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The Effects of Cannabis on Dystonia and Spasticity on Pediatric Patients A clinical trial is planned to study the effects of cannabis on dystonia and spasticity in children with neurological Cannabis in the Treatment of Dystonia, Dyskinesias, and Tics Cannabis has been used for many medicinal purposes, including management of spasms, dystonia, and dyskinesias, with variable success. Dystonia You might be aware of medical marijuana and its ability to offer symptom relief of major diseases like cancer, epilepsy and multiple sclerosis (MS). However, you might not know that

The Effects of Cannabis on Dystonia and Spasticity on Pediatric Patients

A clinical trial is planned to study the effects of cannabis on dystonia and spasticity in children with neurological diseases. The clinical trial will include 40 children divided into two groups: children with spasticity and dystonia due to cerebral palsy, and children with spasticity and dystonia due to genetic neurodegenerative diseases. Each group will be randomly divided into two arms and will receive Avidekel cannabis oil 6-to-1 ratio of CBD to THC or enriched Avidekel cannabis oil 20-to-1 ratio of CBD to THC. During the study, various variables will be collected including: medication intake, spasticity, dystonia score, pain scale, restlessness scale, quality of life measures, safety tests, side effects, and an addiction test. The investigators hypothesize that cannabis consumption will reduce dystonia and spasticity in children with motor disability related to genetic neurodegenerative diseases and cerebral palsy and as a result improve motor function, non-motor functions and quality of life.

Condition or disease Intervention/treatment Phase
Spasticity Dystonia Drug: Avidekel oil Drug: Enriched Avidekel oil Phase 2

The purpose of this study is to examine the effect of two cannabis oils containing the main cannabinoid Δ9 -THC and CBD ratio of 1 to 6 and 1 to 20 (respectively), on spastic movement disorder and dystonia.

40 children will be enrolled in this study and will be divided into 2 groups:

  1. Children with spasticity and dystonia due to cerebral palsy
  2. Children with spasticity and dystonia due to genetic neurodegenerative diseases

Each group will be randomly divided into two groups:

I. Active comparator: 6-to-1 ratio of CBD to THC oil II. Active comparator: 20-to-1 ratio of CBD to THC oil

During the study the following variables will be collected:

  • Consumption of drugs medication intake
  • Spasticity grade measured using the Modified Ashworth Scale
  • Dystonia grade measured using the Barry Albright Dystonia Scale
  • Patient’s subjective report about the severity of his/her spasticity and/or dystonia
  • Pain scale
  • Restlessness scale
  • Quality of life measures: function, sleep, mood , appetite , weight
  • Safety tests: liver function, renal function, blood tests (complete blood chemistry), blood pressure, pulse, ECG
  • Side effects
  • Addiction test

The purposes of the study:

  1. Efficiency – examining the effect of cannabis on dystonia and spasticity.
  2. Examining the most effective cannabinoid ratio for the relief of dystonia and spasticity. (Δ9 -THC and CBD ratio of 1 to 6 or 1 to 20, respectively).
  3. Examining the effect of cannabis on quality of life measures (such as medication intake, mood, appetite, sleep).
  4. Safety: examination of side effects and unwanted effects of cannabis and its limitations.

Research Hypothesis: The investigators hypothesize that cannabis consumption will reduce dystonia and spasticity symptoms in children with motor disability related to genetic neurodegenerative diseases and cerebral palsy and as a result improves motor function, non-motor function and quality of life.

Cannabis in the Treatment of Dystonia, Dyskinesias, and Tics

Cannabis has been used for many medicinal purposes, including management of spasms, dystonia, and dyskinesias, with variable success. Its use for tetanus was described in the second century BCE, but the literature continues to include more case reports and surveys of its beneficial effects in managing symptoms of hyperkinetic movement disorders than randomized controlled trials, making evidence-based recommendations difficult. This paper reviews clinical research using various formulations of cannabis (botanical products, oral preparations containing ∆ 9 -tetrahydrocannabinol and/or cannabidiol) and currently available preparations in the USA (nabilone and dronabinol). This has been expanded from a recent systematic review of cannabis use in several neurologic conditions to include case reports and case series and results of anonymous surveys of patients using cannabis outside of medical settings, with the original evidence classifications marked for those papers that followed research protocols. Despite overlap in some patients, dyskinesias will be treated separately from dystonia and chorea; benefit was not established beyond individual patients for these conditions. Tics, usually due to Tourettes, did respond to cannabis preparations. Side effects reported in the trials will be reviewed but those due to recreational use, including the dystonia that can be secondary to synthetic marijuana preparations, are outside the scope of this paper.

