Muscular pseudohypertrophy due to cysticercosis

Journal of the neurological Sciences Elsevier Publishing Company, Amsterdam

155

- Printed in The Netherlands

Muscular Pseudohypertrophy S. S. JOLLY

AND

due to Cysticercosis c.

PALLIS

Medical College, Patiala, Punjab (India) and Department of Medicine (Neurology), Royal Postgraduate Medical School, London WI2 (Greet Britain) (Received 30 January, 1970)

INTRODUCTION Cysticercosis (human infestation by Cysticercus cellulosae, the larval or bladder-worm stage of the tape-worm Tuenia solium) usually presents to the neurologist as epilepsy (MACARTHUR1934; DIXONANDHARGREAVES 1944) intermittent obstructive hydrocephalus (BICKERSTAFF 1955; BICKERSTAFF et al. 1956; KUPER et al. 1958; DIXONAND LIPSCOMB1961) or as dementia with cerebral atrophy (OBRADOR1948). These facts are well known in areas where cysticercosis is endemic, the diagnosis being routinely considered when neurologists or neurosurgeons are confronted with one or other of these clinical problems. It is the purpose in this communication to draw attention to another way in which cysticercosis may present to neurologists or general physicians, namely as a relatively painless, massive, symmetrical enlargement of muscles, superficially resembling primary muscle disease. This presentation of cysticercosis is rare, only 7 cases having previously been reported (all from India). PRIEST(1926) described a young soldier with diffuse muscular enlargement affecting particularly the shoulder girdles. The muscles were weak and tender and at biopsy were found to be “extensively invaded” by cysticerci. There was presumed to be a concomitant myositis but no pathological details are given. MCROBERT(1944) mentions the dramatic case of a young cultivator who had always been of slim build. “He went to hospital because of a gross alteration in his physical appearance which had come to resemble that of a professional wrestler-to the amusement of his friends and dismay of his household”. Palpation of the thighs and shoulders revealed “thousands of slippery, blubbery swellings” which proved to be cysticerci. Under the evocative title “Cysticercosis resembling a myopathy” MCGILL (1947) described 2 young adults with a short history of painless, progressive muscle enlargement. The first presented as a “veritable Hercules, the calf muscles being especially large” (the admitting officer had provisionally diagnosed him as a case of pseudohypertrophic muscular dystrophy). Weakness was a prominent feature, an unusual occurrence in this unusual syndrome. In the second patient “the calf was incised and J. neurol. Sci.,

1971 12: 155-162

S. S. JOLL.Y,C'.PALLIS

156 the whole substance

of the muscle

mass of cysts, many

hundreds

was found to have been replaced

in number.

JACOB AND MATHEW

by a grape-like

(1968) mention

the

case of a mentally retarded man of 30 who had had fits for many years. The patient gradually developed symmetrical muscular enlargement (the nuchal musculature being strikingly involved) weakness of the enlarged contraction. fibres”.

but continued to work as a farmer. There was only minimal muscles which were slightly tender and felt “nodular” on

On blind biopsy “several

The latter

showed

white cysts were seen lying between the muscle

a “conspicuous

absence”

of cellular

infiltration,

genuine

hypertrophy or increase in connective tissue. For reasons that are not clear the authors state “numerous cysts alone could not account for the diffuse and persistent increase in muscle bulk”. AND KUMAR

The single cases mentioned

by SINGH AND JOLLY (1957) and PRAKASH

(1965) were seen by the present

Cases 1 and 2. Cases 3 and 4 have not hitherto

authors and are referred been reported.

