case of head nodding

One year old girl child normal growth and development started showing this head nodding and difficulty in sitting and standing . Mother attributes everything to a fall which happened 9 days back, fall while she was sitting and playing. She noticed this problem next day onward.

(consent taken from parents for publishing this for teaching purpose)

No significant illness in the past.
No fever during the illness or immediate past
No history of any drug intake (  Possibility of drug ingestion was thought of and excluded)
Only child,born at term No significant perinatal illness, Development in all fields normal till now.
No family history of any neurological illness

On Examination

Vitals stable. She was irritable disturbed by this unsteadiness and inability to hold neck and sit straight.
No pallor
No lymph node
No neurocuaneous markes
No rash
Pulse 110/mt normal volume,. BP normal for age

CNS 

Head nodding and unsteadiness in sitting. Trunkal ataxia.Intention tremor when she is reaching toys.
Eye movements bilaterally rapid in all directions
Pupils normally reacting , fundus normal
Other cranial nerves normal, No long tract signs.
Skull ,spine was palpated for defects or swellings. None

Abdomen examinations ,

 no hepatosplenomegaly. No mass palpable

Heart examination within normal limits

Discussion 

Sudden onset of abnormal nodding head and eye movement in a child of this age most probable diagnosis of dancing eye dancing feet syndrome (Opsoclonus Myoclonus syndrome ) was most likely
Drug poisoning is another possibility , but history not supporting and it is gradually worsening since the onset nine days back .
Demyelination  ADEM is one possibility which can present this way and is the closest DD
Trauma , Intracranial injury may cause this, but patient did nt have any features of raised ICT or other features of head injury or bleeding from other sites
Intracranial space occupying lesion is another possibility , but no other focal deficit or raised ICT to support this
Neurological entities like spasmus nutans will have long history with combination of torticollis,nystagmus and head nodding. Here it is acute onset and many of the features missing
Neurodegenerative diseases like   Pelizaeus- Merzbacker disease 
 would have been a possibility if it presented in  a male child with gradual onset.  Abrupt onset and progression within short period and female sex all are against this possibility

So we considered two possibilities. 

1. Opsoclonus Myoclonus syndrome, as a paraneoplastic condition. Commonest entity presenting at this age is neuroblastoma.
2.We had cases presenting like this at this age without malignancy, few of them following viral infection. An immunological basis , which responds to High dose steroids. There need not always be imaging finding  supporting ADEM
With this in mind we did Ultrasonogram of abdomen, which came normal all organs normal ,adrenal normal no mass
Peripheral smear showed leucocytosis with lymphocyte predominance, normal platelets, Hypochromic RBCs
We did a marrow examination,which came as normal
We send urine VMA and blood for meta nephrine. We had earlier experience of false negative result of urine VMA.(Results awaited

MRI brain Normal 

MRI spine also done 

I must admit that here i made a major mistake. I missed a large mass just because i concentrated on the brain and spinal cord

This is the X Ray taken then

How did this mistake occur?

I just looked at the different Brain cuts ,all normal , I just checked the spinal cord continuing down. I passed it as normal.
This interpretation was earlier than i got the radiologists report and made a major blunder
this was the report


Baby is referred to RCC today.

Why this case presented?

1. Dancing eye dancing feet syndrome is not a common entity
2. Neuroblastoma originating from sympathetic chain from chest ,neck or abdomen can occur , but rare. 
3. This baby did nt have any of the features of malignancy eg no anemia, no organomegaly, no mass,and bone marrow came as negative
4. So highlight of this case is occasionally dancing feet dancing eye syndrome may precede all the other manifestation of malignancy . 

( This case presented with Help of Dr.Divya.Dr.Indu, my post graduates.)