Electronic supplementary material

The online version of this article (doi:10.1007/s13311-015-0376-4) contains supplementary material, which is available to authorized users.

Introduction

Endocannabinoid receptors (CB1R and CB2R) are plentiful in the basal ganglia [1], implying they play a role in normal motor function and that pharmaceutical (or recreational) cannabis formulations, which are agonists at both sites, might alleviate symptoms of movement disorders. CB1R are expressed in γ-aminobutyric acid (GABA)ergic neurons of the caudate and striatum, presynaptic terminals in the globus pallidus externa and interna, substantia nigra pars reticulate and pars compacta, and are present in glutamatergic projections to and from the cortex and the subthalamic nucleus. In addition to GABAergic and glutamatergic pathways, dopaminergic inputs are also influenced by endocannabinoids. In general, the cannabinoid signals would be upregulated if a disease was marked by hypokinesis, such as in Parkinson disease (PD), as the cannabinoid ligands act overall to suppress movement, and would be decreased in hyperkinetic movement disorders such as Huntington disease (HD). However, paradoxical responses can occur during degeneration as the receptors in various parts of the basal ganglia die (Table ​ (Table1 1 ).

Table 1

Cannabis formulations used in various movement disorders

Generic name Trade name Use Dosage and component(s) Reference
PD
Cannabis extract Cannador
Not stated
Dopa-induced dyskinesias/dystonia
Tremor/dystonia
dopa-induced dyskinesias
Δ 9 -THC 2.5 + CBD 1.25
100–600 mg/daily
0.03 mg/kg/daily
25mgTHC/kg//daily
75 mg/daily
[7, 10* 16, 17*, 18*]
Rimonabant (CBD antagonist) SR141716 Dopa-induced dyskinesias Experimental [20]
Inhaled botanical marijuana Parkinson tremor and dyskinesias 1 cigarette 2.9 % THC
Survey of unsupervised smoked marijuana 0.5gm/cig
[13*,14, 15, 19]
Nabilone Cesamet Levodopa-induced dyskinesias Synthetic cannabinoid
0.03 mg/kg
0.03 mg/kg
[10*, 16]
Dystonia
Dronabinol Marinol Cervical dystonia
Dystonia and tics in MS
7.5 mg twice daily
2.5 mg twice daily
[5*, 11]
CBD or cannabis extract None Primary dystonias 10 mg/kg/day up to 75 mg/day
100 mg CBD
[7, 18*, 23]
Inhaled botanical marijuana Spasms
Hemidystonia (in Wilson disease)
Not stated
1 MJ Cigarette/dy
3–4 g/day
[6, 8, 9]
HD
Nabilone Cesamet Motor score no improvement, some in chorea and NPI 1–2 mg/day [23* † , 24*]
CBD or cannabis extract None 10 mg/kg/day, mean 700 mg [21* ‡ ]
Tourette syndrome
Botanical smoked marijuana 0.5–2.0 cigarettes/day
One “cone”/night survey (self-prescribed)
[25–27]
THC capsule None Tics and vocalizations 2.5 mg Δ 9 -THC, maximum 10 mg/day [28 † , 29 ‡ ]

If no evidence classification is indicated, it means it was class IV. PD = Parkinson disease; Δ 9 -THC = Δ 9 -tetrahydrocannabinol (the principal psychoactive agent); CBD = cannabidiol (a lesspsychoactive resin extract constituent of the plant Cannabis Sativa); MS = multiple sclerosis; HD = Huntington disease; NPI = Neuropsychiatric index

The complexity of feedback loops in this region, with indirect actions of the endocannabinoid system modulating other inhibitory, excitatory or dopaminergic transmission, partially explains this. In addition, in degenerative diseases such as HD and PD, progressive loss of specific structures, along with their endocannabinoid receptors occurs. The receptor loss will dampen any effects of CB1/2R agonists such as Δ 9 -tetrahydrocannabinol (THC) and cannabidiol (CBD). Other endocannabinoid receptors, such as transient receptor potential vannilloid-type 1, may be successfully stimulated in early stages of HD, but medications do not exist yet to test this. In fact, CB1R antagonists, similarly experimental at this stage, might be beneficial for hypokinetic symptoms such as hypokinesia in PD.

