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  IN THIS Article
 ::  Abstract
 ::  Introduction
 ::  Case Report
 ::  Discussion
 ::  Acknowledgements
 ::  References
 ::  Article Figures

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ARTICLE
Year : 1976  |  Volume : 22  |  Issue : 4  |  Page : 194-197

Infantile polymyoclonia


Department of Paediatrics, K. E. M. Hospital and Seth G. S. Medical College, Parel, Bombay-400 012., India

Correspondence Address:
N B Kumta
Department of Paediatrics, K. E. M. Hospital and Seth G. S. Medical College, Parel, Bombay-400 012.
India
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Source of Support: None, Conflict of Interest: None


PMID: 1032835

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 :: Abstract 

A case of dancing feet and dancing eye is presented for its peculiar features of ataxia, nystagmoid movements of the eye and asynchronous random jerks of the muscles, extreme irritability but total lack of mental clouding.
Most of the laboratory investigations are normal except that the electrophoretic analysis of CSF protein shows a distinct pre­-albumin band and increase in gamma globulin.
There is uniformly favourable response to corticosteroids.



How to cite this article:
Kumta N B, Irani SF, Punwani D V. Infantile polymyoclonia. J Postgrad Med 1976;22:194-7

How to cite this URL:
Kumta N B, Irani SF, Punwani D V. Infantile polymyoclonia. J Postgrad Med [serial online] 1976 [cited 2020 Feb 26];22:194-7. Available from: http://www.jpgmonline.com/text.asp?1976/22/4/194/42822



 :: Introduction Top


Infantile Polymyoclonia is proposed as more suitable precis for the dancing feet dancing eyes syndrome as it emphasizes the infantile onset and total clinical mani­festation including extreme irritability, opsoclonus and ataxia with absence of mental clouding thus distinguishing it from other similar disorders of infancy, childhood and adult life.


 :: Case Report Top


A four and a half year old female child was admitted with a history of fever, inability to stand and sit, involuntary movements of all four limbs and jerky movements of the eyes since fifteen days prior to admission. The illness started with moderate fever. Three days later the parents noticed continuous jerky movements of all the limbs and the trunk, inability to sit or stand, and abnormal twitching of the eyes. During this period, the child had frequent falls while trying to stand, but there was no history of altered consciousness, convulsions or vomiting.

The child's neonatal period and infancy period were normal. There was a history of measles six months before the present complaints. There is no family history of similar complaints.

On Examination, the child was found to be irritable and had short-lived asyn­chronous random jerks of the muscles of the trunk and extremities. There were rapid repetitive ocular oscillations of vari­able amplitude. These nystagmoid move­ments were usually horizontal, but some­times in the vertical or oblique planes, with an occassional rotatory element. The movements were associated with a fine flutter of the eyelids and were intensified by emotion. They were not present during sleep. The pupils were dilated and react­ing equally to light. The fundi were nor­mal. The other cranial nerves were ap­parently normal. There was rapid head titubation of small amplitude. Muscle tone and power was normal, though the deep jerks were not elicitable and the plantars equivocal. Sensations were normal. The skull and spine were normal, and no meningeal signs were present.

Examination of the other systems re­vealed no abnormality.

A tentative diagnosis of encephalitis was entertained and the child was put on parenteral antibiotics (Inj. Ampicil­lin 100 mg/kg/day for six weeks), ste­roids (Prednisolone 2 mg/kg/day), and anticonvulsants. The steroids were gra­dually tapered off over a period of six weeks and the anticonvulsants were dis­continued after four weeks, with no worsening of symptoms. During its stay in the hospital the child could sit alone and stand with support.

The following investigations were carried out to establish the diagnosis.

Investigations

Tuberculin test was negative (BCG had not been given). Routine hemogram was normal, (revealed a hemoglobin of 12.5 gram per cent, total white cell count of 9000 per cu. mm. with 42 per cent lym­phocytes, 54 per cent polymorphs and 4 per cent eosinophilis). The sedimentation rate was 22 mm. at the end of the first hour. Urine analysis and stool examina­tion revealed no abnormality. The blood sugar was 82 mg, per cent, blood urea nitrogen was 12 mg. per cent, and the serum electrolytes and liver function tests were within normal limits. The electro­phoretic protein pattern was as follows: Total proteins 6.1 gram percent, albumin 2.65 gm. per cent, globulins: alpha 1 -0.28 gm. Per cent, alpha 2 -0.85 gm. per cent, beta 0.63 gm. per cent and gamma 1.69 gm. per cent. Study of im­munoglobulin levels by passive hemagglu­tination inhibition technique revealed IgG 192.4 WHO Units, IgA 95.3 WHO Units and IgM 96.2 WHO Units, the con­trols being IgG 96.2, IgA 95.3 and IgM 96.2 WHO Units. The cerebrospinal fluid was normal and the pressure was not increased. Electrophoretic analysis of CSF proteins on polyacrylamide gel show­ed a distinct prealbumin band, and a marked increase in the number of bands in the gamma globulin region See [Figure 1]. Differential staining of the centrifu­galized CSF deposit did not show any plasmocytes. X-Rays of the skull, EMG and EEG were within normal limits. The Paul Bunnel test for hetero­phil antibody was negative. The lateral brain scintiphotos were within normal, limits. The blood brain barrier for the bromide molecule seemed intact (nor­mal).


