Scientists announced today that they have discovered a cure for apathy. However, they claim that no one has shown the slightest interest in it.” ~ George Carlin
I hear the word apathy tossed around all over the place from different places and people who use this term loosely to imply “lack of interest,” or ‘deep depression.’
However, as a neurologist the word apathy has not only a very special meaning but it also carries with it a specific diagnosis.
So, what does apathy really mean. Apathy as described by the Webster dictionary as “a state of perfunctoriness.” I love that word. It is a state of complete and total indifference in all realms of our being, emotional, spiritual, and social. People who suffer from apathy are completely and utterly devoid of concern, emotions, and feelings. Not because they don’t want to but because they are incapable of caring due to disconnect of fronto-cortical pathways.
Therefore, the term “apathy” in neurology is always a harbinger of brain illness particularly organic etiologies such as head injury, strokes, tumors, schizophrenia (although the latter usually considered a mental illness -I maintain it is an organic neurological disease because of involvement of dopamine- in this case too much) and of course the number one cause and almost synonymous with term of apathy is dementia – (e.g. Alzheimer’s, Parkinson’s, vascular, Lewy body, fronto temporal, etc.)
Therefore, giving someone a diagnosis of apathy in of itself is a poor prognosis which implies typically a rapid cognitive decline.
Having said this, one must always identify the cause and try to treat it as best as possible and prevent further cognitive decline.
Medications employed in the treatment of apathy:
1) [of course are] medications used to treat dementia – i.e the acetylcholinesterase inhibitors like Exelon (Rivastigmine), Aricept (Donezepil), Razadyne (Galantimine), and Namenda (Memantine) an NMDA inhibitor. Several of these compounds have extended release doses and come either in patch or liquid as well or both.
Other medications include:
2) Dopamine replacement– no better feel good drug than dopamine especially if deficiency is what is causing the apathy but it is not always as easy as that in dementia patients particularly those who have dementia of Parkinson’s disease or other dementias associated with parkinsonism because the addition of dopamine will increase visual hallucinations and other hallucinations and worsen confusion. therefore, this has to be measured in scale of risk and benefits but usually in the majority of cases in PD associated apathy combined with other medications like antichlolinerasterase and antipsychotics.
3) Stimulants like Provigil (Modafinil)/ Nuvigil – used in the treatment of Narcolepsy but also for hypersomnolance or increased sleepiness. I prefer this class as first line of treatment over amphetamines after dopamine replacement. In my practice, I had a moderate to great success with them. Other stimulants include amphetamine derivatives such as those employed in the treatment of attention deficit disorder (Methylphenidate-e.g. Concerta, Ritalin; Aderall; Straterra).
4) Antipsychotics are also a good source to combat apathy but best if use atypical drugs like Clozaril or Seroquel because of decrease potential for extrapyramidal side effects like tardive (late onset) dyskinesias and parkinsonism. This is especially crucial if already dealing with apathy related to Parkinson’s or Parkinson’s plus syndrome. We don’t want to make motor symptoms worse resulting in freezing and falls or increase dyskenesias.
5) Anti -depressants– Ssri’s/SNri’s -E.g. Zoloft, Lexapro, Effexor, Cymbalta, etc.
6) ECT– electro convulsive therapy when all else fails -it works tremendously well despite all of the bad the media has given it based on past history and portrayal of patient abuse in movies like One Flew Over the Cuckoo’s Nest. Currently, there are several state of the art centers in the country which provide these much needed services which can help patients have a higher quality of life than the would otherwise -one may be near you. The alternative is earlier institutionalization.
As always early recognition of apathy is Key to improved quality of life by securing proper diagnosis followed by prolonged watchful supervision and treatment by a team of experts which include Neurologist, Neuropsychologist, counselors, social workers, therapist and loved ones to help with financial issues of caring for a chronically ill patient as well as help prolong nursing home admittance for as long as possible.
” 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:
- 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.
- 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:
- The first thing is prevention! it is always easier to prevent than try to fix a problem.
- Second, treat acute pain still easier to treat with less disturbance of a person’s QOL.
- Third, treat chronic pain appropriately to break cycle.
Effective interdisciplinary treatment is necessary to efficiently reduce pain in our lives. This includes :
- physical therapy
- occupational therapy
- massage therapy
- Botox (dysport, myobloc)
- balanced diet
- sleep regimen
- adjust dopamine medications
- Neck surgeries when necessary or as last resort-e.g. laminectomies
- 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)
The things I have learned about dyskenesias in a nut shell.
They are not a sign that you are having fun because you look like you are dancing even if they occur at the beat of your favorite tune.
yes, although most of the time you prefer over being off or stiff, you can’t wait to go to sleep so the movements can stop.
although, they are a great form of exercise you wish you did not have to exercise so much to look so great!
dyskenesias are great for doing the twist and Mixing fruit salad but not for putting on make up or reading a favorite book.
