Dealing with the 50 shades of pain in Parkinson’s to avoid addiction-by Dr. De Leon

Pain is only relevant if it still hurts.” Ed Sheeran

I was reading an article recently regarding the epidemic of pain killers in women in the USA and recent changes in healthcare in an attempt to curtail this problem. It got me thinking about pain as an initial presentation in neurological diseases especially in Parkinson’s disease. Like many other neurological illnesses, PD has a big pain component which varies from person to person. It is believe that 40-80 % of people who have PD have pain depending on the source. The pain can occur at any stages of disease and can take many shades within the same individual even if there has been no advance to the disease from one stage to another. It is of note that pain can be an initial presentation even before motor symptoms manifest themselves. As it was the case for me and many other women particularly of young onset. Thus, pain is more frequent and more pronounced in younger patients especially as an initial presentation. However, because pain is subjective it is too often misunderstood and misdiagnosed by many physicians. Hence, I believe this is the number one reason the authors of Opioid Addiction: The Gender Gap, stated that “addiction is much more common in men than women killing more women per year than men.”

Since, women with young onset PD have a higher rate of pain at presentation. They are the ones to be more likely misdiagnosed and also placed on opioids for chronic intractable pain. Hence it is imperative that we take a proactive approach to getting not just diagnosed earlier but starting treatment early. Furthermore, getting our pain treated properly to avoid going down the slippery slope of opioid addiction with a potential increased risk of mortality for women compared to men. This is because women across the board not excluding PD have more chronic pain then men. I for one have pain as one of the major impacting symptoms of my Parkinson’s disease which disrupts my life more than any other single symptom. However, the key to getting the right treatment is getting the right diagnosis as the source of the pain. In the case of Parkinson’s – there should rarely be a time where opioids (like hydrocodone, fentanyl, morphine, codeine, etc.) are required. In my experience, because patients have an increased risk of herniating disc the initial pain that comes from a pinched nerve will only respond to strong pain killers like hydrocodone and should not be withheld. However, the intake of these should have a set finite amount of time. They are not meant to be taken long term where addiction is most likely to occur.  Having experience multiple types of pain as a patient from dystonia, to joint pain, to thalamic central pain and herniated disc I can attest without a doubt that dopamine agents will treat and reverse the majority of pain syndromes without the need of opioids. I took a few doses of hydrocodone prior to my back surgery since it was the only thing that would break the intractable pain and then a day or two post-surgery. However, in my practice the use of Vioxx was excellent for joint pain caused by PD and no other cox2 inhibitors since its recall has been able to mimic this response in any of the people I have treated or talked to since. There was something definitely special about that drug that helped all PD patients I ever put on. However for my central –non localized, intractable boring pain. Azilect was a life saver. I understand this has been the case for many of whom I spoken to as well the experience of my physician with many of her patients.

As I said at the beginning, there are many types of pain in people with Parkinson’s. One of the most common and frequent pain syndromes is radicular pain. This occurs from severe neck and back rigidity as well as dystonia’s and dyskinesia’s which can distort the normal curvature of the spine.  The lack of sufficient dopamine in the brain is typically the cause for the abnormal postures as seen with stooped posture or increased kyphosis can lead to stretching and pinching of nerve roots mimicking symptoms of radiculopathy. This has happened to me many a time, all I need is an adjustment of my medications; usually a higher dose to solve the problem. However, in my experience, not only can a PD person have pinched nerves in the spine but can have symptoms of trapped nerves like carpal tunnel and ulnar syndrome caused by severe rigidity of muscles elongating nerves passing through. No need for surgery for most of these problems usually increased dopamine dosing is the answer without need to recur to opioids either.

A deceased level of dopamine is the common culprit often times for dystonia and joint pain. The increased muscle stiffness can be so profound as to actually dislocate joints. Patients with PD often times have a shoulder dislocation but can also involve other joints rarely. Thus, before you pursue any other treatment ensure that your PD medications are at their optimal. Perhaps also use centrally acting muscle relaxants like baclofen and toxins like Botox.

In cases, where dyskinesia’s have caused the pain by distorting curvature and musculature of axial muscles and even herniated disc, the first order of business is treat the problem –STOP the dyskinesia’s either through medication or surgically (DBS and other) or combination. Then you can begin to treat the pain by performing neck/back surgery if necessary and using prophylactic medications anticonvulsants-like Keppra, Topamax, anti -depressants in the tricyclic family such as Elavil, Remeron and antidepressants in ssri and snri family- like Cymbalta, Effexor and so on.

Thus, as Parkinson’s community opioid addiction as a whole should not be something we need to contend with if we follow the mantra that the majority of pain syndromes CAN and should be treated by increasing and adjusting PD medications as a first step.

sources:

Kam Katherine. (September 2017) “Opioid addiction: the gender Gap” medical editor- Neha Pathak, WeBMD magazine. 69-71.

