Peripheral nervous system disorders

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10 Peripheral nervous system disorders

Part 1: Guillain–Barré syndrome (GBS)

GBS is classified as a disease of the peripheral nervous system, those nerves controlling movement and senses, and is quite rare, with a prevalence of approximately 1 in 100 000 in European countries. It affects about 1500 people in the UK every year. It is slightly more common in men than women and can affect people of any age, including children. It is also sometimes referred to as acute inflammatory polyradicular neuritis.

The exact cause of GBS is unclear and there is no way to pinpoint who is most at risk from the condition. However, in most cases of GBS, the person affected will have had a viral or bacterial infection a few weeks before getting the condition. It is likely that the infection causes the immune system to attack the body’s own nerves.

In GBS, the body’s immune system attacks these nerves, causing them to become inflamed (swollen). Although axonal demyelination is an established pathophysiological process in GBS, the rapid improvement of clinical deficits with treatment is consistent with Na+ channel blockade by antibodies or other circulating factors, such as cytokines [1]. Most people with GBS make a full recovery within a few weeks or months and do not have any further problems. Some people may take longer to recover and there is a possibility of permanent nerve damage.

Symptoms

Symptoms usually appear after a preceding infection. They increase in intensity over a period of time, varying from a few hours in serious cases to around 4 weeks in most patients. The symptoms also vary with regard to the pattern in which they appear. Their distribution is usually symmetrical and double-sided. This means that the symptoms appear on both the left and right side of the body, but they may also appear randomly, especially in the beginning. They may also appear and disappear quite randomly. Inflammation of the peripheral nerves leads to a tingly, numbing sensation in the arms and legs. This can eventually result in a short-term loss of feeling and movement (temporary paralysis).

Fever is not a symptom of GBS, but may be caused by the preceding infection that triggered the syndrome. GBS symptoms vary, depending on whether the syndrome has affected the sensory nerve fibres or the motor nerve fibres. When both motor and sensory nerves are affected, the patient experiences a mixture of symptoms. The patient may also experience disruption in the working of the autonomous nervous system.

Damage to sensory nerves

Symptoms generally begin in the patient’s feet, hands or face, spread to the legs or arms, and increase in intensity as they move towards the centre of the body. They generally appear on both left and right sides of the body. However, GBS is unpredictable, and cases have been reported in which this ‘glove and stocking’ pattern is not followed. Instead, motor symptoms or disruptions in the autonomous system may be observed. GBS may also affect an arm or a leg alone, without spreading to the rest of the body.

Gradually muscle pain is experienced in the large muscles, such as the thighs, back and shoulders. Pain in the lower back, buttocks or thighs is common, and is often the earliest symptom. Stiffness and cramping pain or deep, aching muscle pain is common. The sensory symptoms then make themselves felt, as the sensory nerves are attacked. The patient experiences loss or reduction of the sense of touch, or abnormal sensations such as burning, tingling, pins and needles, ‘ants under the skin’, vibrations and numbness.

In some patients, the skin develops hyperalgesia, or tenderness to touch, made worse by bed covering, socks and tight-fitting shoes; in some cases, pain may limit walking. Patients with symptoms at first limited to the feet and ankles may observe similar symptoms in the fingertips; as the symptoms extend to the knees, they may also extend to the wrists. The symptoms usually remain peripheral, i.e. beyond the knee and the elbow. The patient loses the ability to tell the difference between hot and cold, and may feel cold or may sweat for no apparent reason. Minor injuries may occur without being noticed. The patient’s sense of taste can be affected too.

Damage to motor nerves

The motor nerves control movement, and damage to them results in partially or completely blocked signals, causing reduced movement or coordination. The patient’s muscles weaken and atrophy. Tendon reflexes are reduced or lost. Progressive weakening or paralysis may occur, typically beginning in the feet, hands or face. The paralysis characteristically involves more than one limb, most commonly both legs. The paralysis is progressive and usually ascending, spreading to the rest of the limb, and from there may spread to the legs, arms and the rest of the body.

