This is the most common form of MND, accounting for about 66% of all patients [2], with both upper and lower motor neurones affected. It is characterized by weakness and wasting in the limbs, often first indicated by clumsiness in walking or handling things. Most commonly the upper limbs and hands are affected first with evident wasting of the thenar eminence. Average life expectancy with this form can be between 2 and 5 years from onset of symptoms.

Progressive bulbar palsy (PBP)

PBP involves both upper and lower motor neurones and affects about a quarter of the patients diagnosed. This form of MND often causes difficulties with speech or swallowing. If the lower motor neurones are affected, the tongue tends to atrophy with visible fasciculation and reduced mobility. This results in a rather nasal type of speech. If the upper motor neurones are affected the tongue is spastic and tends to cause dysarthria, difficulty with the mechanics of speech, i.e. stuttering.

Wild mood swings may also occur with little apparent cause. The limbs are less affected but as the disease progresses the patient may experience weakness in the arms and legs [3]. Life expectancy is between 6 months and 3 years from the onset of symptoms.

Progressive muscular atrophy (PMA)

PMA affects only a small group of people, with damage mainly occurring in the lower motor neurones. It tends to start earlier, predominantly affecting men below the age of 50 years. It first presents with wasting in the arms, manifesting as weakness and clumsiness of the hands. There may be progression to the lower limbs but bulbar involvement is rare until late in the disease. The rate of progression is much slower, with patients living 5–10 years after diagnosis.

There are other much less common variants: Kennedy’s disease, Guam variant MND and primary lateral sclerosis.

Although ALS, PBP and PMA are the three main forms of MND there is an enormous variation in symptoms between patients. Control of bladder or bowels is rarely affected. Mental deterioration and dementia are found in less than 5% of patients [3]. There is increasing evidence of frontal lobe involvement which may lead to emotional lability. Anxiety and depression are quite commonly found, unsurprisingly, in such a serious disease.

Risk factors

There are no apparent risk factors although, as mentioned above, there can be, rarely, a familial link.

Diagnosis

Differential diagnosis

MND presents some problems with diagnosis for several reasons: primarily because it is comparatively rare, but also because the early symptoms do not appear serious to the patient. These can include clumsiness, mild weakness or slightly slurred speech, all of which could have an innocent cause. It may be some time before a doctor is consulted.

The classification and terminology used to describe the different MND syndromes are not always clear or consistent, with ALS being frequently used in US information sources as the general title.

Tests

There is rarely a clear pattern or a single indicative symptom and no definitive test. MND does not usually affect the bladder or bowel. It can be confirmed by electromyography later in the progress of the disease when the motor action potentials are of increased amplitude and duration but fewer in number than normal. Other investigations may be necessary to exclude syringomyelia or cervical spondylosis, both of which may present with weakness in the upper limbs.

Medical treatment

Pharmacology

The National Institute for Clinical Excellence has recommended the use of riluzole (Rilutek) to treat ALS. It is thought to prolong life for a couple of months and may delay the need for a tracheostomy, but it does not really help with many of the symptoms. The drug reduces the natural production of the neural transmitter glutamate, which carries signals to the motor neurones. It is believed that too much glutamate can damage these and inhibit nerve signalling.

Other medicine may help with symptoms. Muscle relaxants such as baclofen, tizanidine and the benzodiazepines may reduce spasticity. Glycopyrrolate and atropine may reduce the flow of saliva. Quinine or phenytoin may decrease cramps. Anticonvulsants and non-steroidal anti-inflammatory drugs may help relieve pain, and other drugs can be prescribed to treat depression. Tranquillizers often help with sleeping problems.

Some patients with PPS develop sleep apnoea (a potentially life-threatening condition characterized by interruptions of breathing during sleep), which can be treated with decongestant therapy or assisted breathing at night. Panic attacks over fears of choking to death can be treated with benzodiazepines. Botulinum toxin may be used to treat jaw spasms or drooling. Amitriptyline and other anticholinergic drugs can help control excess drooling. Some patients may eventually require stronger medicines such as morphine to cope with musculoskeletal abnormalities or pain, and opiates are used to provide comfort care in terminal stages of the disease.

Surgery

Surgery can occasionally be used to remove any blockage to the airway but is not generally applied to this disease.

Prognosis

As stated previously, the prognosis is a poor one for sufferers of this disease, with a small variation according to the focus of the disease. Symptom control is undertaken by the multidisciplinary health team and the main goal is greater comfort for the patient.

Impact on the patient

The manifest problems can be subdivided into the following categories.

Pain

This is a common problem, with up to 73% of patients reporting pain of some kind [4]. The causes are mainly muscular, muscle cramps, spasticity or spasms as a result of weakening muscles. These produce abnormal stresses on the joints and often result in changed posture, leading to further stresses and pain. If the patient is unable to change position easily then areas of pressure will also lead to pain. Chronic back pain produced by poor posture is very common.