Contradictory and confusing efficacy has been reported when cannabis medications (or smoked phytocannabinoids) are used to treat movement disorder symptoms [2, 3]. Although the loss of receptors plays some role, more likely currently available cannabis preparations, which contain different amounts and combinations of cannabinoids (at least 60 have been described) with variable potency and psychoactive content (present in ∆ 9 -THC but not CBD) with different, usually low, doses, make standardized comparisons impossible. Scoring methods, other than counting individual tics or choreiform movements, add to the researcher’s difficulty in measuring efficacy. The potency, especially ∆ 9 -THC content, is kept deliberately low to limit side effects (or patient recognition in those experienced with marijuana), which contributes to treatment failure. Studies are small with problematic recruitment for a substance that will require limitation of activities such as driving, use of a stigmatized medication, and of short duration (sometimes single dose) to avoid abuse or addiction [4]. Even obtaining study drug status in the USA requires working with the various government agencies, and is only becoming easier in the face of a serious epidemic of opiate overuse and toxicity. The Drug Enforcement Agency continues to classify medical cannabis as a Schedule I drug (that with no therapeutic use).

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Investigation into cannabis’ medicinal use began with promising case reports and anonymous surveys, followed by limited clinical research often conducted outside the USA, which has not proved sufficient to allow many evidence-based recommendations. Although overlap exists in individuals, in this review dyskinesias, dystonia, and chorea will be considered separately. Although tremor was studied in patients suffering from multiple sclerosis, there is no information on essential tremor. Tics will be considered separately, as the mechanism differs.

Dystonia

Dystonia involves overactivity of muscles required for normal movement, with extra force or activation of nearby but unnecessary muscles, including those that should be turned off to facilitate movement, and is often painful in addition to interfering with function. It can be primary, as in torticollis and blepharospasm/orofacial dyskinesias or dystonias (Meige syndrome) or as part of another condition such as HD and tardive dyskinesia after dopa-blocking drugs. The globus pallidus and substantia nigra pars reticularis contain CB1R, with cannabinoids acting as neuromodulators and enhancing GABA release and reducing its reuptake [5].

In 1981, Marsden described improvement in a patient with torticollis who smoked cannabis [6]. An open-label series followed [7], and self-reported improvement with smoking marijuana was described in 2002 in a patient with central pain and dystonia and in a patient with Wilson disease [8, 9]. Cannabidiol showed improvement of 20–50 % by videotape review in 5 patients [9], but higher doses exacerbated tremor and hypokinesis in 2 patients with PD and levodopa-induced dystonia. Nabilone, a synthetic oral form of ∆ 9 -THC, which is available in the USA for other indications, was not found to be effective in 1 administration of escalating doses of 0.03 mg/kg using a dystonia rating scale; however, 3/15 patients felt better for several days after its use [10]. Another currently available oral form of ∆ 9 -THC, dronabinol, did not improve symptoms of cervical dystonia, as demonstrated by the Toronto Western Hospital Spasmodic Torticollis Rating Scale in a 3-week treatment trial [5]. Finally, response to dronabinol 2.5 mg twice daily in a case report of dystonia (and tics) in a patient with multiple sclerosis who had previously reported symptom improvement after smoking marijuana [11].

Side effects described in these studies included hypotension and sedation at higher doses of nabilone [10], insomnia and tachycardia from dronabinol [5], and hypokinesia and tremor of PD [10].

Dyskinesias from Levodopa (in Advanced PD)

The plethora of endocannabinoid receptors in the basal ganglia, especially the globus pallidus interna, pars reticulata, and cerebellum indicate they must be playing a role in regulating tone and motor function through the effect of the endogenous cannabinoid ligand, arachidonylethanolamide (anadamide), on modulation of GABA transmission [12]. Many studies have been done with primate or rat models to determine if cannabinoid agonists or antagonists could act to suppress dyskinesias without exacerbating hypokinesis; however, translation to patients has proved difficult.