It will be difficult to stand up or climb stairs, to walk or stand and the patient will often say that the legs feel heavy. The patient may have difficulty holding and manipulating small objects, and because the arms feel weak they can no longer lift heavy objects. The weakness is often accompanied by pain and muscle spasms. Constipation can sometimes be a problem, due to the reduced activity of the intestines, change of diet and weakened stomach muscles that resist efforts by the patient to empty the bowel.

Damage to the cranial nerves can affect the face, producing a form of facial palsy. The speech muscles and vocal cords may also be affected, causing unintelligible speech. If swallowing and breathing are involved then the disease becomes life-threatening. Admission to hospital may be necessary if the GBS develops very quickly. Patients showing signs of weakness are carefully observed for signs such as paralysis of the throat, which signals a potential respiratory failure. In this case a ventilator will be necessary and if the heart rhythm becomes unstable a heart monitor may also be used.

Physiotherapy treatment

Recovery generally begins within a month of the height of the illness and has the potential to be complete. Unfortunately approximately 30% of patients will retain a residual paralysis or paresis, most usually in the lower limbs. Statistically significant correlations have been found between the degree of residual motor deficit and the severity of the weakness in the acute phase, the duration of the plateau phase or the duration of artificial ventilation [2].

Complications such as contractures, particularly those around joints, will delay or prevent full recovery and physiotherapy often concentrates treatment on these. Gentle stretching is undertaken to prevent the patient remaining for long periods in a bent or contracted posture and positioning in bed or when seated needs to be carefully controlled to ensure a good position. Splints may be used and active-assisted exercises slowly introduced.

Another complication to recovery is fatigue, so any exercises need to be carefully graduated in order to strengthen without overtiring the muscles. Temporary use of mobility aids such as wheelchairs and orthoses may be desirable to prevent overstrain. Patients with GBS do seem to have a reduced quality of life and functioning with persistent levels of distress even after the recovery period [3].

Pain can also be a problem, mostly caused by the affected muscles. Correct positioning and comfortable support will assist with this but often analgesia is required, especially at night. Anxiety is undoubtedly a factor in such a sudden and serious illness and is associated with perceived slowness in recovery.

TCM theories

The link is with the Wei syndrome since early stages are often characterized by fullness and febrile disease. Interestingly, antibiotics are sometimes thought to be a causative factor as they destroy bacteria but fail to expel the External Pathogenic factor, resulting in a residual or latent Damp Heat in the interior.

Empty patterns are likely to be the result, particularly Spleen Qi Xu or Spleen Yang Xu. The two given below are not specific for GBS but are likely to manifest in one form or another.

Case study 10.1: level 1 case study: Guillain–Barré syndrome – pain in feet preventing walking

A 28-year-old man presented with severe pain and hypersensitivity in the soles of the feet. He was unable to put any weight through the feet due to pain. Pain also disturbed his sleep at night; pain medications had not helped with the foot pain. He was also feeling low in mood; he was very tired and irritable during the day. The patient was recovering from GBS which had developed 3 months previously.

Part 2: Diabetic neuropathy

Research

Acupuncture has been used to control diabetic symptoms, most notably by Wang et al. [6], who treated the dyspeptic symptoms of gastroparesis by electroacupuncture at ST 36 Zusanli and LI 4 Hegu.

A small pilot study evaluated two clinical styles of acupuncture in the treatment of diabetic neuropathy. Japanese acupuncture, characterized by very shallow needle insertion, was compared to traditional Chinese acupuncture [7]. Interestingly, those given Japanese acupuncture reported decreased neuropathy-associated pain according to daily diary scores whereas those in the other group reported minimal effects. Both styles lowered pain as measured by the McGill Short Form Pain Score. The TCM style improved nerve sensation according to quantitative sensory testing while the Japanese style had a more equivocal effect. However, with such a small group – only 7 patients in all – no significant conclusions can be drawn. It remains possible that acupuncture could be useful in this situation.

Further work on peripheral neuropathy was undertaken by a German group [8]. This was a larger study, with 47 patients involved, all of whom were evaluated over 12 months. The results suggested that nerve conduction showed an objective improvement with treatment. Acupuncture analgesia has been compared with standard medication and proved as successful in alleviating the pain from this type of neuropathy [9].

