This disabling movement disorder affects the person’s professional task, whether be it a writing – as seen here, or playing music……… this too can be cured!!
Dystonia is an involuntary, sustained muscle contraction with abnormal posturing. Focal dystonia affects a part of the body. Writer’s cramp is a type of focal dystonia of the fingers, hand and/or forearm, it is specific to the task of writing, thus the name, and is the most common focal primary dystonic disorder.
It typically begins with an abnormally tight grip when a person writes and gradually progresses, making simple tasks such as holding a pen and writing very difficult.
Though initially thought to be associated with only writing later on it has been found that it is also associated with any particular task that a person performs repeatedly.
This could be a musician playing a musical instrument, Asst Surgeon performing surgery and his dystonia would occur when he holds the scalp, or a barber or a hairdresser while dressing of the head, etc.
Writer’s cramp can be primary or secondary (painful) and simple or dystonic. In some cases, patients with DYT1 dystonia (primary and generalized dystonia) can have a writer’s cramp as the only symptom. Writer’s cramp is often mistaken for over-use conditions.
Over-use syndromes or repeated-use syndromes are usually characterized by pain, whereas the writer’s cramp is more likely to cause problems with coordination and is rarely painful.
It is due to both sensory impairment with decreased spatial sensitivity and a motor abnormality. The main pathophysiology being the disorganization of the sensory-motor system due to loss of inhibition, aberrant neural plasticity, and defective learning-based sensory-motor integration.
It is most prevalent between the ages of 30 to 50 years. Males are more commonly affected than females, but the latter present earlier.
The initial complaint involves loss of precision muscle coordination, cramping, and aching of the hand with task-specific movements.
The hand assumes a semi flexed position of the fingers with possible hyperextension of the fingers and hyperflexion or extension of the wrist with supination or pronation.
The symptoms increase in attempting to do 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 manifest a high-amplitude tremor only with writing. Although clinical manifestations in FHD are typically unilateral in the beginning, the disorder is considered as a bilateral dysfunction of the basal ganglia.
This is demonstrated by the fact that up to 25% of patients develop bilateral FHD if they switch to the previously unaffected hand.
Physical examination
A neurological examination is usually normal. Subtle findings comprise mild dystonic postures developing either impulsively or with movement and reduced arm swing. One-third of patients have a tremor in the affected hand when the extremity is outstretched or while writing.
Observation of the limb all through writing is an important part of the examination; the supposition of dystonic postures should be demonstrable. Patients having tremors with primarily writing, a variant of writer’s cramp may manifest a large-amplitude tremor only during writing.
In contrast to crucial tremors, sustentation or action tremors are not seen with other chores such as holding a full cup of liquid or coordination testing. Dystonic posture is usually not seen with this condition.
One of the most important features that distinguish the writer’s dystonia from other dystonia is the absence of dystonia during other activities involving hand and no evidence of dystonia in any other part of the body.
Differential Diagnosis
Dopamine-Responsive Dystonia
Multiple Sclerosis
Surgery can be very promising and rewarding. Two types of surgeries can be done using stereotactic techniques.
This is the most common surgical procedure that is performed at our Institute. Thalamotomy involves making a small lesion in the ventro anterior part of the thalamus.
This is performed using the stereotactic technique. It is a very short procedure performed under stereotactic conditions and the improvement from the procedure is visualized right on the operation table itself.
The patient is assessed by the team of doctors before undergoing surgery for the various dystonia scales that have been described above.
Video recording of his disability is done to understand the exact amount of disability. Following this, he is taken to the Operation Theatre wherein through a small hole brain on the skull of the head an electrode is passed and the area of the cells which are responsible for dystonia is stimulated.
When these cells are stimulated the patient finds instantaneous improvement in the dystonia on the operation table. For example, a musician who is not able to play the drum can comfortably start playing the drum (referred to the video attached) https://youtu.be/oWVTuXn7qTs a person who cannot write can start writing comfortably.
Once we have confirmed the level of improvement that is required we then make a permanent lesion in the brain bypassing a little higher level of current and increasing the temperature of the tip of the probe in order to destroy a few groups of cells which are responsible for dystonia.
