DRD, parkinson's disease

Dopa responsive dystonia:diagnosis & treatment – by Dr. De León

This is a topic that is long over due since many of you who have Parkinsonism and dystonia may actually carry this diagnosis. At one time, early on in my clinical presentation, I and my neurologist also entertained this diagnosis as the etiology of my symptoms. DRD – Dopa Responsive Dystonia also known as Segawa syndrome in the recessive form.

This is a diagnosis that is often overlooked especially in childhood and early adulthood when most patients begin to exhibit dystonia as is the case for autosomal dominant DRD (DYT5a) caused by a GTP cyclohydrolase 1 deficiency. this deficiency can also be present in autosonal recessive DRD. Further, this enzyme is important in the production of BH4 (tetrahydrobiopterin) which is the substrate that converts phenelalanine to tyrosine. Tyrosine is the first step in conversion of dopamine.  bh4

In autosomal dominant disease symptoms begin early by age 6 often leading to a common misdiagnosis of cerebral palsy. (can also be the cause spastic paraplegia, early onset of generalized dystonia, and of early childhood parkinsonism very frequently).

One of the key characteristics of this DRD is gait dysfunction which worsen gradually with time but typically stabilizes by the third decade. Also more importantly is the diurnal variation in symptom presentation with symptoms being more pronounced in the evening after a full days activity. Improving with rest and sleep. Hence symptoms are usually significantly better in the morning. Plus the symptoms are exquisitely sensitive to dopa requiring very small doses (25-50mg) a day to control symptoms. Although some children and adolescents may develop dyskinesias with initial low doses of dopamine they always resolve with a lowering of dose and do not return with increasing doses again.

Unlike Parkinson’s, this is not considered a neurodegenerative disorder rather a metabolic abnormality. Typical symptoms include uncoordinated, clumsy gait something I had all my life. I lived my childhood with scraped knees and torn tights/leggins. Plus these patients are born with with club feet something I also had which required casting for my entire first year of life. So symptoms begin in lower extremities then migrate over time to involve upper extremities bilateral..again something I had. So of course this was high on list of suspects.

In general patients progress to develop generalized dystonia- I don’t have this. Furthermore, although some depression and sleep disturbances can occur with this syndrome, DRP patients do not exhibit autonomic dysfunction, intellectual, or sensory problems (common of PD) nor cerebellar disturbances (which are common of parkinson’s plus syndromes like MSA).

Rarely, these patients develop symptoms in adulthood instead in which case parkinsonism is the predominant feature rather than dystonia. unlike childhood onset these do not have a diurnal variation. But, on the plus side the movement disorder progresses extremely slow.

Diagnosis: ( I underwent genetic testing and had normal levels of these enzymes but did have an abnormality in another enzyme – which i have to find – of unclear etiology. Hence i am listed as rare cause of PD/dystonia at NIH)

  • as always a good history is a MUST! personal and family.
  • Genetic testing – as i did-look at DYT5 (dominant-GTPH1); look at tyrosine hydroxylase –TH deficiency (recessive)- this enzyme is the limiting factor in the pathway production of dopamine*; sepiapterin deficiency (both autosomal &dominant in children)
  • Mri’s of brain/dat scan normal-there is NORMAL uptake of dopamine
  • Spinal tap and look at CSF enzyme levels
  • blood and urine tests
  • sometimes muscle biopsies ( i almost had)

Although there is still much we don’t know about this illness especially since it has variation in presentation depending on age as well as enzyme involved and penetrance of gene (having the abnormality does not guaranty disease like many other neurological illnesses).- some even think there may be a DRD plus syndrome. Note_ so unless there are symptoms, there is no need to go fishing for genetic testing that might show abnormality that may never develop into anything.

Treatment is symptomatic with mainstay treatment being Levodopa! most patients do well with a dose between 200-400mg a day of levodopa.

In extremely rare cases there have been reports of patients having both DRD and PD- could I be one ? after a decade my dopamine dose remains low compared to my counterparts with same years of illness however, i also need many other dopamine agonists and MAO inhibitors to function ..so i guess time will tell especially if NIH ever links other illnesses to my rare enzyme deficiency.

