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ULY CLINIC

16 Mei 2025, 19:20:07

Ataxia

Ataxia refers to the incoordination and irregularity of voluntary, purposeful movements and is classically categorized into cerebellar and sensory ataxia. It may present acutely or chronically and can affect gait, limbs, trunk, or speech depending on the underlying etiology.

Classification

Cerebellar ataxia – due to cerebellar dysfunction.

Sensory ataxia – due to impaired proprioception (posterior column or peripheral nerve lesions).

Types of ataxia and their clinical features

Type	Key Characteristics
Cerebellar Ataxia	- Staggering, zigzag gait - Difficulty turning - Loss of balance (with eyes open or closed)
Sensory Ataxia	- Stomping, high-stepping gait - Relies on visual input (worse in dark or eyes closed) - Romberg’s test positive
Gait Ataxia	- Wide-based, unsteady, irregular gait
Limb Ataxia	- Dysmetria: Inability to control range, speed, or strength of limb movements - Intention tremor, puppet-like movements
Speech Ataxia	- Dysarthria with irregular rhythm, stress on syllables - Normal content
Truncal Ataxia	- Inability to sit or stand without support - Titubation (swaying or bobbing of trunk/head)

Emergency interventions for acute ataxia

Suspect raised intracranial pressure or brain herniation.
Immediate steps:
- Assess LOC, pupils, respiratory rate, motor status
- Monitor vital signs
- Elevate head of the bed
- Prepare for CT scan/surgery
- Keep resuscitation equipment ready

History taking and physical examination

Ask about:
- Onset (sudden or gradual)
- Associated diseases: MS, diabetes, infection, stroke, alcohol abuse, toxins
- Family history
Romberg Test:
- Positive with eyes closed = sensory ataxia
- Positive with eyes open = cerebellar ataxia

Medical causes of ataxia

Condition	Features
Cerebellar abscess	Ipsilateral limb, gait, truncal ataxia; headache, fever, oculomotor palsy
Cerebellar hemorrhage	Acute, vomiting, vertigo, dysarthria, LOC changes
Creutzfeldt-Jakob disease	Rapid dementia, myoclonus, aphasia, ataxia
Diabetic neuropathy	Sensory ataxia, pain, weakness, skin/bowel changes
Diphtheria	Neuropathy leading to sensory ataxia, limb paralysis
Encephalomyelitis	Post-viral; rare cerebellar involvement
Friedreich’s ataxia	Genetic, progressive; gait → truncal → limb/speech ataxia
Guillain-Barré syndrome	Ascending paralysis; rare sensory ataxia
Hepatocerebral degeneration	Post hepatic coma; cerebellar signs, chorea
Multiple sclerosis	Cerebellar and sensory ataxia, dysarthria, visual signs
Olivopontocerebellar atrophy	Gait → limb/speech ataxia, chorea, dysphagia
Poisoning (Arsenic/Mercury)	Sensory/gait ataxia, tremors, confusion
Polyneuropathy (paraneoplastic)	Ataxia, weakness, sensory deficits
Porphyria	Motor/sensory neuropathy, mental symptoms
Posterior fossa tumor	Early ataxia, vomiting, papilledema, vertigo
Spinocerebellar ataxia	Progressive; gait, limb ataxia, dysarthria, cramps
Stroke (vertebrobasilar)	Sudden ataxia with motor/sensory deficits, oculomotor signs
Wernicke’s encephalopathy	Gait ataxia from thiamine deficiency; reversible with thiamine

Differentiating motor weakness vs. ataxia

Weakness can mimic ataxia—always assess motor strength.
Severe gait ataxia can exist with minimal limb signs.

Key diagnostic tools

Lumbar puncture – if infection suspected or autoimmune process is suspected
Blood tests – glucose, toxins, liver function, B12, thiamine, autoimmune panels
Genetic testing – for hereditary ataxias
Neuroimaging: CT scan or MRI (especially posterior fossa)
Electrophysiology: Nerve conduction studies for peripheral neuropathy

Clinical pearls

Romberg’s test is crucial in distinguishing ataxia types.
Cerebellar ataxia affects balance with eyes open or closed.
Sensory ataxia worsens with eyes closed.
Always rule out stroke or herniation in sudden ataxia.
Watch for reversible causes (e.g., thiamine deficiency, toxins).

Management overview

Acute emergencies: Stabilize ABCs, manage raised ICP, treat underlying cause
Chronic ataxia: Supportive care, rehabilitation, physical therapy, genetic counseling
Disease-specific treatments:
- Wernicke’s encephalopathy: IV thiamine
- MS relapse: Corticosteroids
- Paraneoplastic syndromes: Treat underlying malignancy

Conclusion

Ataxia is a multifactorial neurological sign rather than a diagnosis. Its accurate classification, prompt recognition of emergency causes, and a systematic approach to diagnosis are essential in guiding appropriate management and improving patient outcomes.

References