Since the initial observation in 1991 of no improvement of resting tremor in 5 patients who smoked one marijuana cigarette [13], surveys have been done asking patients with PD to say if they had tried marijuana on their own (presumably the smoked botanical form) and if so with what effect. Venderova sent surveys to 630 patients attending a movement disorders clinic in Prague, and of the 339 respondents, 25 % had used marijuana. Of these 85 patients, 39 benefitted in rest tremor (31 %), bradykinesia (45 %), and dyskinesias (14 %), and continued its daily use [14]. A recent survey of Colorado residents with Parkinson using all types of complementary therapies found 9 using medical marijuana (4 %), reporting improvement of mood and sleep, but only 2 with improvement of motor symptoms, not specifically dyskinesias [15].

A small but class III randomized double-blind study using nabilone (synthetic ∆ 9 -THC) showed a significant reduction in Rush score on levodopa-induced dyskinesias in 7 patients, observed and measured with the Rush dyskinesia scale [16], and 2 reported improvement in dystonia occurring in the off period for dopa. In a study of 17 patients using the oral preparation Cannador (2.5 mg ∆ 9 -THC/1.25 mg CBD) no improvement was noted in dyskinesias as measured in Q32–34 of the Unified Parkinson’s disease rating scale scale (the primary outcome), or other scales including Parkinson Disease Questionnaire-39 scale, nor was there a dose response [17]. The study by Carrol et al. [17] was the only one considered class I for the purposes of evidence-based recommendations. Various doses of cannabidiol were given to 21 patients over 6 weeks; no change was found in the total motor score, despite improvement in the quality of life section of the Unified Parkinson’s disease rating scale if the target dose of 300 mg daily was reached, which was possible in only 35 % of the patients [18]. Finally, an open-“label” study of smoked marijuana in 22 Israeli patients showed improvement in the number of dyskinesias observed after dopa challenge, 30 min after smoking the cigarette [19]. In an interesting twist, a single dose of the CB1R antagonist rimonabant (SR141716) was found to have no effect on motor symptoms, or induced dyskinesias in 8 patients [20].

Side effects mentioned in the studies included hypotension, vertigo, hyperacusis, and disorientation and visual hallucinations [15], somnolence, dizziness and bad taste, with hypoglycemia in 1 patient [19], and, rarely, bradykinesia, but in the few studies where it was measured [17], there was no effect on cognitive function as measured by Mini Mental State Examination; in fact, an improvement in Mini Mental State Examination was noted, which was attributed to a practice effect but may have been precognitive (the 4-week trial was too short to call cannabis neuroprotective).

In summary in PD, the symptom that responded best to cannabis, levodopa-induced dyskinesias, is a fairly rare complication of dopamine replacement in advanced cases. The role of cannabis in other symptoms of PD is unclear, and these symptoms vary according to the stage of neurodegeneration and also to the state of treatment with dopamine transmitter replacement. As there is the potential of cannabis worsening some symptoms, especially hypokinesia, very careful research must be done with PD.

Dyskinesias in HD

Abnormalities of motor function, along with psychiatric and cognitive dysfunction, are a main feature of HD, a dominantly inherited neurodegenerative disease. Cannabis products have been most often used to ameliorate agitation and other psychiatric symptoms, but the presence of endocannibinoid receptors in the striatum, where they modulate GABA transmission and affect glutamate release, suggests a role for management of the excessive involuntary movements (choreoathetosis or dystonia) of HD. As in other degenerative processes, these receptors can decrease as the disease progresses, leaving less response to cannabinoid agonists as the brain’s substrate changes. Obviously, current or prior use of dopamine-blocking medication (neuroleptics), which can superimpose tardive dyskinesias on the direct movements of HD, will also change the therapeutic effects of cannabis.

Consroe et al. [21] first reported the clinical use of CBD in HD in 1991. This class III study of 15 patients receiving cannabis extract in capsule form (10 mg/kg), crossing to placebo over a 15-week period, off neuroleptics for at least 2 weeks, found no difference in the chorea severity score of Marsden and Quinn, or on videotape and live assessment of chorea severity, nor on secondary end points of Shoulson and Fahn disability scores, finger tapping, or manipulation. Side effects, as checked off a symptom inventory, did not vary between placebo and treated patients. After a case report in a patient who had improved mood and movements after smoking marijuana and then taking prescribed nabilone, 1 mg daily [22], the authors gave 1 or 2 mg of nabilone to 37 patients in a class II study [23]. Despite improvement in the secondary measures of neuropsychiatric index and chorea score, the primary outcome, total motor score of the Unified Huntingdon Disease Rating Scale, showed only a modest response, with no dose response (i.e., 2 mg was not better than 1 mg).