Find a Movement disorder specialist whom you can talk and listens to you and follow up routinely. Patients have done well with low dopamine for more than 2 decades. want to prevent contractions so PT and OT are crucial. this may also involve treatment with botox and centrally acting muscle relaxants like baclofen, dantrolene or baclofen pump. As well as rest to replenish dopamine.







All rights reserved Maria De Leon



depression and suicide, Dystonia and parkinson's, dystonia treatments, parkinson's disease

Role of Marijuana in the Treatment of PD & Dystonia: Weeding through the Hype:By Dr. De Leon

AS consumption of recreational marijuana has skyrocketed over the last few years reaching 20 million people in the last month alone, the use in medicine has also begun to loosen in several states. But is this a good thing? or are we setting our selves for greater disappointment and worst problems down the road? My personal opinion is a resounding Yes!  Any drug that heightens the depressive factors and magnifies the potential for memory loss, sleep problems, slows reaction time, causes dizziness, paranoia, anxiety, and hallucinations in a disease that already has the potential to do this can’t be a good thing….

In fact, there is a 1% risk of serious psychiatric side effects including suicide…this is a risk I as a d PD doctor would not be willing to take on behalf of my patients who already have tendency to get depressed and become suicidal.

However, I know many a patient that swear by the medicinal effects of this drug. So, is this a placebo effect or is there something really to this?

The American Academy of Neurology just spent an inordinate amount of time reviewing this issue. The first statement they said was “weed may be easy to find but finding credible information about its role in medicine is becoming increasingly challenging.”

Part of the challenge in identifying its efficacy in neurological disease such as PD or dystonia is the number of preparations available on the market which include synthetic which mimic the effects of whole marijuana plants or its extracts. Furthermore, the plant can be consumed in a variety of ways which then can influence its chemical composition, dosing and efficacy. For instance smoking it can have a completely different effect than if it is chewed or eaten. Another challenge in determining its potential benefit is the lack of quality control for testing, labeling, etc.  Thus, like most holistic remedies which are outside of the FDA jurisdiction have no objective consistent information about its effects on patients upon which physicians can make reliable informed decisions to treat their patients.

Yet, despite all the limitations to their review the AAN report concluded that to date the use of smoked marijuana is uncertain but has shown to worsen posture and balance. Oral cannabinoid drugs were not considered effective in the treatment of levodopa induced dyskinesias.

An open label observational study revealed improvement in pain and sleep in PD however because it was open there is potential for a lot of bias and more research is needed in this area. another study done in Israel revealed improvement in pain and tremors of PD patients however tremors in MS patients were not improved …

As far as cervical dystonia is concerned in a study partially-funded by DMRF, the results were inconclusive necessitating further studies to confirm or deny efficacy. But of concern to me is the fact that dystonia when hereditary and familial starts at a young age and instituting a drug like marijuana at a young age before brain has completely developed could be more detrimental. According to a study looking at routine marijuana use in young people, those who smoked pot routinely had smaller brains, less white matter, lower IQ’s, more psychiatric problems, even caused poor driving. We need more extensive research into the effect of prolonged use in adults as well before we as physicians can sign off on it as a routine therapy, I believe.

Nevertheless if you are still interested- A word of caution: when considering new treatments not approved by FDA or non traditional treatments ALWAYS consider the SOURCE CAREFULLY when pondering over anecdotal information especially on the internet!!! the internet can be a cesspool of unverifiable accounts of all sorts of treatments including marijuana which could potentially cause a great deal of harm if not discussed with the physician first.

But, I know that this will not deter those who are determine to find an alternative treatment to their disease – if you are thinking about it before you do, you should follow these simple guidelines for your own safety:

  • Always initiate the subject of outside non-traditional treatment interests as well as complementary treatment options.
  • Openly discuss with your physician the risks, benefits, safety issues, possible interactions, concerns you and physician might have.
  • only after an open discussion will your physician be able to make full informed decisions about your disease and make strategies to avoid unsafe conditions and interactions.


