Journal of Neurology, Neurosurgery, and Psychiatry, 1972, 35, 81-87

Cysticercosis of the central nervous system withamyotrophic lateral sclerosis: case report and

review of the literaturePAULINE KAHN'

Fromn the Pathology Department, Maida Vale Hospital, London

SUMMARY A case is described in which the clinical and pathological features of amyotrophiclateral sclerosis (ALS) occurred in a patient with cysticercosis of the central nervous system andassociated pachymeningitis of the cervical spinal cord. This is an extremely rare finding. The patientalso presented the two symptom complexes more commonly encountered in this condition: focaland generalized epilepsy, and signs of obstructive hydrocephalus. The literature relevant to this case,and to cysticercosis in Great Britain, is briefly reviewed. The case described adds to the concept ofmotor neurone disease as a syndrome rather than a disease entity of undetermined aetiology.

Chronic adhesive leptomeningitis of the uppercervical spinal cord may be associated withsevere muscular atrophy and spastic paraplegia,with histological evidence of degeneration ofanterior horn cells and lateral columns, andpresent with the syndrome of amyotrophiclateral sclerosis (ALS). The essential features ofALS were first described in 1869 by Charcot andJoffroy and, in one of their two cases, a hyper-trophic cervical meningitis was found at nec-ropsy.Racemose cysticercosis of long standing,

affecting the cerebral ventricular system and theposterior fossa, is frequently accompanied byfibrous adherent thickening of the basal lepto-meninges. If this process is severe and extends toinvolve the meninges investing the cervical cord,amyotrophy and spastic paresis may occur(Meyer, 1906; Redalie, 1921; Guillain, Perisson,Bertrand, and Schmite, 1927; Guillain, Bertrand,and Thurel, 1933; Lopez and Feijoo, 1936).These features may be found in combinationwith the better-known clinical manifestations ofintracerebral cysticercosis-namely, epilepsy andsymptoms of raised intracranial pressure.A clinicopathological study of a patient is

presented to illustrate these facts.

CASE REPORT

A 53 year old man, an ex-soldier, was admitted to1 Present address: Department of Neuropathology, Maudsley Hospi-tal, Denmark Hill, London S.E.5.

81

hospital in June 1966. He had been stationed inIndia (1934-1943). In 1937 he was treated for porktapeworm (Taenia solium) infestation. Seven yearslater he had a generalized fit, after which focal motorattacks in the right arm and occasional grand malattacks occurred. In 1949 a diagnosis of epilepsysecondary to cysticercosis was made and he wasinvalided out of the Army. Subsequently, thespasms in the right arm became painful, and henoticed that his right hand was clumsy. Mild weak-ness of the hand and astereognosis were found.

In February 1965 he began to have frequent severeoccipital headaches. The pain radiated to his fore-head and could be produced by moving his neck. Heexperienced bouts of vomiting, an episode of intract-able hiccough, and noticed unsteadiness on walking.In June 1966, there was ataxia of all limbs, spasticparesis of the right arm and leg, and astereognosisand apraxia in the right hand. Radiographs showedcalcified intracranial cysts and cysts in the intercostaland pelvic girdle muscles. Myodil ventriculographydemonstrated a dilated fourth ventricle with obstruc-tion to its outlets. A diagnosis of posterior fossa neo-plasm, or racemose cysticercosis was made.At exploration of the posterior fossa (Professor

Valentine Logue) a thick-walled cyst measuring 4 cmby 5 cm was encountered. This was partially re-moved, leaving the anterior portion which wasclosely adherent to the brain-stem and was seen toextend down to the Cl spinal cord segment. A freeflow of cerebrospinal fluid was established. Micro-scopical examination of the tissue showed a racemosecysticercosis cyst (Dr. M. el Batata).

