteye and difficulty in starting micturition. Heimproved with prednisolone treatment. Hewas a student at the University in 1993, and,except for his visual symptoms, was reason-ably well. He is considered to have clinicallydefinite multiple sclerosis.

Case 10(Mpilo Hospital, Bulawayo) In 1977 a 22 yearold man noticed that he could not control theuse of his left arm. When he was examined atthat time by Dr I Rachman he had nystagmusand ataxia of the left upper limb and there waspallor of the left optic disc. He was admittedto hospital where he developed mild rightfacial weakness and difficulty with coughingand swallowing. Ataxia in the left armincreased and the left leg was also unsteady.He gradually improved but in 1978 he devel-oped poor vision in the left eye and was foundto have a large central scotoma. He recalledtwo previous episodes of impaired vision inthe left eye in March and September of 1977.He was considered to have multiple sclerosis.In 1978 he had an epileptic seizure and lateradmitted that he had had a previous seizure inAugust 1976. During 1988 and 1989 he wasattended by one of us (LFL). At this time hehad a loss of balance and was unable to walkwithout using a stick. He was totally blind inhis right eye and had poor vision in the lefteye. He is considered to have clinically defi-nite multiple sclerosis.

Case 11(Harare and Parirenyatwa Central Hospitals,Harare) This 29 year old woman lost thevision in her left eye in 1974 and in her right

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eye in 1975. In 1983, she developed hemi-paresis of the right side, which recovered over,one month. At that time she was noted tohave bilateral optic atrophy, worse on theright side. A cranial angiogram showed nopathology. Routine testing of her CSF wasnormal.

She was seen again in 1986, when she hadbilateral optic atrophy, weakness of both legs,extensor Babinski's responses, and oligoclonalbands in her CSF. By June 1989 she spentmost of the day in a wheelchair. She com-plained of painful flexor spasms in her legsand constipation. By June 1992 she had spastictetraplegia.By September 1992 power in her legs had

improved but she was totally blind. Her mentalacuity remains undiminished and she enjoysbooks provided by tape aids for the blind. Sheis considered to have clinically definite multiplesclerosis.

Case 12(Harare and Parirenyatwa Central Hospitals,Harare) In 1988, a 40 year old man presentedwith difficulty with balance. He also com-plained of pain in his knees and forgetfulness.In 1990, he developed almost total loss ofvision in the right eye and, six months later,blurring of vision in the left eye. By then thevision in the right eye had partially recovered.He also complained of a strange sensation inhis feet. He had no bowel or bladder symp-toms. On examination his gait was unsteadyand his speech was slurred. There was ataxiaof both arms and legs. The limb reflexes werehyperactive and the Babinski's responses wereextensor. His vision was very poor so that hecould just see his fingers. From his history andthe clinical findings, he is considered to haveclinically definite multiple sclerosis.

All of the five Zimbabwean patients withmultiple sclerosis maintain that they had nowhite ancestors. A further young black manfrom Zimbabwe attended the University ofPennsylvania as a medical student in the early1 970s and was diagnosed there as having mul-tiple sclerosis.

DiscussionOur case series is strong evidence that multi-ple sclerosis does occur in black SouthernAfricans, although there is, as yet, no post-mortem confirmation. Multiple sclerosis is,however, very rare among them. There seemsto be a gradient in Southern Africa from a rel-atively high prevalence of multiple sclerosis inwhite immigrants from Europe to a lowerprevalence in white English speaking Africanborn, a somewhat lower prevalence in whiteAfrikaans speaking African born, a low preva-lence among the coloured and Asian popula-tion (Indian and Chinese) and, lastly, a verylow multiple sclerosis prevalence among theblack population.89 14

Devic's syndrome,'5 or neuromyelitisoptica, seems to be as common among theblack population in Africa,'7'0" in compari-son with multiple sclerosis, as it is in Japan

and China.'6 17 In the present report, six of the12 black patients considered to have multiplesclerosis became blind, or nearly blind, anuncommon occurrence in multiple sclerosis inEurope or North America or among whiteSouth Africans. By contrast with the blackpatients with multiple sclerosis in Africa, theblack people of North America, or the Afro-Caribbean people who have immigrated toEngland and who have a largely black ances-try, have a similar pattern of multiple sclerosisto that which occurs among the white popula-tion.18-21

Seven black immigrants to England fromAfrica'819 are known to have been diagnosedas having multiple sclerosis, one of whombecame totally blind. One has died and therewas a postmortem examination. She was an11 year old girl when, in 1970, she developedan insidious right hemiparesis over a period oftwo months and unsteadiness of both arms.She was investigated at the National Hospital,Queen Square, by Dr Michael Kremer, whoconsidered that she had multiple sclerosis.She made a complete recovery. She had arelapse in July 1971 and again recovered. InJuly 1973, she was readmitted to the NationalHospital because of seizures.

