Symptoms typically start between the ages of 5 and 15, but in late-onset<unk> , they may occur after age 25 years. The symptoms are broad, but consistently involve gait and limb ataxia, dysarthria and loss of lower limb reflexes. Classical symptoms There is some variability in symptom frequency, onset and progression. All individuals with<unk>  develop neurological symptoms, including dysarthria and loss of lower limb reflexes, and more than 90% present with ataxia. Cardiac issues are very common with early onset<unk>  . Most individuals develop heart problems such as enlargement of the heart, symmetrical hypertrophy, heart murmurs, atrial fibrillation, tachycardia, hypertrophic cardiomyopathy, and conduction defects. Scoliosis is present in about 60%. 7% of people with<unk>  also have diabetes and having diabetes has an adverse impact on people with<unk> , especially those that show symptoms when young. Other symptoms People who have been living with<unk>  for a long time may develop other complications. 36.8% experience decreased visual acuity, which may be progressive and could lead to functional blindness. Hearing loss is present in about 10.9% of cases. Some patients report bladder and bowel symptoms. Advanced stages of disease are associated with supraventricular tachyarrhythmias, most commonly atrial fibrillation. Other later stage symptoms can include, cerebellar effects such as nystagmus,<unk> st saccadic eye movements, dysmetria and loss of coordination (truncal ataxia, and stomping gait). Symptoms can involve the dorsal column such as the loss of vibratory sensation and proprioceptive sensation. The progressive loss of coordination and muscle strength leads to the full-time use of a wheelchair. Most young people diagnosed with<unk>  require mobility aids such as a cane, walker, or wheelchair by early 20s. The disease is progressive, with increasing staggering or stumbling gait and frequent<unk> lling. By the third decade, affected people lose the ability to stand or walk without assistance and require a wheelchair for mobility. Early-onset cases Non-neurological symptoms such as scoliosis, pes cavus, cardiomyopathy and diabetes are more frequent amongst the early-onset cases.
Friedreich's ataxia
Spinocerebellar ataxia type 6
Stratton Parker syndrome
Autosomal recessive cerebellar ataxia type 1
Spinocerebellar ataxia type-13
Sandhoff disease
Jokela type spinal muscular atrophy
Parkes Weber syndrome