In February 1967 he became confused. Recurrenceof the obstructive hydrocephalus was demonstrated

Protected by copyright.

on October 3, 2020 by guest.

http://jnnp.bmj.com

/J N

eurol Neurosurg P

sychiatry: first published as 10.1136/jnnp.35.1.81 on 1 February 1972. D

ownloaded from

82Pauline Kahn

by air ventriculography, and insertion of a Pudenzventriculocaval shunt resulted in considerable im-provement in his general and mental state. Shortlyafter this, he started to suffer from pain in hisshoulders, radiating to his hands, and associatedwith paraesthesiae in the fingers, and similar, lessintense pain and paraesthesiae of the legs. After aleft ventricular tap, contralateral hemiparesis andhemianaesthesia developed due to a capsularhaemorrhage.

Neurological examination two months latershowed nystagmus on right lateral gaze and aresidual right facial weakness. There was sym-metrical muscular wasting in the arms, mainly offorearm and hand muscles, with normal tone. Finemovement and coordination were impaired. Hecould not sit up without assistance due to weaknessof the trunk muscles. Stereognosis and two-pointdiscrimination were impaired. In the legs, spasticparaparesis with adductor spasm was severe. Hebecame cachectic and died in May 1968.

LABORATORY FINDINGS In June 1966 routine bio-chemical, haematological, and serological tests were

normal. The erythrocyte sedimentation rate (ESR)was 5 mm in one hour. Ventricular fluid contained13 mg protein/100 ml. Nine months later, the ESRwas 36 mm in one hour; ventricular fluid protein was171 mg/100 ml. with a cell count of 4,000 red cellsand 45 white cells per mm3.

PATHOLOGICAL STUDIES Post mortem examinationwas performed 24 hours after death. The body wasthat of a small emaciated man. There was broncho-pneumonia and a left pleural effusion.

CENTRAL NERVOUS SYSTEM The cerebral convexitiesappeared normal on external examination. Denseadhesions bound the cerebellum to the dura materof the posterior fossa, and a thick mass of fibroustissue was adherent to the anterior rim of the fora-men magnum and encircled the brain-stem. Theentire basal surface of the brain was covered by athick grey membrane, dense over the medial struc-tures, and this gross adhesive leptomeningitisextended from the interpeduncular cistern, over thebrain-stem, into the spinal canal. The ventriculo-caval shunt was patent. Coronal sections of the fixedbrain showed small lateral ventricles. Four calcifiedcysts, 0 3 to 0 4 cm in diameter, were found in thecerebrum. One cyst was in the left superior frontalgyrus, and three were in sulci. Microscopicalexamination of these lesions showed a centralnecrotic zone and a surrounding band ofeosinophilicacellular material containing small lamellated foci ofcalcification. A narrow band of gliosis surroundedthe frontal lesion. There was a linear necrotic zone

in the left internal capsule.The fibrous tissue on the ventral aspect of the

brain-stem incorporated the vertebral and basilararteries, and many large and small cysts with yellowgelatinous contents were applied to the lateral sur-faces of pons and medulla (Figs. 1 and 2). Thesewere seen microscopically to be composed of acentral folded mass of acellular eosinophile andSchiff-positive material surrounded by a cellularzone, composed of polymorph-leucocytes, lympho-cytes, macrophages, and very large multinucleategiant cells which were closely applied to the cyst wall.The contiguous meninges showed an intense inflam-matory cellular exudate, and a few arterioles withsevere endarteritis, although in general this was nota common feature.

