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By the time some patients with writer’s cramp come to Renee Portenier, OTR, CHT, a certified hand therapist in rehabilitation services at UCLA Medical Center in Los Angeles, they have difficulty even signing their names. “Many can’t write for more than a few minutes before they have a hand spasm or their handwriting deteriorates,” she says.

In many cases, patients unable to hold a pen and write normally think they are simply suffering from fatigue or overuse. In reality, they may have a neurological abnormality that causes a progressively worsening tightness and cramping in the hand muscles.

Tom Hill, MD, PA, a neurologist in private practice in Austin, Texas, says, “The main issue is that these patients can’t write. The harder they try, the smaller the letters and the worse the cramp until they just can’t do it.”

Patients may blame their dystonia on arthritis, Hill says, but arthritis has a vastly different pathology and treatment approach. “The more common thing is to think it is overuse syndrome, but that doesn’t occur with writing, simply because no one writes by hand that much these days,” he says.

Head to Hand Connection

Writer’s cramp is a focal hand dystonia that specifically affects a person’s handwriting. A number of studies show a correlation with neurological abnormalities, such as a sensory processing defect. Other studies attribute the problem to a dysfunction of the basal ganglia. As reported in the journal of neurology Brain, patients with writer’s cramp show hyperactivity in basal ganglia, an enhanced response to tactile input from the affected hand, and task-related overactivity in visual cortical areas, the left anterior insula, and the right intraparietal sulcus.

One recent study, however, demonstrated the presence of structural abnormalities in brain structures interconnected within the sensorimotor network, including the cerebellum and cortical representation of the affected hand.

“There are more reports coming in that the cerebellum is involved,” says Joel S. Perlmutter, MD, professor of neurology, radiology, and physical therapy at Washington University School of Medicine in St. Louis. This doesn’t negate the more traditional view that abnormalities in the basal ganglia are a cause. Evidence for that includes the association of various lesions, tumors, or strokes with dystonia, he says, and the fact that drugs that block the basal ganglia cause dystonia. But this study adds the possibility, he contends, that the cerebellum is involved as well. How the two factors are connected isn’t yet clear, and calls for further research.

“Writer’s cramp is diagnosed by examination and by people who know what it looks like,” Perlmutter says. “It’s almost tautological. These are really peculiar postures that typically occur with selective actions. The co-contraction of agonist and antagonist muscles can occur in lots of other things.”

Other task-specific dystonias of the hand include those that occur when typing or playing musical instruments.

“What seems to be common are highly learned, specific tasks,” Perlmutter adds.

Writing on the Wall

The treatments are not very good in any modality, according to Hill. Some patients show improvement with therapy that targets the way the pen is held. Different types and sizes of writing instruments can help. He had one patient who learned to write with the opposite hand, but then developed writer’s cramp on that side as well.

Some patients respond to carefully selected site injections of botulinum toxin, Perlmutter says, which is typically thought to interfere with nerve transmission that causes the muscle to contract. But because some people get results at a dose that is too low to produce muscle weakness, the mechanism may instead be affecting the muscle spindles, which are controlled through the cerebellum. That, in fact, may be how the cerebellum is connected to dystonia, Hill adds.

To Beth Crowner, PT, DPT, NCS, MPPA, director of clinical practice for faculty at Washington University School of Medicine, one benefit of treating with botulinum is that it is reversible. “You can adjust the dose, and if you don’t like it, you can stop,” she says. But although medication often is the first line of treatment of writer’s cramp, it has drawbacks. If you just use medication, you aren’t training the patient to do anything differently, Crowner says. Also, oral medications usually have side effects, and she says about half of the people who might benefit can’t take them.

Another problem with using botulinum, Hill observes, is that it leaves the patient with weakness that affects other activities. “Most people with writer’s cramp just get by. No one has to write that much anymore, and these patients can usually type just fine.”

But new developments and increased visibility of the field may mean fewer people have to get by. When Perlmutter started seeing patients with writer’s cramp nearly three decades ago, the average number of physicians a patient would see before a diagnosis was seven. “Now it is two or three, so that is really an improvement. Patients don’t feel like they are crazy anymore,” he says.

PT Strategies

Physical therapy can help patients by training the brain to redevelop the individuated movements involved in writing, but one problem is the inability to selectively activate the desired set of muscles in the hand without overflowing into others. Therapists sometimes restrain the fingers and have patients practice moving a single finger, essentially retraining the brain and hand into a more appropriate motion.

“That may have to do not only with the basal ganglia dysfunction, but abnormal spindle sensitivity,” Perlmutter says. “That needs to be proven, and it could make a difference in PT strategies. One of the things we’re trying to do is see if that really works, whether people benefit from that, and so can they get less frequent injections. Right now those treatments are faith-based, and it would be nice to have evidenced-based medicine.”

Crowner believes that PT is underutilized in managing patients with focal dystonias. “The level of PT involvement depends on the physician involved. There are different ways a PT can intervene. We can work on ergonomics or adapt the instrument, including through use of straps or devices that help the person hold it, and we can adapt the way a person performs the task,” she asserts. “Ultimately, you want to minimize compensatory movements of the dystonic muscles and surrounding joints and muscles that are compensating for the dystonic muscles. You have to isolate natural motions and down-train abnormal movements. It takes a lot of training.”

It is important to actively avoid reinforcing abnormal motion, Portenier says, noting, “I work to change how they write mechanically, which can bring about neurological changes as well. We approximate normal motion by having them write just a couple of words, then their name, and then a sentence. It is like reps. I’ve also had patients draw shapes. These patients can often drum their fingers or do other motions and are fine, but they pick up a pen and it is downhill from there.”

In terms of energy use, she continues, writing longhand uses the least, followed by printing in upper and lower case. Printing in all capital letters uses the most energy. “You can change the writing style and type of pen, and sometimes that makes a difference,” Portenier says. “It is really individual to each case.” She has not found typical modalities such as heat, cold, or ultrasound to be helpful with writer’s cramp.

Crowner views the recent studies supporting a neural connection as positive developments. “The exciting thing in the neural world is we are seeing that the nervous system is much more plastic than we used to think. A number of studies show that there are adaptations in the brain. For training a person with focal dystonia in proper movements, that’s pretty exciting,” she says.

Although it is difficult to know how common the problem actually is, writer’s cramp usually develops in midlife, Crowner notes, typically growing more severe and then leveling off. “People need to have the attitude that PT can have a positive influence. Not only can we prevent [conditions] from getting worse, but as an adjunct to medical intervention, we can help them get better,” she says.

Until the need to write with pen or pencil is completely eliminated — which isn’t likely to be anytime soon — more research can clarify the neurological mechanism behind writer’s cramp, and PTs can continue to refine their approach to this debilitating problem.

Resources

Delmaire J, Vidahilhet M, Elbaz A, Bourdain F, Bleton JP, Sangla S, et al. Structural abnormalities in the cerebellum and sensorimotor circuit in writer’s cramp. Neurology. 2007;(69):376-80.

Kristeva R, Chakarov V, Losch F, Hummel S, Popa T, Schulte-Mönting J. Electroencephalographic spectral power in writer’s cramp patients: evidence for motor cortex malfunctioning during the cramp. Neuroimage. 2005;27(3):706-14.

Peller M, Zeuner KE, Munchau A, Quartarone A, Weiss M, Knutzen A, et al. The basal ganglia are hyperactive during the discrimination of tactile stimuli in writer’s cramp. Brain. 2006;129(Pt 10):2697-708.

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Melissa Gaskill is a medical writer for the Gannett Healthcare Group. To comment, e-mail pteditor@gannetthg.com

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