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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
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
Children ages 1-3 yrs Diagnosis of exclusion Follows a viral illness varicella, coxsackievirus, echovirus and is suspected autoimmune response
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
Normal CT CSF is typically normal, but may have mild pleocytosis (increases with time) May last weeks to months Prognosis is excellent
Ingestions/Toxins
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
Intermittent Ataxia
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
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
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