Sections of cerebellum and pons showed a dilatedfourth ventricle, a granular ependymal lining, andsoftening of surrounding white matter (Fig. 1).Sections of the spinal cord showed marked fibrousthickening of the dura mater, arachnoid, and pialmembranes of the cervical segments and, to a lesserdegree, of the thoracic leptomeninges. The fibroustissue encircled emerging dorsal nerve roots (Fig. 3).Microscopically, a little collagen, derived from thethickened pial membrane encroached on the peri-pheral substance of the spinal cord. There wasconcentric fibrosis of many small pial arteries andassociated pallor of the peripheral cord substance inthe myelin preparations. The anterior spinal arteryappeared normal. The intrinsic spinal cord vesselsshowed concentric mural fibrosis. Inflammatory cellcuffing was not present. The number of anterior horncells was decreased at all levels, and was always mostseverely reduced in the medial zones. Many of theremaining cells were either pale and shrunken, orlarge and distended with lipochrome pigment. Therewere several extremely large neurones with one tothree vacuoles in the cytoplasm. Gliosis in theanterior horns was generally moderate. In myelinpreparations of cervical, thoracic, and lumbar cordsegments there was bilateral pallor of the pyramidaltracts and the ventral nerve roots in the cervicalsegments were wasted (Fig. 3).

Severe neurogenic atrophy was seen in sections ofthe small muscles of the hand, thenar, forearmmuscles, hamstrings, quadriceps femoris, and tibialisanterior muscles (Fig. 4). Calcified cysts weredemonstrated in shoulder girdle and proximal thighmuscles. Other organs examined showed no relevantchanges.

DISCUSSION

The case presented had three clinical syndromeswhich correlated with the neuropathologicalfindings. Focal and generalized epilepsy was dueto calcified encysted larvae in the cerebrum.Symptoms of raised intracranial pressure andnerve root irritation resulted from basal lepto-meningitis and obstruction of the outlet foramina

82P

rotected by copyright. on O

ctober 3, 2020 by guest.http://jnnp.bm

j.com/

J Neurol N

eurosurg Psychiatry: first published as 10.1136/jnnp.35.1.81 on 1 F

ebruary 1972. Dow

nloaded from

Cysticercosis of the central nervous system with amyotrophic lateral sclerosis

FIG. 1. Cerebellum andbrain-stem. The fourth ventricleis enlarged. The surroundingparenchyma shows necrosis.Note the grossly thickenedleptomeninges (arrow).

:

f

.1 C

V"4 t

.*ii; I

*~~~~~~~~~~~~~~~1

Of

/A

u...4

41'.,x *..:

FIG. 2. Medulla. There is a degenerate larval cyst (arrow) and an intense inflammatory reaction around it.Fibrous tissue incorporates nerve roots and vessels. Nissl x 65.

44

83

i

'w

ol.

er:

Protected by copyright.

on October 3, 2020 by guest.

