Spasmus nutans


Spasmus nutans

Description, Causes and Risk Factors:

Spasmus nutans is a disorder affecting infants and young children. It involves rapid, uncontrolled eye movements, head bobbing, and occasionally, abnormal positioning of the neck.

Most cases of spasmus nutans begin between age 4 months and 1 year. It usually goes away by itself in several months to years.

The cause is unknown, although it may be associated with other medical conditions. (Rarely, symptoms similar to spasmus nutans may be due to certain types of brain tumors or other serious conditions).

The origins of spasmus nutans are obscure, although we have some notions about the underlying pathogenesis. Something in the infant's early environment, particularly the nature of the stimuli to which he was exposed, was thought to cause spasmus nutans. It is ironic that spasmus nutans should become a clinical rarity at a time when increasing attention is being paid to the influence of sensory elements in early environment upon later behavior. Spasmus nutans could provide an excellent medium through which the contribution of such sensory elements to the genesis of pathologic phenomena might be investigated.

Some studies have found an association with children from lower socioeconomic status, as well as coexisting strabismus and refractive error. Chiasmal glioma can present with an identical appearing nystagmus prior to affecting the anterior visual pathway.

Symptoms:

    Small, quick, side-to-side eye movements (nystagmus) - both eyes are involved, but each eye may move differently.

  • Head nodding.

  • Head tilting.

Diagnosis:

Laboratory investigation usually is not required for spasmus nutans.Exceptions include workup for metabolic or infectious etiology in congenital cataracts, serology in suspected toxoplasmosis, toxicology in optic atrophy, endocrine assay for pituitary dysfunction in optic nerve hypoplasia, and others.Children with opsoclonus who are otherwise well should undergo measurement of urine vanillylmandelic acid (and abdominal CT scan) to rule out neuroblastoma.

Ocular ultrasonography is indicated in nystagmus patients with persistent hyperplastic primary vitreous (PHPV), cataract, Peters anomaly, and other disorders in which the ocular fundus cannot be visualized. It also is useful to assess the status of the retina in advanced retinopathy of prematurity, familial exudative vitreoretinopathy, and perinatal trauma.

Electroretinography is an essential component of evaluation in early acquired nystagmus in which intrinsic retinal disease is suspected, such as Leber congenital amaurosis, achromatopsia, congenital stationary night blindness, and other disorders.

Visual-evoked response (VER) has limited use in the evaluation of infantile nystagmus due to the inability of infants to perform pattern VER. Flash VER provides little insight into visual pathway dysfunction but may be of some value in documenting abnormal chiasmal crossing in albinism.

Treatment:

The benign form of spasmus nutans requires no treatment. If the symptoms are caused by another condition, that condition must be treated appropriately.

There are also non-drug therapies that can be used to treat spasmus nutans. The ketogenic diet is a high-protein, low-carbohydrate diet. When given in a study to infants with spasmus nutans who had not responded to other therapies, the diet reduced the number of spasms in 36% of infants and completely stopped the spasms in 19%. Side effects of the diet can include kidney stones, nausea, vomiting, and fatty liver. The researchers recommended the diet should not be used as first-line therapy, but should be tried as an alternative.

Surgery is an additional option that may be considered when babies have not responded to medical therapy and/or have brain abnormalities that can be identified on brain scans. Some of the conditions that may be considered for surgery include cortical dysplasia, porencephaly, or tuberous sclerosis. In such cases, this approach may control seizures and improve developmental outcomes.

NOTE: The above information is for processing purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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