 

copy right2017

all rights reserved Maria De Leon

 

 

 

 

 

 

Is numbness in my feet caused by my Parkinson’s disease/dystonia? : By Dr. De Leon

“I have become comfortably numb.” Unknown

On a weekly basis, I get the question can PD cause numbness? Well today, I thought I would tackle this sensitive topic from a different angle than before. hopefully this will help so many of you who like me are  plagued with this problem and are not sure where to turn. Image result for images of radiculopathy

First, let me explain a couple of things. we have learned in the last decade or so that Parkinson’s disease can cause pain and central sensory deficits. this means that the brain does not process sensation normally because these connections pass through the basal ganglia on their way to the thalamus (sensory control center in the brain). This usually manifest in abnormal sensations when touch is involved. Plus, this abnormal basal ganglia dysfunction is the source for what we call “central” pain in PD. This is a burning, searing, excruciating pain that is constant deep in the bone aggravated by touch and follows the normal pathways of the brain – usually unilateral but can become bilateral. But, this does not cause numbness or tingling typically. as you might recall one of my initial symptoms was pain- this central pain. I could not stand anyone to touch my limbs which started on my left side, showering was torture. I felt like the water touching me was pure acid.

However, peripheral sensory deficits like toe, feet, and hand numbness has not been shown to be caused by PD itself. The usual test we use to detect neuropathies  called  NCV (nerve conduction velocity) study are normal in PD. Yet, many of us have been down with severe numbness particularly in our lower extremities.

So, why is this?

Well you are neither alone nor crazy. Well, I think I have a logical explanation for these symptoms from my own experience, living with my Parkinson’s and also having treated many patients in the past who regularly complained of this problem.

The cause is a simple mechanical problem. I have had two herniated discs in the past and know full well the symptoms of radiculopathy – numbness and pain shooting down your leg along with accompanying weakness. Of course, the first time I had numbness in my feet and back pain I immediately thought I was having exacerbation of my injured back. however, I had no pain nor weakness only numbness. I discovered that the usual treatments for back spasm like Lidoderm patches, Nsaid’s (non-steroid anti-inflammatories), wearing a brace did not alleviate this problem completely. However, increasing dose of dopaminergic medication e.g. levodopa stopped symptoms instantaneously as soon as medicine kicked in.  Also, over the years I have noticed that when muscles get very tight i.e. dystonia especially in the neck and arms it can mimic cervical radiculopathy, carpal tunnel syndrome, and ulnar neuropathy. as a matter of fact when I was in residency because I was starting to get dystonia carrying my heavy doctors bag I was erroneously diagnosed and given a splint for carpal tunnel. since it was difficult to carry my bag with the brace the symptoms stopped abruptly.

As far as foot and toe numbness, I know too well the feeling. Although not painful it is extremely uncomfortable and if severe it can lead to falls due to loss of proprioception (not knowing where floor is). The main cause for this as I stated earlier is a mechanical one. as we stiffen rather our backs get rigid it decreases the normal curvature of the spine putting tension in our back muscles which then in turn pull on our spine to maintain posture this stretches the space between the vertebra and compresses the nerves as they exit the foramen. end result, the nerves get stretched and compressed decreasing their function acting as if they were being pinched  permanently. As I sit here writing my back is getting tighter and I begin to feel some discomfort in my feet telling me I have to move about. however, when someone is under-medicated you get the same effect severe rigidity of axial muscles ( neck and back) with loss of normal curvature and subsequently the entire nerve collection known as a plexus gets compressed and stretched causing numbness in feet because these are the biggest fibers- so get affected first. the key is that usually this occurs bilaterally simultaneously and without pain or weakness and gets better quickly after taking medication or realigning spine like laying down supine in a flat surface.

Of  course this is not to say that Parkinson’s patients do not get neuropathies ( progressive nerve ending atrophy) from other common causes like B12 deficiency, diabetes, or vitamin deficiencies, or presenting symptom of cancer.

My recommendation to evaluating this problem and feeling better soon is to maintain diary.

  • When it happens?
  • How long it lasts?
  • Does it follow a pattern close to your medications wearing off?
  • Other symptoms associated with it? like changes in bowel/bladder function, weakness, pain, one sided?
  • Does it improve? or is it constant?
  • Have you had dyskinesia’s/ dystonia involving more commonly neck to cause cervical radiculopathy and myelopathy but will have other symptoms.  (the constant gyration of neck can lead to herniated discs, as well as torticollis – abnormal posture of neck due to involuntary sustained contractions).

Talk to your physician and discuss problem. as they check for metabolic problems ask them to adjust your dose of medication ( up usually) before proceeding with any more invasive or expensive tests because in my experience this will remedy problem if caused by Parkinson’s-indirectly.

