Abnormal prion proteins accumulate in the brain and the spinal cord, damaging neurones (see NEURON(E)) and producing small cavities. Diagnosis can be made by tonsil (see TONSILS) biopsy, although work is under way to develop a diagnostic blood test. Abnormal prion proteins are unusually resistant to inactivation by chemicals, heat, X-RAYS or ULTRAVIOLET RAYS (UVR). They are resistant to cellular degradation and can convert normal prion proteins into abnormal forms. Human prion diseases, along with scrapie in sheep and BSE in cattle, belong to a group of disorders known as transmissible spongiform encephalopathies. Abnormal prion proteins can transfer from one animal species to another, and variant CJD has occurred as a result of consumption of meat from cattle infected with BSE.
From 1995 to 1999, a scienti?c study of tonsils and appendixes removed at operation suggested that the prevalence of prion carriage may be as high as 120 per million. It is not known what percentage of these might go on to develop disease.
One precaution is that, since 2003, all surgical instruments used in brain biopsies have had to be quarantined and disposable instruments are now used in tonsillectomy.
Measures have also been introduced to reduce the risk of transmission of CJD from transfusion of blood products.
In the past, CJD has also been acquired from intramuscular injections of human cadaveric pituitary-derived growth hormone and corneal transplantation.
The most common form of CJD remains the sporadic variety, although the eventual incidence of variant CJD may not be known for many years.... creutzfeldt-jakob disease (cjd)
The basal ganglia play a vital part in producing smooth, continuous muscular actions and in stopping and starting movement.
Any disease or degeneration affecting the basal ganglia and their connections may lead to the appearance of involuntary movements, trembling, and weakness, as occur in Parkinson’s disease.... basal ganglia
It usually arises in alcoholics after withdrawal or abstinence from alcohol.
Early symptoms include restlessness, agitation, trembling, and sleeplessness.
The person may develop a rapid heartbeat, fever, and dilation of the pupils.
Sweating, confusion, hallucinations, and convulsions may also occur.
Treatment consists of rest, rehydration, and sedation.
Vitamin injections, particularly of thiamine (see vitamin B complex), may be given.... delirium tremens
(See anxiety; anxiety disorders.)... generalized anxiety disorder
Treatment of acute hypoglycaemia depends upon the severity of the condition. Oral carbohydrate, such as a sugary drink or chocolate, may be e?ective if the patient is conscious enough to swallow; if not, glucose or GLUCAGON by injection will be required. Comatose patients who recover after an injection should then be given oral carbohydrates. An occasional but dangerous complication of coma is cerebral oedema (see BRAIN, DISEASES OF – Cerebral oedema), and this should be considered if coma persists. Emergency treatment in hospital is then needed. When the patient has recovered, management of his or her diabetes should be assessed in order to prevent further hypoglycaemic attacks.... hypoglycaemic coma
Cause Although this is one of the most common diseases of the central nervous system in Europe – there are around 50,000 affected individuals in Britain alone – the cause is still not known. The disease comes on in young people (onset being rare after the age of 40), apparently without previous illness. The ratio of women-to-men victims is 3:2. It is more common in ?rst and second children than in those later in birth order, and in small rather than big families. There may be a hereditary factor for MS, which could be an autoimmune disorder: the body’s defence system attacks the myelin in the central nervous system as if it were a ‘foreign’ tissue.
Symptoms These depend greatly upon the part of the brain and cord affected by the sclerotic patches. Temporary paralysis of a limb, or of an eye muscle, causing double vision, and tremors upon exertion, ?rst in the affected parts, and later in all parts of the body, are early symptoms. Sti?ness of the lower limbs causing the toes to catch on small irregularities in the ground and trip the person in walking, is often an annoying symptom and one of the ?rst to be noticed. Great activity is shown in the re?ex movements obtained by striking the tendons and by stroking the soles of the feet. The latter re?ex shows a characteristic sign (Babinski sign) in which the great toe bends upwards and the other toes spread apart as the sole is stroked, instead of the toes collectively bending downwards as in the normal person. Tremor of the eye movements (nystagmus) is usually found. Trembling handwriting, interference with the functions of the bladder, giddiness, and a peculiar ‘staccato’ or ‘scanning’ speech are common symptoms at a later stage. Numbness and tingling in the extremities occur commonly, particularly in the early stages of the disease. As the disease progresses, the paralyses, which were transitory at ?rst, now become con?rmed, often with great rigidity in the limbs. In many patients the disease progresses very slowly.
People with multiple sclerosis, and their relatives, can obtain help and guidance from the Multiple Sclerosis Society. Another helpful organisation is the Multiple Sclerosis Resources Centre. Those with sexual or marital problems arising out of the illness can obtain information from SPOD (Association to Aid the Sexual and Personal Relationships of People with a Disability). (See APPENDIX 2: ADDRESSES: SOURCES OF INFORMATION, ADVICE, SUPPORT AND SELFHELP.)
Treatment is di?cult, because the most that can be done is to lead a life as free from strain as possible, to check the progress of the disease. The use of INTERFERON beta seems to slow the progress of MS and this drug is licensed for use in the UK for patients with relapsing, remitting MS over two years, provided they can walk unaided – a controversial restriction on this (expensive) treatment. CORTICOSTEROIDS may be of help to some patients.