Sources: “Weeding through the Hype :American Academy of neurology Reviews Use of Medical Marijuana:”(Winter 2015) Dystonia Dialogue 20-21.

dystonia in PD, Parkinson's Diagnosis, parkinson's disease, Parkinson's Health, Parkinson's symptoms, treatments in dystonia withPD

Rare Dystonia Syndromes and Parkinson’s Disease: By Dr. De Leon

About a year before my dad passed away, he and I were at the store about to enter the store to do some grocery shopping when comes along a lady stooped at 90 degree angle pushing a buggy full of groceries. My heart immediately sank at this site. This lady apparently had been shopping all alone.  I wonder how it was possible to live like this (bringing back memories of some of my dystonic patients further gripping my heart). Sensing my anguish, before I could say or do anything being completely paralyzed at the site of this phenomenal woman who ventured all alone despite the pain she must be have likely been enduring, my father quietly stated- “I so hate that you are sick.”

I turned and looked at him with intrigue in my eyes. “But dad that poor woman…” as I trailed off he said: “I know you are a great doctor and if you were working I bet you could help her get better.”

A lump formed in my throat thinking about this person and so many more that could use my skills as a doctor, after all I was nowhere near as disabled as this person in front of us.

Before I could get to her someone came and help her load her truck and I had to watch and see how she was going to drive. Sure enough as I though, when she climbed unto the vehicle and set down she was able to straighten up.

My dad was amazed at this. I explained to him this was a form of dystonia (abnormal sustained involuntary contraction of the muscles) called Camptocormia (a.k.a bent spine). This is a dystonic posturing linked to atrophy of the paraspinal muscles. This is posturing is known as one of the extreme postures impairments linked or associated with Parkinson’s disease (PD). Camptocormia in Parkinson’s is defined as extreme anteroflexion of the trunk which abates with recumbent position or standing up against a wall or hard surface. Thus this lady possibly had PD –dystonia at the very least. PD patients who have this tend to have early involvement of axial muscles and long duration of illness as well as tend be older of male gender with history of spondyloarthric spine increasing risk of developing camptocormia. This particular dystonia does not respond to levodopa or have very minimal response. Interestingly, the Pet scans looking at dopamine transporters of PD patients with and without Camptocormia are identical.

Although dad knew little of neurology or dystonia he had a good 6th sense and was correct in assuming this woman’s condition was in my area of expertise. I frequently think of both that lady who I have not seen again and my dad who is now gone to be with the Lord. Yet, the resolve to understand the connection between the two movement disorders in an attempt to find relief to so many people in need has only strengthen with time.

There are two other dystonic syndromes involving the AXIAL muscles commonly seen by MDS that are frequently related to parkinsonian patients:

  1. Head drop (anterocollis- cervical dystonia)
  2. Pisa Syndrome- a condition in which there is sustained involuntary flexion of the body and head to one side and slight rotation of the trunk when sitting and standing. This gives the appearance of leaning tower of Pisa – hence the name! This condition also commonly seen in schizophrenia.

These two are typically related to Multisystem atrophy (MSA)! However, Pisa syndrome can be seen infrequently in PD as well.  Those that have this syndrome tend to have lower body mass index; higher disability due to longer duration of disease and more advanced disease. Subsequently, PD patients with Pisa syndrome had poorer quality of life partly due to increased falling. Severe osteoporosis and arthrosis were significantly part of the life of these patients as well. Although, anterocollis is a sin-qua- non of MSA in its early presentation but when it occurs in the late stages of PD it is invariably due to medication effect (wearing off).

Despite the fact that dystonia is the third most common movement disorder after essential tremors (ET) and PD is still not well recognized or understood; even though there have been many advances in the last several decades identifying specific genes causing dystonic syndromes.

One of the main problems in dealing with dystonia from a medical point of view is that it is both a symptom as well as a diagnosis of primary neurological disease.  This notion has always intrigued me particularly after I developed YOPD and my initial symptoms as it is common in the majority of YOPD to commence with some sort of focal dystonia. Despite the fact that I have been trying for a number of years to get the Parkinson’s foundations to work side by side the dystonia foundation to focus on the common path ways.  After a decade, I am happy to report progress by a final acknowledgement of dystonia as a presenting symptoms of PD by the MJfox foundation. Further, they are beginning to recognize a pattern of presentation which is essential in the diagnostic armamentarium of every physician (who depend on pattern recognition) but also for the PD community to be aware of the possible initial symptoms.

