Dystonia Health Dictionary

Dystonia: From 3 Different Sources


Abnormal muscle rigidity, causing painful spasms, unusually fixed postures, or strange movements. Dystonia may affect a localized area of the body, or may be more generalized. The most common types of localized dystonia are torticollis (painful neck spasm) and scoliosis (abnormal sideways curvature of the spine). Generalized dystonia may be due to neurological disorders such as Parkinson’s disease, or may also be a side effect of antipsychotic drugs.

Dystonia may be resolved with anticholinergic drugs or with benzodiazepine drugs.

In some cases, biofeedback training may help.

Injections of botulinum toxin into the affected muscles are effective in treating some types of dystonia.

Health Source: BMA Medical Dictionary
Author: The British Medical Association
Dystonia refers to a type of involuntary movement characterised by a sustained muscle contraction, frequently causing twisting and repetitive movements or abnormal postures, and caused by inappropriate instructions from the brain. It is sometimes called torsion spasm, and may be synonymous with ATHETOSIS when the extremities are involved. Often the condition is of unknown cause (idiopathic), but an inherited predisposition is increasingly recognised among some cases. Others may be associated with known pathology of the brain such as CEREBRAL PALSY or WILSON’S DISEASE.

The presentation of dystonia may be focal (usually in adults) causing blepharospasm (forceful eye closure), oromandibular dystonia (spasms of the tongue and jaw), cranial dystonia/Meige syndrome/Brueghel’s syndrome (eyes and jaw both involved), spastic or spasmodic dysphonia/laryngeal dystonia (strained or whispering speech), spasmodic dysphagia (di?culty swallowing), spasmodic torti/latero/ ante/retrocollis (rotation, sideways, forward or backward tilting of the neck), dystonic writer’s cramp or axial dystonia (spasms deviating the torso). Foot dystonia occurs almost exclusively in children and adolescents. In adults, the condition usually remains focal or involves at most an adjacent body part. In children, it may spread to become generalised. The condition has always been considered rare, but commonly is either not diagnosed or mistakenly thought to be of psychological origin. It may, in fact, be half as common as MULTIPLE SCLEROSIS (MS). Similar features can occur in some subjects treated with major tranquillising drugs, in whom a predisposition to develop dystonia may be present.

One rare form, called dopa-responsive dystonia, can be largely abolished by treatment with LEVODOPA. Particularly in paediatric practice this drug will often be tried on a child with dystonia.

Health Source: Medical Dictionary
Author: Health Dictionary
n. muscle dysfunction characterized by spasms or abnormal muscle contraction. One form is a postural disorder often associated with disease of the *basal ganglia in the brain. There may be spasm in the muscles of the face (see hemifacial spasm), shoulders, neck, trunk, and limbs; the arm is often held in a rotated position and the head may be drawn back and to one side. Other forms of dystonia include *torticollis, *blepharospasm, and writer’s *cramp. Dystonic conditions, including blepharospasm, may be helped by the injection of *botulinum toxin. —dystonic adj.
Health Source: Oxford | Concise Colour Medical Dictionary
Author: Jonathan Law, Elizabeth Martin

Botulinum Toxin

The toxin of the anaerobic bacterium CLOSTRIDIUM botulinum is now routinely used to treat focal DYSTONIA in adults. This includes blepharospasm (see EYE, DISORDERS OF), SPASMODIC TORTICOLLIS, muscular spasms of the face, squint and some types of tremor. Injected close to where the nerve enters the affected muscles, the toxin blocks nerve transmissions for up to four months, so relieving symptoms. The toxin is also used in cerebral palsy. Although very e?ective, there are many possible unwanted effects, especially if too high a dose is used or the injection is misplaced.... botulinum toxin

Blepharospasm

Prolonged, involuntary, contraction of one of the muscles controlling the eyelids, causing them to close. It may be due to photophobia, damage to the cornea, or dystonia, for which botulinum toxin (a muscle relaxant) treatment is highly effective.... blepharospasm

Atropa Acuminata Royle Ex

Lindl.

Synonym: A. belladonna auct. non L.

Family: Solanaceae.

Habitat: Kashmir and Himachal Pradesh up to 2,500 m.

English: Indian Belladonna, Indian Atropa.

Ayurvedic: Suuchi.

Unani: Luffaah, Luffaah-Barri, Yabaruj, Shaabiraj.

Action: Highly poisonous; sedative, narcotic, anodyne, nervine, antispasmodic (used in paralysis); parkinsonism; encephalitis; carcinoma; spastic dysmenorrhoea; whooping cough, spasmodic asthma; colic of intestines, gall bladder or kidney, spasm of bladder and ureters; contraindicated in enlarged prostate.

Key application: In spasm and colic-like pain in the areas of the gastrointestinal tract and bile ducts. (German Commission E, The British Herbal Pharmacopoeia.) It is contraindicated in tachycardiac arrhythmias, prostate adenoma, glaucoma, acute oedema of lungs.

