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5 results

  • Treatment with High-Dose Prednisolone in Vigabatrin-Refractory Infantile Spasms

  • Part of
  • Wafaa Al-Shehhi, Vann Chau, Jennifer Boyd, Carter Snead, Rohit Sharma, Elizabeth Donner, Cristina Go, Puneet Jain
  • Journal:
    Canadian Journal of Neurological Sciences / Volume 49 / Issue 4 / July 2022
    Published online by Cambridge University Press:
    02 July 2021, pp. 532-539
    • Article
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  • Objectives:

    This research aimed to study the short-term seizure outcomes following treatment with 8 mg/kg/day prednisolone in children with infantile spasms (IS) refractory to vigabatrin. We hypothesized that high-dose prednisolone may result in similar rates of electroclinical remission when compared to published ACTH rates.

    Methods:

    All consecutive children with hypsarrhythmia or hypsarrhythmia variant on EEG with/without IS, who had been treated with vigabatrin as first-line anti-seizure medication (ASM) followed by high-dose oral prednisolone (8 mg/kg/day; maximum 60 mg/day) in cases who did not respond to vigabatrin, were included. Clinical and electroclinical response (ECR) at 2 weeks following initiation of treatment and adverse effects were assessed.

    Results:

    Sixty-five children were included. A genetic etiology was seen in 38.5% cases. Complete ECR was seen in 30.8% (20/65) of the patients 2 weeks after vigabatrin. Complete ECR was noted in 77.8% (35/45) of the patients, 2 weeks after prednisolone initiation in children who failed vigabatrin, and this was sustained at 6 weeks in 66.7% (30/45) patients. Prednisolone was generally well tolerated.

    Conclusions:

    High-dose (8 mg/kg/day) oral prednisolone resulted in sustained complete ECR (at 6 weeks) in two-thirds of the children with hypsarrhythmia or hypsarrhythmia variant on EEG with/without parentally reported IS. It was generally well tolerated and found to be safe.

  • Hemispherotomy for Epilepsy: The Procedure Evolution and Outcome

  • Faisal Alotaibi, Raidah Albaradie, Salah Almubarak, Saleh Baeesa, David A. Steven, John P. Girvin
  • Journal:
    Canadian Journal of Neurological Sciences / Volume 48 / Issue 4 / July 2021
    Published online by Cambridge University Press:
    01 October 2020, pp. 451-463
    • Article
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  • Hemispherectomy is a unique epilepsy surgery procedure that has undergone significant modification and evolution since Dandy’s early description. This procedure is mainly indicated to treat early childhood and infancy medically intractable epilepsy. Various epileptic syndromes have been treated with this procedure, including hemimegalencephaly (HME), Rasmussen’s encephalitis, Sturge–Weber syndrome (SWS), perinatal stroke, and hemispheric cortical dysplasia. In terms of seizure reduction, hemispherectomy remains one of the most successful epilepsy surgery procedures. The modification of this procedure over many years has resulted in lower mortality and morbidity rates. HME might increase morbidity and lower the success rate. Future studies should identify the predictors of outcomes based on the pathology and the type of hemispherectomy. Here, based on a literature review, we discuss the evolution of hemispherectomy techniques and their outcomes and complications.

  • Chapter 61 - Non-accidental brain injury

  • from Section 3 - Symptomatic epilepsy
  • Edited by Simon D. Shorvon, Frederick Andermann, Renzo Guerrini
  • Book:
    The Causes of Epilepsy
    Published online:
    05 March 2012
    Print publication:
    14 April 2011, pp 425-432

  • Summary

    Hemimegalencephaly often presents in early infancy with either cerebral hemisphere affected, and occurs in every ethnic group and both genders. Hemimegalencephaly may be the primary etiology of several epilepsy syndromes. In Ohtahara syndrome, tonic seizures, frequently asymmetric, present early in association with a burst suppression pattern. West syndrome is another frequently encountered epilepsy type with infantile spasms present in up to 50% of patients with hemimegalencephaly. The diagnosis of hemimegalencephaly is made definitively by magnetic resonance imaging (MRI) examination; however, early detection using postnatal cranial ultrasound and computed tomography (CT) is possible. Brain single photon emission computed tomography (SPECT) imaging often reveals hypoperfusion of the malformed hemisphere in the interictal state and hyperperfusion during the ictus. The choice of antiepileptic medication rests largely on the seizure type present in each case. Anatomic or functional hemispherectomy have been employed in patients with hemimegalencephaly and both show similar rates of postoperative seizure-freedom.

  • Chapter 104 - Introduction – how status epilepticus is caused

  • from Section 5 - Status epilepticus
  • Edited by Simon D. Shorvon, Frederick Andermann, Renzo Guerrini
  • Book:
    The Causes of Epilepsy
    Published online:
    05 March 2012
    Print publication:
    14 April 2011, pp 723-729

  • Summary

    This chapter presents the definition, epidemiology, pathology, physiology, clinical features, diagnostic tests and principles of management for Rasmussen encephalitis (RE). The histopathological properties of RE are non-specific and are suggestive of chronic multifocal encephalitis. Rasmussen hypothesized a viral etiology, based on the constituents of the immune reaction in the brain such as lymphocyte infiltration and microglial nodules. Magnetic resonance imaging (MRI) of the brain shows progressive hemiatrophy of variable degree in the involved cerebral hemisphere. Fluorodeoxyglucose positron emission tomography (PET) studies showed large areas of hypometabolism which were confined to the affected hemisphere. An early surgery seems to be favored in view of the progressive neurologic devastation and dementia that may directly result from the catastrophic epilepsy. The late complications of the early surgical techniques, including superficial hemosiderosis and hydrocephalus, have been largely overcome by the introduction of disconnective procedures such as functional hemispherectomy and hemispherotomy.

  • Case 75 - Mechanicalventilation

  • from Section II - Neurocritical care
  • Edited by George A. Mashour, Ehab Farag
  • Book:
    Case Studies in Neuroanesthesia and Neurocritical Care
    Published online:
    03 May 2011
    Print publication:
    03 February 2011, pp 253-256

  • Summary

    Patients who are refractory to medical management can be candidates for surgical treatment such as anatomical or functional hemispherectomy. This chapter presents a case study of a 10-month-old male with left-sided hemiparesis. The patient subsequently developed seizures refractory to medical treatment and presented for a right functional hemispherectomy. Postoperatively, antithrombin III (ATIII) levels were checked twice daily and infusions of thrombate III were dosed accordingly. Early surgery for intractable epilepsy is recommended as it has been shown to improve functional outcomes. Anatomic hemispherectomy consists of the resection of the frontal, parietal and occipital cortices, complete temporal lobectomy and insular resection. Perioperative complications associated with this procedure include significant changes in systemic and pulmonary vascular resistance, arrhythmias, cardiac arrest, neurogenic pulmonary edema, seizures, cerebral edema, massive blood loss, and coagulopathy. Patients undergoing hemispherectomy are usually on chronic anticonvulsant therapy.