Drowsiness and forgetfulness were the main reported adverse events in both groups, with no increase in psychosis or euphoria in the treated group. In 1 case nabilone actually caused increased chorea [24].

Tourette Syndrome Tics

As in any condition influenced by anxiety, a nonspecific beneficial effect of cannabis might be expected, but given the presence of endocannibinoid receptors in the striatum, it is possible that a direct effect of cannabis is reducing the number of tics.

Success in treating symptoms of Tourette sydrome, including involuntary movements (tics) and compulsive behaviors, was first mentioned in an observation of 3 patients who, in 1988, noted improvement in tics and urges while smoking marijuana cigarettes [25], followed by another case of a patient remaining symptom free for a year while smoking marijuana daily [26]. In 1998 a survey of a larger population confirmed a reduction in tic or complete remission in 82 % of patients [27]. The same authors used Δ 9 -THC capsules of varying strengths in a single dose in 12 patients (class II study) and reported improvement in scores of the Tourette Syndrome Symptom List and obsessive–compulsive behavior scores, with a decreased number of complex motor tics observed by the examiner [28]. The following year, in a study of 24 patients using the maximum-strength Δ 9 -THC capsule from the pilot study (10 mg) for 6 weeks, a significant response in self-rated Tourette score and observer-rated scores, including the Tourette Syndrome Clinical Global Impression Scale, the Shapiro Tourette Syndrome Severity Scale, and the Yal Global Tic Severity Scale, as well as the review of video, was noted [29]. These were then summarized in a Cochrane review [30]. Little additional work was summarized in a more recent review [31]. Of note, improvements occurred without exacerbating performance on neuropsychologic testing [32].

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Side effects were minimized by a simple technique of providing dronabinol after breakfast in order to slow its absorption and provide a steady level acting in the brain [29].

Conclusions

Although clinical studies in this area are difficult to do, even in countries where the use of cannabis has been allowed for years, there is a clear role for cannabis products in symptom management for these difficult conditions. The movement disorders are well-known to be worsened in patients who are anxious, but the careful observations reviewed above lead to the conclusion that there is a direct effect of cannabis in various formulations in some conditions, especially hyperkinetic symptoms. Caution in using a potential central nervous system depressant is always required in patients whose neurologic function is already compromised by disease, but larger studies will prove there is a promising role for this class of drug in the therapy of dyskinesias, tics, and possibly dystonia.

Dystonia

You might be aware of medical marijuana and its ability to offer symptom relief of major diseases like cancer, epilepsy and multiple sclerosis (MS). However, you might not know that medical marijuana for dystonia can help if you or a loved one has the condition. Dystonia afflicts close to 300,000 people in North America and can interfere with daily living.

Marijuana and Dystonia

Types of dystonias depend on the regions of the body they affect. Because so much of the disease entails muscle spasms and involuntary contractions, cannabidiol treatment, or medical marijuana, can be incredibly beneficial for treating involuntary muscle spasms. There has been research to support CBD treatment as particularly beneficial for reducing dystonic movement disorders.

How and Why Marijuana Is an Effective Treatment for Dystonia

Research has found that medical cannabis for dystonia does improve symptoms. Cannabis contains two main cannabinoids that activate the CB1 and CB2 receptors of your endocannabinoid system. These are tetrahydrocannabinol (THC) and cannabidiol (CBD), and they help regulate the inhibitory and excitatory neurotransmitters necessary to diminish muscle tremors and spasticity.

A study of Sativex, or cannabis medication, in people who had spasticity symptoms due to MS revealed that after four weeks of weed treatment, the spasms traditional efforts didn’t work on were significantly reduced.

The Journal of Pain and Symptom Management published a 2002 case study that reported improved dystonia symptoms in a 42-year-old chronic pain patient. Her subjective pain score, which was at a nine, fell to zero after she inhaled the cannabis smoke. She also needed no additional analgesic drugs for the next 48 hours. Researchers said no other types of treatment provided her with significant relief in her condition.

What Symptoms of Dystonia Can Marijuana Treat?

