From the Guidelines
Spinocerebellar ataxia (SCA) primarily causes progressive problems with movement and coordination, with common symptoms including unsteady gait, slurred speech, abnormal eye movements, and poor hand coordination. The symptoms of SCA can vary depending on the specific type, but generally include a range of movement and coordination problems. According to the most recent study 1, ataxia is the inability to generate coordinated voluntary movement, which can manifest clinically with signs and symptoms such as:
- Clumsiness
- Nystagmus
- Dysmetria
- Abnormal or unsteady gait
- Dysdiadochokinesis
- Dyssynergia Additionally, other causes such as muscle weakness and hypotonia may manifest with gait and postural abnormalities that may mimic ataxia 1. The specific symptom pattern varies depending on the SCA type, with symptoms typically worsening gradually over years, resulting from degeneration of the cerebellum and sometimes other parts of the nervous system, affecting the brain's ability to coordinate movement and other functions 1. While there's no cure for SCA, symptom management includes physical therapy, occupational therapy, speech therapy, and medications to address specific symptoms like tremor or muscle stiffness. It is essential to note that the evaluation of ataxia requires careful review of demographics, history, clinical examination, laboratory testing, and neuroimaging to reach a cohesive diagnosis 1. In the setting of ataxia unrelated to recent trauma, MRI is the preferred imaging modality, tailored to assess the brain or spine depending on the area of suspected pathology 1. The use of intravenous contrast is generally helpful in these cases 1. Overall, the management of SCA should focus on improving the patient's quality of life by addressing their specific symptoms and providing supportive care.
From the Research
Symptoms of SCA Ataxia
- Dysphagia is a common symptom in patients with spinocerebellar ataxias (SCAs) and may be a cause of death 2
- Patients with dysphagia have a longer disease duration and more severe ataxia than patients without dysphagia 2
- Other symptoms of SCA include:
- Gait ataxia
- Dysarthria
- Oculomotor disturbances
- Pyramidal and extrapyramidal disease signs
- Rigidity
- Bradycardia
- Sensory deficits
- Mild cognitive and executive function decline 3
- Early symptoms of SCA can vary, but common initial symptoms include:
- Gait difficulty
- Double vision
- Dysarthria
- Impaired hand writing
- Episodic vertigo 4
- The prevalence of dysphagia in different disease stages of SCA 1,2,3, and 6 has been studied, with dysphagia being most common in SCA1, followed by SCA3, SCA 6, and SCA 2 2
- Dysphagia in SCA1 and 3 is associated robustly with ataxia severity, whereas this association is less obvious in SCA2 and 6, demonstrating genotype-specific clinical variation 2
- Evaluation of swallowing ability by videofluoroscopic examination of swallowing (VF) is essential for preventing aspiration in patients with SCA 5