A. belladonna L. (European sp. Belladonna, Deadly Nightshade) is cultivated in Kashmir and Himachal Pradesh.

The herb contains tropane (tropine) or solanaceous alkaloids (up to 0.6%), including hyoscamine and atropine; flavonoids; coumarins; volatile bases (nicotine).

Tropane alkaloids inhibit the para- sympathetic nervous system, which controls involuntary bodily activities; reduces saliva, gastric, intestinal and bronchial secretions, and also the activity of urinary tubules. Tropane alkaloids also increase the heart rate and dilate the pupils. These alkaloids are used as an additive to compound formulations for bronchitis, asthma, whooping cough, gastrointestinal hy- permotility, dysmenorrhoea, nocturnal enuresis and fatigue syndrome.

Atropine provides relief in parkin- sonism and neurovegetative dystonia.

The root is the most poisonous, the leaves and flowers less, and the berries the least. (Francis Brinker.)

Dosage: Leaf, root—30-60 mg powder. (CCRAS.)... atropa acuminata royle ex

Spasmodic Torticollis

A chronic condition in which the neck is rotated or deviated laterally, forwards, or backwards, often with additional jerking or tremor. It is a form of focal DYSTONIA, and should not be confused with the far commoner transient condition of acute painful wry-neck.... spasmodic torticollis

Chlorpromazine

n. a phenothiazine *antipsychotic drug used in the treatment of schizophrenia and mania; it is also used to control nausea and vomiting in terminal illness. Common side-effects are drowsiness and dry mouth. It also causes abnormalities of movement, especially *dystonias, *tardive dyskinesia, and *parkinsonism.... chlorpromazine

Cramp

n. prolonged painful contraction of a muscle. It is sometimes caused by an imbalance of calcium and potassium in the body, but is more often a result of fatigue, imperfect posture, or stress. Spasm in the muscles making it impossible to perform a specific task but allowing the use of these muscles for any other movement is called occupational cramp. It most often affects the hand muscles for writing (writer’s cramp), a form of *dystonia.... cramp

Deep Brain Stimulation

(DBS) a surgical treatment involving the implantation of a medical device that sends electrical impulses to specific parts of the brain. DBS in selected brain regions can provide benefits for treatment-resistant movement disorders, such as Parkinson’s disease, tremor, and *dystonia.... deep brain stimulation

Dyskinesia

n. a group of involuntary movements that appear to be a fragmentation of the normal smoothly controlled limb and facial movements. They include *chorea, *dystonia, *athetosis, and those involuntary movements occurring as side-effects to the use of levodopa and the phenothiazines (see tardive dyskinesia).... dyskinesia

Hemifacial Spasm

a type of *dystonia that results in irregular spasms affecting the facial muscles on one side. It is usually due to irritation of the facial nerve by an overlying artery within the skull base. Treatment is with injections of *botulinum toxin.... hemifacial spasm

Rhizotomy

n. a surgical procedure in which selected nerve roots are cut at the point where they emerge from the spinal cord. In posterior rhizotomy the posterior (sensory) nerve roots are cut for the relief of intractable pain in the organs served by these nerves. An anterior rhizotomy – the cutting of the anterior (motor) nerve roots – is sometimes done for the relief of severe muscle spasm or *dystonia.... rhizotomy

Torticollis

(wryneck) n. an irresistible turning movement of the head that becomes more persistent, so that eventually the head is held continually to one side. This is a form of *dystonia; the spasm of the muscles is often painful. It may be caused by a birth injury to the sternomastoid muscle (see sternomastoid tumour). Relief may be obtained by cutting the motor nerve roots of the spinal nerves in the neck region or by injection of the affected muscles with *botulinum toxin.... torticollis

Brain, Diseases Of

These consist either of expanding masses (lumps or tumours), or of areas of shrinkage (atrophy) due to degeneration, or to loss of blood supply, usually from blockage of an artery.

Tumours All masses cause varying combinations of headache and vomiting – symptoms of raised pressure within the inexpansible bony box formed by the skull; general or localised epileptic ?ts; weakness of limbs or disordered speech; and varied mental changes. Tumours may be primary, arising in the brain, or secondary deposits from tumours arising in the lung, breast or other organs. Some brain tumours are benign and curable by surgery: examples include meningiomas and pituitary tumours. The symptoms depend on the size and situation of the mass. Abscesses or blood clots (see HAEMATOMA) on the surface or within the brain may resemble tumours; some are removable. Gliomas ( see GLIOMA) are primary malignant tumours arising in the glial tissue (see GLIA) which despite surgery, chemotherapy and radiotherapy usually have a bad prognosis, though some astrocytomas and oligodendronogliomas are of low-grade malignancy. A promising line of research in the US (in the animal-testing stage in 2000) suggests that the ability of stem cells from normal brain tissue to ‘home in’ on gliomal cells can be turned to advantage. The stem cells were chemically manipulated to carry a poisonous compound (5-?uorouracil) to the gliomal cells and kill them, without damaging normal cells. Around 80 per cent of the cancerous cells in the experiments were destroyed in this way.

Clinical examination and brain scanning (CT, or COMPUTED TOMOGRAPHY; magnetic resonance imaging (MRI) and functional MRI) are safe, accurate methods of demonstrating the tumour, its size, position and treatability.