Contorting muscles and repetitive spasms not only characterize dystonia, but they’re also often accompanied by neuropathic pain. The pain can target any extremity, including your hands. Disabling and painful dystonias can occur in your shoulder, neck and facial areas, and prevent you from being able to speak, move or walk if left untreated.

Now, although cannabis for dystonia can’t cure the condition or reverse any side effects, it’s been said that it can, in many cases, eliminate your pain and cause your muscles to unclench and relax to the point where you’re not severely disabled anymore and may be able to function and move normally.

CBD does seem to help relieve some muscle spasming associated with dystonia but doesn’t appear to help as much as it does when it’s combined with high levels of THC. In treatments of CBD without THC, patients don’t seem to get the relief from residual pain.

Marijuana for dystonia helps ease symptoms of severe pain, nausea and appetite loss due to its antiemetic, analgesic and appetite-stimulating properties. It also has antispasmodic properties that help counteract the involuntary movements that go along with dystonia.

Best Strains of Marijuana to Use for Dystonia

Not only does dystonia cause symptoms of insomnia, tremors and pain similar to restless leg syndrome, but there may also be secondary effects of the continuous brain and muscle activity, including:

  • Blurred vision
  • Mental stress
  • Depression
  • Difficulty concentrating
  • Mood swings
  • Disrupted sleep patterns

When you have dystonia, it affects your overall well-being and ability to function normally each day, which can be stressful and depressing. Fortunately, there are some cannabis and dystonia strains that can help with a number of symptoms. The strains for each symptom are as follows:

For pain related to dystonia, try:

  • Death Star
  • Purple Trainwreck
  • Shishkaberry

For tremors and involuntary muscle movements associated with dystonia, try:

  • Purple Wreck
  • Snoop Dogg OG
  • Shishkaberry

For insomnia related to dystonia, try:

  • Death Star
  • Afgoo
  • Purple Wreck

For depression and stress accompanying dystonia, try:

  • Orange Kush
  • Death Star
  • White Berry

Take the Next Steps in Your Fight Against Dystonia by Finding a Dispensary

Of course, you’ll find some conventional treatments for dystonia. For some people, they may be enough. For others, however, they may find these treatments don’t offer much relief, particularly for pain and muscle spasms, so they look to cannabis.

Medical marijuana can help provide relief from dystonia symptoms. All it takes is a simple search for a medical marijuana doctor or dispensary. Dispensaries make it much easier to find prescribing doctors and get your medical weed quickly. There’s no hassle, and you save time.

When it comes to pain, you don’t want to waste any time finding relief. Here at Marijuana Doctors, we can help you improve your quality of life while living with dystonia. There can be ups and downs with dystonia, but living successfully with it is possible.

More Information About Medical Marijuana and Dystonia

What Is Dystonia?

Dystonia is a movement disorder causing your muscles to involuntarily contract. These contractions lead to repetitive movements and twisting, which can be painful at times. Researchers believe faulty brain signals cause muscles to pull on your body incorrectly, spasm and force your body to twist, move repetitively and form into an abnormal posture. Dystonic tremors sometimes accompany these symptoms.

Types of dystonia can be divided into three groups:

  • Idiopathic dystonia refers to dystonia that occurs without an apparent cause.
  • Genetic dystonia refers to dystonia genetically present at birth.
  • Acquired dystonia, also known as secondary dystonia, has resulted from either environmental or other forms of brain damage.

Some individuals inherit dystonia. Others have another disease that causes it. Dystonia can be so severe voluntary movements can make the condition worse.

Possible causes of the condition are:

  • Genetic inheritance
  • Carbon monoxide and lead poisoning
  • Physical trauma
  • Stroke
  • Infections

Pharmaceutical drugs may cause dystonia, particularly neuroleptics, which are often used to treat bipolar disorder and schizophrenia.

Is There a Cure for Dystonia?

There’s no known cure for dystonia as of yet. However, in many cases, the condition doesn’t shorten the lifespan of a person. Dystonia is thought to come from a part of your brain known as basal ganglia.

This group of subcortical nuclei in your brain monitors the speed of movement and controls undesired actions. They’re responsible for sending your muscles signals that instruct them when to move and when to stop moving. With dystonia, these basal ganglia instructions become chaotic and irregular and cause your unwanted muscle contractions and movements.

