From the Guidelines
Ataxia is a neurological disorder characterized by impaired coordination of movement, and the main types of ataxia include cerebellar ataxia, sensory ataxia, vestibular ataxia, and optic ataxia, as noted in the most recent study 1. The types of ataxia can be categorized based on their underlying causes, which can be acquired or hereditary.
- Cerebellar ataxia results from damage to the cerebellum and causes unsteady gait, poor coordination, and slurred speech.
- Sensory ataxia stems from proprioception problems due to damage in sensory nerves or spinal cord pathways.
- Vestibular ataxia results from inner ear or vestibular system dysfunction causing balance issues.
- Optic ataxia involves difficulty with visually guided movements. Ataxia can be acquired through conditions like stroke, multiple sclerosis, alcoholism, vitamin deficiencies, or brain tumors, or it can be hereditary, with major hereditary forms including Friedreich's ataxia, spinocerebellar ataxias (SCA), ataxia-telangiectasia, and episodic ataxias, as discussed in 1. The most appropriate initial imaging for ataxia is MRI, tailored to assess the brain or spine depending on the area of suspected pathology, as recommended in the most recent study 1. Treatment depends on the underlying cause, with some vitamin deficiencies correctable with supplements, while hereditary forms may be managed with physical therapy, occupational therapy, speech therapy, and supportive devices to maintain function and independence, as noted in 1. Genetic counseling is important for those with hereditary forms, and research continues on potential treatments targeting specific genetic mutations, as discussed in 1.
From the Research
Types of Ataxia
- Cerebellar ataxia: This is the more common and easier to identify type of ataxia, resulting from cerebellar disorders 2.
- Sensory ataxia: This type of ataxia is often mistaken for cerebellar ataxia, leading to diagnostic errors and delays, and is caused by sensory disorders 2, 3.
- Spinocerebellar ataxia type 3 (SCA3): This is the most common autosomal dominant inherited spinocerebellar disorder, and can be easily misdiagnosed due to its diverse and not obvious symptoms in the early stages 4.
- Cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (CANVAS): This is a multisystem ataxia defined by the presence of cerebellar ataxia, bilateral vestibulopathy, and a somatosensory deficit 5, 6.
Characteristics of Ataxia
- Poor coordination and balance disorder 3.
- Somatosensory deficit contributing to a significant level of disability in CANVAS 5.
- Abnormal visually enhanced vestibuloocular reflex in CANVAS 5.
- Dizziness and unstable gait, which can be misdiagnosed as persistent postural-perceptual dizziness 4.
Diagnosis of Ataxia
- Clinical assessment and diagnostic algorithm to separate sensory from cerebellar ataxia 2.
- Novel non-contact sensing technique using microwave sensing platform and machine learning approaches to detect and distinguish sensory ataxia and cerebellar ataxia 3.
- Neurophysiological investigation, including sensory nerve action potentials, blink reflexes, and somatosensory evoked potentials, to identify sensory neuronopathy in cerebellar and/or vestibular ataxias 5.
- Genetic testing to diagnose SCA3 and other inherited ataxias 4, 6.