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Table 4 Symptomatic treatments

From: Guidelines on the diagnosis and management of the progressive ataxias

4.1 Spasticity
 Recommendation Grade
Careful assessment by a neurologist, with advice from a physiotherapist, is required to decide on the type of treatment of spasticity. GPP
Consider physiotherapy first to treat spasticity, and if that does not provide complete benefit use pharmacological treatment. Surgery should be considered in cases where physiotherapy and pharmacological treatments have not worked. GPP
For pharmacological treatment of generalised spasticity consider using the following oral medications (usually in this order due to the profile of side effects and better tolerability): baclofen, tizanidine, gabapentin, clonazepam, dantrolene sodium or diazepam. GPP
To treat focal spasticity refer to a specialised clinic for treatment with intramuscular botulinum toxin injections, followed by physiotherapy. GPP
4.2 Tremor
 Recommendation Grade
Patients with ataxia who have tremors should be offered pharmacological treatment using Propranolol, Primidone, Propranolol and Primidone in combination, Topiramate, Clonazepam and Gabapentin (in this order). GPP
In patients where tremor is extremely debilitating and not responsive to medication a referral to a centre specialising in functional neurosurgery should be considered. D [15,16,17,18]
4.3 Dystonia
 Recommendation Grade
Focal dystonia should be treated with botulinum toxin injections. GPP
Generalised dystonia should be treated with oral medications, followed by surgery if this is not effective. GPP
Patients with dystonic tremor should be offered physiotherapy and oral medications followed by surgery if the former are ineffective. GPP
4.4 Scoliosis
 Recommendation Grade
Regular surveillance of the development of scoliosis in FRDA patients (especially children) is recommended as it is important for it to be treated. GPP
If scoliosis is detected, referral to a physiotherapist and spinal surgeon is recommended. GPP
For mild scoliosis the patient should be kept under close observation and the spinal surgeon should consider treatment with bracing. B [19,20,21]
For severe scoliosis consider surgery to straighten the spine. B [22, 23]
Regular follow-up by a spinal surgeon is recommended after an operation on the spine. B [22, 23]
4.5 Pain
 Recommendation Grade
Treat pain with physiotherapy and/or pharmacological treatments. GPP
Consider use of the following drugs to treat neuropathic pain: Amitriptyline, Nortriptyline, Carbamazipine, Pregabalin, Gabapentin and Duloxetine. GPP
Consider referral to a pain management clinic if pain is severe or limiting daily activities. GPP
4.6 Cardiac involvement in FRDA
 Recommendation Grade
When FRDA is diagnosed a referral to a cardiologist is recommended for the early diagnosis of cardiac problems and the management of cardiac complications, where required. GPP
Regular screening by a cardiologist is recommended in FRDA patients; once every two years before any cardiac disease is documented, and at least annually after manifesting features of asymptomatic cardiac disease. GPP
Transthoracic Echocardiography and ECG should be used for the diagnosis and monitoring of the myocardial changes. GPP
Holter monitoring should be undertaken to detect silent cardiac arrhythmias or the association of symptoms (such as palpitations, shortness of breath) with the underlying rhythm. GPP
A cardiologist should consider pharmacological treatment (including the use of anticoagulants), and in some cases the implantation of pacing devices, in collaboration with the neurologist. GPP
4.7 Bladder problems - lower urinary tract dysfunction
 Recommendation Grade
In primary care, test for urinary tract infection and measure post-void residual (to exclude common causes of urgency and frequency). If these are normal, check for other common causes such as prostate enlargement. GPP
Practical advice should be given about cutting down caffeine, fizzy drinks and alcohol, as well as information about timed voiding and bladder retraining whenever appropriate. The fluid intake should be individualized; a fluid intake of between 1 to 2 L a day is recommended (taking into consideration possible concurrent cardiac issues). GPP
Advice on pelvic floor exercises should be given as it may be helpful especially when symptoms are mild. GPP
Most individuals with overactive bladder symptoms will require antimuscarinic medications (such as tolterodine, oxybutynin, propiverine and solifenacin). GPP
In patients with cardiac complications and/or cognitive problems caution is advised when using antimuscarinic medications. GPP
In patients with cognitive problems, more selectively-acting antimuscarinic medications, such as trospium chloride or darifenacin should be considered. GPP
