October 27, 2009

Generalized Dystonia

Watch the video.

Dystonia is a movement disorder that consists of directional, sustained muscle contractions that result in abnormal, twisting postures. There are usually combined contractions of agonist and antagonist muscles. The movements are frequently stereotyped.

Dystonia is also described by the region or regions it involves, a limb, the neck, the face, or in this case the whole body. This latter form, generalized dystonia, is disabling, and usually begins in young people. At 26 years old, this woman is old to develop generalized dystonia, but the onset of her disease is not typical of primary dystonia. Neurologic disorders can be divided into primary diseases and those that are acquired or secondary. Since this condition began acutely and in association with a flu shot - association not proving causality - it should be characterized as a secondary dystonia.

Secondary dystonia has a long list of potential causes, but it is not fully necessary to review them. The initial evaluation of a movement disorder begins with phenomenology. It frames the search for an etiology and the eventual treatment. The video demonstrates one of the features of dystonia: it is frequently activity-dependent, so walking backward and running can be completely normal while walking forward is completely abnormal. One way to conceptualize why this might happen is to consider running, walking forward, and walking backward as drawing on different motor programs. Task-specific dystonia vividly illustrates this idea. In this case, however, the complete resolution of all symptoms with running and walking backward is not consistent with dystonia. Additionally, her pattern of speech is not consistent between interviews and is typical of a psychogenic dysarthria.Here is a more definitive statement from the Dystonia Medical Research Foundation.

This young woman is ill and should be seen by a movement disorder neurologist at an academic center. I mention an academic center because she needs to see a doctor who will be able to take the amount of time with her she needs. And if she is not seen by someone with a practiced eye, she may receive unnecessary and potentially harmful medical and even surgical treatment.

Here's another news account.
This story is certainly not a reason to forgo a flu shot, especially if you're in a high risk group.

1 comment:

Anonymous said...
