Lack of coordination of muscles during voluntary movements is called ataxia. The part of brain that controls muscle coordination is called cerebellum. When this cerebellum fails in maintaining coordination, ataxia occurs. Ataxia is a neurological sign and symptom. Ataxia may affect movements, speech, eye movements and ability to swallow. Men and women both are equally affected.
Cerebellum is a part of a brain that has an important role in motor control. Cerebellum has also some involvement in cognitive functions like attention,language and regulation of fear and pleasure.Cerebellum has no role in initiation of movement but it helps in coordination and accurate timings of the movement.Sensory system of spinal cord and other parts of the brain sends impulses to the cerebellum.These impulses are combined by the cerebellum to form fine tune motor activity.How To Classify Ataxia ?
1. Cerebellar ataxia: In this cerebellum is affected and causes symptoms of ataxia such as:
• There is no co-ordination between organs, muscles, limbs or joints.
• Inability to control distance, power, and speed of an arm, hand, leg or eye movement.
• The body parts tremble as voluntary movement is carried out.
• Slurred speech.
2. Sensory ataxia: In this type of ataxia, dorsal column of spinal cord is affected.
• Loss of perception about body parts.
• An unsteady stamping gait.
3. Vestibular ataxia: Impairment of inner ear causes vestibular ataxia. It results in vertigo with nausea and vomiting. Following symptoms appears with advancement of the disease:
• Difficulty in swallowing.
• Poor facial expressions.
• Arch of the foot becomes high.
It is progressive disease with high mortality rate.
It is most commonly seen in teen years.
• Deficiency of vitamin B12
• Brain tumor.
• Congenital cerebellar malformations, cerebellar palsy, stroke, multiple sclerosis.
• Abuse of alcohol.
• Lead or mercury poisoning.
• Head injury.
Patient presents himself with following signs and symptoms:
• Lacking in coordination.
• Gait becomes unsteady.
• Difficulty while eating, writing, swallowing.
• CT scan or MRI
• Blood tests
• Urinalysis- 24-hour urine is advised if Wilson’s disease is suspected.
• Genetic tests, to rule out Hereditary Ataxia.
No specific treatment is there for ataxia.In some cases beta adrenergic blockers may help in muscle co-ordination.
• Speech therapy
• Supplementary diet
• Adaptive devices are used, such as canes or walkers for walking, modified utensils for eating, communication aids for speaking. Difficulties for patients suffering from ataxia and how to help these patients.People with ataxia often encounter difficulties with speech and swallowing.
Following are some problems faced by patients with ataxia and some helpful tips for the patients. Problems with swallowing (‘dysphagia’). Ataxia can affect the muscles needed for chewing and swallowing in the same way that other groups of muscles may be affected. They may be weak and their movements slow or inaccurate or mistimed.
Dysphagia can involve problems with chewing, food sticking in the throat or difficulty moving the food back through the mouth. Eating can become very slow, exhausting and messy. People may cough or choke during and after eating, which can be very frightening. Too much saliva means dribbling from the mouth. These problems can combine to make eating stressful and unpleasant. In addition, some food might remain in the mouth after eating, which can cause oral hygiene problems.
People may also experience aspiration, where liquid and food from the throat trickle into the airways and down into the lungs.When detected, the symptoms of dysphagia can often be successfully managed to make swallowing easier. Diagnosis and treatment are a team effort, perhaps involving a radiologist to carry out a moving X-ray of the swallow, speech and language therapist, dietitian, the person with ataxia, their family and carers.
Early referral can help in identify and even avoid later problems. It is important to have regular follow-ups. Some practical changes can improve the ability to eat and drink more safely and comfortably.
A softer diet requires less chewing; eating is not so tiring.
•Avoid lumpy or very dry food; add gravy (for example) to drier food.
•Pureed or liquidized food may be easier to manage.
•Thickened drinks (add a cornstarch-based preparation) slows the flow, giving more time and control When eating.
•Alternate liquid with solid food – sip fluid during a meal
•Eat little and often through the day – less tiring than full meals
•If full meals seem ok, eat the main meal at midday when most people have more energy and strength, which makes swallowing safer and easier.
•Drink a small amount of water at the end of a meal to remove any food that lingers in the throat; that clear your mouth by coughing deliberately.
•Posture and atmosphere Good posture: use comfortable, supportive seating; tuck the chin down towards your chest when swallowing to help close the airway and reduce the chance of choking
•Eat in a relaxed atmosphere; cut out distractions (talking or TV) to increase concentration.
•Don’t eat the meal in rush: take time to chew well,allow time between swallows.
•Remain upright for at least 30 minutes after the meal.People who need help with feeding should feel confident in their career(s).Carers should take their time and be particularly careful to make sure each mouthful is swallowed before offering more food or drink. Ideally, everyone caring for a person with ataxia should learn the Heimlich manoeuvre, a first aid treatment for choking. Problems with speech (dysarthria).
Ataxia can also cause difficulty with speaking, disrupting the smooth and efficient working of the lips, tongue, soft palate and larynx (voice box).Everyone is different but there are some recognized features of dysarthria that tend to occur in ataxia: – Some slurring of words (the most common symptom)-Slower speech – Distortion of sounds – Difficulty in varying intonation – Quiet voice or difficulty controlling loudness A small number of people with ataxia can also experience problems with finding words quickly, formulating ideas or following complex instructions. Strategies for communicating with people with ataxia
•Before you start a conversation with somebody new, explain that you sometimes have difficulty with your speech. Ask them to tell you if they don’t understand.This usually helps to relax both speaker and listener;alerts the listener to pay extra attention; and gives them permission to say if they have difficulty understanding (otherwise they may keep quiet so as not to cause embarrassment).
•Talking face-to-face helps.Listeners watching a speaker’s face gain extra help from watching the mouth and if they are closer, will also are able to hear well.
•Reduce background noise if possible – switch off the TV, shut the door or move to another room.
•Depending on how severe the dysarthria is,other tactics may help: – slow the speaking rate to give the tongue more time to get around words – reduce the amount to say by getting to the main point quickly – Speak in short utterances – it allows you to spend more energy on each word – Clearly introduce new topics – the listener will understand much better if they know what the subject is, even when speech is not very good – Clearly highlight the important words in the message – like newsreaders on TV or the radio – if you have to repeat,change the words slightly so that you are not just repeating the same thing, or just say the important words(comparable to a telegram) if the listener still doesn’tunderstand Strategies for listeners.
•Remind the person with ataxia of their communication strategies if they have a tendency to forget to use them.Agreed signals such as raising a hand if tempo becomes too fast help to limit interruptions to conversations when such reminders are necessary.
• Allow plenty of time; talking with someone who has dysarthria always takes longer, and they’ ll manage better if they feel relaxed, not rushed.
• Be an active listener, giving the person with ataxia your full attention. This may well involve concentrating more and be listening harder than usual.
• Be honest. If you don’t understand, don’t pretend to. Acknowledge what you do understand, so that the speaker doesn’t have to repeat the whole message unnecessarily, and then let the speaker try to fill in the gaps.
There is variation in life expectancy.Early death is common.
• Recurrent lung infections.
• Progressive movement disorder.
• Inability to stand with the eyes closed
• Legs weak and calves feel bruised
• Incontinence of urine.
• Loss of libido.
• The patient is nervous his hands tremble.
• There are flashes of light.
• Trembling of the hands while writing.
• Great nervous prostration.
• Great sexual excitement.
• Limbs feels heavy, patient can scarcely lift them.
• Lightning like pain in the back.
• Sensation of cobweb on the face
• Ptosis and diplopia.
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