As the surgery is done under the fully awake condition the chances of any collateral damage bar side effects or complications are very minimal. The improvement from the surgery is instantaneous.
The patient is observed for a couple of days in the hospital and repeats scoring and video recordings are done. Following this, the patient is discharged and followed up only once after 6 months to assess if there is any change in the outcome that was seen after the surgery.
One of the second largest experiences of performing this surgery in the world. However, results have been widely appreciated by the international Scientific Community. We have also published our results in prestigious journals like Movement Disorders Clinical Practice and Annals of Indian Academy of Neurology.
HP 47/M, head clerk by profession approached to us with difficulty in writing with his right hand since last 12 years. His symptoms gradually progressed and at present he had severe cramping of his hand involving thumb, middle & index finger with abnormal posturing. It was associated with severe pain. This was brought on by attempt to write. He was treated conservatively without any relief of his symptoms. Meanwhile he learned to write with his left hand as he had to continue his profession as clerk; but of late his left hand has also been developing similar symptoms.
On examination he was neurologically well preserved without any deficits. On making him write (TASK SPECIFIC) he developed dystonic posturing of his elbow, wrist, thumb, middle & index finger. He had difficulty in holding the pen and the grip on it was weak. The written words were unclear and speed of writing slow. MRI Scan was normal.
Note the dystonic posturing of fingers before surgery” – Below respective image
Ventro-oralis anterior nucleus of thalamotomy was performed using Radio Frequency Thermo-coagulation under Stereotactic conditions. Test lesions were performed before making a definite lesion to assess the improvement and development of any side effects. Two lesions were performed. There was an instantaneous improvement in his dystonia.
Post operatively he could write well and the written words were clear. His speed of writing
and grip on the pen improved. Pain and dystonic posturing of his elbow, wrist & fingers subsided and there were no complications.
HP 47/M, head clerk by profession approached to us with difficulty in writing with his right hand since last 12 years. His symptoms gradually progressed and at present he had severe cramping of his hand involving thumb, middle & index finger with abnormal posturing. It was associated with severe pain. This was brought on by attempt to write. He was treated conservatively without any relief of his symptoms. Meanwhile he learned to write with his left hand as he had to continue his profession as clerk; but of late his left hand has also been developing similar symptoms.
On examination he was neurologically well preserved without any deficits. On making him write (TASK SPECIFIC) he developed dystonic posturing of his elbow, wrist, thumb, middle & index finger. He had difficulty in holding the pen and the grip on it was weak. The written words were unclear and speed of writing slow. MRI Scan was normal.
Ventro-oralis anterior nucleus of thalamotomy was performed using Radio Frequency Thermo-coagulation under Stereotactic conditions. Test lesions were performed before making a definite lesion to assess the improvement and development of any side effects. Two lesions were performed. There was an instantaneous improvement in his dystonia.
Post operatively he could write well and the written words were clear. His speed of writing
and grip on the pen improved. Pain and dystonic posturing of his elbow, wrist & fingers subsided and there were no complications.
Preoperative posture of hand
Postoperative normalization of hand posture
AT 15/M, college student of std. XII had been suffering from a form of dystonia called writer’s cramp. He had been falling grades and was getting depressed with increasing difficulties in daily activities also. The most marked symptom was abnormal posturing of right hand on performing specific tasks.
On examination he was neurologically well preserved without any deficits. On making him write (TASK SPECIFIC) he developed dystonic posturing of his elbow, wrist, thumb, middle & index finger. He had difficulty in holding the pen and the grip on it was weak. The written words were unclear and speed of writing slow. He had no difficulty in walking or other side of the body. MRI Scan was normal.
Gpi deep brain stimulation was performed using on one side. Test stimulation was performed whilst the patient was fully awake and cooperative to identify the write spot with maximal improvement and minimal side effects. There was an instantaneous improvement in his dystonia on the operating table itself.
Post operatively he could write well and the written words were clear. His speed of writing and grip on the pen improved. Pain and dystonic posturing of his elbow, wrist & fingers subsided and there were no complications.