The NATIONAL INSTITUTE FOR CLINICAL EXCELLENCE (NICE) ruled in 2001 that the use of the drugs interferon beta and glatiramer acetate for patients with multiple sclerosis was not cost-e?ective but recommended that the Department of Health, the National Assembly for Wales and the drug manufacturers should consider ways of making the drugs available in a cost-e?ective way. Subsequently the government said that it would consider funding a ‘risk-sharing’ scheme in which supply of drugs to patients would be funded only if treatment trials in individuals with MS showed that they were e?ective.
The Department of Health has asked NICE to assess two CANNABIS derivatives as possible treatments for multiple sclerosis and the relief of post-operative pain. Trials of an under-thetongue spray and a tablet could, if successsful, lead to the two drugs being available around 2005.
It is important to keep the nerves and muscles functioning, and therefore the patient should remain at work as long as he or she is capable of doing it, and in any case should exercise regularly.... multiple sclerosis (ms)
The main side effects of sympathomimetics are palpitations and trembling.
Anticholinergics may cause dry mouth, blurred vision, and, rarely, difficulty in passing urine.
Xanthines may cause headaches, nausea and palpitations.... bronchodilator drugs
The main symptoms include sweating, hunger, dizziness, trembling, headache, palpitations, confusion, and sometimes double vision. Behaviour is often irrational and aggressive. Coma may occur in severe cases. Hypoglycaemia may also be the cause of seizures and jittery behaviour in newborns.
Sugar should be eaten at the first sign of a diabetic attack.
An injection of either glucose solution or the hormone glucagon may be given in an emergency.... hypoglycaemia
Symptoms (a sense of breathing difficulty, chest pains, palpitations, feeling light-headed, dizziness, sweating, trembling, and faintness) begin suddenly. Hyperventilation often occurs, causing a pins-and-needles feeling, and feelings of depersonalization and derealization. The attacks end quickly.
Panic attacks are generally a feature of an anxiety disorder, agoraphobia, or other phobias. In some cases, such attacks are part of a somatization disorder or schizophrenia. Behaviour therapy and relaxation exercises may be used in treatment of this condition.... panic attack
Parkinson’s disease A neurological disorder that causes muscle tremor, stiffness, and weakness. The characteristic signs are trembling, rigid posture, slow movements, and a shuffling, unbalanced walk. The disease is caused by degeneration of, or damage to, cells in the basal ganglia of the brain, reducing the amount of dopamine (which is needed for control of movement). It occurs mainly in elderly people and is more common in men.
The disease usually begins as a slight tremor of 1 hand, arm, or leg, which is worse when the hand or limb is at rest. Later, both sides of the body are affected, causing a stiff, shuffling, walk; constant trembling of the hands, sometimes accompanied by shaking of the head; a permanent rigid stoop; and an unblinking, fixed expression. The intellect is unaffected until late in the disease.
There is no cure.
Drug treatment is used to minimize symptoms in later stages.
Levodopa, which the body converts into dopamine, is usually the most effective drug.
It may be used in combination with benserazide or carbidopa.
The effects of levodopa gradually wear off.
Drugs that may be used in conjunction with it, or as substitutes for it, include amantadine and bromocriptine.
Surgical operations on the brain are occasionally performed.
Untreated, the disease progresses over 10 to 15 years, leading to severe weakness and incapacity.
About one third of sufferers eventually develop dementia.... parity
Alcohol withdrawal symptoms start 6–8 hours after cessation of intake and may last up to 7 days. They include trembling of the hands, nausea, vomiting, sweating, cramps, anxiety, and, sometimes, seizures. (See also confusion, delirium tremens, and hallucinations.)
Opioid withdrawal symptoms start after 8–12 hours and may last for 7–10 days. Symptoms include restlessness, sweating, runny eyes and nose, yawning, diarrhoea, vomiting, abdominal cramps, dilated pupils, loss of appetite, irritability, weakness, tremor, and depression.
Withdrawal symptoms from barbiturate drugs and meprobamate start after 12–24 hours, beginning with tremor, anxiety, restlessness, and weakness, sometimes followed by delirium, hallucinations, and, occasionally, seizures. A period of prolonged sleep occurs 3–8 days after onset. Withdrawal from benzodiazepine drugs may begin much more slowly and can be life-threatening.
Withdrawal symptoms from nicotine develop gradually over 24–48 hours and include irritability, concentration problems, frustration, headaches, and anxiety. Discontinuation of cocaine or amfetamines results in extreme tiredness, lethargy, and dizziness. Cocaine withdrawal may also lead to tremor, severe depression, and sweating.
Withdrawal symptoms from marijuana include tremor, nausea, vomiting, diarrhoea, sweating, irritability, and sleep problems. Caffeine withdrawal may lead to tiredness, headaches, and irritability.
Severe withdrawal syndromes require medical treatment.
Symptoms may be suppressed by giving the patient small quantities of the drug he or she had been taking.
More commonly, a substitute drug is given, such as methadone for opioid drugs or diazepam for alcohol.
The dose of the drug is then gradually reduced.... withdrawal syndrome