Is there a solution to dyskenesias?
mind you is not an easy feat….
The first thing to remember iS that is much easier to prevent than to stop once they occur.
Key points women are more likely to develop dyskenesias than men.
altough, We as patients know our bodies best and know a lot about Parkinson’s disease we should not manage our own symptoms without the guidance of a professional (MDS).
One, because it is very hard to be objective when it comes to our own disease state.
Second, most of the time what we intuitively think should be the right thing to do is not always the BEST treatment plan for the long run of the disease…this is where experts like myself and other movement disorder specialists come in handy.
Third, as I have mentioned many times before, anyone can treat Parkinson’s in the first stages because it will respond to any and all PD medicines; however as disease advances the expertise in the art of treatment provided by a seasoned specialist is indespensible. This is where knowing how to treat rather than what to treat with becomes crucial.
Fourth, when I was in practice it was common to see my first stage PD patients 2-3 x a years unless they needed something because they were pretty autonomous; but as Parkinson’s progressed the frequency of the office visits increased as should your visits to your physicians. It was usually every month to every 6 weeks evaluation in my late stage disease patients and 4-6 times a yeAr for middle stages. Even though, I understand as a patient we all get tired of visiting and going to the doctor, unfortunately this is the type of commitment that is required to keep DYSKENESIAs at bay and control before they become intrusive by adding medications like amantadine, Keppra, zonegran, comtan or Tasmar (or STALEVO which I love because it has so many doses ranging from 50 mg to 225mg) and adding long term release intermixed with short term and intermediate release. Also things like DBS and other surgeries like pallidotomies; apomorphine and even Botox injections if have focal DYSKENESIAs or dystonias are great treatment options with proven benefit and low risk in the right hands of experienced physicians.
Fifth, although certain autonomy is allowed for patients it needs to be evaluated frequently because invariably most patients will overdose creating more side effects and DYSKENESIAs. More is not always better…we feel bad can’t walk, move, talk, swallow so we not only increase our doses but start increasing frequency sure way to develop DYSKENESIAs faster ! The key is to mix dopa agonists( especially NEUPRO because the continuous release will potentials the effect of levodopa giving it a more continous star in your body without having so many peaks and valleys), MAO inhibitors along with dopamine in various forms…don’t be afraid of taking several types of medications this is how you get the most benefit without having to take such large doses of levodopa and keep number of times you take medications down ( e.g. 4-5 x a day instead of every 2 hours). We have so many new choices now.
Sixth, remember that dopamine agonists and amantadine effect usually work for several years in the range of 5-7 years after which their effect starts to wane but after a small period of rest ( 1-2 years off these medications) they can be again reintroduced having again a positive effect.
Seventh, It is recommended that around the time you enter the middle stages of disease (3-4th stages) every patient should start keeping a calendar of every medication taken. This needs to include time of intake of each medicine along with name, how long it takes to kick in ( what do you notice when it kicks in), how long each medication last, can you tell when it wears off- what do you notice and the does medicine wear off suddenly. your doctors usually have copies of these calanders in their office, you can get from them or get from the website Or create your own. For instance, I can tell you that my azilect helps my pain & walking, my Neupro helps my vision and my STALEVO helps my brain feel “on.”
Eight, most important advice to prevent and stop these intrusive involuntary movements which make our lives miserable is early DBS before they start…if you Wait till after they start it will take longer to find right setting. when DBS setting is adjusted in brain It is imperative to adjust medications at the same time for maximum result so need to find a doctor who can program in office and has extensive experience – this is one of those art things where experience truly dictate outcome. Patients with DYSKENESIAs by definition have already more advanced disease taking more medications therefore adjustments have to go even slower for maximum results which can be extremely frustrating. so find someone close by because will need a lot of visits; if not able to travel frequently to maximize benefit then should consider doing pallidotomies instead.
Parkinson’s disease is an extremely complex disease encompassing multiple body systems outside of the brain thus in order to have the best quality of life is to have disease managed by a movement disorder specialist who serves as the conductor in a well orchestrated performance involving many other subspecialties (including Gi, neurosurgeons, dermatologists. Urologist,internist, anesthesiologist, ENT, and ST, PT, and OT). Together they can allow you to dance in whatever fashion you desire without the presence of DYSKENESIAs.
First, dystonia which falls under the umbrella of the movement disorders diseases implies an abnormal contraction of the muscles that causes involuntary twisting resulting in abnormal postures. It can be painful. Some common known dystonia are writer’s cramp-a type of dystonia that occurs only while writing affecting the muscles of the hand and forearm.
Dystonia is a symptom like a cough, or nausea. We need to know the cause. It can be a primary disease such as dopa responsive dystonia (DRD), or dystonia muscularoum deformans (also known as Openheimer’s syndrome or torsion dystonia-DYTI ).