http://jnnp.bmj.com

/J N

eurol Neurosurg P

sychiatry: first published as 10.1136/jnnp.35.1.81 on 1 February 1972. D

ownloaded from

Pauline Kahn

FIG. 3. Spinal cord-cervical. Marked thickening of dura mater, arachnoid, and pia mater. Myelin loss ismost marked in the direct and indirect corticospinal tracts. There is atrophy of the ventral roots (arrows).Haematoxylin-van Gieson x 8.

~~~~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ J # %

FIG. 4. Longitudinal section throughtibialis anterior muscle. There is groupatrophy of muscle fibres indicatingdenervation atrophy. H and E, x 100.

84P

rotected by copyright. on O

ctober 3, 2020 by guest.http://jnnp.bm

j.com/

J Neurol N

eurosurg Psychiatry: first published as 10.1136/jnnp.35.1.81 on 1 F

ebruary 1972. Dow

nloaded from

Cysticercosis of the central nervous system with amyotrophic lateral sclerosis

of the fourth ventricle. Finally, there wasamyotrophic lateral sclerosis, secondary topachymeningitis of the cervical spinal cord.

Meningeal fibrosis, accompanying the race-mose form of cysticercosis, is frequently men-

tioned in the literature, particularly from SouthAmerica and Poland (Lopez and Feijoo, 1936;Stepien and Chorobski, 1949; Arana andAsenjo, 1945; Stepien, 1962). Some reports ofspinal cord involvement deal with the rare findingof an intramedullary cyst (Cabiese, Vallenas, andLanda, 1959; Dixon and Lipscomb, 1961;Hesketh, 1965). These reports are mostly fromneurosurgeons.

In a comprehensive review of all aspects ofcysticercosis in man, particularly in relation tothe incidence in Great Britain, Dixon andLipscomb (1961) analysed 450 cases. In 49 inwhich the disease was demonstrated at necropsy,

nine had ventricular obstruction with associatedintracranial hypertension. Lesions in the spinalcord were found in two cases, one with a cyst inthe cord substance, and one with a meningealreaction.

Bickerstaff, Cloake, Hughes, and Smith (1952)reviewed the pathology of the racemose form ofcysticercosis, and Bickerstaff, Small, and Woolf(1956) detailed the pathological findings in theposterior fossa. More recently Trelles, Palomino,and Caceres (1967) published a general andcritical review of the histopathology of cerebralcysticercosis. These authors, and Martinez(1961), have studied the fine structure of thecyst membrane, in the basal, racemose form, andconcluded that the metabolites of the parasiteexert an influence on the host tissue by leakagethrough a digestive pore. Trelles et al. (1967)emphasize the importance of the inflammatoryreaction, which amounts to a true encephalitis,and above all the predominant vascular lesions.The histopathology of the basal meningeal

reaction in the present case corresponded very

closely with the description of Trelles, notably inthe occurrence of large giant cells seen exclu-sively in the inner lamina of inflammatory cellssurrounding the degenerate larval remains, andalso in that, while there was prominent vascularinvolvement, true endarteritis was exceptional.This does not exclude the possibility of mildischaemic changes or more severe sequelae ofvascular insufficiency in the vicinity of theaffected vessels.

In 1925 Purdon Martin coined the term'amyotrophic meningo-encephalitis' to describe

the pathology of spinal progressive muscularatrophy associated with syphilis, and in his casesthe vascular abnormalities were by contrast,widespread, severe and typically obliterative. Inthe disease under discussion, the relative impor-tance of toxic, immunological, and ischaemicfactors, in producing changes in the spinal cord,and the resulting clinical manifestations, cannotbe completely determined. However, the degreeof vascular involvement in the investing mem-branes and spinal cord substance suggests thatischaemia may at least in part have contributedto the neuronal destruction and resulting amyo-trophy.The rarity of the combination of calcified

cerebral cysts and the racemose larvae isreflected in the retrospective statistical surveymade by Barrientos, Schirmer, Schenone,Aranda, Concha, and Rojas (1967). In theirreview of 5,132 necropsies at Santiago Psy-chiatric Hospital there were 31 examples ofhydatid disease and 39 cases of cysticercosis, ofwhich 36 were cerebral, and of the latter 23 werecystic, 11 racemose, and only two combined bothforms. No mention was made of pathologicalchanges in the spinal cord.

