Pediatric Ataxias Clinical Practice Guidelines (SNLG, 2022)

Italian National Guideline System

These are some of the highlights of the guidelines without analysis or commentary. For more information, go directly to the guidelines by clicking the link in the reference.

December 08, 2022

Clinical practice guidelines on the diagnosis and management of pediatric ataxias from the Italian National Guideline System (SNLG) were published in October 2022 in Frontiers in Neurology.[1]

Acute ataxia

For children who present with signs and symptoms of acute ataxia, assessment by a specialist is recommended after their condition has been stabilized.

If the etiology remains unclear following the history taking and physical examination, toxicologic screening should be performed.

Electroencephalography is recommended for children with acute ataxia who have an epileptic seizure, altered state of consciousness, or fluctuating signs. If infection of the central nervous system is suspected, lumbar puncture with cerebrospinal fluid analysis should be performed.

Magnetic resonance imaging (MRI) and/or computed tomography of the brain should be performed in children with acute ataxia that persists for more than 3 days, as well as those who exhibit focal signs, asymmetric ataxic signs, altered state of consciousness, cranial neuropathy, papilledema, and ophthalmoplegia.

If paraneoplastic ataxia is suspected, a workup to rule out occult neuroblastoma is recommended.

Chronic ataxia

An MRI scan of the brain, which should be performed early in the workup, is recommended for all children with suspected chronic ataxia.

Clinical, electrocardiographic, and echocardiographic examinations to detect potential cardiac disorders, such as hypertrophic cardiomyopathy, are recommended for all children with Friedreich ataxia.

Clinical and radiographic assessments to detect scoliosis and other malformations are recommended for children who have chronic ataxia.

For children with chronic ataxia who have low levels of vitamin E and/or coenzyme Q10A as a result of a genetic mutation, supplementation is recommended.

Genetic testing and counseling should be offered to all children with chronic ataxia and their families.

For more information, please go to Friedreich Ataxia and Ataxia with Identified Genetic and Biochemical Defects.


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