All primary dystonias are caused by some type of genetic abnormality. Dystonia can also be part of another neurological disease; this type is known as secondary dystonia.
Secondary dystonia or acquired causes:
- Strokes (CVA’s)
- Multiple Sclerosis
- brain tumors
- carbon monoxide poisoning
- oxygen deprivation- e.g. strokes
- brain trauma
- birth injury ( some people think cerebral palsy fall into this category)
- heavy metal poisoning
- drug interactions ( side effects) usually to antipsychotics after long use
- drug use,
It can affect any muscle of the body. The dystonia can be:
- Focal – affect one specific body part e.g. writer’s cramp, cramp with piano playing.
- Generalized- all or most body parts
- Multifocal- several unrelated body parts
- Segmental –adjacent body parts like torticollis.
- hemidystonia –arm and leg on the same side In Parkinson’s disease it is usually more commonly seen as a side effect of levodopa when wearing off and can look like any of the above.
Typically Parkinson’s patients experience focal or hemidystonia. In YOPD it is not uncommon to see dystonia as one of the presenting symptoms again it can be focal, hemidystonia, and occasionally segmental. Unfortunately sometimes dystonia can remain the most prominent feature for years before other motor pd symptoms to develop. But, during this time persons with YOPD should have other non-motor symptoms developing. I have seen patients treated with focal dystonia for 6 years only to see signs of PD in the 7th year. With time DRD develops pd symptoms sometimes confounding picture for those not so well versed in the field erroneously labeling Parkinson’s when in fact is primary dystonia with secondary parkinsonism. The treatment unfortunately is the same but the prognosis is quite different. One being static or mildly progressive while the other one will have a progressive neurodegenerative course (PD). DRD can be controlled with large doses of dopamine while the other initially maybe controlled but will eventually lead to dyskenesias. Independent of whether dystonia is primary or secondary’s the treatment armamentarium is essentially the same. The basic principle is finding an underlying treatable cause such can reverse the symptoms if not is back to symptomatic control. In the case of Parkinson’s is really as much an art as it is a science in being able to adjust the Parkinson’s medications. This is where open communication between a doctor and patient come in handy to pinpoint exactly if the dystonia is being caused by wearing off, end of dose, or a biphasic response!
Symptoms of dystonia:
- A “dragging leg”
- Cramping of the foot
- Involuntary pulling of the neck- a.k.a. spasmodic torticollis or cervical dystonia
- Uncontrollable blinking a.k.a. blepharospasm
- Speech difficulties- a.k.a. spasmodic dysphonia – 2 types adductor & abductor. Adductor is by far the most common and responds best to botox injections (no anesthesia needed only feel a prick through front of neck). Adductor dysphonia voice sounds strangled or strained because the vocal cords are so stiff it is difficult to produce sounds this is what it is typically seen with PD; but normal while laughing, crying or shouting. In abductor dysphonia, the voice involuntarily sounds like a whisper when attempting to talk. Botox is also used for this- more complicated has to be given into actual vocal cord muscles.
All dystonias, as all other movement disorders, get worst during stress and fatigue!
Treatments for dystonia:
- Medications oral: baclofen, Valium, xanax, klonopin, neurontin, dantrolene, levodopa,tetrabenazine
- Medications injectable: botox (there are several types including disport), baclofen pump; e.g. botox works great for spasmodic dysphonia
- Surgeries: DBS, thallomotomies, pallidotomies, ramisectomy and rhizotomies (spinal nerves are cut in order to relieve pain), pheripheral deenervation (cutting nerves in pheripheal nervous system), myectomies (taking out a portion of muscle), myotomy (dissecting the muscles).
- Ancillary treatments: PT, OT, ST, adaptive equipment-e.g. orthotics, leg braces
- Sensory tricks: stimulation applied to affected muscles or muscles nearby people can reduce and control their own contractions. This man is touching back of his neck as a sensory trick to keep from going back.
- Non-medical treatments: massage, acupuncture, behavioral therapy (biofeedback), yoga.
In the hands of a skilled and knowledgeable specialist all dystonias should be able to be controlled providing the person living with this condition, whether it is a symptom of something else or a syndrome itself, a good quality of life since we now have so many treatment options. However, most dystonias unless related to medication side effects or are focal in nature (except spasmodic dysphonia which require ENT specialist to do injections) will require a team approach (ancillary services, MDs, neurosurgeon, pain specialist, orthopedist, massage therapist) and a combination of therapeutic modalities to achieve the best possible outcome. These treatments should be accompanied along a life style in which sleep is given priority and methods of stress relief are integrated into everyday life to maximize benefit.
In order to properly diagnose and decide proper treatment need to be seen by a movement disorder specialist (MDS). He or she will run blood test (which may include genetic testing), imaging test (CT/MRI Brain) along with a detail neurological exam to find the cause.
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