Saturday, March 28, 2009

Neck tongue syndrome (NTS)

Generically, neck-tongue syndrome is an acute presentation brought about by rapid neck movements, and involves a distribution of symptoms only within the face/head, most often reported as numbness in the tongue. This is usually caused by an acute subluxation (partial dislocation) of the top two vertebrae of the neck, which causes abnormal input to cranial nerves and can cause symptoms in the cranium (head).

According to McGraw-Hill Concise Dictionary of Modern Medicine; NTS is a condition characterized by sharp pain and tingling of the upper neck and/or occiput on sudden neck rotation, with numbness of the ipsilateral half of the tongue; the NTS is attributed to stretching the C2 ventral ramus, which contains proprioceptive fibers from the lingual nerve to the hypoglossal nerve and 2nd cervical root.
There are 2 categories of NTS: complicated NTS due to the presence of an underlying disease process (inflammatory or degenerative) and uncomplicated NTS (idiopathic or trauma-related) (2).

Patient’s description:

Case 1: A typical description- It's an intense blinding pain at the base of the head and the tongue always goes numb and feels like it swells up and occasionally vision blacks out for a few seconds, and then it all fades away.

Case 2: Strong, sharp pain followed by numbness and tingling of face and tongue, after this episodes plus symptoms on neck, limbs and back are also found in many cases.

Case 3: Neck feels like somethings "popping" and then my face and tongue go numb for a short time.

The summary of descriptions:
1. The pain is usually starts behind ear right where head meets my neck.
2. Seconds later the tongue goes numb.
3. Sometimes the patients also find that dropping his/her head for a short while relaxes the neck enough to stop the throbbing pain.
4. Many patients say that episodes became less violent (only the pain in face/neck are present with numbness of face, tongue and sometimes arms.) with increase in age.

Cause & pathology:
According to N Bogduk (1981); the most likely cause of the simultaneous occurrence of suboccipital pain and ipsilateral numbness of the tongue is an abnormal subluxation of one lateral atlanto-axial joint with impaction of the C2 ventral ramus against the subluxated articular processes. Radiographs revealed a narrowing of the left para-odontoid space. Physical examination revealed a mildly painful restriction in rotation at C1-2 with no apparent muscular hypertonicity (2).
Afferent proprioceptive fibers from the tongue normally travel with the lingual nerve via the hypoglossal nerve to the ventral ramus of the C2 spinal nerve. It is believed that mechanical injury to the C2 ventral ramus can thus cause a referred sensory disturbance of the tongue. In the evaluation of these patients, upper cervical spine, especially atlantoaxial, instability should be considered, along with muscle spasm (1).

The difference between VBI & NTS:

It is important to differentiate between the signs and symptoms of vertebral artery insufficiency, particularly if it is causing dizziness, loss of balance, or drop attacks (fainting). Sustained full rotation of the head can kink/obstruct the artery that goes to your brainstem, as can extending your head backward, and both should never be performed together. Rapid, full range rotation can also cause these. They likely were more severe as a child, because several key stabilizing components of your spine were not yet developed (until about 18 years old).

The hyper-mobility syndrome "double-jointedness":

NTS is so rare condition & so less studied that there are currently no research papers associating NTS to hyper-mobility of cervical joints. How ever it provides a plausible explanation in many cases. Hyper-mobility syndrome is a connective-tissue disorder. Seeing a geneticist for connective tissue disorders can be worth while who can diagnose hyper-mobility syndrome through genetics others can be diagnosed through blood/skin tests, etc.

Scoliosis & NTS:

Scoliosis can cause some issues such as this. As for example A DL spine scoliosis can affect a secondary compensatory curve that can affect upper cervical & cranium positioning severly. Hence it is plausible that if the scoliosis improves through physical therapy or surgery, if it's necessary, the neck-tongue syndrome may too.

Not many treatment options are suggested in the literature when the condition is so rarely encountered & studied.
Borody C treated uncomplicated NTS that responded favorably to spinal manipulative therapy directed at the cervical spine. In the absence of upper cervical instability, spinal manipulative therapy appears to be beneficial and should be considered in all cases of uncomplicated NTS (2).

1. Orrell RW, Marsden CD. The neck-tongue syndrome. J Neurol Neurosurg Psychiatry
2. Borody C. J Manipulative Physiol Ther. 2004 Jun;27(5):e8.

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