Table 4.

Treatable causes of episodic ataxia.

	GENE OR MECHANISM	TREATMENT
Primary EA
EA1	KCNA1	Carbamazepine, other anticonvulsant drugs, (Acetazolamide)#
EA2	CACNA1A	Acetazolamide, 4-AP, dalfampridine, fampridine
EA3	unknown	Acetazolamide
EA4	unknown	Gabapentin
EA5	CACNB4	Acetazolamide
EA6	SLC1A3	Acetazolamide
EA8	UBR4	Clonazepam
Other genetic EA		Carbamazepine
EA9?	FGF14	Acetazolamide
EA10?	CACNA1G	Carbamazepine
EA9?	SCN2A	(Acetazolamide)#
	PRRT2	Carbamazepine
	SLC2A1/GLUT-1 deficiency	Ketogenic diet, Carbamazepine
	ATP1A3	Acetazolamide
Secondary EA
Metabolic	Hypothyroidism	Thyroxine
	Thiamine pyrophosphate deficiency	Thiamine
	Thiamine transporter (SLC19A3)	Biotin, Thiamine
	Biotinidase deficiency (BTD)	Biotin
	Hartnup disease	Niacin supplement
	Maple syrup urine disease (BCKDHA, BCKDHB, DBT)	Dietary restriction of branched-chain amino acids, Thiamine supplement
	Ornithine transcarbamylase deficiency	Dietary restriction of nitrogen intake
	Pyruvate dehydrogenase deficiency (PDHX, PDHA1)	Thiamine, alpha-lipoic acid, ketogenic diet, dichloroacetate
	Mitochondrial	Mitochondrial cocktail
Inflammatory	Multiple sclerosis	Steroids, disease-modifying therapies
	Behcet’s	Immunomodulatory treatment
	Kawasaki disease	High-dose aspirin, IVIG, steroids
Autoimmune	Autoimmune (CASPR2, anti-NMDA-R, anti-Hu/ANNA-1)	Steroids, immunomodulatory treatment
	Bickerstaff brainstem encephalitis	IVIG, Plasmapheresis
Miller-Fisher syndrome	IVIG, Plasmapheresis
Toxic	Toxicity (lead, alcohol, AEDs)	Discontinue medications/toxic exposure
Vascular	TIA/Stroke	Secondary stroke prevention, rehabilitation
Epilepsy	Epileptic pseudoataxia	Antiseizure medications
Iatrogenic	Thalamic deep brain stimulation	Programming adjustment
Functional	Functional	Functional motor and cognitive rehabilitation

# A response is not reliably observed.