Spasmus nutans

Delorme C, Gras D, Roze E. Spasmus nutans: more than meets the eye. Pediatr
Neurol. 2015 Oct;53(4):367-8.

“Spasmus nutans is a rare transient movement disorder of early childhood, defined by the clinical triad (1) nystagmus, (2) head nodding, and (3) torticollis, in the absence of any associated ophthalmological or neurological condition. [1] This condition might be difficult to recognize and is rarely reported in the literature.”

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Adrenocorticotrophic hormone (ACTH) therapy in infantile spasms

Mytinger JR, Weber A, Heyer GL. The response to ACTH is determined early in
the treatment of infantile spasms. Epileptic Disord. 2015 Mar;17(1):52-7.

Although adrenocorticotropic hormone is the most commonly used treatment for infantile spasms in the United States, the optimal regimen for this indication is not known. The purpose of this study was to elucidate the optimal adrenocorticotropic hormone treatment duration. We conducted a retrospective chart review of response to adrenocorticotropic hormone among all patients with infantile spasms managed at our institution from January 2009 to September 2013. Treatment response was defined as clinical remission for greater than or equal to 28 days starting at any point within the adrenocorticotropic hormone course and remission of hypsarrhythmia (or definite EEG improvement if hypsarrhythmia was absent at baseline). For responders, the diagnostic and post-treatment EEG tracings were reviewed. Electroclinical remission was achieved in 21 of 39 patients (54%) receiving adrenocorticotropic hormone, including 11/25 (44%) receiving a long course (typically 12 weeks) and 10/14 (71%) receiving a short course (typically four weeks). The mean time to clinical remission was 5.8 days (median: 5 days; range: 1-20 days). Only one patient responded beyond two weeks of treatment. This study provides Class IV evidence that among patients with infantile spasms, the response to adrenocorticotropic hormone is most often determined early in the treatment course. Given the importance of rapid remission, clinicians should consider adding or changing treatment if infantile spasms do not resolve within two weeks of adrenocorticotropic hormone initiation. Further study is needed to determine the optimal adrenocorticotropic hormone regimen for infantile spasms.

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Riikonen R. Recent advances in the pharmacotherapy of infantile spasms. CNS Drugs. 2014 Apr;28(4):279-90.

“The optimal dosage and duration of ACTH therapy is still unknown, and low doses of ACTH are as effective as high doses. Shorter treatment lag probably improves long-term cognitive outcome. ACTH is superior to vigabatrin for cognitive outcome in cryptogenic groups. Different corticosteroids and AEDs can be given as second-line drugs when first-line drugs are ineffective or are contraindicated.”

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Infantile spasms

Hancock EC, Osborne JP, Edwards SW. Treatment of infantile spasms. Cochrane
Database Syst Rev. 2013 Jun 5;6:CD001770.

To date, few well-designed RCTs have considered the treatment of infantile spasms, and the numbers of patients enrolled have been small. In the majority, methodology has been poor, hence it is not clear which treatment is optimal in the treatment of this epilepsy syndrome. Hormonal treatment resolves spasms in more infants than vigabatrin, but this may or may not translate into better long-term outcomes. If prednisolone or vigabatrin is used, high dosage is recommended. Vigabatrin may be the treatment of choice in tuberous sclerosis. Resolution of the EEG features may be important, but this has not been proven. Further research using large studies with robust methodology is required.

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