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Table 2 Metabolic and other etiological diagnoses that respond to specific therapy

From: Infantile spasms (West syndrome): update and resources for pediatricians and providers to share with parents

Diagnosis

Specific therapy

Pyridoxine-dependent seizures

Pyridoxine

Phenylketonuria

Diet

Maple syrup urine disease

Diet

Biotinidase deficiency

Biotin

Menkes disease

Copper histidinate

Hyperammonemia disorders

Possibly diet, depending on which disorder

Nonketotic hyperglycinuria

Benzoate

Tumor

Surgery

Arterial-venous malformation

Surgery

Sturge-Weber syndrome

Surgery if medications fail

Tuberous sclerosis complex

Vigabatrin, ACTH (if vigabatrin fails), and possibly surgery if medications fail

Cortical dysplasias: focal cortical dysplasias, hemimegalencephaly

Possible cortical resection if medications fail

Malformations of cortical development

Epilepsy surgery

  1. Courtesy of Dr Shields.