By Dr Ananya Mandal, MD
Ataxia has no cure. There are no medications that can specifically treat and cure the symptoms of ataxias. However, medications may ease the symptoms by treating the underlying condition that causes the ataxia.
The aim of treatment of ataxia is to improve the quality of life though patient education and provision of aids and devices to maintain self sufficiency and independence for as long as possible.
Ataxias due to underlying causes may be treated by treating these underlying causes that include stroke, a low vitamin E level or exposure to a toxic drug or chemical.
Treatment also revolves around managing the co-existing conditions such as muscle cramps, stiffness, tremor, spasticity as well as depression, anxiety, sleep disorders etc.
Ataxia management team
Ataxia is managed by a team of specialists in different fields. This is called the multi-disciplinary team.
A usual team of specialists who treat ataxias include a neurologist (one who specializes in diseases of the brain and nervous system), an ophthalmologist or eye specialist, a urologist who treats problems like urinary incontinence in these patients, a cardiologist or heart specialist who deals with cardiomyopathy in ataxia patients, a physiotherapist, a speech and language therapist, a psychologist, an occupational therapist, a social worker, diet advisor and a specialist neurology nurse.
A geneticist or a genetic counsellor may be part of the team dealing with hereditary ataxias.
Treatment of ataxias
Symptomatic treatment - Some types of hereditary ataxias may be treated by easing the symptoms. This however does not cure the situation. For example in patients with episodic ataxia type 2, symptomatic relief may be obtained by treating with acetazolamide. Similarly ataxia with vitamin E deficiency may be treated with vitamin e supplements.
Physiotherapy - Physiotherapy helps prevent muscles from weakening or becoming stiff and spastic. This is achieved with a number of physical exercises that strengthen the muscles and maintain flexibility. Special braces may be prescribed to stretch the muscles.
Speech and language therapy - With progression of ataxia there may be slurring of speech and difficulty in communicating. A speech and language therapist may help in preventing these problems to a great extent.
In addition the therapist may also help with difficulties in swallowing. This may be achieved by performing several exercises that can stimulate the nerves used in the swallowing reflex and strengthen the muscles of swallowing.
Nutrition advice - A nutrition advisor may help develop a diet plan for these patients. With worsening swallowing reflexes, eating becomes difficult resulting in malnutrition. A diet plan that includes healthy and balanced options helps prevent malnutrition.
Occupational therapy - Occupational therapy helps the patient maintain as much self sufficiency and independence as possible for as long as possible. It helps patients adapt to their gradual loss of mobility and develop new skills to perform daily activities independently.
The occupational therapist helps patients adopt and use successfully wheelchair and walking aids (crutches), guiderails, stair lifts etc.
Medications for muscle stiffness, spasticity, cramps and pain - Patients with muscle spasms, cramps, pain and stiffness are treated with medications that are termed muscle relaxants. These agents include baclofen or tizanidine. Muscles can be loosened up by injections of botulinum toxin or botox as well. The effects of the injection normally last for up to three months.
Involuntary eye movements of oscillopsia may be treated using medications such as gabapentin. Gabapentin helps control the eye movements. Double vision is corrected by using special lenses called prism lenses.
Tiredness and fatigue may be seen in ataxia patients. This could be due to disturbed sleep. Patients with multiple sclerosis may experience severe fatigue. This may be treated using sleeping aids like benzodiazepines and physiotherapy.
Medications may also be prescribed for urinary incontinence and bladder difficulties seen in hereditary ataxias. These agents are antimuscarinic and help relax the bladder and reduce the urge to urinate frequently. The bladder may be catheterized (a small tube is inserted into the bladder via the urethra) in order to drain out the collected urine.
Erectile dysfunction may be seen in some patients with ataxia. This can be treated using medications known as phosphodiesterase-5 (PDE-5) inhibitors including Sildenafil.
Nerve pain is seen as burning, aching or rapidly moving pain along with tingling and numbness in the affected areas. This can be treated with little efficacy using pain relievers like non steroidal anti-inflammatory drugs (NSAIDs) including ibuprofen, diclofenac, paracetamol etc. Agents that are more effective in treating nerve pain include amitriptyline, gabapentin or pregabalin.
Cardiomyopathy may result in certain ataxias. It is commonly seen in Friedreich’s ataxia resulting in abnormal thickening of the heart muscles. Left untreated it may result in heart failure and arrhythmias. Cardiomyopathy is difficult to treat and has no cure.
Antidepressants and cognitive behavioral therapy
Depression is seen with many of the long term conditions. It is commonly seen after stroke and in movement disorders like ataxia that severely limit activities of daily living and increase dependence on others for routine activities. Depression may be treated using antidepressant medications as well as cognitive behavioral therapy.
Occupational and physiotherapy
Idiopathic late onset cerebellar ataxia (ILOA) is treated similarly as hereditary ataxias using occupational and physiotherapy and providing symptom relief.
Treatment based on cause
The recommended treatment for acquired ataxia depends of the cause of the ataxia. Ataxias caused due to infections for example may be treated with antibiotics. In patients with ataxias after a stroke or a head injury the symptoms may persist until the brain has recovered from the injury. Recovery may be of varying degrees.
Reviewed by April Cashin-Garbutt, BA Hons (Cantab)