Essential tremor usually affects the upper extremities and the hands, but it may also involve head, lower extremities, tongue and voice. Patients usually complain of their handwriting becoming sloppier, large and irregular. Patients also complain of trouble holding things like screwdriver, a cup of coffee or soup. They may spill things and feel embarrassed about going to eat out. Sometimes writing cheques may be a challenge for these patients.
Tremor usually affects both sides but initially may be noticed only on one side. It could occur at any age, but more commonly it is seen in patients above 55 years of age. The intensity of tremor increases with advancing age. This type of tremor may be temporarily dampened by g alcohol intake. The tremor of the hands is usually of medium frequency. It is usually seen when patients have their arms outstretched and by finger-nose-finger movements. Essential tremor is usually not present at rest in contrast to the tremor of Parkinson's disease.
The patients usually give family history of tremor in their parents, siblings or close relatives. However, sporadic cases are seen very frequently. The exact mechanism of inheritance is not found. In essential tremor the arms are affected in about 90% of the patients whereas head is affected only in about 30% of the patients, voice is affected in about 10% to 15% of the patients and legs are affected in about 15% of the patients. Spiral drawing and handwriting specimen is consistent with tremor. Essential tremor is more common than Parkinson's disease tremor. It affects about 5% to 6% of the patients over the age of 65. To detect essential tremor, patients are asked to make a pointer with their index finger and then touch the finger of the examiner and their own nose back and forth. They are also told to hold their arms with fingers apart in front of their body as well as in a wing beating position. Also the patients are tested for pouring of liquids, writing and spiral drawing. Before making diagnosis the other causes such as drugs, anxiety, hyperthyroidism, a task specific tremor like writing tremor, or tremor due to other focal neurological abnormalities should be excluded.
As mentioned earlier, most of the cases are sporadic; however, some of the cases may show inheritance. The first degree relatives of patients with essential tremor have more risk of developing the essential tremor. Essential tremor occurs in all populations across the world.
The diagnosis of essential tremor is usually made by history and examination. The brain scans are usually normal in these patients. However, conditions like Parkinson’s disease, other focal neurological problems, history of use of medications like valproic acid, thyroxin and lithium should be excluded carefully. As mentioned before, essential tremor gradually worsens with age and eventually may interfere with activities of daily living, writing, employment, etc.
For the management of essential tremor, any exacerbating factors should be addressed first. The two medications commonly used are beta-blockers and primidone. About 60% of the patients would have a 60% response from these medications.
Among the beta-blockers the most effective medication is propanolol. Some patients might take only 10 mg to 20 mg of propanolol tablet before performing social activities whereas other patients would prefer taking everyday medications. If propanolol is to be taken on daily basis, the dosage varies from 60 mg to 320 mg per day. Side effects could be a slight drop in blood pressure, feeling tired, depression and impotence. Propanolol is contraindicated in patients with asthma, COPD or heart failure. Diabetes mellitus is also a relative contraindication as Propranolol can mask symptoms of hypoglycemia.
BOTOX ® injections may offer some improvement but may cause finger or wrist weakness in these patients. Gabapentin, benzodiazepines like clonazepam, Topiramate may have mild to moderate effect in reducing essential tremor. The initial starting dose of topiramate is 25mg once a day which can be slowly increased overtime. Surgical treatments are used for patients who have very advanced essential tremor and they fail pharmacological management.
Primidone is a medication used for epilepsy that was found to be helpful in the treatment of essential tremor. The initial dose is 62.5 mg once daily which could be increased slowly, however some patients have difficulty tolerating, and they might feel drowsy, dizzy with Primidone. If patients are compliant with this medication the side effects may go away in 2 to 3 weeks.
Blepharospasm is characterized by the increased frequency of blinking and involuntary forceful closure of eyelids. It may lead to functional blindness in some cases. It is usually caused by the abnormal contraction of orbicularis oculi and other neighboring facial muscles. Blepharospasm may become worse in bright light. Like cervical dystonia, blepharospasm sometimes may be relieved by a sensory trick of placing a finger lateral to the orbit. The onset of blepharospasm is usually gradual and women are affected more than men.