to below as

CASE REPORTS Case 1 A 22-year-old woman was admitted to hospital with a 3-month history of enlarging muscles. While under observation, she had several major epileptic fits (without localising or lateralising features) and her behaviour was noted to be episodically abnormal, with marked emotional lability and occasional delusions. There was no past history of epilepsy and no family history of muscular disorder, epilepsy, or dementia. Examination revealed symmetrical enlargement (with minimal tenderness) of the calves, glutei, biceps, triceps and rhomboids, the picture superficially resembling pseudohypertrophic dystrophy. There was however no paresis of any of the enlarged muscles (or of any of the muscles of normal size). The gait and stance were normal and the tendon reflexes were brisk. No myotonic features could be demonstrated, either on percussion or following voluntary contraction. A subcutaneous nodule was felt over the anterior abdominal wall but no individual nodules could be palpated in relation to the enlarged muscles. The cerebrospinal fluid (CSF) was normal in pressure and composition and X-rays of the skull and soft tissues of the chest, arms and thighs failed to show any abnormal calcification. The electrocardiogram (ECG) was not recorded. Biopsy of the abdominal nodule confirmed the diagnosis of cysticercosis, and on “blind” biopsy of a gastrocnemius muscle at least 3 cysticerci presented. The rest of the muscle was histologically normal. Case 2 A 14-year-old youth was admitted to hospital with a 1%month history of diffuse enlargement of the body musculature. In the months preceding admission he had developed fits with a visual prodrome, headache, and mental deterioration of sufficient severity to necessitate discontinuation of his studies. There was a past history of nodular swellings appearing and disappearing in the tongue, which had gradually increased in size. There was no family history of muscular disorder, epilepsy or dementia. He was mentally dull, with marked symmetrical enlargement of the calves, glutei, rhomboids, trapezii, deltoids, biceps and brachio-radialis, the affected muscles being tender. Gait and stance were normal. Power was normal in the affected groups and there was no myotonia, muscular weakness or wasting elsewhere. The tendon reflexes were not impaired. Several nodules were seen in the tongue and a few subcutaneous nodules were palpated over the left side of thechest, but no nodules were felt in the enlarged muscles themselves. The CSF was normal in pressure and composition. Radiological examination of the calves revealed a few “spotty” opacities, not exceeding 2 mm in length, the significance of which will be discussed further on. The EGG was abnormal (in the absence of any symptoms or physical signs of cardiac disease) with inversion of the T waves in lead 1, avL, and in all the precordial leads. Blind biopsy of the calf muscles revealed numerous tense cysticerci (Fig. l), at least a dozen presenting in the incision 5 cm long. The muscle itself was histologically normal. Case 3 A 22-year-old

man presented

with epileptic

fits of 8 months’ duration and with rapid diffuse J. neural. Sci., 1971, 12: 155-162

MUSCULAR

PSEUDOHYPERTROPHY

Fig. 1. Case 2. Blind biopsy of gastrocnemius

DUE TO CYSTICERCOSIS

157

muscle. At least a dozen cysticerci can be seen.

Fig. 2. Case 3. Subconjunctival

cysticercus. J. neural. Sci., 1971, 12: 155-162

158

S. S. JOLLY,

C. PALLIS

enlargement of the thighs and calves over the previous 6 months. The fits had been of major type, without focal features or sequelae. There was no family history of epilepsy or muscular disorder. Examination of the central nervous system revealed no abnormal signs apart from diffuse, firm, symmetrical enlargement of the calves, thighs and buttocks. The enlarged muscles were neither weak nor tender and clinically unaffected muscles all showed normal power. There was no myotonia. The gait and tendon reflexes were normal. The tongue was enlarged and contained 2 raised opalescent nodules over its dorsal surface. A s&conjunctival nodule was present near the outer canthus of the right eye (Fig. 2). No subcutaneous or intermuscular nodules were palpable. The CSF was normal in pressure and composition. Full radiological examination of the skull and of the soft tissues of upper and lower limbs failed to reveal any pathological calcification. The ECC was abnormal (in the absence of any symptoms or physical signs of cardiac disease) with T wave inversion in avL, V4 and V.5. A blind biopsy was taken from the right thigh. Numerous extremely tense cysts

Fig. 3. Case 4. Enlargement

of thighs and calves. J. neural. Sci., 1971, 12: 155-162

-VI VI L 5

& .I ” .iz.

