The influence of etiology upon ictal semiology, treatment decisions and long-term outcomes in infantile spasms and West syndrome

Prognosis research in people with coronary heart disease

Prognosis Research in Health Care ◽

10.1093/med/9780198796619.003.0012 ◽

2019 ◽

pp. 258-267

Author(s):

Adam Timmis ◽

Pablo Perel ◽

Peter Croft

Keyword(s):

Coronary Heart Disease ◽

Heart Disease ◽

Risk Stratification ◽

Secondary Prevention ◽

Treatment Decisions ◽

Long Term Outcomes ◽

Prognosis Research

Coronary heart disease (CHD) outcomes have improved in recent decades because of better treatment, improved investigations, and better secondary prevention. The results of prognosis research have contributed to the development and evaluation of these new components of healthcare for CHD, but have also critically questioned traditional classifications of CHD, emphasized the importance of long-term outcomes in judging the success of healthcare in CHD patients, and highlighted the potential of risk stratification to guide better treatment decisions for individual patients with CHD. This chapter uses example studies to illustrate this story.

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West syndrome: ethiology, management, long-term outcomes

Bulletin of Siberian Medicine ◽

10.20538/1682-0363-2009-4(2)-137-141 ◽

2009 ◽

Vol 8 (4(2)) ◽

pp. 137-140

Author(s):

L. S. Krayeva ◽

V. M. Alifirova ◽

O. V. Grebenyuk

Keyword(s):

Hormone Therapy ◽

Antiepileptic Drugs ◽

Age Of Onset ◽

Neurological Status ◽

West Syndrome ◽

Long Term Outcomes ◽

Long Term Observation

The article is based on long-term observation of 27 patients with West syndrome, age of onset, etiology of symptomatic forms, neurological status, efficiency of hormone therapy and antiepileptic drugs.

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Optimized Treatment for Infantile Spasms: Vigabatrin versus Prednisolone versus Combination Therapy

Journal of Clinical Medicine ◽

10.3390/jcm8101591 ◽

2019 ◽

Vol 8 (10) ◽

pp. 1591

Author(s):

Hahn ◽

Park ◽

Kang ◽

Lee ◽

Kim ◽

...

Keyword(s):

Combination Therapy ◽

Initial Treatment ◽

Dose Group ◽

Treatment Initiation ◽

Infantile Spasms ◽

Second Line ◽

Long Term Outcomes ◽

First Line ◽

Optimized Treatment

Hormone therapies and vigabatrin are first-line agents in infantile spasms, but more than one-third of patients fail to respond to these treatments. This was a retrospective study of patients with infantile spasms who were treated between January 2005 and December 2017. We analyzed the response rates of initial treatment and second-line treatment. Responders were defined as those in whom cessation of spasms was observed for a period of at least one month, within 2 weeks of treatment initiation. Regarding the response rate to initial treatment, combination therapy of vigabatrin with prednisolone showed a significantly better response than that of vigabatrin monotherapy (55.3% vs. 39.1%, p = 0.037). Many drugs, such as clobazam, topiramate, and levetiracetam, were used as second-line agents after the failure of vigabatrin. Among these, no antiepileptic drug showed as good a response as prednisolone. For patients who used prednisolone, the proportion of responders was significantly higher in the higher-dose group (≥40 mg/day) than in the lower-dose group (66.7% vs. 12.5%, p = 0.028). Further studies of combination therapy to assess dosage protocols and long-term outcomes are needed.

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Infantile Spasms and Injuries of Prematurity: Short-Term Treatment-Based Response and Long-Term Outcomes

Journal of Child Neurology ◽

10.1177/0883073817712587 ◽

2017 ◽

Vol 32 (10) ◽

pp. 861-866 ◽

Cited By ~ 3

Author(s):

Adam Wallace ◽

Victoria Allen ◽

Kristen Park ◽

Kelly Knupp

Keyword(s):

Developmental Delay ◽

Intraventricular Hemorrhage ◽

Refractory Epilepsy ◽

Age Of Onset ◽

Periventricular Leukomalacia ◽

Retrospective Chart Review ◽

Infantile Spasms ◽

Long Term Outcomes ◽

Short Term Treatment

The association of infantile spasms and periventricular leukomalacia and/or intraventricular hemorrhage is well documented. Data regarding early treatment-based and long-term outcomes are limited. A retrospective chart review identified children with infantile spasms born prematurely (<37 weeks) with diagnoses of periventricular leukomalacia and/or intraventricular hemorrhage. Thirteen children were included. Median gestational age was 30 weeks and age of onset of infantile spasms was 8 months. Nine children had intraventricular hemorrhage, 10 had periventricular leukomalacia, and 6 children had both. Twelve of 13 children had resolution of spasms. In responders, the successful medication was adrenocorticotropic hormone (ACTH) in 7, topiramate in 3, and vigabatrin in 2. Follow-up after a median of 7.1 years found that all patients had developmental delay but only 1 had refractory epilepsy. Standard therapies (ACTH and vigabatrin) appeared to be more effective than other treatments. Developmental delay is common in children with periventricular leukomalacia / intraventricular hemorrhage and infantile spasms, but refractory epilepsy might be less frequent.

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