Reports which include paraparesis, or thesymptomatology of ALS in cysticercosis of thenervous system are rare (L6pez and Feijoo, 1936;Dixon and Lipscomb, 1961; Martinez, 1961).Full necropsy reports which include examinationof the spinal cord are extremely uncommon, andnone is recent. Meyer (1906) reported a case ofALS combined with multiple cysts of cysti-cercosis in the brain. The spinal cord wasexamined and there was a severe cervicalpachymeningitis. He emphasized a perivascularinflammatory reaction, and attributed the neuralchanges to vascular insufficiency. The Frenchauthors Redalie (1921) and Guillain et al. (1927,1933) described cases with similar pathologicalchanges including degenerative features in theneurones and fasciculi of the spinal cord.With regard to surgical treatment, Dixon and

Lipscomb (1961) concluded that decompressionfor intracranial hypertension due to obstructionto the cerebrospinal fluid was rarely successful,but one case is quoted (case 279) in whichremoval of a large arachnoid cyst from thecisterna magna resulted in marked improvementfor four years, after which the patient's con-dition deteriorated progressively. It is ofinterest that no case has been recorded in theliterature, or could be found on review of the

85P

rotected by copyright. on O

ctober 3, 2020 by guest.http://jnnp.bm

j.com/

J Neurol N

eurosurg Psychiatry: first published as 10.1136/jnnp.35.1.81 on 1 F

ebruary 1972. Dow

nloaded from

Pauline Kahn

records of the Royal Army Medical College, ofa patient having had a ventriculocaval shunt inobstructive hydrocephalus due to cysticercousleptomeningitis. In the present case the patient'ssymptoms of raised pressure were successfullyalleviated by means of the Pudenz ventriculo-caval shunt procedure. Kassis, Nathanson, andTinsley (1967) suggest that a shunt is contra-indicated because of the possibility of haemato-genous spread of the cysts. No evidence of thiswas revealed in this case.

In Great Britain most cases of this diseasehave occurred in soldiers or their relatives whohave lived in India. This applied particularly tothe period preceding 1930, when stricter hygienicmeasures were instituted in the affected parts ofIndia. Dixon and Smithers (1935), continuingthe original research on cysticercosis started byColonel W. P. (later Lieutenant-General SirWilliam) MacArthur (1933, 1934), made acomprehensive survey, including the historicaland literary references, of the occurrence andmeasures of prevention of Taenia solium infesta-tion in man. They reported on a series of casesin British families between 1892 and 1934. Dixonand Lipscomb (1961) continued and enlarged onthis study. Many of the patients included in theiranalysis have been the subjects of further reportsand references to these are given in their report.The condition was also relatively common inpersons of Polish origin who came to thiscountry during or shortly after the second WorldWar. In Bickerstaff's review of five cases, twopatients were Polish, one was Ukrainian, andtwo were English.At present, Taenia solium infestation is still

common in many countries, and with the facilityof modern travel occasional cases may beexpected to occur sporadically in any part of theworld. In 1968 and in 1970, biopsy material fromposterior fossa leptomeninges, taken from twopatients in the National Hospitals for NervousDiseases, London, was found microscopically ineach case to contain fragments of the racemoseform of cysticercosis. Both these patients camefrom Greece (Professor W. Blackwood, personalcommunication).

I wish to thank Dr. Marcia Wilkinson who wasresponsible for the late medical care of the patient,and who suggested the pathological study. Pro-fessor Valentine Logue agreed to the publication ofthis report, and Professor W. Blackwood and Dr.Robin Barnard made helpful suggestions. ColonelR. P. Bradshaw, L/RAMC, kindly placed the relevant