Strokes When a blood vessel, usually an artery, is blocked by a clot, thrombus or embolism, the local area of the brain fed by that artery is damaged (see STROKE). The resulting infarct (softening) causes a stroke. The cells die and a patch of brain tissue shrinks. The obstruction in the blood vessel may be in a small artery in the brain, or in a larger artery in the neck. Aspirin and other anti-clotting drugs reduce recurrent attacks, and a small number of people bene?t if a narrowed neck artery is cleaned out by an operation – endarterectomy. Similar symptoms develop abruptly if a blood vessel bursts, causing a cerebral haemorrhage. The symptoms of a stroke are sudden weakness or paralysis of the arm and leg of the opposite side to the damaged area of brain (HEMIPARESIS), and sometimes loss of half of the ?eld of vision to one side (HEMIANOPIA). The speech area is in the left side of the brain controlling language in right-handed people. In 60 per cent of lefthanders the speech area is on the left side, and in 40 per cent on the right side. If the speech area is damaged, diffculties both in understanding words, and in saying them, develops (see DYSPHASIA).

Degenerations (atrophy) For reasons often unknown, various groups of nerve cells degenerate prematurely. The illness resulting is determined by which groups of nerve cells are affected. If those in the deep basal ganglia are affected, a movement disorder occurs, such as Parkinson’s disease, hereditary Huntington’s chorea, or, in children with birth defects of the brain, athetosis and dystonias. Modern drugs, such as DOPAMINE drugs in PARKINSONISM, and other treatments can improve the symptoms and reduce the disabilities of some of these diseases.

Drugs and injury Alcohol in excess, the abuse of many sedative drugs and arti?cial brain stimulants – such as cocaine, LSD and heroin (see DEPENDENCE) – can damage the brain; the effects can be reversible in early cases. Severe head injury can cause localised or di?use brain damage (see HEAD INJURY).

Cerebral palsy Damage to the brain in children can occur in the uterus during pregnancy, or can result from rare hereditary and genetic diseases, or can occur during labour and delivery. Severe neurological illness in the early months of life can also cause this condition in which sti? spastic limbs, movement disorders and speech defects are common. Some of these children are learning-disabled.

Dementias In older people a di?use loss of cells, mainly at the front of the brain, causes ALZHEIMER’S DISEASE – the main feature being loss of memory, attention and reasoned judgement (dementia). This affects about 5 per cent of the over-80s, but is not simply due to ageing processes. Most patients require routine tests and brain scanning to indicate other, treatable causes of dementia.

Response to current treatments is poor, but promising lines of treatment are under development. Like Parkinsonism, Alzheimer’s disease progresses slowly over many years. It is uncommon for these diseases to run in families. Multiple strokes can cause dementia, as can some organic disorders such as cirrhosis of the liver.

Infections in the brain are uncommon. Viruses such as measles, mumps, herpes, human immunode?ciency virus and enteroviruses may cause ENCEPHALITIS – a di?use in?ammation (see also AIDS/HIV).

Bacteria or viruses may infect the membrane covering the brain, causing MENINGITIS. Viral meningitis is normally a mild, self-limiting infection lasting only a few days; however, bacterial meningitis – caused by meningococcal groups B and C, pneumococcus, and (now rarely) haemophilus – is a life-threatening condition. Antibiotics have allowed a cure or good control of symptoms in most cases of meningitis, but early diagnosis is essential. Severe headaches, fever, vomiting and increasing sleepiness are the principal symptoms which demand urgent advice from the doctor, and usually admission to hospital. Group B meningococcus is the commonest of the bacterial infections, but Group C causes more deaths. A vaccine against the latter has been developed and has reduced the incidence of cases by 75 per cent.

If infection spreads from an unusually serious sinusitis or from a chronically infected middle ear, or from a penetrating injury of the skull, an abscess may slowly develop. Brain abscesses cause insidious drowsiness, headaches, and at a late stage, weakness of the limbs or loss of speech; a high temperature is seldom present. Early diagnosis, con?rmed by brain scanning, is followed by antibiotics and surgery in hospital, but the outcome is good in only half of affected patients.

Cerebral oedema Swelling of the brain can occur after injury, due to engorgement of blood vessels or an increase in the volume of the extravascular brain tissue due to abnormal uptake of water by the damaged grey (neurons) matter and white (nerve ?bres) matter. This latter phenomenon is called cerebral oedema and can seriously affect the functioning of the brain. It is a particularly dangerous complication following injury because sometimes an unconscious person whose brain is damaged may seem to be recovering after a few hours, only to have a major relapse. This may be the result of a slow haemorrhage from damaged blood vessels raising intracranial pressure, or because of oedema of the brain tissue in the area surrounding the injury. Such a development is potentially lethal and requires urgent specialist treatment to alleviate the rising intracranial pressure: osmotic agents (see OSMOSIS) such as mannitol or frusemide are given intravenously to remove the excess water from the brain and to lower intracranial pressure, buying time for de?nitive investigation of the cranial damage.... brain, diseases of




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