;

0 .? 2

z! f

4

M

MCROBERT (1944)

M

JACOBAND

M

M

Case 4

M

Case 3

Case 2

Present series Case 1

F

M

Case 2

MATHEW (1968)

M

(1947) Case 1

MCGILL

M

(1926)

PRIEST

Sex

Age

25

25

14

22

30

20

25

25

24

(years)

+

++

++

++

++

+

++

++

++

Muscle enlargement

MUSCULAR

0

0

+

f

*

0

0

0

+

Muscle tenderness

CLINICAL

i

0

+

0

It

Muscle weakness

PREVIOUSLY

OF MAIN

REPORTED

SUMMARY CYSTICERCOSIS

TABLE OF

+

0

normal

abnormal

0

abnormal

only 0

done

not

done

not

done

not

done

not

done

not

done

not

ECG

? scolices

0

+

+

0

0

Tongue nodules

0

only

? scolices

0

0

OF CASES

IN THE CASESOF THE PRESENTSERIES

Muscle calcijication

AND

FEATURES

1

++

0

+

+

+

+

+

++

++

Subcut. nodules

dementia

+

+

+

+

0

+

0

+

Epilepsy

+

+

+

+

+

+

done

not

+

+

Muscle biopsy

160

S. S.JOLLY,

C. PALLlS

bulged up on incision of the muscle, and one of these was excised. Microscopic examination of the excised cyst confirmed the diagnosis of cysticercosis. The muscle itself was histologically normal. Case 4

A 25year-old male was admitted to hospital with an 18-month history of enlargement of the thighs and calves which had come on in the course of a few weeks. For about 6 months he had been aware of subcutaneous “swellings” in various parts of his body. There was no past or family history of epilepsy. The patient was retarded, disorientated and grossly lacking in memory, judgment and insight (it was not even possible to ascertain whether this represented a recent deterioration). There was no evidence of raised intracranial pressure and there were no lateralising neurological signs. There was considerable symmetrical enlargement of the thighs and calves (Fig. 3), enlarged muscles being neither weak nor tender and showing no evidence of myotonia. The tendon reflexes were retained. Subcutaneous nodules, about 1 cm in length, could easily be palpated over the chest wall, abdomen and forearms. Nodules were also seen in the tongue. A few nodules were palpable in the forearm musculature where, characteristically, contraction would make some nodules disappear and render others more prominent. No nodules could be felt in the enlarged thighs and calves. The CSF was normal in pressure and composition and X-rays of the skull and soft tissues failed to show any pathological calcification. The ECG was normal. Blind biopsy of the right calf confirmed the diagnosis of cysticercosis, several tense cysts becoming obvious as soon as the skin and fascia overlying the gastrocnemius had been incised and the superficial fibres of the muscle separated. The muscle itself was histologicallynormal.

main clinical features of the 4 cases and of those previously literature are summarised in Table 1.

The

recorded

in the

DISCUSSION

Involvement of skeletal and cardiac muscle in cysticercosis has been known since the condition was first recognised, although in early accounts (VOSGIEN 1911; SCHMITE 1928) muscle involvement was considered to be one of the rarer manifestations of the disease, being found, for instance, in only 3.5% of cases in VOSGIEN'S series. What is not generally known, however, is that infiltration of muscles by cysticerci may at times be massive enough to result in clinically obvious, symmetrical, muscular pseudohypertrophy. The fact is not documented in any of the standard neurological textbooks, nor is it mentioned in a number of recent published in the British, French, ltalian and Russian

review articles on cysticercosis, literature (MOLINARI et ul. 1954; BRANCACCIO AND GRAZIANO 1959; DIXON AND LIPSCOMB 1961; MAIZLIN AND IURCHENKO 1962; GHERMAN et al. 1965.) It is doubtful whether muscular enlargement in cysticercosis occurs at the time of the original haematogenous dissemination of the hexacanth embryos and whether it represents an inflammatory response to such invasion. The classical studies of MACARTHUR (1934) showed that when “new” subcutaneous lesions developed in a patient with known cysticercosis the larvae in such cysts were dead, and usually showed degenerative changes. Such cysts had one feature in common: a very tense capsule. This was due to the large amount of fluid contained in them. MACARTHUR postulated that live larvae in muscle (or elsewhere) were usually well tolerated by the host but that when the larvae died they acted as a foreign irritant. Disintegration of the larvae (which usually occurred from 1 to 2 years-and sometimes up to 10 yearsafter parasitisation) was associated with an increase in the quantity of cyst fluid. Cysts might then easily double or treble their volume. “A cyst originally flaccid and not differing appreciably in consistency from the surrounding muscle would be J. neural. Sci., 1971, 12: 155-162

