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Case of the week 77  ( October 2012 )

Spasmus nutans


Mother of this 5 years old child complains that her child has a dancing eye, she claims that this disorder started since only 3 weeks.
VA , Fundus , refraction are within normal
The child appears fine with no obvious illness or neurological disease.


 See video of this patient
 


Spasmus nutans

 Clinical picture of spasmus nutanes includes three signs, head tilt, Head Nodding and Nystagmus.

 Nystagmus is usually unilateral, it may be horizontal vertical or rotary. it is usually rapid with low amplitude. Spasmus nutans usually appears at the age of 6 months to approximately 3 years old.

The cause is unknown, the condition is usually harmless and self-limiting.

Some types of central nervous system disease can result in acquired, primarily unilateral nystagmus which mimics spasmus nutans. If a patient with unilateral nystagmus is suspected of having spasmus nutans, and you cannot be sure that the patient does not actually have CNS disease as a cause of the nystagmus, a head CT scan or MRI should be done.  If neuroblastoma is suspected, an abdominal CT scan should be done along with a urine test for catacholamines.

Some serious CNS conditions that have been diagnosed in patients with spasmus-nutans-like finding are the following:

1. Chiasmal glioma

2. Nonspecific CNS degenerative disease

3. Achromatopsia - congenital stationary night blindness

4. Third ventricle tumor

Imaging should be done if a patient with spasmus-nutans-like finding has one or more of the following:

1) afferent pupillary defect

2) optic atrophy

3) failure to thrive

4) or seem to have some suggestion of any type of neurologic disease

Spasmus nutans should be differentiated from latent nystagmus which is a unilateral nystagmus occurring when the fellow eye is occluded.

When a child demonstrates congenital or acquired nystagmus which has a null or nearly null in the chin up or chin down position, serious CNS disease should be ruled out.  Down beating nystagmus with chin down and upbeating nystagmus with chin up can be signs of posterior fossa lesions.  Convergence - retraction nystagmus is seen with dorsal midbrain syndrome (Parinaud's syndrome). Other "nystagmoid" eye movement could also be considered indication for obtaining imaging of the head.  Acquired childhood nystagmus is always a concern!

In the final analysis, the clinician must weigh each case of childhood nystagmus including spasmus nutans on its own merits and employ a conscientious, careful and systematic clinical approach.

Imaging in cases of spasmus-nutans-like picture is done selectively.  The management is tailored to each patient's specific needs and includes careful follow up, which includes accurate monitoring by the family.  Studies may be done on the basis of the patient's unique clinical picture.  A spasmus nutans "plus" clinical picture always requires investigation; plus means any other important clinical neurological sign such as optic atrophy, afferent pupil defect,etc.

If a patient demonstrates spasmus nutans, regardless of an otherwise healthy appearance, an MRI of the head is done.  In most cases where spasmus nutans is the only sign the study will be normal.  But, no one will praise you for saving the health care system the cost of an MRI and you will be found wanting if an MRI was not done and significant other disease is present.

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