What is Ataxia?
Ataxia is a neurological sign characterized by loss of coordination of voluntary muscle movement due to dysfunction of the nervous system structures responsible for integrating movement information, maintaining balance, or transmitting position sense. 1
Core Definition and Pathophysiology
Ataxia refers to the inability to generate coordinated voluntary movement, manifesting clinically as clumsiness, unsteady gait, poor coordination of extremities, and difficulty with precise movements. 1 The underlying pathology can involve multiple nervous system components:
- Cerebellar dysfunction affects neural information integration, coordination planning, and movement execution 1
- Spinal cord and peripheral sensory nerve damage disrupts proprioception (position sense) 1, 2
- Vestibular system impairment affects balance and equilibrium maintenance 1
- Cerebral lesions involving the thalamus, pons, corona radiata, or internal capsule can cause ataxia with hemiparesis 1
Clinical Manifestations
Common Presenting Features
- Wide-based, unsteady gait is the hallmark feature across all ataxia types 1, 2
- Poor coordination of extremities with dysmetria (inability to judge distance) 1
- Truncal ataxia (instability of the trunk) particularly with midline cerebellar pathology 1
- Dysarthria (slurred or unclear speech) 1, 3
- Abnormal eye movements including nystagmus and ocular dysmetria 1, 3
Critical Distinction: Types of Ataxia
Cerebellar Ataxia
- Results from damage to the cerebellum itself 1, 4
- Symptoms do not significantly worsen with eye closure 2, 5
- Associated with nystagmus, dysmetria, and intention tremor 2
- Negative or unchanged Romberg test 1, 5
Sensory (Proprioceptive) Ataxia
- Dramatically worsens when eyes are closed (positive Romberg test) - this is the key distinguishing feature 1, 2, 5
- Caused by dorsal column, dorsal root ganglia, or peripheral sensory nerve dysfunction 2, 5
- Patients rely heavily on vision to compensate for lost position sense 2, 5
- Associated with sensory loss, hyporeflexia, and sometimes weakness 1, 2
- Does not present with nystagmus, dysmetria, or intention tremor unlike cerebellar ataxia 2
- Characterized by a stamping, unsteady gait quality 5, 4
Vestibular Ataxia
- Associated with nausea, vomiting, and vertigo 1
- Lurching gait triggered by head rotation 1
- Results from vestibular system dysfunction 1
Relevance to Older Adults with Parkinsonian Features
In older adults presenting with grip loss and motor symptoms resembling Parkinson's disease, ataxia is not a typical feature of idiopathic Parkinson's disease. 6 However, several important considerations exist:
- Multiple system atrophy (MSA) can present with either cerebellar ataxia or parkinsonism at onset, representing a critical differential diagnosis 6
- Some rare forms of parkinsonism, particularly juvenile or early-onset types, may have ataxia as a prominent feature 6
- Cerebellar degenerative diseases can develop parkinsonian features that may become predominant 6
- The coexistence of both parkinsonian and cerebellar features represents a diagnostic challenge requiring careful evaluation 6
Common Etiologies
Acute Onset (hours to days)
- Stroke (cerebellar or brainstem) 1
- Head trauma 1
- Acute cerebellitis (infectious or inflammatory) 1
- Drug toxicity (metronidazole, alcohol, opiates) 1
- Miller Fisher syndrome (variant of Guillain-Barré) 1
Chronic/Progressive
- Genetic disorders (spinocerebellar ataxias, Friedreich ataxia) 1, 7
- Multiple system atrophy 6
- Paraneoplastic cerebellar degeneration 1
- Chronic alcohol abuse 1
- Vitamin deficiencies (vitamin E, B12) 1, 5
- Structural abnormalities (Chiari malformation, cerebellar tumors) 1
Diagnostic Approach
Clinical Bedside Testing
- Romberg test is essential to distinguish sensory from cerebellar ataxia 1, 2, 5
- Assessment of gait pattern, including observation with eyes open and closed 2, 5
- Evaluation for associated neurological signs (nystagmus, dysmetria, sensory loss, reflexes) 1, 2
Imaging Recommendations
- MRI brain without and with contrast is the primary imaging modality for evaluating ataxia 8
- MRI of cervical and thoracic spine is recommended when proprioceptive ataxia is suspected to evaluate dorsal column pathology 2, 5
- CT has limited utility in non-emergent settings compared to MRI 1
Management Principles
Individuals with ataxia who have poor balance, low balance confidence, and fear of falls or are at risk for falls should be provided with a balance training program. 1
Evidence-Based Interventions
- Assistive devices (canes, walkers) should be prescribed and properly fitted to improve balance and stability 1, 2, 8
- Balance training programs are indicated for all patients with poor balance and elevated fall risk 1, 8, 5
- Postural training and task-oriented therapy may be considered for rehabilitation of ataxia 1
- Treatment of underlying etiology when identified (vitamin supplementation, management of neuropathy) 5
Prognosis Considerations
- Ataxia typically improves during acute rehabilitation after stroke 1
- Ataxia without concurrent hemiparesis has better prognosis for functional recovery 1
- Intensive task-oriented therapy may improve motor performance in stroke-related ataxia 1
Critical Clinical Pitfalls
- Do not assume all unsteady gait is cerebellar - always perform Romberg testing to identify sensory ataxia, which has different implications and workup 2, 5
- Ataxia in the context of parkinsonian features should raise suspicion for multiple system atrophy rather than idiopathic Parkinson's disease 6
- Acute ataxia with altered consciousness, headache, or additional neurological deficits may indicate cerebellar mass effect with herniation risk requiring urgent intervention 1
- Cardiovascular risk factors significantly increase likelihood of vertebrobasilar insufficiency in patients with episodic ataxia and dizziness 8