Insomnia

This is a common problem, reported in 48% of patients admitted to a hospice [5]. There are of course many reasons why this might be so. Firstly, any breathlessness will tend to get worse when it is time to sleep, especially if there is associated anxiety. Again, this is often linked to weak musculature. It may also be associated with depression and may respond to antidepressants or sedatives.

Fatigue

A form of general fatigue occurs in most patients and can in itself be seriously disabling. Any physical activity needs to be undertaken with care but ‘a little and often’ is very useful. Corticosteroids are sometimes prescribed for fatigue but if they are used for long periods that may produce unwanted side-effects of their own.

Other less common problems may include sore eyes resulting from the weakness of the eye muscles. Urinary retention may occur, due more to the weakness of the abdominal walls rather than to problems with the kidney or bladder. Catheterization is sometimes necessary. Pressure sores may result if movement is severely limited and regular turning and the use of pressure-relieving cushions and mattresses are vital.

MND and physiotherapy

Physiotherapists are part of a multidisciplinary team dealing with the problems of MND and the main thrust of physical management is the control of symptoms.

The pain can be dealt with in several ways. As discussed previously, a lot of it is due to the weakening of muscle, the imbalance caused by this and the subsequent inappropriate increase in muscle tone, leading to cramps and spasticity. Correct positioning, education of handlers in moving and handling and encouragement of physiological movement with adequate rest periods underpin the treatment programme. Gentle stretching to maintain soft-tissue length can sometimes alleviate pain. Acupuncture may also now be routinely considered as part of the pain relief [6]. Transcutaneous electrical nerve stimulation (TENS), hot packs or even warm baths can all be useful.

Respiratory problems are difficult to treat and many modalities are used. Fundamental failure of chest expansion is dealt with by maximizing ventilation and gas exchange and assisting with the removal of secretions. Physical methods will include non-invasive ventilation, drug therapy, advice and education for the family as well as the patient.

Acupuncture in MND

There is not much evidence supporting the use of acupuncture in MND, or ALS. This is surprising considering the manifestations of the disease but probably reflects the overall rarity of the condition. It is possible that much of the recorded acupuncture research on patients receiving palliative care does include a fair number of MND sufferers. Specific problems, such as anxiety, depression, insomnia and shortness of breath, are fairly common in this unfortunate group [7]. The calming and anxiolytic effect of acupuncture will contribute significantly in this situation.

A recent Cochrane review included five acupuncture studies and decided that there was low evidence that acupuncture and acupressure were useful [8]. Acupuncture certainly is used [9], but precise details of the interventions are often lacking.

Body acupuncture

Local points are used in dealing with the pain to facilitate freer joint movement; a concentration on the major Yang meridians will be helpful, with the addition of the powerful distal points. The limbs are thought to be regulated through the Governor Vessel so acupuncture can be used to strengthen and augment the flow of Qi which may be blocked by pathogenic invasion. Table 11.1 offers a selection of useful points.

Table 11.1 Motor neurone disease – useful points

Scalp acupuncture

Some authorities use scalp acupuncture but MND is not generally cited as an indication. However Jiao suggests that two variations of bulbar paralysis will respond [10]. What he terms as ‘true bulbar paralysis’ with symptoms of dyslalia, dysphagia and the disappearance of the pharyngeal reflex can be treated by stimulating the balance areas bilaterally. What he terms ‘false bulbar paralysis’, with additional symptoms of dysphagia, choking and excess salivation, requires the addition of the lower two-fifths of the motor area on each side (see Chapter 12). Good results are claimed, but there is no very convincing research evidence as yet.

TCM approach

The picture in traditional Chinese medicine (TCM) literature for MND is unclear. The disease itself is not commonly described in TCM and certainly not in terms that Western medicine would be comfortable with. The links to the Wei syndrome are tenuous but seem to be the best match. Wei syndrome refers to weakness and motor impairment of the limbs, possibly accompanied by numbness and muscular atrophy. The fact that this seems to be localized in either the upper or lower portions of the body serves to confuse the picture somewhat, although there are well-established methods for balancing Qi in the two halves of the body, often by using the extraordinary meridians: in this case the Dai Mai might be helpful.

The most likely syndromes will be Lung and Spleen Qi Xu, Qi and Blood deficiency or Liver and/or Kidney Blood deficiency.

Nicholas Haines provides a good example of a case history in the book Acupuncture in Practice [11]. In a useful flow diagram (Figure 11.1) he indicates how a succession of pathogenic influences may produce the right circumstance for the symptoms of MND. It is not suggested that these are the root cause of the disease but the TCM points to treat them will be useful as part of the wider management of the patient.