Subsequently she developed abdominalpain, a macular skin rash, jaundice, hae-matemesis, and died. Necropsy showed mas-sive hepatic necrosis with many petechialhaemorrhages. The brain was atrophic butwas too poorly preserved for reliable histologi-cal assessment.

Multiple sclerosis is uncommon not onlyamong indigenous black people of Africa, butalso among the semitic people of NorthernAfrica and of Malta. There is a steep fall inmultiple sclerosis prevalence from Sicily,where the prevalence is around 50 to 60 per1oo 000,22 to the nearby island of Maltawhere, in a population of 350 000 people,only 13 patients with multiple sclerosis couldbe found and one man, totally blind, who hadbeen diagnosed as having Devic's syndrome.2'The pattern of multiple sclerosis prevalenceseems to change from Southern Europe,where it is relatively high, to Malta, where it islow, and to the indigenous people of Africa.Further studies of the genetic and environ-mental factors that are responsible for the dif-ference in the pattern of multiple sclerosis inAfrica compared with Europe and NorthernAmerica should shed light on the cause of thisstrange disease.

The South African Bureau of Statistics, Pretoria, providedcopies of the death certificates of the South African blackpatients in which multiple sclerosis was mentioned. Mrs LoinaRobinson and the Multiple Sclerosis Society of Zimbabweassisted us in obtaining the histories of the Zimbabwe patients.Professor Ian McDonald of the National Hospital, QueenSquare, advised us.

1 Goldstein B. Two cases of disseminated sclerosis inAfrican natives. East Afr MedJ1 1946;23:170-3.

2 Foster RM, Harries JR. Multiple sclerosis in the African.BMJ 1970;3:628.

3 Adam AM. Multiple sclerosis: epidemic in Kenya. East AfrMedJ 1989;66:503-6.

4 Kanyerezi BR, Kiire CF, Obace A. Multiple sclerosis inMulago Hospital, Uganda. East Afr Med J 1980;57:262-6.

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5 Mbonda E, Larnaout A, Maertens A, et al. Multiple sclero-sis in a black Cameroonian woman. Acta Neurol Belg1990;90:218-22.

6 Haimanot R. MS-a case report on an Ethiopian. EthiopMedJ 1985;23:27.

7 Vigneront F. La sclerose en plaques au Senegal. Nancy,France: University of Nancy, 1976 (Thesis).

8 Dean G. Disseminated sclerosis in South Africa. BMJ1949;1:842-4.

9 Dean G. Annual incidence, prevalence, and mortality ofmultiple sclerosis in white South African-born and inwhite immigrants to South Africa. BMJ 1967;2:724-30.

10 Hift W, Moodley T. A possible case of neuromyelitisoptica in a Bantu patient. SAfrMedJr 1973;47:987-8.

11 Cosnett JE. Multiple sclerosis and neuromyelitis optica. SAfrMedJ3 1981 ;60:249-51.

12 Bhigjee Al. Multiple sclerosis in a black patient. A casereport. S AfrMedJ 1987;72:873-5.

13 Poser CM, Paty DW, Scheinberg L, et al. New diagnosticcriteria for multiple sclerosis: guidelines for researchprotocols. Ann Neurol 1983;13:3:227-3 1.

14 Dean G, Elian M. Motor neuron disease and multiplesclerosis in Australia, New Zealand and South Africacompared with England and Wales. J Neurol NeurosurgPsychiatry 1993;56:633-7.

15 Devic M. Myelite subaigue compliquee de neurite optique.Le Bulletin Medical 1894;8:1033-4.

16 Kuroiwa Y, Kurland LT. Multiple sclerosis Eastand West. Proceedings of the Asian multiple sclerosis work-shop, Kyoto, 1981. Kyushu University Press, 1982.

17 Arnason BGW, Davis FA, Dean G, Kelly R, Sever JL,Waksman BH. Round the world from our correspon-dents: China-demyelinating diseases. Lancet 1982;1:734.

18 Dean G, McLoughlin H, Brady R, Adelstein AM, Tallett-Williams J. Multiple sclerosis among immigrants inGreater London. BMJ 1976;1:861-4.