The diagnosis is by history and examination. History of exposure to neuroleptic or antipsychotic medications should be asked. The brain scans in patients with blepharospasm are usually normal. There are no other diagnostic tests.
The most effective treatment is Botulinum toxin (BOTOX ®) injected intramuscularly. The effect of BOTOX ® usually lasts for 3 months. Once the effect of BOTOX wears off, the injections have to be repeated again.
Hemifacial spasm is characterized by unilateral twitching of the muscles of the face around the eye and or mouth. The twitching is usually intermittent and occurs many times a day. Women are affected slightly more than men. It causes significant social embarrassment. The etiology of this condition is unknown. The twitching is involuntary and usually involves the muscles supplied by facial nerve ie 7th cranial nerve. Most of the cases are idiopathic whereas some cases may result due to compression of the facial nerve by an aberrant blood vessel in the brainstem area. Most of the cases are sporadic. The twitching of the muscles may become worse with stress, fatigue, anxiety and is relieved in sleep.
Hemifacial spasm may be confused with blepharospasm. Hemifacial spasm is usually unilateral where as blepharospasm is mostly bilateral. Other conditions which can be confused with hemifacial spasms are facial tics, facial myokymia, hemimasticatory spasm. If the neurological examination is otherwise normal, then no further tests are done. However, if patients have focal abnormalities on neurological examination, then MRI is useful. MRI should be obtained with special attention to the brain stem structures.
The treatment of choices for hemifacial spasm is Botulinum toxin(BOTOX®) injected intramuscularly. The effect of BOTOX® usually lasts for 3-4 months. Once the effect of BOTOX® wears off, the injections have to be repeated again. Surgical treatment or medications such as carbamazepine may be effective in some cases.
Prevalence of Gilles de la Tourette’s syndrome in adolescents ranges from 3 to 5 per 10,000. It is slightly more common in males than in females. Tourette’s syndrome is characterized by multiple motor and vocal tics that usually start before the age of 21 years and lasts for more than one year. The symptoms may change in character over time and wax and wane in intensity.
Tourette’s syndrome is the most common cause of tics which might range from simple brief jerks to complex involuntary movements which could be preceded by an unpleasant sensation which is relieved by movement. Tics could be suppressed briefly but this builds up an inner sensation which may needs to be relieved by excessive tics later. The head and neck region in the body is most commonly involved. Tics may involve limbs as well as sounds. Some of the examples of tics include eye blinking, grimacing, head shaking, throat clearing, barking sounds, repeating words or parts of words or sniffing. Tics could be very fast in speed called clonic tics or could be sustained contractions called dystonic tics.
Supressability is a key feature to distinguish tics from other movement disorders. Tics usually begin in childhood and increase in severity around age 9 to 10 years. With age they may improve. The tics might be associated with obsessive compulsive or attention deficit disorder. CT scan and MRI are usually normal.
When tics are mild and not bothering the patient no treatment is necessary. However if the tics become socially disabling treatment might be needed. Some of the medications used in the treatment of Tourette’s syndrome include Clonidine which is an antihypertensive drug but does help Tourette’s syndrome. Other medications used include Tetrabenzine and Clonazepine. Prophenazine are used less commonly due to more serious side effects of tardive dystonia.
Restless legs syndrome affects about 5- 10 % of people. Most of the patients report a desire to move their legs, restlessness and worsening of symptoms at rest and a relief with walking and the symptoms are worse at night. Patient usually describe a sensation as crawling, creeping, tingling, cramping, pulling, pain or electric tension, or discomfort or itching in their legs. Some patients may feel the sensation in their arms as well. These patients may have difficulty sitting for a prolonged ride in a car or plane, watching television and sitting in movie theatre without getting up and walking to relieve the abnormal feelings in their legs.
The exact cause of restless leg syndrome is not known but in some cases it could be inherited in an autosomal dominant fashion. Symptoms of RLS could occur secondary to other conditions like polyneuropathy, renal failure, iron deficiency and pregnancy. Patients with Restless Leg Syndrome may have a rapid jerking of their arms or legs when they are sleeping; called PLMS or periodic limb movements of sleep. Their spouses usually report these patients have kicking movements of legs when they are sleeping, these may be irregular and random. The incidence of this condition increases with age.