161

MUSCULARPSEUDOHYPERTROPHYDUE TO CYSTICERCOSIS detected for the first time when it became tense and firm”. Calcification curred at a still later stage, after the fluid had gradually been resorbed.

only

oc-

If MACARTHUR is right the finding of tense cysts on muscle biopsy in our Cases 3 and 4 is interesting, suggesting fairly recent larval death. No data are available on this morphological in the literature.

aspect of the cysts in our first 2 cases or on those previously The “spotty”

MCGILL’S (1947) first patient,

radiological

changes

are also of interest,

reported suggesting

reported

in our Case 2 and in calcification

confined

to the scolices (probably an earlier stage than the usual radiological appearance, in which the major part of the parasite is calcified). The presence of such calcification reinforces

the suggestion

-possibly

years-earlier

that invasion

of the muscle must have occurred

and that the massive

muscle “enlargement”

many months in these cases is

more likely to be a consequence of larval death, on a large scale, than a manifestation of recent invasion. Muscle enlargement implies that the previous infestation must have been considerable. The massive nature of this invasion in the 4 cases presented is attested by the presence of tongue nodules and electrocardiographic abnormalities in 2 of the 4 cases, by the presence of obvious subcutaneous nodules in 3 cases and by the presence of neurological abnormalities (epilepsy or dementia) in all 4 cases. In cases of cysticercosis it is well known that whereas individual subcutaneous lesions are often readily palpable, individual intramuscular cysts may often be difficult or even impossible to feel. This is particularly so if nodules are deeply embedded in large muscle masses of the thighs and calves. In the cases under discussion it is possible that the very multiplicity of cysts in close proximity to one another, although capable of imparting to the muscle a generally firm and “lumpy” consistency, made it even harder to palpate the diagnostically helpful single intramuscular nodules. The contrast between the appearance on biopsy of a muscle packed with parasites and the relatively normal findings on muscle palpation is certainly most striking. When diffuse, symmetrical, muscle enlargement is the presenting feature in cysticercosis, difficulties in diagnosis are likely, particularly if one is unfamiliar with such a possibility. The cases differ clinically from pseudo-hypertrophic muscular dystrophy in the absence of gross paresis in either the enlarged or normal muscles, in the rapidity with which muscle enlargement may occur, in the occasional presence of muscle tenderness, in the age of onset of the disorder (on the whole later than in the Duchenne type of muscular dystrophy) and of course in the absence of any genetic factor in aetiology. The condition can be differentiated from the muscular enlargement of myotonia congenita by the absence of cramps, demonstrable myotonia or a positive family history. None of our patients showed evidence of hypothyroidism or of primary amyloidosis, conditions which may occasionally be associated with considerable symmetrical muscular enlargement. In trichiniasis the onset is acute and although generalised muscular swelling may occur, the muscles are painful and more obviously oedematous. Fever and eosinophilia may be prominent features. Diagnosis presents no difficulty when subcutaneous nodules are visible or palpable. Biopsy of such a nodule will then confirm that one is dealing with cysticercosis but muscle biopsy is probably still necessary before the muscle enlargement itself can with confidence be attributed to the same cause. In such cases “blind” muscle biopsy J. neural. SC’., 1971, 12: 155-162

S. S. JOLLY, C. PALLIS

162 (i.e. biopsy of an enlarged palpable

nodule)

the diagnosis

will usually

of cysticercal

muscle, the site of the incision

not being determined

result in several tense cysts presenting. muscular

by a

This will confirm

pseudohypertrophy.