Keeping above in mind we also have to remember that dystonia itself is not a common /classic presenting symptom of PD. However, when it is the presenting symptoms it’s in YOPD. In YOPD, dystonia is more commonly isolated in the lower limb as it was my presenting symptom.  As a symptom in general isolated lower limb dystonia is very rare in adults. This dystonia is also referred as kinesigenic foot dystonia this is because this type of dystonia is brought on by exercise and/ or movement of the foot- typically causing toes to curl involuntarily and sometimes exaggerated hyperflexion of foot  causing much pain and discomfort.
Common trigger is putting on shoes for women particularly if attempting to put on high heels as they cause us to arch our foot even further.

Treatments: Vary depending on cause

As with any illness – the key to successful treatment is first deciding its cause- whether the dystonia in any individual is a symptom of an underlying neurological disease or a disease itself such as primary dystonia or Dopa- responsive -dystonia (DRD)?

Once you have ascertained that these syndromes are part of Parkinson’s or Parkinson’s plus disorders then we must consider their treatment.

  1. Anterocollis– Commonly causes neck pain and cervical radiculopathy. Botox works wonders; adjusting PD meds; therapy, cervical collar & DBS. Must recognize and treat early before cervical damage can occur and or cause fracture of c-spine!
  2. Pisa syndrome– Commonly causes back pain. Discontinue using antipsychotic drugs and usage of amantadine and other anti-cholinergics; no good treatment for those caused by neuroleptics.
  3. Camptocormia-  Commonly causes back pain and lumbar/thoracic radiculopathy. Since levodopa has little or no effect we must consider alternative treatments. These include: withdrawal of causative drugs like antipsychotics or neuroleptic drugs such as Haldol; orthotic aids; Physical therapy; steroid injections; Botox injections which have proven of great value in many patients with this type of dystonia- would be my first choice after excluding triggering causes/medical causes; muscle relaxants – baclofen, dantrolene, etc.; tetrabenazine; amantadine; and surgical intervention -such as DBS- deep brain stimulation- subthalamic
  4. Lower limb dystonia– start dopamine agonists; levodopa; physical therapy; foot orthotics; Botox; muscle relaxants; DBS when necessary.

In summary having one of the first 3 syndromes in the presence of PD is a sign of disabling advanced disease leading to higher comorbidities which in turn can lead to spine fractures of neck and back requiring surgery if not properly treated and managed early in its presentation. The latter two frequently result in severe falls due to loss of balance and “veering” of gait. However, presence of lower limb dystonia in a young adult could signify the onset of Parkinson’s disease. Thus, need to follow up with a neurologist/ MDS for all these types of dystonia for a better prognosis and quality of life.


Pisa syndrome in Parkinson disease

 R Djaldetti and E Melamed   Camptocormia in Parkinson’s disease: new insights.   J Neurol Neurosurg Psychiatry. 2006 Nov; 77(11): 1205.
 Sako W, Nishio M, Maruo T, Shimazu H, Matsuzaki K, Tamura T, Mure H, Ushio Y, Nagahiro S, Kaji R, Goto S. Subthalamic nucleus deep brain stimulation for camptocormia associated with Parkinson’s disease. Mov Disord. 2009 May 15;24(7):1076-9.
Finsterer J, Strobl  W. Presentation, etiology, diagnosis, and management of camptocormia. Krankenanstalt Rudolfstiftung, Vienna, Austria. European Neurology. 01/2010; 64(1):1-8.

Dolhun, R. The Michael J. Fox foundation for Parkinson’s Research. (September 2015) :”Dystonia and Parkinson’s Disease.” Practical Neurology. 14(7):43-46.