19 Dean G, Brady R, McLoughlin H, Elian M, AdelsteinAM. Motor neurone disease and multiple sclerosisamong immigrants in Britain. British J7ournal ofPreventional Social Medicine 1977;31:141-7.

20 Elian M, Dean G. Multiple sclerosis among the UnitedKingdom-bom children of immigrants from the WestIndies. I Neurol Neurosurg Psychiatry 1987;50:327-32.

21 Elian M, Nightingale S, Dean G. Multiple sclerosis amongUnited Kingdom-born children of immigrants from theIndian Subcontinent, Africa, and the West Indies.J Neurol Neurosurg Psychiatry 1990;53:906-1 1.

22 Dean G, Grimaldi G, Kelly R, Karhausen L. Multiplesclerosis in southern Europe I: prevalence in Sicily in1975. JEpidemiol Community Health 1979;33:107-10.

23 Vassalo L, Eliam M, Dean G. Multiple sclerosis in southernEurope II: prevalence in Malta in 1978. J EpidemiolCommunity Health 1979;33:111-3.

Neuralgia

Neuralgia covers a multitude of disorders in literature as

well as in neurology.Not only novelists have been confused by the term

tic douloureux and whether it can be simply regarded asa synonym for trigeminal neuralgia. Early accounts oftrigeminal neuralgia stressed the occurrence of reflexmuscle spasms during the painful paroxysm; hence thealternative title for the condition. Gowers called thesemovements tic convulsif, ignoring the fact that Frenchneurologists had applied that term to cases of hemifacialspasm.Mr Weller's condition sounds in fact, more like

hemifacial spasm than trigeminal neuralgia whereasthe neuralgic pains described by Joyce, Saki, and EdithWharton are insufficiently detailed to be confident thatthey refer to trigeminal neuralgia. Dostoyevsky places a

paroxysm of pain in the temple, not a particularlycommon site for the condition, and Elizabeth Gaskell'scoupling the problem with sickness and fever suggeststhat she was dealing with a different disorder. Theresistance of the pain to even potent analgesics was

clearly known to Sheridan Le Fanu, and its severity toHerman Melville. The last word belongs to Proust.Charcot, incidentally, receives frequent mention inRemembrance of things past.

Charles Dickens, 1836-7, The Pickwick papersMr Weller communicated this secret with great glee,and winked so indefatigably after doing so, that Sambegan to think he must have got the tic douloureux inhis right eye-lid.

Herman Melville, 1851, Moby Dick"It's a kind of tic-dolly-row they say-worse nor atoothache. Well, well; I don't know what it is, but theLord keep me from catching it".

Elizabeth Gaskell, 1857, The life of Charlotte BronteMartha has suffered from tic-douloureux, with sick-ness and fever, just like you.

Sheridan Le Fanu, 1864, Uncle SilasTo this hour I cannot say what was the nature of those

periodical seizures. I have often spoken to medicalmen about them, since, but never could learn thatexcessive use of opium could altogether account forthem. It was, I believe, certain, however, that hedid use that drug -in startling quantities. It was, indeed,something of a topic of complaint with him that hisneuralgia imposed this sad necessity upon him.

F Dostoyevsky, 1871, The devilsMrs Stavrogin raised her head slightly and pressed thefingers of her right hand gingerly to her right temple,evidently feeling an acute twinge of pain there.

Edith Wharton, 1912, The reef"Oh, don't-I hate the lights!' Owen exclaimed,catching her by the wrist and pushing her back into theseat. He gave a nervous laugh and added: "I'm halfblind with neuralgia. I suppose it's this beastly rain."

Marcel Proust, 1921-2, Remembrance of things past:cities of the plainThen, when Cottard was scarcely known to the public,if you spoke toM Verdurin of his wife's facial neuralgia,"There is nothing to be done," he would say, with thenaive complacency of people who assume that anyonewhom they know must be famous, and that everybodyknows the name of their daughter's singing-teacher."If she had an ordinary doctor, one might look for asecond opinion, but when that doctor is calledCottard" (a name which he pronounced as though itwere Bouchard or Charcot) "one simply has to bow tothe inevitable."

J7ames joyce, 1922, UlyssesAn old man widower, unkempt hair, in bed, with headcovered, sighing: an infirm dog, Athos: Aconite,resorted to by increasing doses of grains and scruplesas a palliative of recrudescent neuralgia ...

Saki, The toys of peaceMiss Louise has been upstairs all the afternoon,Ma'am, reading to the second kitchenmaid, who hasthe neuralgia. G D PERKIN

Regional Neurosciences Centre,Charing Cross Hospital,London W6 8RF, UK

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