West syndrome

Shan Yao is sweet and neutral, and enters the Lung, Spleen and Kidney meridians. It can either tonify the Qi or nourish the Yin. Its astringent property enhances its tonifying and stabilizing function. This herb is particularly useful in chronic conditions of Qi and Yin deficiency, such as in Thirst and West syndrome (diabetes) to moisten the internal organs and stabilize the body essence. 

In 138 Japanese patients with West syndrome treated with low-dose tetracosactide, the initial effects on seizures and long-term outcome were not related to dose (daily dose 0.005-0.032 mg/kg, 0.2-1.28 IU/kg total dose 0.1-0.87 mg/kg, 4—35 IU/kg) (7). There were moderate or severe adverse effects in 30% of the patients. There was slight loss of brain volume on CT/MRI scans in 64% of the patients, moderate loss in 23%, and severe loss in 4%. The severity of adverse effects correlated with the total dose of corticotropin, and the severity of brain volume loss due to corticotropin correlated well with the daily and total doses. The authors recommended a reduction in the dose of corticotropin in order to avoid serious adverse effects. 

Ito M, Miyajima T, Fujii T, Okuno T. Subdural hematoma during low-dose ACTH therapy in patients with West syndrome. Neurology 2000 54(12) 2346-7. 

Vigabatrin is used as an adjunctive antiepileptic in patients with resistant partial epilepsy with or without secondary generalization, unresponsive to other therapy [2]. Nowadays, vigabatrin is rarely used in the treatment of partial seizures due to several irreversible visual field constrictions associated with its chronic use [57-62], It is regarded by many authorities as a drug of choice in infants with west syndrome (infantile spasms), particularly in cases associated with tuberous sclerosis [62],... 

X-linked infantile spasm syndrome ARX (ISSX West syndrome, WS) Partington syndrome (PRTS) Transcription factor (Tract 1 16 23) (Tract 2 12 20)... 

Ito M, Aiba H, Hashimoto K, Kuroki S, Tomiwa K, Okuno T, Hattori H, Go T, Sejima H, Dejima S, Ikeda H, Yoshioka M, Kanazawa O, Kawamitsu T, Ochi J, Miki N, Noma H, Oguro K, Ozaki N, Tamamoto A, Matsubara T, Miyajima T, Fujii T, Konishi Y, Okuno T, Hojo H. Low-dose ACTH therapy for West syndrome initial effects and long-term outcome. Neurology 2002 58(1) 110-14. 

Patient 16 had presented with West syndrome at 2 yr of age, with electroencepha-lographic changes typical of this disorder. Although seizures responded to clonazepam therapy, she was tetraplegic and totally unresponsive at 3 yr of age. She had repeated episodes of bronchopneumonia. DHA therapy was begun at 3 yr. She died 3 wk later during an episode of bronchopneumonia similar to those she had suffered prior to therapy. 

Schlumberger, E. Dulac, 0. (1994) Dev. Med. Child Neurol. 36, 863-872. A simple, effective and well-tolerated treatment regime for West syndrome. 

There are other seizure conditions in which pyridoxine therapy finds a place. Infantile spasm (spastic convulsions), in combination with diffuse electroenceph-alographic abnormalities (hypsarrhythmia), is referred to as West syndrome. Mental retardation is associated with this condition (32). ACTH is effective for the short-term treatment of infantile spasms. In view of the elevated therapy-associated morbidity, valproic acid and vigabatrin have been used (33). Following reports of beneficial effects of high doses of pyridoxine, initial treatment for one to two weeks with high doses of pyridoxine is the established therapy in some European countries and in Japan (34). Combined therapy with high-dose pyridoxine in association with low-dose corticotrophin has also been reported as a promising therapy for seizure control, normalized EEG, and intellectual outcome. 

H. Yamamoto, Y. Sasamoto, Y. Miyamoto, H. Murakami, and N. Kamiyama. A successful treatment with pyridoxal phosphate for West syndrome in hypophosphatasia. Pediatr. Neurol. 30 216-218 (2004). 

N. Fejerman, R. Cers-Simo, and R. Caraballo. Vigabatrin as a first-choice drug in the treatment of West syndrome. 7. Child Neurol. 15 161-165 (2000). 

Pineda M, Vilaseca MA, Artuch R, et al. 3-Phosphoglycerate dehydrogenase deficiency in a patient with West syndrome. Dev Med Child Neurol 2000 42 629-633. 

Yum MS, Ko TS. Zonisamide in West syndrome an open label study. Epileptic Disord 2009 11(4) 339-44. 

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