Parkinson's Health, Parkinsons disease, parkinsons symptoms, parkinsons treatments

How to Deal with the 6 Common Causes of Leg pain in PD? : By Dr. De Leon

One of the biggest complaints I hear from people in PD support groups is a continuous relentless severe leg pain. Prior to a decade ago, I as all my fellow movement disorder specialist would have not thought leg pain to be a direct precursor of PD or an initial non-motor symptom. My grandmother often complained of pain and deep aches in her calves and in her legs which started before her tremors and shuffling were noticeable. But, I was unaware of the connection at the time and erroneously assumed her pain was neuropathic in nature due to her diabetes but was always somewhat surprised that she continue to complain of this pain on and off throughout her illness despite neuropathic medication. With hind sight what she was experiencing was central pain of PD. I too had severe pain first in one leg then the other which would come on suddenly without warning stopping me in my tracks throughout the day. I was constantly asking my husband to massage my legs just as my grandmother had asked of us time and time again.

So why do we have leg pain in PD and what can we do to relieve the discomfort?

First, some believe that lower limb pain is a specific non-motor phenotype variant of central pain in Parkinson’s disease. I, too, believe this; more importantly it can be one of the very first signs of PD as it was for me. This pain is usually bilateral.

Second, leg pain can also occur secondary to dystonia as an initial symptom or as a consequence of long term levodopa use (most common). When related to levodopa it usually occurs as a wearing off but can also occur at peak dose. In most cases this leg pain is unilateral and has direct correlation to medication intake.  When is due to dystonia pain is more common in early morning. This type of leg pain is usually accompanied by toes curling and foot abnormally posturing. dystonia in feet

Third, musculoskeletal pain due to rigidity, abnormal posturing and lack of mobility affects legs commonly causing pain in the legs, however this pain is usually more pronounced on the more affected side.

Fourth, pain in legs can also be caused by radiculopathy; nerves can become trapped or temporarily pinged in the spine or as they exit the spinal canal due to stiffness/rigidity of the muscles which exert an abnormal lordotic (curvature) of spine. Once again, this type of pain is usually confined to only on one side of the body and is positional meaning it is worst with standing and sitting and relieved by laying down. Pain usually radiates from back or hip down to leg and can also get worst with coughing or straining.

Fifth, pain in legs can also be due to medication effects or withdrawal from certain types of medicines like NEUPRO. In the latter, the pain which can be in both legs is more cramping.

Sixth, Let’s us not forget that we do not live in a vacuum and that just because we have PD does not make us immune to other common diseases such as peripheral vascular disease (PAD). Men are more likely to have this but women are not exempt. Risk factors include diabetes (remember PD may increase this risk), high blood pressure (again some PD meds may increase this risk), heart disease, high cholesterol, smoking, stroke, kidney disease.

The symptoms of this are:

– walking fast or uphill or for long periods to point of hurting immediately when walking

-Feet and legs feel numb at rest and skin is pale and cool to touch

Symptoms are worse with elevating legs and better with dangling over the bed.

Sometimes pain in legs can be a combination of all of the above.

Pain can be the most disability of all PD features interfering with all activities of living. Despite this fact it is often under treated and frequently overlooked. Any pain in PD should be promptly and effectively treated especially that of leg pain before the pain becomes chronic and your brain reorganizes itself completely to be able to handle the pain. I feel terrible because I did not fully understand the phenomena of central pain in PD at the time of caring for my grandmother. Fortunately, with adjustment of her levodopa meds her pain subsided for the most part. As I said before we have come a long way in understanding pain in PD, so there should be no reason why anyone should be subjected to dealing with pain on a daily basis when we have so many treatment options.


Treatments therefore depend on properly identifying the source of pain.

