ATAXIA

The Cerebellum

Coordinates or fine-tunes movements Located in the posterior cranial fossa Bi-hemispheric with ipsilateral function Midline lesions cause truncal instability (vermis)

The Peduncles

Proprioception and sensory afferent via the INFERIOR PEDUNCLES Frontal motor cortex impulses travel via the MIDDLE PEDUNCLES Purkinje fibers course along the SUPERIOR PEDUNCLES to muscular tracts

Ataxia

Inability to make smooth, coordinated movements Usually manifests as unsteady gait May be difficult to access in younger children Vast differential, but can be narrowed down

Approaching Ataxia

History and physical Define time course Recent illnesses? Trauma? Access to medications?

Acute Ataxia

Need to rule out the bad stuff Focal deficits, history of trauma, evidence of ICP, shunt malfunction Fever, nuchal rigidity, toxic appearance

Life-threatening Causes of Ataxia

Meningitis Increased ICP with herniation (2 to mass, bleed, obstruction) Drug intoxication These rarely present as ataxia alone

Evaluation

Computed Tomography for evidence of ICH, hydrocephalus, Posterior fossa tumors Consider spinal tap for meningitis/encephalitis Question caregivers about medicines If negative history, physical and workup then consider other causes

Acute Cerebellar Ataxia

Children ages 1-3 yrs Diagnosis of exclusion Follows a viral illness varicella, coxsackievirus, echovirus and is suspected autoimmune response

Acute Cerebellar Ataxia

Explosive onset and most severe in beginning Wide-based gait, unsteadiness, imbalance while sitting, horizontal nystagmus (50%) and dysarthria May have vomiting, but fever and nuchal rigidity should be absent

Acute Cerebellar Ataxia

Normal CT CSF is typically normal, but may have mild pleocytosis (increases with time) May last weeks to months Prognosis is excellent

Ingestions/Toxins

Alcohol Phenytoin Carbamazepine Benzos Antihistimines

Dextromethorphan Lead Phenothiazines Ethylene glycol Mercury

These would be based on possibility of exposure

Guillain-Barr syndome

Miller Fisher Variant Ataxia, opthalmoplegia and areflexia Usually preceded by viral illness (5-10d) Paralysis of upward, then lateral and finally downward gaze Limb ataxia>truncal ataxia Elevated protein in CSF Recovery in weeks to months

Conversion Reaction

Usually girls age 10-15 Involuntary, but provides a secondary gain Sits without difficulty, but sways upon standing No wide-based gait Normal strength, tone, and reflexes

Other things to consider

Postconcussion Labrynthitis Occult intoxication MS Benign vertigo

Intermittent Ataxia

Basilar migraines Pseudoataxia Metabolic diseases

Basilar Migraine

Recurrent attacks of ataxia followed by severe throbbing headaches May have vision loss, vertigo, parasthesias May evolve into classic migraines N/V in less than 1/3 of cases EEG to rule out benign occipital epilepsy

Pseuodataxia (epileptic ataxia)

Sudden and episodic May be only manifestation of seizure Can be confused with seizure med toxicity May be confused/disoriented Clonazepam and valproate are drugs of choice

Metabolic disease

Hartnup disease DD, photosensitive, decreased tone, episodic ataxia Maple Syrup Urine Disease neonatal seizures, encephalopathy, urine odor and recurrent ataxia Pyruvate Dehydrogenase Deficiency intermittent ataxia and lactic acidosis

Chronic

May be just as worrisome as acute CT/MRI Hydrocephalus Posterior fossa tumors Congenital/hereditary

Brain tumors

Cerebellar astrocytoma Brainstem glioma Ependymoma Meduloblastoma Can also be 2 to supratentorial masses

Hereditary ataxias

Ataxia-telengectasia Friedrich Ataxia Ramsey-Hunt Syndrome

Management

Dependent on cause Most common causes are self-limiting and have great prognosis Head-protection may be necessary Consult neurology Increased concern if other abnormal findings on neuro exam