Parkinson’s disease and related conditions

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7 Parkinson’s disease and related conditions

Introduction

Risk factors

The root cause remains obscure but parkinsonism results from many different pathological processes, including ageing, environmental and genetic factors. Ageing is not thought to be a primary cause of PD, although the substantia nigra containing dopamine-producing neurons declines with age. It is possible that injury or infection in early life may predispose the patient to accelerated loss of this tissue. It has been suggested that PD can be a side-effect of certain psychotropic drugs [2]. Analytic studies generally reveal an inverse association between PD and cigarette smoking, although epidemiologic evidence does not support a direct protective effect of smoking [3].

It has also recently been suggested that gout can protect from PD. The value of the increased uric acid present in the system needs to be fully evaluated but first results are interesting [4]. The association between ischaemic stroke, vascular risk factors and PD has been addressed in several studies [5].

Shy–Drager syndrome is very similar to Parkinson’s in symptomatology but differs in one major respect, respiratory problems. It is described as multiple-system atrophy with autonomic failure and is a progressive disorder but is often first recognized by an increase in loud and strident snoring while the patient is sleeping.

This disorder is generally characterized by postural hypotension – an excessive drop in blood pressure which causes dizziness or momentary blackouts upon standing or sitting up. There are three types of Shy–Drager syndrome: (1) parkinsonian type, which may include symptoms of PD such as slow movement, stiff muscles and mild tremors; (2) cerebellar type, which may include problems such as loss of balance and the tendency to fall; and (3) combination type, which may include symptoms of both types.

There has been some argument over the years about other similar pathologies, known as vascular parkinsonism. The condition has been named and renamed several times, with terms such as arteriosclerotic parkinsonism, arteriosclerotic pseudoparkinsonism and lower-body parkinsonism. Despite the progress in our understanding of other parkinsonian syndromes, such as progressive supranuclear palsy and multiple-system atrophy, and significant developments in neuroimaging techniques, the concept of vascular parkinsonism is still unclear and the clinical diagnosis is often difficult [5], but from a physiotherapy or acupuncture perspective it does not differ greatly from PD.

Diagnosis

Differential diagnosis

Early symptoms are subtle and make their appearance gradually, with those closest to the patient often unaware of what the diminished energy and depression may foreshadow. Normal voluntary and spontaneous movements are lacking. In some people the disease progresses more quickly than in others, leading to a rapid decrease in the ability to perform daily activities due to the shaking or tremor.

The definitive symptoms of PD are tremor at rest; involuntary trembling in the hands, arms, legs or jaw; rigidity or stiffness of the limbs and trunk; a general slowness of movement (bradykinesia); and impaired balance and coordination, often involving postural instability. ‘Dropped-head’ syndrome is characterized by severe neck flexion but minor thoracic or lumbar curvature. It results from neck extensor weakness or increased tone of the flexor muscles. This symptom is usually reported in neuromuscular diseases such as amyotrophic lateral sclerosis, myasthenia gravis and polymyositis or in extrapyramidal disorders, but does also occur in PD [6].

Patients may also have difficulty in walking or talking and completing small motor tasks becomes problematic.

In addition to these motor changes, there may be other symptoms, including depression and other emotional changes; difficulty in swallowing, chewing and speaking; urinary problems or constipation; skin problems; and sleep disruptions. There is noticeably decreased facial expression, apathy, fatigue and sometimes pain. This fatigue, combined with worsening functional status, can be a significant contributor to poor quality of life [7].

Some cognitive changes can be suspected early in the disease, particularly frontal lobe executive dysfunction. Parkinsonians find it difficult to turn thought into action. A slowing-down of mental processes is sometimes mistaken for dementia, but as a rule of thumb, if you give a Parkinson’s patient time to answer a question, he or she will answer. A patient suffering from Alzheimer’s will tend to forget the question. As the disease develops psychiatric problems can dominate the clinical picture. Depression is the most common of these, with about a third of patients suffering from depression at some stage in their disease [1].

Medical treatment

Pharmacology

Various pharmacologic and surgical therapies have been developed to deal with the dysfunction caused by this disease. A variety of drugs provide some relief from the symptoms. Usually, patients are given levodopa combined with carbidopa. Carbidopa delays the conversion of levodopa into dopamine until it reaches the brain. Nerve cells can use levodopa to make dopamine and replenish the brain’s dwindling supply. Although levodopa helps at least three-quarters of parkinsonian cases, not all symptoms respond equally to the drug. Bradykinesia and rigidity respond best, while tremor may be only marginally reduced.

Problems with balance and other symptoms may not be improved at all. Anticholinergics may be used to help control tremor and rigidity. Other drugs, such as bromocriptine, pramipexole and ropinirole, mimic the role of dopamine in the brain, causing the neurons to react as they would to dopamine. An antiviral drug, amantadine, also appears to reduce symptoms. A relatively new drug, rasagiline (Agilect), can also now be used along with levodopa for patients with advanced PD or as a single-drug treatment for early PD. Constipation is a common problem, often as a result of decreased intestinal peristalsis induced by anticholinergics.

