a lack of muscle coordination which may affect speech, eye movements, the ability to swallow, walking, picking up objects

and other voluntary movements. An inability to coordinate muscle activity during voluntary movement; most often results from disorders of the cerebellum or the posterior columns of the spinal cord; may involve the limbs, head, or trunk A person with persistent ataxia may have damage in the part of the brain that controls muscle coordination - the cerebellum. causes: multiple sclerosis, a head injury, alcohol abuse, stroke, cerebral palsy, a faulty gene, or a tumor. Ataxia may also be a symptom of incoordination linked to infections. indicate a lack of coordination in a physiological process: - optic ataxia (usually part of Balint's syndrome which includes a lack of coordination between visual inputs and hand movements) - ataxic respiration (uncoordinated respiratory movements, usually due to dysfunction of the respiratory centers of the medulla oblongata). TYPES OF ATAXIA 1. CEREBELLAR ATAXIA caused by a dysfunction of the cerebellum - a region in the brain which is involved in the assimilation of sensory perception, coordination and motor control. causes some basic neurological problems, such as: Floppiness (hypotonia). Lack of co-ordination between organs, muscles, limbs or joints (asynergy). Impaired ability to control distance, power, and speed of an arm, hand, leg or eye movement (dysmetria). Difficulty in accurately estimating how much time has passed (dyschronometria). An inability to perform rapid, alternating movements (dysdiadochokinesia). How the patient is affected depends on which parts of the cerebellar are lesioned, and whether lesions occur on one side (unilateral) or both sides (bilateral). VESTIBULOCEREBELLUM If the vestibulocerebellum is affected the patient's balance and eye movement control will be affected. The patient will typically stand with feet wide apart in order to gain better balance and avoid swaying backwards and forwards (posterior-anterior oscillations). Even when the patient's eyes are open balance is difficult when the feet are together. SPINOCEREBELLUM If the spinocerebellum is affected the patient will have an unusual gait, with unequal steps, sideways steps, and uncertain starts and stops. The spinocerebellum regulates body and limb movements. CEREBROCEREBELLUM If the cerebrocerebellum is affected the patient will have problems carrying out voluntary, planned movements. The head, eyes, limbs and torso may tremble as voluntary moves are carried out. Speech may be slurred, with variations in rhythm and loudness. 2. SENSORY ATAXIA ataxia due to loss of proprioception (Proprioception = the sense of the relative position of neighboring parts of the body, a sense that indicates whether the body is moving with the required effort, and also where the various parts of the body are located in relation to each other. Even if I am blindfolded I know through proprioception if my arm is above my head or hanging down in front of me, by my side, or behind me). A patient with sensory ataxia typically has an unsteady stomping gait, with the heel striking hard as it touches the ground with each step. Postural instability becomes worse in poorly lit environments. If a doctor asks the patient to stand with eyes closed and feet together his/her

instability will clearly worsen. This is because loss of proprioception makes the patient much more reliant on visual data. The patient may find it hard to perform smoothly coordinated voluntary movements with the limbs, trunk, pharynx, larynx, and eyes. 3. VESTIBULAR ATAXIA The vestibular system is affected. In acute (sudden) unilateral cases the patient may experience vertigo, nausea, and vomiting. In slow-onset chronic bilateral cases the patient may only experience unsteadiness, giddiness. SYMPTOMS Initial ataxia symptoms usually include: Poor limb coordination. Dysarthria - slurred and slow speech that is difficult to produce. The patient may also have difficulties controlling volume, rhythm, and pitch. If the ataxia advances other symptoms may also appear: Swallowing difficulties, which may sometimes result in choking or coughing. Facial expressions become less apparent. Tremors - parts of the body may shake or tremble unintentionally. Nystagmus - involuntary rapid rhythmic repetitious eye movement. Movements may be vertical, horizontal, or circular. Pes cavus - a foot with too high an arch. Cold feet - because of a lack of muscle activity. Problems with balance. Walking difficulties - in severe cases the patient may need a wheelchair. Vision problems. Hearing problems. Depression - as a result of having to live and cope with the symptoms. Cerebellar ataxias - Early onset : between the ages of 4 and 26. - Late onset : after the patient is 20 years old. less severe symptoms Ataxia telangiectasia symptoms generally include: Small veins appear around the corner of the eyes, cheeks and ears. Physical and sexual development is usually delayed. Friedreich's ataxia symptoms generally include: The spine curves sideways (scoliosis). The heart muscle becomes weaker (cardiomyopathy). Diabetes. For patients whose symptoms are caused by injury or illness, symptoms often improve over time, and eventually go away completely. CAUSES Vitamin B12 deficiency - as well as other neurological abnormalities, vitamin B12 deficiency can cause overlapping cerebellar and sensory ataxia. Inherited ataxia If you have one of these conditions, you were born with a defect in a certain gene that makes abnormal proteins. The abnormal proteins hamper the ability of nerve cells, primarily in your cerebellum and spinal cord, to function properly and cause them to degenerate over time. As the disease progresses, coordination problems worsen