  • If bilateral always assume it is central pain- pain due to PD and treat accordingly. As I mentioned many times before, Azilect works great for this type of pain.
  • Massage therapy works for all types of leg pain-my favorite.
  • If having pain due to dystonia first find out if occurring at end of dose or at peak dose so meds can be adjusted. If medication adjustment don’t work consider DBS. Pain due to dystonia also responds well to Botox, Myobloc, or Dysport injections, baclofen, Dantrolene, and Klonopin work well alone or in combination with other treatment modalities. Physical therapy (PT) can go a long way to alleviating pain of this type.
  •   If having radicular pain try trigger point injections, epidurals, nerve block, surgery, DBS (deep brain stimulator) for pain in the spine, Botox, Lidoderm patches, muscle relaxants, anti-inflammatories and steroids and PT.
  • To avoid and alleviate pain caused by stiff muscles the best treatment is activity in the form of stretching exercises- any number of activities will do such as walking, tai-chi, water aerobics, swimming, dancing, bicycling, yoga; of course if needed can use a Tylenol plus a Motrin or Advil as needed. Sometimes may need to up levodopa if stiffness is persisting or add a centrally acting muscle relaxant like baclofen or Neurontin.
  • To avoid cramps stay well hydrated. Make sure your patches don’t fall off! Eat food high in potassium like avocados, bananas, and strawberries. When cramp hits quick remedy spoonful of mustard with warm water or take some pickle juice.
  • If you have symptoms of PAD or suspect consult your physician immediately this can be a life threatening problem!!
Dystonia and parkinson's, Parkinson's Diagnosis, parkinson's disease, Parkinson's Health

Tips to dealing with cramping toes: By Dr. De Leon

  • Purple Clover's photo.

It is extremely painful to have one little toe or worse all toes suddenly decide to have a mind of their own and begin to curl, twist and bend at their own free will. Believe me, I know. This type of pain can bring a grown man to their knees.

When I first began having symptoms of PD, I noticed that every time I arched my foot to put on my shoes especially if the shoes had a narrow circumference, the shaft was high  (e.g. boots),my toes along with my entire foot would curl up into an extremely painful contorted mess that could only be relieved by stoking deeply for several minutes. At first I thought I had cramps due to potassium deficiency since I was on diuretics (water pills) for my blood pressure. But, since they were potassium sparing of course this was not the case. Since I love shoes, this little problem was turning into a huge problem since I could no longer wear most of my shoes. Each time I attempted to wear high heels even medium heels my toes would automatically curl down. Because of the exaggerated angle in which the foot would have to be positioned  wearing these shoes not only was it difficult to put on but even more painful to wear often causing me to be off balance. Now, I was forced to make a drastic change to my wardrobe throwing away most of my shoes including all my favorites to prevent falling and pain.

For the first time in my life I was wearing tennis shoes all the time (extra wide) with little to no arch.

Although, cramping toes can be one of the initial presentation of Parkinson’s disease as it was for me it can also be a sign and symptom of many other possible conditions.

 In fact, cramping toes usually are a sign that medications are causing side effects ( as I alluded before especially in the elderly many of whom take water pills for heart problems), not working very well, and a first sign of Diabetes and other neuropathies (loss of nerve endings).  Yet, if you already have a neurologic illness such as PD or idiopathic dystonia, the presence of these are an indication of poor medication control.

What to do at first sign of cramping in toes/ legs /foot:

Go to PCP to make sure your electrolytes are okay like potassium, magnesium and calcium (have both your total calcium and ionized calcium).

Toes cramping can also be a sign of diabetes, as I stated earlier. So your doctor may have you undergo a blood sugar tests especially if older or have risk factors like family history or repeated use of steroids. Usually they will order a 3- hour glucose test but others prefer a 5- hour test. This requires you drinking a nasty sugary orange drink while fasting while you have your blood drawn at baseline then every 1/2 hour to an hour for the duration of test.

2) There is a condition known as painful legs moving toe syndrome which also can cause this problem. This extremely rare, usually in adults. Pain is that of nerve burning, searing. This is associated with writhing movement of  one or more toes and can be in one foot or both. sometimes can have movement of toes without pain- “painless legs- moving toes

3) this can also occur in dystonia syndromes – focal idiopathic or secondary dystonia as seen with PD and other neurological disorders (e.g. Cerebral palsy, DRD, parkinsonism).

If all blood test initially are normal and symptoms persist seek opinion of a Movement disorder specialist (MDS).