histopathological material of the Royal ArmyMedical College at my disposal. Technical, photo-graphic, and secretarial assistance were provided byMrs. M. Triggs, Mr. Gordon Cox, and Mrs. F. M.Jones, to whom I am grateful.

REFERENCES

Arana, R., and Asenjo, A. (1945). Ventriculographic diagno-sis of cysticercosis of the posterior fossa. Journal ofNeurosurgery, 2, 181-190.

Barrientos, J., Schirmer, E., Schenone H., Aranda, R.,Concha, L., and Rojas, A. (1967). Investigati6n decisticercosis e hidatidosis en 5,132 autopsias practicadasen el Hospital Psyquiatrico de Santiago (1939-1966).Boletin Chileno de Parasitologia, 22, 150-155.

Bickerstaff, E. R., Cloake, P. C. P., Hughes, B., and Smith,W. T. (1952). The racemose form of cerebral cysticercosis.Brain, 75, 1-18.

Bickerstaff, E. R., Small, J. M., and Woolf, A. L. (1956).Cysticercosis of the posterior fossa. Brain, 79, 622-634.

Cabieses, F., Vallenas, M., and Landa, R. (1959). Cysticerco-sis of the spinal cord. Journal ofNeurosurgery, 16, 337-341.

Charcot, J.-M., and Joffroy, A. (1869). Deux cas d'atrophiemusculaire progressive avec lesions de la substance grise etdes faisceaux antero-lateraux de la moelle epiniere.Archives des Sciences Physiologiques, 2, 345-367, 629-649,744-760.

Dixon, H. B. F., and Smithers, D. W. (1935). Cysticercosis(Taenia solium). Journal of the Royal Army Medical Corps,64, 227-234.

Dixon, H. B. F., and Lipscomb, F. M. (1961). Cysticercosis.An analysis and follow-up of 450 cases. Medical ResearchCouncil Special Report Series, 299, 1-58.

Guillain, G., Perisson, J., Bertrand, I., and Schmite, P. (1927).Cysticercose cerebrale racemeuse. Revue Neurologique, 34,ii, 433-444.

Guillain, G., Bertrand, I., and Thurel, R. (1933). 1tudeanatomique et clinique d'une meningite basilaire etspinale a Cysticercus racemosus. Revue Neurologique, 40,114-125.

Hesketh, K. T. (1965). Cysticercosis of the dorsal cord.Journal of Neurology, Neurosurgery, and Psychiatry, 28,445-448.

Kassis, H., Nathanson, M., and Tinsley, M. (1967). Cysti-cercosis cerebri. Chicago Medical School Quarterly, 26,188-194.

L6pez Albo, W., and Feij6o, A. (1936). Parapleiia progresivay eosinofilia subaracnoidea. Cisticercosis meningea dorsal(tercer caso diagnosticado en vida en Espania). AnnalesMedicinae Internae Fennial, 5, 137-151.

Mac Arthur, W. P. (1933). Cysticercosis as a cause of epilepsyin man. Transactions of the Royal Society of TropicalMedicine and Hygiene, 26, 525-528.

Mac Arthur, W. P. (1934). Cysticercosis as seen in theBritish army, with special reference to the production ofepilepsy. Transactions of the Royal Society of TropicalMedicine and Hygiene, 27, 343-363.

Martin, J. P. (1925). Amyotrophic meningo-myelitis (spinalprogressive muscular atrophy of syphilitic origin). Brain,48, 153-182.

Martinez, A. (1961). Neurocysticercosis. Chilean Institute ofNeurosurgery and Brain Research, pp. 178-183.

Meyer, E. (1906). Amyotrophische Lateralsklerosekombiniertmit multiplen Hirncysticerken. Archiv fur Psychiatrie undNervenkrankheiten, vereinigt mit Zeitschrift fur die gesamteNeurologie und Psychiatrie, 41, 640-652.

86P

rotected by copyright. on O

ctober 3, 2020 by guest.http://jnnp.bm

j.com/

J Neurol N

eurosurg Psychiatry: first published as 10.1136/jnnp.35.1.81 on 1 F

ebruary 1972. Dow

nloaded from

Cysticercosis of the central net-rous system with amyotrophic lateral sclerosis

Redalie, L. (1921). Deux cas de cysticercose cerebro-spinaleavec meningite chronique et endarterite obliterantecerebrale. Revue Neurologiqute, 28, 241-266.

Stepien, L. (1962). Cerebral cysticercosis in Poland. Clinicalsymptoms and operative results in 132 cases. Jouirnal ofNeurosurgery, 19, 505-513.

Stepien, L., and Chorobski, J. (1949). Cysticercosis cerebriand its operative treatment. Archives of Neltrology andPsychiatry (Chicago), 61, 499-527.

Trelles, J. O., Palomino, L., and Caceres, A. (1967). Histo-pathologie de la cysticercose cerebrale. Acta Neuropatho-logica (Berlinz), 8, 115-132.

87P

rotected by copyright. on O

ctober 3, 2020 by guest.http://jnnp.bm

j.com/

J Neurol N

eurosurg Psychiatry: first published as 10.1136/jnnp.35.1.81 on 1 F

ebruary 1972. Dow

nloaded from