History of Dystonia

Dystonia’s clinical features were first announced in 1911, reports the International Parkinson and Movement Disorder Society. This was when three authors described several children afflicted by a syndrome that represented genetic cases of DYT1 dystonia.

In 1975, more than six decades later, the first dystonia conference was held in New York. During this time, dystonia was identified, in addition to severe generalized forms, to encompass poorly progressive segmental and focal cases with adulthood onset like torticollis, blepharospasm and writer’s cramp.

These forms were previously classified among neuroses and were considered independent disorders. In 1984, dystonia received a modern definition. Years after, it became apparent dystonia syndromes are diverse and numerous, prompting the introduction of new terminological descriptors, i.e. heredodegenerative dystonias, dystonia plus, etc., and other classifications. Dystonia’s clinical complexity became fully recognized.

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Symptoms of Dystonia

In many cases, dystonia is displayed as abnormal posturing. Opposing muscles will contract at the same time as if competing for total control over a particular part of the body. There are many forms of dystonia, and each case varies in severity.

Symptoms may include:

  • Tremors
  • Difficulty producing speech
  • Trembling within the diaphragm while breathing
  • Lack of sleep
  • Heightened anxiety and stress
  • Similar pain to restless leg syndrome

Due to the constant muscle and brain activity, disturbed sleeping patterns, mood swings, mental stresses, difficulty concentrating and blurred vision are all common symptoms.

Effects of Dystonia

Complications depend on the type of dystonia and may include:

  • Physical disabilities that impact how you perform specific tasks and daily activities.
  • Difficulty with speech, swallowing and jaw movement.
  • Functional blindness affecting your eyelids.
  • Fatigue and pain due to repetitive muscle contractions.
  • Anxiety, depression and social withdrawal.

Some areas of your body and its functions that dystonia may affect include the following.

Common with cervical dystonia, you experience contractions that cause you to twist your head and turn it to one side. You may also pull forward or backward that may cause pain.

Forearm and Hand

Certain forms of dystonia occur when you’re performing a repetitive activity, like playing a musical instrument or writing.

Tongue or Jaw

Typical with oromandibular dystonia, you may experience drooling, slurred speech and difficulty swallowing or chewing. This type of dystonia can become painful and frequently occurs together with blepharospasms or cervical dystonia.

Vocal Cords and Voice Box

You may experience a whispering or tight voice, particularly if you have spasmodic dystonia, which affects the muscles of the tongue and mouth, which may impact voice and speech. You might also find you have difficulty swallowing. Voice, speech or specialized swallowing therapy may help, along with botulinum toxin injections.

Eyelids

Common with blepharospasms, you may experience involuntary spasms or rapid blinking that cause your eyes to close and result in you becoming functionally blind. Although not typically painful, the spasms may increase when you’re under stress, in bright light or interacting with others. Your eyes may also feel dry.

Dystonia gradually develops in most cases. An exception to this may include acute dystonic reactions caused by certain antipsychotic medications and rapid-onset dystonia parkinsonism that could come on within hours or days.

Spasms, Cramping and Speech

You may begin experiencing mild symptoms of dystonia at first, such as difficulty chewing or subtle jaw or facial spasms that suggest early jaw or face dystonia. Your speech may change in rhythm or pitch that suggests early signs of spasmodic or laryngeal dysphonia. Early signs of cervical dystonia could cause mild head jerking movements, local neck discomfort or stiff neck.

Your hands may cramp up or become fatigued during manual activities like writing, or you may have difficulty walking, suggesting limb dystonia. Children developing generalized dystonia might start out complaining of leg cramps or a foot turning in. In some cases, an injury to a particular body area may cause focal dystonia.

Breathing

The dystonia condition can affect your breathing in a few ways. Severe neck dystonia may impact your upper airway and cause you difficulty breathing. You may experience a shortness of breath with dystonia that involves your vocal cords when they close tight, usually when you speak. When you breathe, it requires muscles located between your ribs and your diaphragm, the latter of which is a major muscle of respiration.

Your muscles between your ribs may become stiff due to dystonia causing shortness of breath. Sometimes it may affect your diaphragm. Also, if your dystonia affects your spine, it could cause your torso to twist and make it hard for your lungs to expand when you breathe, causing you shortness of breath.

