AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Background

Writer's cramp is a form of task-specific focal dystonia. Dystonia is an involuntary, sustained muscle contraction causing twisting movements and abnormal postures; focal dystonia means only one body part is affected. Writer's cramp is the most common dystonia occurring in the setting of repetitive movement disorders. A focal dystonia can sometimes be the first manifestation of a generalized dystonia.

Pathophysiology

Normally, an antagonist muscle relaxes when an agonist muscle is contracted. Patients with dystonia have simultaneous contraction of both groups of muscles. Spinal reciprocal inhibition, a process that inhibits the antagonist muscles when the agonist muscles are active, is reduced in patients with writer's cramp. This is most probably due to aberrant descending commands.

Abnormalities in the basal ganglia lead to abnormalities of sensory processing and motor output. The normal increase in cerebral blood flow in the supplementary motor area is reduced in response to vibration and abnormal somatosensory evoked potentials. These provide evidence for the abnormal sensory processing in patients with dystonia. Increased motor cortex excitability along with decreased cortical inhibition causes abnormal motor output.

Frequency

United States

One study estimated the prevalence to be 69 cases per 100,000 population; this is thought to be an underestimation because a high percentage of patients never seek medical assistance.

International

Again, because of the small percentage of affected patients seeking medical attention, accurate prevalence estimates are not available.

Sex

Prevalence is slightly higher in men; the male-to-female ratio is 1.3:1.

Age

Typically, patients present in the third to fifth decades, and women usually present earlier than men.

CLINICAL

Section 3 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

History

• Incoordination, cramping, and aching of the hand with task-specific movements are usually the initial complaints.
• The hand assumes a semiflexed position of the fingers with possible hyperextension of the fingers and hyperflexion or extension of the wrist with supination or pronation.
• The symptoms become exaggerated with attempts to perform a specific task, such as writing. In some cases, the hand may dart across the page with a sudden jerk.
• A related condition is primary writing tremor. Affected individuals exhibit a prominent large-amplitude tremor only with writing.

Physical

• Neurologic examination findings are usually normal.
• Subtle findings include mild dystonic postures developing either spontaneously or with movement and reduced arm swing.
• One third of patients have a tremor in the affected arm or hand while writing or when the extremity is outstretched. Observation of the limb during writing is an important part of the examination; the assumption of dystonic postures should be demonstrable. Patients affected with primary writing tremor, a variant of writer's cramp, may exhibit a large-amplitude tremor only during writing. In contrast to essential tremor, action or sustentation tremors are not seen with other tasks such as holding a full cup of liquid or coordination testing. Dystonic posturing is usually not seen with this condition.

Causes

• Writer's cramp frequently affects persons who write a great deal or perform other repetitive hand movements such as typing.
• Approximately 5% of patients have a positive family history of a similar condition.
• Five to ten percent report an accident or injury to the hand or arm immediately preceding the onset of symptoms.
• Several possible, but rare, associations have been reported, including C6 ruptured disk, lithium use, basal ganglia or cortical tumors, arteriovenous malformations (AVMs), and stroke. However, most cases are idiopathic.

DIFFERENTIALS

Section 4 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Other Problems to be Considered

Primary writing tremor
Postural tremor
Idiopathic torsion dystonia

WORKUP

Section 5 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Imaging Studies

• MRI - If a structural lesion is suspected

Procedures

• Electromyography/nerve conduction studies
• • To evaluate for nerve injury, if trauma is the suspected cause
  • Confirm diagnosis by showing simultaneous contraction of agonist and antagonist muscles

TREATMENT

Section 6 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical Care

• Approximately 5% of patients have spontaneous remission, most probably in the first 5 years. However, the majority of these patients have relapses.
• Treatment is generally disappointing.
• Transcutaneous electrical nerve stimulation (TENS) delivered to the forearm flexor muscles for a 2-week period has been found to improve symptoms for up to 3 weeks after treatment.
• Behavioral changes may help.
• Biofeedback, hypnotherapy, and relaxation therapy have been tried.

Activity

• Altering the grip of the pen and/or increasing the diameter of the pen used is the first step in treatment.
• Patients can use a writing device or other means of transcription, such as typing or dictation.

MEDICATION

Section 7 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Several classes of drugs have been used in patients with writer's cramp. Anticholinergics have had conflicting results. Beta-blockers have helped the tremor in a few patients. Botulinum toxin injections seem to have the best results.

Drug Category: Anticholinergic

Blocking cholinergic innervation of the basal ganglia is hypothesized to increase the dopaminergic effect, thereby reducing dystonia.

Drug Category: Beta-adrenergic blockers

These agents help reduce the tremor.

Drug Category: Neurotoxins

Neurotoxin complex blocks neuromuscular conduction by binding to receptor sites on motor nerve terminals, entering the nerve terminals, and inhibiting the release of acetylcholine. When injected intramuscularly, it produces a localized chemical denervation muscle paralysis.

FOLLOW-UP

Section 8 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Prognosis

• The prognosis is good.

Patient Education

• Patients should reduce the task-specific activities that precipitate the condition.

MISCELLANEOUS

Section 9 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical/Legal Pitfalls

• Do not consider patients with writer's cramp and other action dystonias as having a psychiatric disorder. This could deprive the patient of evaluation by a movement disorder specialist and hence the benefit of adequate treatment. Patients with writer's cramp do not have a higher incidence of psychiatric disorder than the general population.

REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

• Baur B, Schenk T, Fürholzer W, et al. Modified pen grip in the treatment of Writer's Cramp. Hum Mov Sci. Oct 2006;25(4-5):464-73. [Medline].
• Chen R, Hallett M. Focal dystonia and repetitive motion disorders. Clin Orthop. Jun 1998;(351):102-6. [Medline].
• Chen RS, Tsai CH, Lu CS. Reciprocal inhibition in writer''s cramp. Mov Disord. Sep 1995;10(5):556-61. [Medline].
• Cohen LG, Hallett M. Hand cramps: clinical features and electromyographic patterns in a focal dystonia. Neurology. Jul 1988;38(7):1005-12. [Medline].
• Epidemiologic Study of Dystonia in Europe (ESDE) Collaborative Group. Sex-related influences on the frequency and age of onset of primary dystonia. Neurology. Nov 10 1999;53(8):1871-3. [Medline].
• Marsden CD, Sheehy MP. Writer''s cramp. Trends Neurosci. Apr 1990;13(4):148-53. [Medline].
• Rhoad RC, Stern PJ. Writer''s cramp--a focal dystonia: etiology, diagnosis, and treatment. J Hand Surg [Am]. May 1993;18(3):541-4. [Medline].
• Schmidt A, Jabusch HC, Altenmüller E, et al. Dominantly transmitted focal dystonia in families of patients with musician's cramp. Neurology. Aug 22 2006;67(4):691-3. [Medline].
• Tinazzi M, Farina S, Bhatia K, et al. TENS for the treatment of writer's cramp dystonia: a randomized, placebo-controlled study. Neurology. Jun 14 2005;64(11):1946-8. [Medline].
• Turjanski N, Pirtosek Z, Quirk J, et al. Botulinum toxin in the treatment of writer's cramp. Clin Neuropharmacol. Aug 1996;19(4):314-20. [Medline].

Article Last Updated: Nov 2, 2006