Sensory (Proprioceptive) Ataxia with Positive Romberg Sign
This 83-year-old man is demonstrating a positive Romberg test, indicating sensory or proprioceptive ataxia rather than cerebellar dysfunction, and requires urgent evaluation for peripheral neuropathy, posterior column spinal cord disease, or posterior fossa pathology affecting vestibular pathways. 1
Clinical Interpretation
The described scenario—falling to one side when closing eyes while seated with arms outstretched—represents a classic positive Romberg test, which specifically indicates:
- Worsening with closed eyes is pathognomonic for sensory/proprioceptive ataxia, distinguishing it from cerebellar ataxia which does not significantly worsen with visual deprivation 1
- The test reveals loss of coordination due to impaired position sense, as patients rely heavily on visual compensation when proprioceptive or vestibular input is compromised 1
- Falling direction (leftward in this case) may indicate lateralized pathology but is not reliably localizing on its own 2
Differential Diagnosis Priority
Primary Considerations
Peripheral sensory neuropathy is the most common cause in elderly patients and presents with:
- Distal proprioceptive loss affecting feet and legs 1
- Preserved strength and reflexes may be diminished 1
- Increased body sway even with eyes open in advanced cases 3
Posterior column spinal cord disease (including vitamin B12 deficiency, tabes dorsalis, or cervical myelopathy) must be considered:
- Loss of vibration and position sense 1
- May have associated sensory level or upper motor neuron signs 1
Vestibular pathology (peripheral or central) can mimic sensory ataxia:
- Acute vestibular neuronitis, posterior fossa stroke, or demyelination 1
- Distinguished by associated vertigo, nausea, nystagmus, or other brainstem signs 1
- A refined Romberg test on foam versus firm surface with eyes closed can differentiate: vestibular patients show dramatically increased sway on foam, while sensory neuropathy patients show greater impairment on firm ground 4, 2
Critical Immediate Evaluation
Bedside Assessment
- Complete three-step test to evaluate for skew deviation or fourth nerve palsy if vertical diplopia present 1
- Assess for additional neurologic signs: cranial nerve palsies, internuclear ophthalmoplegia, Horner's syndrome, hemiparesis, sensory level 1
- Proprioceptive testing: vibration sense at toes, joint position sense 1
- Gait observation: wide-based, stomping gait suggests sensory ataxia 1
- Orthostatic vital signs in this elderly patient to exclude syncope-related falls, as amnesia for loss of consciousness occurs in up to 40% of elderly patients 5
Red Flags Requiring Urgent Imaging
MRI brain and cervical spine with and without contrast is indicated if: 1
- Acute or subacute onset (suggests stroke, demyelination, or mass lesion) 1
- Associated brainstem signs (nystagmus, diplopia, dysarthria, dysphagia, crossed sensory findings) 1
- Upper motor neuron signs suggesting myelopathy 1
- Severe vertigo, nausea, or vomiting suggesting vestibular neuronitis or posterior fossa pathology 1
MRI is the preferred initial imaging modality for evaluating cerebellar ataxia and should include the brainstem; contrast should be added if inflammatory or neoplastic causes are suspected 1
Diagnostic Algorithm
Step 1: Determine Ataxia Type
- Positive Romberg (worsens with eyes closed) → Sensory/proprioceptive or vestibular ataxia 1
- Negative Romberg (no change with eyes closed) → Cerebellar ataxia 1
Step 2: Distinguish Sensory from Vestibular
- Presence of vertigo, nystagmus, or brainstem signs → Vestibular/central pathology requiring urgent MRI 1
- Distal sensory loss, absent ankle jerks, no vertigo → Peripheral neuropathy 1
- Refined Romberg on foam: Dramatically increased sway on foam suggests vestibular; greater impairment on firm ground suggests sensory neuropathy 4, 2
Step 3: Localize Sensory Pathology
- Distal stocking-glove pattern → Peripheral neuropathy (check B12, glucose, TSH, EMG/NCS) 1
- Sensory level, upper motor neuron signs → Spinal cord disease (urgent MRI cervical/thoracic spine) 1
- Isolated proprioceptive loss, normal strength → Posterior column disease (check B12, syphilis serology, MRI spine) 1
Common Pitfalls
- Assuming all positive Romberg tests are benign peripheral neuropathy: Acute onset or associated neurologic signs mandate urgent imaging to exclude stroke or demyelination 1
- Missing posterior fossa pathology: Skew deviation can be comitant and mimic benign conditions, but demands urgent MRI with contrast 1
- Overlooking syncope in elderly patients: Up to 40% have amnesia for loss of consciousness; always check orthostatic vitals 5
- Relying solely on Romberg quotient values: Even quantitative measurements show only modest discrimination between patients and controls; interpret within the full neurological examination context 3
Immediate Management
- If acute onset with brainstem signs: Activate stroke protocol, obtain urgent MRI brain/brainstem with and without contrast, neurology consultation 1
- If subacute with isolated sensory findings: Order vitamin B12, methylmalonic acid, glucose, TSH, EMG/NCS, consider MRI cervical spine 1
- If elderly with falls: Assess orthostatic vitals, review medications (especially antihypertensives, sedatives), fall risk assessment 5
- Refer to neurology for electrodiagnostic testing and further evaluation of spinocerebellar tracts, dorsal columns, or peripheral nerves 1