A Few Important Dystonia Statistics

Statistics on dystonia from the American Association of Neurological Surgeons reveal that:

  • Over 250,000 people in the United States have the condition.
  • Dystonia is the third-most-common type of movement disorder, behind Parkinson’s disease and essential tremor.
  • The older a person is at onset, the more potential for the disorder remaining moderate.
  • Focal dystonia or adult-onset dystonia seems to afflict people between the ages 40 through 50
  • Women are three times more likely to get focal dystonia than men.
  • Among the individuals with focal dystonia, up to 30 percent have spasms next to the primary area.
  • Childhood or adolescent onset of specific primary dystonias have been linked to genetic mutations.

Also, researchers have identified more than 15 different genetic mutations that contribute to dystonia, reports the National Institutes of Health.

Current Treatments Available for Dystonia and Their Side Effects

The initial step in treating dystonia is to learn about the underlying cause. Determining the root cause of your dystonia may have a direct impact on your course of treatment. For instance, a person with primary focal dystonia may require a slightly different treatment approach than an individual who has dystonia related to another neurological disorder.

Because dystonia presents so rarely and varies entirely on a case-by-case basis, standard treatment does not exist for the condition. Physical interventions such as physical therapy have not shown significant progress in their ability to heal patients, though it is not said to worsen the condition. Occupational therapists are often paired with patients suffering from symptoms of dystonia to assist them in daily living activities.

Because physiotherapy has proven useful for patients with dystonia-like symptoms with Parkinson’s disease, the same method of treatment has been used on patients with dystonia. Anti-Parkinson’s medications have been utilized for patients suffering from dystonia as well.

The type of treatment you receive typically depends on what causes your dystonia. In cases of secondary dystonia, treating the underlying condition may improve your symptoms.

In addition to medical marijuana for dystonia, In general, there are four standard methods for treating dystonia, depending on the area of your body it affects and how severe your symptoms are. These approaches include the following.

Botulinum Toxin Injections for Dystonia

Most forms of focal dystonia are treated with botulinum toxin injections. The doctor may inject small amounts of this toxin into your overactive muscles to change the muscle firing and calm the abnormal movements, and it usually lasts for a few months at a time.

Side effects of Botox include:

  • Rash
  • Allergic reactions
  • Itching
  • Back or neck pain
  • Headache
  • Difficulty swallowing
  • Muscle stiffness or weakness
  • Shortness of breath
  • Diarrhea
  • Nausea
  • Loss of appetite
  • Stomach pain

Talk to your doctor about these side effects and if the benefits of this treatment would outweigh the risks.

Medications for Dystonia

Oral medications are usually given for multifocal, segmental and generalized dystonia. These types of medications include anticholinergic drugs and muscle relaxants or anti-spastic agents.

Side effects of anticholinergic drugs may include:

  • Blurry vision
  • Dry mouth
  • Drowsiness
  • Constipation
  • Hallucinations
  • Sedation
  • Delirium
  • Memory impairment
  • Decreased sweating
  • Confusion
  • Decreased saliva
  • Difficulty urinating

Antispastic agents or muscle relaxants come with side effects, too, so be sure you’re only taking them under your doctor’s advice and that you’re being monitored carefully. Some common side effects include drowsiness and dizziness, and the drug carries the potential for abuse.

Physical Therapy for Dystonia

Research into the benefits of physical therapy for dystonia patients remains limited. However, physical intervention is employed to help people with various aspects of daily living. These include improving balance, enhancing mobility, increasing stamina, improving posture and making it easier to accomplish daily living functions, such as going to the bathroom, dressing and undressing, bathing and completing other everyday tasks.

Physical therapy activities for dystonia include strengthening and stretching exercises, along with techniques to increase the range of motion and fine motor skills. Exercises are intended to not only build up underutilized muscles but also prevent weakening of bones. As dystonia is a neurological condition, physical therapy interventions don’t treat the disease directly but rather the symptoms and secondary effects that accompany the disorder.

Surgery for Dystonia

If you have more severe or widespread dystonia that is debilitating, you may benefit from surgery if other treatments have proven ineffective. Deep brain stimulation (DBS) is the most current and widely used surgical approach.

The surgeon implants thin electrodes into a part of your brain’s basal ganglia in this surgery. The electrodes are attached to a device similar to a pacemaker and implanted in your chest wall. The electrodes then distribute controlled electrical pulses to improve your symptoms of dystonia.

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