If symptoms are determined to be neurological and due to PD as were mine then starting treatment with dopamine agonists or levodopa will dissipate the pain and stop the involuntary contraction of your toes. No longer was I bound to wearing tennis shoes all the time! Too bad I had already thrown half of my shoe collection away.

if are already a season PD patient and are now  having this problem: note time of onset and duration along with relation to medication intake. Does is it commence at end of dose, at peak dose, or beginning of medication? This is crucial to help your MDS best adjust your medications to stop your symptoms. If adjusting meds do not help Botox and even DBS can be done.

As an aside: Sometimes NEUPRO can cause cramps when patch wears off or if it falls off. I have noticed that suddenly I start cramping so I look for my patch to find out it has slipped due to humidity and sure enough it has most of the time.

As I previously mentioned neuropathies can trigger these problems, make sure that if you are a PD person your doctor is checking your  B12  and Vitamin D because these deficiency are not only common in Parkinson’s but can cause neuropathy.

Treatments of Curling Toes: will depend largely on the cause but there are some symptomatic treatments. Finding the etiology is key to prevention and successful treatment.

  • Mustard- spoonful of mustard with water- carry some packets with you in case
  • (sour/dill) Pickle juice- when I first started having symptoms I became like all my patients with jars full of dried pickles in the refrigerator.
  • Tonic water (Gin & tonic maybe not so much) a glass or 2 a day-
  • No quinine- no longer used because potential for heart problems
  • B12 injections/ nasal spray/sublingual
  • B1- thiamine 100mg tablets daily- not enough to take multivitamins ( better to take B complex vitamins but may still need to take extra)
  • Neurontin ( or Lyrica)
  • Dopamine medications
  • Klonopin (other anxiolytics)
  • Baclofen ( other centrally acting muscle relaxants)
  • Botox injections for focal dystonia when meds not helping ( may also use other forms of botulinum e.g. disport, myobloc)
  • DBS
  • Sugar control (diet, exercise and medications)
  • Replace electrolytes- K, Mg, Ca
  • Replace Vitamin D
  • parathyroid/thyroid replacement

Great sources of Potassium:

  • Avocados (also rich in Magnesium)
  • Bananas ( Magnesium source as well)
  • Strawberries
  •  Popcorn

Great sources rich in Magnesium:

  • dark  leafy greens ( also have lots of B12 vitamin)
  • fish (mackerel)
  • nuts and seeds -(pumpkin seeds)
  • beans and lentils
  • dark chocolate ( my favorite)
  • non-fat yogurt
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Tips to Prevent and Treat Neck Pain Caused by PD & Dystonia: By Dr. De Leon

” I got bone spurs that jingle, jangle, jingle.” ~ unknown

Neck pain is an extremely common problem which afflicts all of us who suffer from Parkinson’s disease at one time or another. Also is a frequent source of disability for those who have cervical torticollis, and generalized dystonia independent of etiology.

The cause in both cases is increase axial rigidity (stiffness of any of the skeletal muscles that support the head or trunk). The neck joints are on a pivot which allow for sideways movements while the fact that the head rest on the atlas (named after Greek god “holding the weight of the world”  while sitting in second vertebra called the axis allows for front and back movements. …

From the moment we awake until we go to bed these muscles are constantly being pushed in all directions. It’s no wonder that as we age they begin to deteriorate. Now add a chronic neurodegenerative disease like Parkinson’s or dystonia independent of cause and we are bound to have pain as our head and neck muscles are trying hard to compensate for abnormal posturing all throughout the day.

Pain is believed to occur in 30-50% (can go up to 85% when all causes of pain are taken into account) of all patients with PD. Although extremely common, pain remains under-recognized as being one of the non- motor symptoms of PD.  The important thing to remember is that pain can appear any time in the course of the disease and even be the presenting symptoms of Parkinson’s, as it was for me.

Predisposing factors for pain in Parkinson’s are:

  • Agedys
  • Gender- more common in women
  • Duration of disease- worst as Parkinson’s disease advances due to increased rigidity of neck; but also to wearing “off” causing dystonia’s and motor fluctuations causing dyskinesia’s.

Pain is frequently under treated in the settings of both PD and dystonia but even more so in the Parkinson’s population, this is why I felt the need to talk about it today.