Continuous levodopa treatment for PD patients is frequently associated with the development of motor complications such as dyskinesias and a tailing-off of effect towards the end of the dose. Medical management for this includes careful manipulation of the dose to establish the optimum treatment schedule and improve absorption, incorporating catechol-O-methyl transferase inhibition, monoamine oxidase-B (MAO-B) inhibition, dopaminergic agonists, amantadine and continuous dopaminergic infusions.

Surgery

Surgery was in vogue for PD before the 1970s but became less popular until recently. It may be appropriate if the disease fails to respond to drugs or the effect of the drugs diminishes. Transplanted fetal mesoencephalic cells, harvested from aborted fetuses, grown in cell culture and injected into the brain in a form of cell suspension, have been shown to replace the production of natural dopamine, producing some good results, although the technique itself remains controversial [8]. A technique called deep-brain stimulation uses electrodes implanted into the brain and connected to a small pulse generator which can be externally programmed. This can decrease the need for some of the drugs, reducing the unwanted side-effects, most often the increased involuntary movements associated with levodopa [9]. The deep-brain stimulation can also reduce the fluctuation of symptoms, allowing for greater freedom of movement generally. Early results have been good but the technique is not yet widely used.

Prognosis

Impact on patient

PD is similar to other neurological problems in that it varies widely from patient to patient. With some the tremor is the most limiting factor while others seem untroubled by that, but far more concerned by their lack of mobility or difficulty communicating by facial expression. Clinical experience suggests that there is, however, a great deal of unreported depression and often quite a lot of pain brought about by the rigidity and overall poverty of muscle movement [10].

When investigating the lifestyle impact of PD, Kuopio et al. found that women scored significantly lower on five of the eight dimensions of the SF-36 outcome measure. This study found depression to be more common among women than men [11]. They concluded that, to improve the quality of life in PD patients, it is necessary to recognize and treat the depression. Parkinsonian symptoms and symptoms of autonomic dysfunction such as constipation and sexual impotence in males predominate early in the course of the disease and certainly have an effect on mood. Constipation may be unrelenting and hard to manage in some patients.

Shy–Drager syndrome may be difficult to diagnose in the early stages; however, within a year of onset most patients develop postural hypotension. For the majority of patients, blood pressure is unstable – often fluctuating up and down and may cause severe headaches. Other symptoms may also develop, such as generalized weakness, double vision and/or other vision disturbances, impairment of speech, sensory changes, difficulties with breathing and swallowing, often causing pronounced snoring; dysphagia is a late symptom and affects both solids and liquids. This difficulty arises either from an inability to force the food down the throat or rigidity of the voluntary muscles of the throat and leads to complaints of food getting stuck in the throat. Ingestion of only small portions of food and careful chewing, one morsel being swallowed before the next is taken, may help. The problem is important because of the risk of aspiration. Other symptoms may include irregularities in heart beat, inability to sweat and diarrhoea.

Parkinson’s disease and physiotherapy

Exercise has long been a popular form of treatment with physiotherapists, although the effects seem to be best in the short term. A recent study by Morris et al. [12] confirms this. For the exercise group, quality of life improved significantly during inpatient hospitalization and this improvement was retained at follow-up. Inpatient rehabilitation produced short-term reductions in disability and improvements in quality of life in people with PD. Exercise training has also been found to decrease significantly the number of falls experienced by these patients [13]. Other techniques, including auditory cues [14] or the use of treadmills, have been tried with some success [15], but all the studies were relatively small and none appear to hold the key. As a novel way of combining both forward and backward walking in a pleasurable environment, tango lessons have been investigated, with some success [16].

The Royal College of Physicians guidelines have recommended the following:

All of the above will be tackled in a course of physiotherapy treatment at any stage of the disease but the addition of acupuncture may be of considerable benefit. There have been several studies investigating the effect of acupuncture on the symptoms of PD and a recent systematic analysis summed the situation up well, concluding that ‘there is evidence indicating the potential effectiveness of acupuncture for treating idiopathic PD’. The review found that, out of the 10 trials selected, nine claimed a statistically positive effect from the use of acupuncture [18].

The outcome measures most used were the Unified Parkinson’s Disease Rating Scale (UPDRS) and the outdated Webster scales. The Motor Dysfunction Rating Scale for Parkinson’s Disease (MDRSPD) was only used in one of the studies. The results were limited by the methodological flaws, unknowns in concealment of allocation, number of dropouts and blinding methods in the studies, but as acupuncture has been used clinically to treat the symptoms of PD for hundreds of years, the picture is encouraging for further research.

Acupuncture in Parkinson’s disease

Acupuncture remains a popular treatment for PD in far-Eastern countries.

Occidental medicine has a given definition for PD and knowledge of PD pathophysiology has led to development of therapeutic management. PD, even if not named, is likely to have always existed in different parts of the world. Description and management of this neurodegenerative condition are found in ancient medical systems. The following section attempts to introduce the philosophical concepts of traditional Chinese medicine (TCM) and the description, classification and understanding of parkinsonian symptoms in TCM.