Finally, staying active and keeping muscles limber by doing exercises routinely- does not necessarily mean daily but at least couple of times a week, can reduce this problem dramatically. As an aside never start any vigorous exercise program like boxing, or other physical regimen before discussing with your physician especially if having numbness or other symptoms because you can seriously injure yourself.

So next time you have problems with feet being numb do not fret and follow these recommendations as you call your doctor to discuss further. However, if symptoms come on suddenly on one side of the body go to ER asap. This could be a stroke!

@copyrights 2015
all rights reserved by Maria De Leon

Tardive Syndromes- Tardive dystonia, Parkinsonism, and Other Movement Disorders: By Dr. De Leon

What is a Tardive (late) Dyskinesia?

This is a persistent, at times irreversible, abnormal involuntary movement disorder appearing after a prolonged (usually 1-2 years) or extensive intake of dopamine antagonist (blockers) such as old traditional neuroleptics. Prior to onset of atypical antipsychotics like Seroquel, there was a slew of elder patients in nursing homes and mental facilities with tardive dyskinesias mainly due to antipsychotics like Haldol (usually referred as Vitamin H among health professionals). They can develop in the course of treatment (at least 3 months), after dose reduction, or even after the causative drug has been withdrawn. Stopping or decreasing dopamine blockers causes tardive dyskinesias in about 40% of people previously asymptomatic.

These movements usually present as hyperkinesias (excessive movement) involving the trunk, limbs and orofacial muscles. Exposure to these drugs can reproduce any of the involuntary movements that are hyperkinetic inn nature such as chorea, dystonia, myoclonus, tics, and tremors. Tardive dyskinesias is a generic form used to refer to all these syndromes. But a more restrictive syndrome of involuntary movement of face and mouth (buccolingual masticatory syndrome) also seen in older patients with maladjusted /poor fitting dentition-was the first type of tardive dyskinesia ever written about. Patients look like they are constantly chewing or ruminating. This involves the tongue twisting, persistent protrusion of tongue with lip smacking, puckering and chewing motions. Sometimes may be accompanied by trunk swaying, rocking movements and pelvic trusts. while standing in place these individuals tend to pace or march in place or shift their weight from one leg to another. in addition these patients exhibit moaning and grunting as well as abnormal breathing patterns causing frequent referral to pulmonary and cardiac specialists. They often have respiratory dyskinesias (arrhythmic breathing, deep inspirations and fast breathing).

An unusual clinical manifestation of tardive dyskinesia is a painful sensation in the oral and genital region.

Drug Induced Dyskinesias:

  • Lithium-Parkinsonism
  • Dilantin (phenytoin-with high or normal levels)- disappear with dose reduction or withdrawal- causes chorea, other movements are dystonia, ballismus, tremor, asterixis, and myoclonus.
  • Tegretol (carbamazepine)-tics (vocal and motor)in children and dystonia in brain damaged children.
  • Tricyclic antidepressants ( Elavil, imipramine)- either acute intoxication or after chronic use. 1) Acute abnormal movements are tremors, myoclonus, choreathetosis.2) Chronic use leads to chorea. Symptoms stop with drug cessation.
  • Fluoxetine-myoclonus
  • B-adrenergic drugs used for respiratory disease ( like albuterol, terbutaline) cause tremors.
  • oral contraceptives– can cause chorea
  • calcium channel blockers- (e.g. verapamil, Norvasc)- can cause parkinsonism, and acute dystonia
  • reserpine- parkinsonism
  • tetrabenazine-parkinsonism
  • Antiemetics-metoclopramide, Phenergan-parkinsonism, chorea, dystonia.

Risk factors:

  1. old age
  2. female gender
  3. mentally impaired
  4. duration of illness
  5. length of drug exposure
  6. cumulative drug exposure
  7. history of mood disorder
  8. diabetes (2x greater risk)
  9. structural brain abnormalities
  10. history of electroconvulsive therapy
  11. early side effects like parkinsonism

Risk factors for acute dystonia:

  • male gender (2:1)
  • younger than 30
  • high dose of neuroleptic
  • potency of drug
  • underlying psychiatric disorder like schizophrenia
  • family predisposition

Akathisia (restlessness-feelings of inner tension causing patients to shift positions continuously); frequently misdiagnosed as anxiety. Caused by prolonged exposure to dopamine blockers.

Treatment:

  • reserpine
  • tetrabenazine
  • clozapine
  • tardive dystonias requires a combined approach with levodopa and anticholinergics; clonazepam (klonopin)

If you are on any of these medications and have movement disorder follow up with an MDS or if your current symptoms have worsen and are on one of these meds make sure you follow up immediately.

 

 

 

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.

Sources:

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.