Although, pain in dystonia is more frequently accepted and recognized by MDS other physicians are not as attuned to this problem. Therefore, cervical pain in dystonia is still poorly managed and treated; even though 75% of patients with dystonia complain of some type of pain with 7/10 sufferers rate their pain moderate to severe. In fact when cervical dystonia sufferers were compared to PD patients in QOL (quality of life) they scored just as poorly. Yet, these patients scored lower in physical limitations than PD despite having higher physical function scores.

So, we have a whole population of over 1.5 million people living in pain and unable to get on with their lives and at times not even be able to do basic daily activities of living. This in my opinion is simply unacceptable and we have to be more proactive in our care advocating for better treatment. This entails going to the doctor as soon as pain begins and not wait so cause can be found and appropriate treatment instituted.

Poorly controlled PD & dystonia symptoms as well as over medication in PD patients is the number one cause for cervical pain in my experience often leading to severe spondylosis (narrowing of canal) and herniated discs of the neck.

Cervical Dystonia more common:xray of anterocollis

  • In women
  • In the US 30/100,000> Europe 1-2/100,000
  • Ethnicity- Jewish Ashkenazi

The key to successful treatment of neck pain in both groups is rapid diagnosis along with immediate, effective treatment to prevent acute pain from becoming chronic and affecting a person’s day to day living.

The reason for advocating rapid effective treatment is to prevent brain changes from setting in; with chronic pain the chemical composition of the brain alters as well as it remaps itself after experiencing prolonged pain.  The pain center in the brain moves and expands out of the usual sensory centers (parietal lobe) to encompass areas of memory, and emotion causing people to become irritable, quick-tempered, and impatient. This is because chronic pain increases the threshold of our ability to focus (trouble multi-tasking) on every day activities. This transformation  occurs because the brain’s area responsible for processing emotion begin to deteriorate at a faster pace than normal. We then expand so much energy trying to deal with pain that even a minor incident like being caught in traffic for 5 minutes can set us off!

This in turn can lead people to go in search of solitude manifesting as isolation creating a vicious cycle of depression, hopelessness and pain intensification.
Many years of experience have shown me that  the best way to combat pain is to bombard it with whatever necessary to halt the process before it becomes an insidious recurring problem.

This is the one exception to the rule of more is not always better!

Unlike other symptoms of PD where we don’t want to just keep adding medications, in pain particularly those caused by nervous system e.g. radicular pain has to be stopped before the spine and brain have a chance to rewire itself?

Cervical pain treatment:

  1. The first thing is prevention! it is always easier to prevent than try to fix a problem.
  2. Second, treat acute pain still easier to treat with less disturbance of a person’s QOL.
  3. Third, treat chronic pain appropriately to break cycle.

Effective interdisciplinary treatment is necessary to efficiently reduce pain in our lives. This includes :

  1. physical therapy
  2. occupational therapy
  3. acupuncture
  4. massage therapy
  5. Botox (dysport, myobloc)
  6. exercise
  7. balanced diet
  8. sleep regimen
  9. adjust dopamine medications
  10. Bio-feedback/counseling
  11. DBS
  12. Neck surgeries when necessary or as last resort-e.g. laminectomies
  13. prescription pain medications :
  • anti-seizure-e.g. Neurontin, Topamax, Tegretol, Keppra;
  • anti-depression-e.g. Elavil, Cymbalta, Effexor;
  • anti-anxiety-e.g. Klonopin;
  • muscle relaxants-e.g. baclofen, dantrolene, zanaflex, relafen;
  • topical anesthetics-e.g. Lidoderm patches, Flector patches;
  • injection- e.g. steroids and Toradol or invasive epidurals, nerve block baclofen pump, Medtronic’s spinal cord stimulator (Neurostimulation);
  • anti-inflammatories-e.g. Celebrex, Mobic, ultram, ultracet; and occasional narcotics -e.g. vicodin, hydrocodone (as acute treatment or short course to break cycle in chronic pain -do not recommend long term use because will worsen symptoms of PD and interfere with medications used for treatment of both PD & dystonia; should not be necessary in most cases if above followed properly)