There has been a serious attempt to re-evaluate the traditional treatments in the light of modern knowledge, in particular the herbal remedies [19], but a scientific review of all TCM therapies in this context is now needed.

In a non-blinded pilot study, 85% of patients reported subjective improvement of individual symptoms, including tremor, walking, handwriting, slowness, pain, sleep, depression and anxiety [20]. There were no adverse effects; however there were only 20 patients in this study.

Electroacupuncture may be a refinement that will be more effective in treating PD. It is used on body needles and also on those inserted into the scalp (see section on scalp acupuncture, below).

Some interesting work has been done by a Chinese research group, where electroacupuncture was applied at a frequency of 100 Hz at the points GV 20 and GV 14 to MFB transected parkinsonian rats who had rotenone (3 μg) administered bilaterally and stereotaxically into the medial forebrain bundle (MFB) to produce parkinsonian symptoms [21]. An extension of this work to human patients might produce significant results.

Nonetheless, the following sections will offer a selection of acupuncture points and techniques with varying rationales. The reason for point selection may ultimately be less important than the point itself. Table 7.1 gives a summary of expected symptoms.

Table 7.1 Symptom picture for Parkinson’s disease (including multiple-systems atrophy)

Symptom Characteristic presentation Parkinson’s disease
Decreased mobility Rigidity image
Fatigue Lack of energy image
Respiratory problems Snoring

Muscle spasm Tremor image Contractures Stiffness and rigidity image Autonomic changes Slowing circulation image image Cognition/mood image Communication image image Bladder and bowel problems Usually drug-induced image Visual problems Rare X

X, usually absent; image, common; image image, very frequent.

Phlegm Heat agitating Wind (affecting Liver)

There are three different syndromes associated with the stirring of Liver Wind. They can be caused by:

Treatment for all three causes will aim to subdue the Liver Wind, which can be very dangerous, frequently leading to Wind Stroke or cerebrovascular accident. Otherwise the Liver energies need controlling or tonifying according to whether the underlying symptoms exhibit excess or deficiency. The prevention of stroke depends on getting this balance correct but it will not really affect or prevent the onset of PD.

This syndrome is often associated with hypertension, stroke, epilepsy and trigeminal neuralgia. It may be exacerbated by prolonged frustration or anger, both of which are said to damage the liver. It is also linked to obesity and a lack of physical exercise.

The symptoms are likely to include vertigo, tremor, convulsion and spasms. Stiff neck, facial paralysis, tinnitus, apoplexy and hemiplegia may also occur.

Liver and Kidney Yin deficiency

This situation is often the precursor to Liver Yang rising. If the cooling Yin of the Liver is depleted the Yang becomes hyperactive and rises upwards, producing the symptoms of headache, dry eyes and tinnitus. The Heat also causes irritability and anger, emotions damaging to the Liver. This syndrome is a combination of both excess and deficiency, although the excess symptoms seem more obvious.

Liver Yang rising is a fairly common pattern and will be mentioned again in this book as it is closely associated with neurological damage. Stress, frustration, anger and resentment build up over a long period of time, obstructing the free flow of Liver Qi. This produces Heat, which dries up Yin. Yin thus cannot control Yang, which rises to the head. In contrast, the person could be relatively cool in the lower part of the body.

Symptoms include general frailty, dizziness, tinnitus, insomnia, headache, night sweats, low-back pain, stiff neck, back and knees and mental restlessness. Often also present will be lack of facial expression, poor memory and general physical clumsiness.

Treatment

Nourish Yin, dispel Wind, energize the meridians.

In summary, in TCM terms PD provides a long and complicated list of symptoms, all of which combine to make up a picture that is not unlike the TCM idea of old age, a slow decline of supporting Kidney energy. It is not suggested that PD can be cured by acupuncture but it is reasonable to suppose that the known physiological effects may help with symptom control.

It is important to remember that these patients are characterized by a slowing-down of body processes and a general lack of energy. Acupuncture can be a draining type of therapy and should be used with caution. However, it can be seen that, with a basic understanding of TCM, a useful prescription can be drawn up for a patient manifesting with a clear neurological disease process [27]. Then it would be sensible to select specific points for symptoms, ensuring that some of the powerful end points are also used. One good argument for using acupuncture for the management of PD might be that it causes fewer adverse effects than drug treatment, particularly levodopa, and often addresses some of the associated problems [28].

However, it is always best to combine acupuncture with the type of physical retraining treatment described in the physiotherapy section of this chapter. PD has been described as a depression with physical symptoms. One of the well-documented side-effects of acupuncture is the improvement in mood. Parkinson’s patients are hard to motivate and it is often difficult to perceive whether there is a response.

Teaching ways to initiate the first step, increasing control over simple tasks like walking or sit to stand will help immeasurably with daily life, while a feeling that all is not lost will help to maintain whatever progress is possible and motivate the patient further.

Case study 7.2: level 3 case study

This patient was a 62-year-old man who had had PD for 8 years, and who was being managed by specialist PD services. He lives with his wife. He has a history of hypertension.

References

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