In some instances, referral to an urologist is recommended eg: in cases of haematuria or suspicion of concomitant urological condition. GPP
4.8 Gastroenterological problems
 Recommendation Grade
Suggest changes in lifestyle (eg: diet, fluid and mobility assistance) for patients with constipation, followed by the use of laxatives or suppositories. GPP
Consider referral for specialist assessment if patients have urgency and faecal incontinence. GPP
4.9 Sexual dysfunction
 Recommendation Grade
Consider discussing sexual function with male patients due to the potential for erectile dysfunction. GPP
Treat erectile dysfunction where appropriate with phosphodiesterase-5 inhibitors. Treatment decisions should balance the needs of the person and the potential side effect of medications e.g., hypotension. GPP
If patients have cardiac pathologies caution should be exercised when considering medication, and consultation with a cardiologist is recommended. GPP
4.10 Swallowing and dysphagia
 Recommendation Grade
If patients show symptoms of dysphagia a referral to a speech and language therapist should be made (see Additional file 1: Table S2). GPP
If there is unintentional weight loss due to dysphagia consider the use of nutritional supplements and refer to a dietician. GPP
If calorie intake cannot be maintained despite supplements, discuss the possibility of a percutaneous gastronomy (PEG) to provide secure feeding. GPP
4.11 Sialorrhoea (excessive salivation)
 Recommendation Grade
Sialorrhoea is normally associated with dysphagia, thus a referral to a speech and language therapist is recommended for assessment of swallow. GPP
Treat sialorrhoea and thick secretions according to Bavikatte et al. 2012 [9] (and the full guidelines). GPP
4.12 Audiology and hearing
 Recommendation Grade
If a patient is experiencing hearing problems refer to Audiology services for a battery of hearing tests. GPP
A hearing aid trial should be considered although it is often not suitable for this patient population. GPP
A trial with an FM hearing device is recommended in cases of ataxia with Auditory Neuropathy Spectrum Disorder (ANSD). C [24, 25]
Refer to hearing therapist or speech and language therapist for guidance on communication tactics. GPP
For those who do not achieve any benefit from hearing aids, consider a referral to a cochlear implant centre. D [26]
In specific cases (e.g. ANSD) a referral to a neuro-otologist should be considered. GPP
4.13 Eye symptoms
 Recommendation Grade
A referral to a neuro-ophthalmologist is recommended if ataxia patients have any eye symptoms. GPP
If disabling nystagmus or oscillopsia is present treatment is recommended, often with either gabapentin or baclofen. B [27,28,29]
Refer to an optometrist or neuro-ophthalmologist for restoration of single vision with prisms in cases of diplopia. GPP
Patients with visual impairment should be offered low vision aids and the possibility of having their visual disability registered. GPP
4.14 Cognition
 Recommendation Grade
When cognitive impairment is suspected (even if mild) referral to a Neuropsychology department is recommended. GPP
Cognitive rehabilitation is recommended for those patients with cognitive impairment. C [30]
Characterising the course of the cognitive impairment is advisable in order to inform the likely prognosis. GPP
4.15 Depression and other psychiatric symptoms
 Recommendation Grade
In many cases depression can be treated in primary care using medications, counselling or cognitive behavioural therapy. GPP
In more severe or complex cases of depression and other psychiatric symptoms a referral to a psychiatrist/neuropsychiatrist in secondary care is recommended. GPP
For adults consult NICE Guidelines for the treatment of depression in patients with a chronic physical disorder [10]. GPP
4.16 Inherited episodic ataxias
 Recommendation Grade
Advise episodic ataxia patients on identification and avoidance of common triggers that may cause attacks such as stress, caffeine and alcohol consumption, and excessive physical exertion. GPP
Acetozolamide is recommended as the first line drug in episodic ataxia types 1 and 2, although not all patients respond. GPP
Patients taking acetazolamide should be advised to keep hydrated to prevent the development of renal calculi and should undergo annual ultrasound screening of the urinary tract. D
Patients with a known hypersensitivity to sulponamides should be counselled at the start of treatment and need to be kept under surveillance. GPP
Consider use of 4-aminopyridine on a named patient basis as second line drug in episodic ataxia type 2 if acetazolamide is not beneficial. C [31]
In episodic ataxia type 1 consider use of carbamazepine, phenytoin or lamotrigine as second line treatment. GPP