ACKNOWLEDGEMENTS

We are grateful

to Dr. C. Prakash

and to the Editors

of the Journal

of Tropical

Medicine and Hygiene for permission to reproduce Fig. 1, and to Professor Chhuttani, Director of the Postgraduate Medical Institute, Chandigarh (Punjab), for permission to report Case 4. SUMMARY

Four patients

are described

in whom diffuse, painless,

symmetrical

muscular

enlarge-

ment, without paresis, was a presenting feature of cysticercosis. In all cases there was additional evidence of the disease in the form of subcutaneous nodules, involvement of the nervous system, or other evidence of muscle invasion (tongue lesions, abnormal ECG, etc.). “Blind” muscle biopsy in these cases will confirm the diagnosis, many tense cysts usually presenting within the operative field. It is argued that the muscular pseudohypertrophy occurs at the time of larval death and not at the time of the original invasion. REFERENCES BICKERSTAFF, E. R. (1955) Cerebral cysticercosis: common but unfamiliar manifestations, Brit. med. J. i: 1055-1058. BICKERSTAFF, E. R., J. M. SMALL AND A. L. WOOLF (1956) Cysticercosis of the posterior fossa, Brain, 79 : 622-634. BRANCACCIO, C. AND S. GRAZIANO (1959) Su di un case di cisticercosi muscolare, Acta med. ital. Mal. infett., 14: 369-370. DIXON, H. B. F. AND W. H. HARGREAVES (1944) Cysticercosis (Taenia sodium): a further ten years clinical study covering 284 cases, Quart. J. Med., 13 : 107-121. DIXON, H. B. F. AND F. M. LIPSCOMB (1961) Cysticercosis: an analysis and follow-up of 450 cases, Spec. Rep. Ser. Med. Res. Coun., London. GHERMAN, I., M. DEBAU, S. SCHIAU AND M. DEBAU (1965) Observations sur trois cas de cysticercose musculaire g&&ali&e, Ann. Parasit. hum. eomp., 40: 427431. JACOB, J. C. AND N. T. MATHEW (1968) Pseudohypertrophic myopathy in cysticercosis, Neurology (Minneap.), 18: 767-771. KUPER, S., H. MENDELOW AND N. S. F. PROCTOR (1958) Internal hydrocephalus caused by parasitic cysts, Brain, 81: 235-242. MACARTHUR, W. P. (1934) Cysticercosis as seen in the British Army with special reference to the production of epilepsy, Trans. roy. Sot. trop. Med. Hyg., 27: 345-357. MCGILL, R. G. (1947) Cysticercosis resembling a myopathy, Indian J. med. Sci., 1: 109-l 14. MCROBERT, G. R. (1944) Somatic taeniasis (solium cysticercosis), Zndiun med. Gaz., 79: 399-400. MAIZLIN, Z. K. AND G. I. IURCHENKO(1962) Two cases of massive multiple cysticercosis (in Russian), Zdravookhr. Beloruss., 8 : 65-66. MOLINARI, V., C. PEZZULO AND R. RICCIARDI (1954) Su di un case di cisticercosi muscolare diffusa, Acta med. ital. Mal. infett., 9: 329-333. OBRADOR, S. (1948) Clinical aspects of cerebral cysticercosis. Arch. Neural. Psych&. (Chic.), 59: 457-468. PRAKASH, C. AND A. KUMAR (1965) Cysticercosis with Taeniasis in a vegetarian, J. trap. med. Hyg., 68:

100-103.

PRIEST, R. (1926) A case of extensive somatic dissemination of cysticercus celfulosae in man, Brit. med. J., ii: 471472. SCHMITE, P. (1928) Les Cysticercoses du Neuraxe, Thesis, Paris, No. 59. SINGH, A. AND S. S. JOLLY (1957) Cysticercosis: case report, Zndkm J. med. Sci., 11: 98-100. VOSGIEN, Y. (1911) Le Cysticercus celhdosae chez I’Homme et chez les Animaun, Thesis, Paris, No. 373. J. neurol. Sri.,

1971, 12: 155-162