Infantile polymyocloniaNB Kumta, Simin F Irani, DV Punwani
Department of Paediatrics, K. E. M. Hospital and Seth G. S. Medical College, Parel, Bombay-400 012., India
Correspondence Address: Source of Support: None, Conflict of Interest: None PMID: 1032835
Source of Support: None, Conflict of Interest: None
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.
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.
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.
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).
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  and is known by other names such as Opsoclonus,  prefixation tremors,  myoclonic encephalopathy of infancy  and acute cerebellar ataxia.  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  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.
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.