Infantile Spasms is a rare disorder consisting of epileptic spasms that occur before the age of one year. This severe epilepsy affects about 0.31 in 1000 births with an average age at onset of 6 months. Treatment usually consists of hormonal therapies (adrenocorticotropic hormone or corticosteroids) or vigabatrin. Decisions about choice of therapy are complicated because these first-line treatm...

BACKGROUND Management of infantile spasms is difficult because current treatment regimens, including many anticonvulsants and hormones, are often ineffective. We conducted this study to determine the effective dose of topiramate (TPM) in Taiwanese children with infantile spasms. METHODS Fourteen patients with infantile spasms were given TPM at an initial dose of 12.5 mg/d, and the dose was ra...

21 new cases of infantile spasms were reported in 1976 from paediatric departments in Denmark. The connection between infantile spasms and the Lennox-Gastaut syndrome is mentioned, because of reports of a significantly higher incidence of HLA-B7 in children with Lennox-Gastaut syndrome. The HLA antigen distribution in 19 of the 21 children was compared with that of 1967 healthy adults. No diffe...

Investigators from the National Infantile Spasms Consortium (NISC) in the USA studied the etiology of new-onset infantile spasms (IS) in 251 infants (mean age at onset, 7.1, range, 0.1-22.7 months).

The modulation of voltage-dependent calcium currents (I(Ca)) by corticotropin was studied in acutely dissociated rat amygdala neurons using whole-cell, patch-clamp recording techniques. Application of corticotropin(1-24) or corticotropin(4-10) increased I(Ca) in a concentration-dependent manner, with half-maximal effective concentrations of 65 and 176 nM and maximal increases of approximately 7...

OBJECTIVE Infantile spasms is diagnosed late even by expert pediatricians. Late diagnosis (later than 3 weeks) can have a negative effect on the long-term prognosis. We aimed to investigate infantile spasms treated with intravenous methylprednisolone pulse. MATERIALS & METHODS In this case series study, 20 infants with infantile spasms in 17-Shahrivar Hospital, Rasht, Iran were enrolled. Drug...

background: irritable bowel syndrome (ibs) with chronic abdominal pain and changes in bowel habits in the absence of any organic cause is identi_ed as the most common known gastrointestinal disorder. ibs affects both sexes and can occur at any age. diagnosis of this disease in younger patients and women is more likely. in the pathophysiology of ibs, hereditary, environmental, visceral, psycholo...

INTRODUCTION The purpose of this study was to describe healthcare resource utilization and costs resulting from early (within 30 days of diagnosis) versus late (>30 days after diagnosis) treatment with prescriptions for H.P. Acthar(®) Gel (repository corticotropin injection; Acthar; Mallinckrodt) to manage infantile spasms (IS). METHODS We included all patients in the Truven Health MarketScan...

BACKGROUND Epilepsy is a serious chronic health condition with a high morbidity impairing the life of patients and afflicted families. Many epileptic conditions, especially those affecting children, are rare disorders generating an urgent medical need for more efficacious therapy options. Therefore, we assessed the output of the US and European orphan drug legislations. METHODS Quantitative a...

BACKGROUND Hemimasticatory spasm is a very rare movement disorder characterized by unilateral, involuntary, paroxysmal contractions of the jaw-closing muscles, causing clinically brief twitches and/or spasms. CASE REPORT A 62-year-old female consulted us with a 30-year history of unusual involuntary twitches in the preauricular region and spasms that hampered jaw opening. During these spasms,...