The diagnosis is clinical by history. The periodic limb movements of sleep could be diagnosed with a sleep study.The iron deficiency, polyneuropathy, electrolyte abnormalities, renal insufficiency, degenerative disc disease of spine and thyroid dysfunction should be ruled out in these patients. The treatment includes Dopamine agonists such as pramipexole, ropinarole, levodopa, gabapentin, and opioids medications.
Cervical dystonia is one of the most common dystonic disorders. Cervical dystonia causes sustained muscle contractions of the head and neck region causing the twisting and repetitive movements of head. Initially it may just start with a sensation of head being pulled to one side or the other. It may have a mild head tremor but eventually it may cause the sustained turning of the head to one side. This may lead to significant neck pain.
Cervical dystonia usually worsens with fatigue and emotional stress but it improves with relaxation and usually disappears in sleep. Sensory tricks are the characteristic feature of cervical dystonia in which if a patient touches the side of face or back of their head they can bring their head in the neutral position transiently. It usually affects patients in their adulthood between the ages of 30 to 50. The most common neck muscles involved are sternocleidomastoid, levator scapulae, splenius capitis, trapezius and scalene muscles. The abnormal contractions of the different combination of these muscles cause the head being turned or tilted to one side. Sometimes the ipsilateral shoulder could be higher than the contralateral shoulder and slightly anteriorly placed. Some patients may have just the sustained turn, tilt, backward bending or forward bending of their head where as other patients may have dystonic head tremor. The head tremor could be side to side or horizontal or vertical tremor ie yes-yes head tremor. If the head tremor is mild then the dystonic tremor may be confused with essential tremor affecting head.
The diagnosis of cervical dystonia is usually clinical and requires examination by a neurologist. The brain scans are usually normal in these patients
The most effective treatment of cervical dystonia is botulinum toxin injections. These injections are given in the neck muscles involved. BOTOX® injections are started from a low dose and the dose is increased slowly. The effect usually lasts anywhere from 3 to 4 months and these injections have to be repeated once the effect is gone.
The medications which may be helpful in some cases include trihexyphenidyl, which is an anticholinergic medication. The starting dose is 1 to 2 mg per day then it is increased as needed divided in three doses up to a maximum of 6 mg per day. Side effects include dry mouth, in elderly patients it may cause confusion at times. Other medication used are baclofen and benzodiazepine especially clonazepam.
Surgical procedures such as thalamotomy and pallidotomy may be tried in cervical dystonia and are found to effective.
These include Selegeline or Eldepryl® and Rasagiline or Azilect®. These drugs reduce the symptoms of Parkinson’s by preventing the breakdown of dopamine. Selegiline or Eldepryl can act as a stimulant which may cause insomnia, otherwise, the side effects are similar to Levodopa.
The effect of this drug is carried out by increasing dopamine release, blocking a specific receptor for glutamate in the brain and possibly by anticholinergic mechanisms. It helps the tremor, may also help slowness and rigidity in the early stages of Parkinson’s. This also helps dyskinesias which are involuntary movements caused by Levodopa and therefore may be used in the advanced stages of Parkinson’s. Its side effects include urinary retention, skin blotches, hallucinations and swelling of feet.
These include Trihexyphenidyl or Artane® and Trihexbenztropine or Cogentin®. These drugs may be used to treat the tremor in the early stage of Parkinson’s. They work by restoring the balance between dopamine and acetylcholine. Acetylcholine is also a neurotransmitter found in the brain. They can cause many side effects including memory problems and therefore there use is limited.
The medications should be taken at the right time in right dosage. Keeping track of when the medication was effective and when it wasn’t, making notes of the side effects, sharing this with your doctor at regular intervals leads to a better treatment. You should use the same pharmacy consistently, so they have a record of your medication history. Thus, there is less of a chance you will buy a prescription free product that may interfere with your drug treatment.
Always try to drink a glass of water with each dose of your medication. This will help the absorption process of the medication. Also, you should swallow controlled-release tablets without chewing them. Thus, the contents will be able to reach their target destination more efficiently.