 For a person to develop Friedreich's ataxia the faulty gene would need to be carried by both the mother and the father (recessive inheritance). Spinocerebellar ataxia, on the other hand, only requires one parent to carry the defective gene (dominant inheritance), and each of his/her offspring would have a 50% risk of developing the condition. *) Autosomal dominant ataxias: Spinocerebellar ataxias. Researchers have labeled 28 autosomal dominant ataxia genes with the designation SCA1 through SCA28, generally numbered according to their order of discovery, and the number continues to grow. Cerebellar ataxia and cerebellar degeneration are common to all types, but other signs and symptoms, as well as age of onset, differ depending on the specific gene mutation. Episodic ataxia. There are six recognized types of ataxia that are episodic rather than progressive EA1 through EA6. All but the first two are rare. EA1 involves brief ataxic episodes that may last seconds or minutes; are triggered by stress, being startled or sudden movement; and often are associated with muscle twitching. EA2 involves longer episodes, usually lasting from 30 minutes to six hours, that also are triggered by stress. If you have this type of ataxia, you also may experience dizziness (vertigo), fatigue and muscle weakness during your episodes. In some cases of episodic ataxia, symptoms resolve in later life. Episodic ataxia doesn't shorten life span, and symptoms may respond to medication, such as acetazolamide (Diamox), which also is used to treat seizures, or the anticonvulsant phenytoin (Dilantin). *) Autosomal recessive ataxias: Friedreich's ataxia. This neurological disorder involves damage to your cerebellum, spinal cord and peripheral nerves. Peripheral nerves carry signals from your brain and spinal cord to your muscles. In most cases, signs and symptoms appear between the ages of 5 and 15, but may occur as early as 18 months or as late as 30 years of age. The rate of disease progression varies. If you have Friedreich's ataxia, however, you're likely to rely on a wheelchair within 15 years of the appearance of symptoms, and your life span may be affected if the disorder includes significant heart disease. The first indication generally is difficulty walking (gait ataxia). The condition typically progresses to the arms and trunk. Muscles weaken and waste away over time, causing deformities, particularly in your feet, lower legs and hands. Other signs and symptoms that may develop as the disease progresses include slow, slurred speech (dysarthria); fatigue; rapid, involuntary eye movements (nystagmus); spinal curvature (scoliosis); and heart disease, including heart enlargement (cardiomyopathy) and heart failure. Ataxia-telangiectasia. This rare, progressive childhood disease causes degeneration in the brain and other body systems. Signs and symptoms usually appear by age 10. The disease causes immune system breakdown (immunodeficiency disease), which increases susceptibility to other diseases. It affects various organs. Telangiectasias are tiny red "spider" veins that may appear in the corners of your child's eyes or on the ears and cheeks. Although they're characteristic of the disease, your child may or may not develop them. Delayed development of motor skills, poor balance and slurred speech are typically the first indications of the disease. Recurrent sinus and respiratory infections are

common. About 1 in 5 children with ataxia-telangiectasia develops leukemia or lymphoma because of dysfunction in the immune system. Most people with the disease need a wheelchair by their teens and die in their teens or early 20s. Congenital cerebellar ataxia. This type refers to ataxia that results from damage to the cerebellum that's present at birth. Wilson's disease. People with this condition accumulate copper in their brains, livers and other organs, which can cause neurological problems, including ataxia. Non-inherited ataxia Even without a family history of ataxia some people may still develop the condition. In some cases, experts never find out what the cause was. The following procedures and circumstances can cause ataxia: Brain surgery. Head injury. Alcohol abuse. Drug abuse. Infections, such as chicken pox (usually goes away after infection has gone). A brain tumor. Exposure to toxic chemicals. Multiple sclerosis, cerebral palsy, and some other neurological conditions. Malformation of the cerebellum while the baby is still in the womb. DIAGNOSIS Magnetic resonance imaging (MRI) or Computerized Tomography (CT) scan - to determine whether there is any brain damage. Genetic tests - to determine whether the patient has inherited ataxia. Blood tests - some types of ataxia affect the make up of blood. Urine tests - urinalysis may suggest specific systemic abnormalities that are linked to some forms of ataxia. If the specialist suspects Wilson's disease the patient may be asked for a 24-hour urine collection to determine how much copper is in the system. In some cases the specialist never knows what caused the ataxia, this is known as sporadic or idiopathic ataxia. TREATMENT use of a cane (walking stick), crutches, walker or a wheelchair. Symptoms such as tremor, stiffness, spasticity, sleep disorders, muscle weakness, depression (or frustration, sadness and anger) may be addressed with targeted physical therapy, speech therapy, medications and counseling. Occupational therapy - the occupational therapist can help the patient manage better around the house and work. This may involve some home adaptations, wheelchair assessments, and making the kitchen more practical for the patient. Speech therapy - the speech therapist can help with swallowing, coughing, choking and speech problems. If speech becomes very difficult the speech therapist can help the patient learn how to use speech aids. Orthopedic care - this can help patients with curvature of the spine (scoliosis). Physical therapy (physiotherapy) - a Physical therapist (physiotherapist) can help maintain

strength and improve mobility. Counseling - patients with ataxia commonly become frustrated and depressed; this usually results from having to cope with some symptoms which affect physical mobility and coordination. Talking to a well qualified counselor, such as a psychotherapist can help. Supplements and nutrition - some patients with ataxia have very low levels of vitamin E and require supplements and/or a special diet. As sensitivity to gluten is more common among ataxia patients, a gluten-free diet also helps. Medication - some patients with ataxia telangiectasia are prescribed gamma-globulin injections to boost their immune systems. There are also drugs for muscle spasms and uncontrollable eye movements.