 :: Discussion Top


The dancing feet dancing eyes syn­drome is a unique disorder with a sud­den onset, protracted course and charac­terized by a violent turmoil of skeletal muscles. It was described as Infantile polymyoclonia [3] and is known by other names such as Opsoclonus, [5] prefixation tremors, [6] myoclonic encephalopathy of infancy [4] and acute cerebellar ataxia. [2] The main clinical features of infantile poly­myoclonia are dancing eye movements, somatic myoclonic ataxia and extreme irritability. There is usually an acute on­set in infancy, a nonprogressive protract­ed course with recurrences and remissions, normal routine laboratory findings and a favourable response to corticoids and corticotropins. Quantitative and qua­litative abnormalities in the IgG immuno­globins and cerebrospinal fluid plasmo­cytosis are also consistent features of this spectacular disorder. These anomalies emphasize the probable immune distur­bance in this disorder and support the contention that infantile polymyoclonia is a distinct clinical and immunological entity.

Myoclonus is the essential ingredient of the disorder. One sees frequent irre­gular and widespread shock like muscular contractions producing incoordination of limb movements and irregular titubation of the trunk. There is extreme irritability but total lack of mental clouding or other clinical or laboratory evidence of an ence­phalitis process.

The pathomorphological basis for the myoclonus has not been established in­spite of many recent reviews on the sub­ject. Myoclonus may represent the clini­cal manifestation of an irritative distur­bance in the dentato-rubro-thalamic pathways and its interconnection at cere­bellar, pontine and mesencephalic levels. Atkin and Bender [1] have performed extensive clinical and electro-oculographic studies on patients with the eye ball fluttering and have implicated the pre­tectal region of the midbrain at fault. No definite destructive lesion has been found by any of the workers, and the anatomi­cal and physiological substrate of any, myoclonus is uncertain.

This case suggests a disturbance in the immune mechanism as seen by the raised IgG immunoglobulins and there is uni­formly favourable response to corticor­tropins and corticoids which is due to its cytolytic effects on the lymphoid tissues.

Increase of plasmocytes and increased gamma globulin levels in the cerebrospi­nal fluid do not prove that there is an im­mune disturbance, even though these two features are seen in diseases with an auto­immune basis. The high cerebrospinal gammaglobulin may be a reflection of neural tissue destruction as seen by changes in a variety of infectious and demyelinating diseases.


 :: Acknowledgements Top


We are grateful to Dr. C. K. Desh­pande, Dean, K. E. M. Hospital for per­mission to publish this article. We thank Dr. V. P. Mondkar, Hon. Neurologist, K. E. M. Hospital for his valuable guid­ance.

 
 :: References Top

1.Atkin, A. and Bender, M. B.: Clinical and electro-oculographic studies on pa­tients with eyeball fluttering, J. Neurol. Sci.. 12: 202-215, 1964.  Back to cited text no. 1    
2.Cottam, D. G.: Acute cerebellar ataxia,Arch. Dis. of Childh., 32: 181-190, 1967.  Back to cited text no. 2    
3.Dyken, P. and Kolar, O.: Dancing eyes dancing feet-Infantile polymyoclonia, Brain, 19: 305-320, 1968.  Back to cited text no. 3    
4.Kinsbourne, M.: Myoclonic encephalopathy of infants, J. Neurol., Neurosurg, Psy.,25: 271-290, 1962.  Back to cited text no. 4    
5.Marmion, D. E. and Sandilands, J.: Opso­clonia-a rare ocular sign in polioence­phalitis, Lancet, 2: 508-517, 1974.  Back to cited text no. 5    
6.Struckland, B.: Prefixation tremors, spasmus nutans or opsoclonus, Lancet, 2: 369-374, 1947.  Back to cited text no. 6    


    Figures

  [Figure 1]



 

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Official Publication of the Staff Society of the Seth GS Medical College and KEM Hospital, Mumbai, India
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