Several ideas have been put forward, including the use of acu-magnets on acupuncture points, particularly for diabetes and insomnia [10].

Bearing in mind that regrowth of peripheral nerves is possible, if the conditions are favourable and destruction has not been complete, acupuncture to improve the local tissue condition and increase local circulation may be very helpful [11].

The following case study is not strictly illustrating diabetic neuropathy but demonstrates the general principles.

Case study 10.2: level 1 case study

This case report describes the treatment of knee pain in a 43-year-old man with alcoholic peripheral neuropathy with axonal damage. He had bilateral dorsiflexor muscle weakness and wore bilateral ankle foot orthoses. He had poor balance but walked independently with one stick. He had been diagnosed with epilepsy 4 years previously.

Part 3: Bell’s palsy

Bell’s palsy is defined as a weakness in one side of the face, causing the facial muscles on that side to droop. There may also be an accompanying feeling of numbness in the area. The patient may have difficulty closing the eye fully and closing the mouth to retain saliva. Occasionally, in addition to the paralysis there is a loss of taste, increased sensitivity to sound and pain or discomfort in or around the ear.

Case study 10.3: level 1 case study – acute facial palsy

The patient was a 27-year-old female who had a middle- and outer-ear infection that was also diagnosed as shingles which affected balance and caused nausea. Her ear became red and swollen. She was given painkillers and antibiotics by her GP. Six days later she noticed that her mouth did not feel right when she was cleaning her teeth. She could not spit properly. Her parents also noticed she looked odd. She realized something was wrong with her facial muscles when she went to wipe her eye and touched her eyeball. Two days later she was unable to close her eye and the left side of her face had slumped. Her smile was asymmetrical. She went to her GP who diagnosed shingles/herpesvirus and gave her antiviral medications. She was also referred to an ear, nose and throat (ENT) specialist who prescribed prednisolone. She was advised that the problem could take 6 months to go – or might not go at all.

Part 4: Restless-legs syndrome (RLS)

This syndrome is described as a common sensorimotor disorder of unknown aetiology. It is sometimes associated with multiple sclerosis. It ranges in severity from merely causing annoyance in the patient to actively affecting sleep and quality of life. Lifestyle changes such as decreased use of caffeine, alcohol and tobacco together with regular sleep and exercise are frequently recommended and these provide some relief. In about half of the patients there seems to be some family tendency.

Part 5: Postherpetic neuralgia

This is a complication of herpes zoster or shingles, and seems to occur most often in middle-aged to elderly patients. It is characterized by persistent pain following the course of the intercostal nerve at the level which was originally infected by the virus. It can be shooting or burning in nature and can sometimes lead to allodynia, a supersensitivity to non-noxious stimuli in the area. This can make normal clothing very uncomfortable. This pain can be very debilitating, often leading to fatigue, insomnia, anxiety and depression.

Treatment is started when the rash first appears and may continue for many months, or even years, once this condition becomes chronic.

Case study 10.4: level 2 case study

The patient was a school teacher, aged 58. He had retired early due to postherpetic neuralgia from which he had suffered for 4 years. This was atypical in that the area most affected seemed to be concentrated on his right eye. He was often forced to cover the eye with an eye patch as he found light painful and he took over-the-counter painkillers constantly to control a ‘burning, stabbing’ pain in that area. This was often spread over the right side of his face.

Part 6: Phantom limb pain

This is the pain felt by the patient in a non-existent limb after amputation, whether accidental or surgical. In fact the limb does not need to be physically lost since this also occurs in conditions in which the brain is dissociated from the body such as in peripheral nerve injury and after spinal cord injury when an area loses sensation and usually movement too. It can be very distressing to the patient and, while it clearly originates in the spinal column, it is usually described in terms of the perceived locality as in ‘my hand is burning’ or ‘my foot feels as though it’s being crushed’.

References

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[7] Ahn A.C., Bennani T., Freeman R., et al. Two styles of acupuncture for treating painful diabetic neuropathy – a pilot randomised control trial. Acupunct Med. 2007;25(1–2):11-17.

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