P.098 The Epilepsy Surgery Experience in Children with Infantile Spasms
                        at the Hospital for Sick Children

Background: Infantile spasms (IS) is an epileptic encephalopathy, characterized by epileptic spasms, hypsarrhythmia, and developmental regression. This is a retrospective case series detailing the experience in children with IS who have undergone epilepsy surgery at The Hospital for Sick Children (HSC). Methods: Records of 223 patients from HSC were reviewed. Patients were included if they had a current or previous history of IS with a lesion detected on MRI/PET scan who underwent epilepsy surgery. Results: Nineteen patients were included. The etiology of IS was encephalomalacia in six patients (32%), malformations of cortical development in 11 patients (58%), atypical hypoglycaemic injury in one patient (0.5%), and partial hemimegalencephaly in one patient (0.5%). The median age at the onset of IS was five months. The median age at surgery was 18 months. Nine patients (47%) underwent hemispherectomy and 10 patients (53%) underwent lobectomy/lesionectomy. Fifteen patients (79%) were considered ILAE Seizure Outcome Class 1. Developmental outcome was improved in 14/19 (74%) and stable in 5/19 (26%) patients. Conclusions: Even with a generalized EEG pattern such as hypsarrhythmia, patients should be considered for focal resective surgery. Early surgical intervention shortens the duration of active epilepsy thus limiting the potentially irreversible effects of on-going seizures.

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B.6 High dose diazepam treatment for Electrical Status Epilepticus in Sleep (ESES): Is it effective?

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/cjn.2021.275 ◽

2021 ◽

Vol 48 (s3) ◽

pp. S17-S17

Author(s):

C Gorodetsky ◽

T Go ◽

S Weiss

Keyword(s):

Status Epilepticus ◽

Epileptiform Activity ◽

Case Series ◽

Epileptic Encephalopathy ◽

Epilepsy Syndrome ◽

Pediatric Epilepsy ◽

High Dose ◽

Retrospective Case ◽

Cognitive Improvement ◽

Clinically Significant

Background: Epileptic encephalopathy with electrical status epilepticus in sleep (ESES) is a pediatric epilepsy syndrome with sleep induced epileptic discharges and acquired impairment in cognition, language and/or behavior. Despite the widespread use of high dose diazepam, there is limited research on its efficacy. Methods: Single-center, retrospective case-series of children presenting with cognitive/ language regression and ESES from 2014-2019. All children underwent baseline overnight EEG followed by diazepam (1mg/kg) administered per rectum, and continuation of 0.5 mg/kg of oral diazepam for 3 months. Follow up EEGs were performed following the first dose and after 6-9 weeks of treatment. Results: 23 children were included [male 14 (60%); mean age 7 years (4 -12)]. 10 children (45%) had symptomatic epilepsy (defined by abnormal MRI and/or genetic evaluation). Decrease in more than 25% of the spike activity was seen in 18 (78%). This effect was sustained in 11 children (47%) after 6 weeks. Only 6 (60%) children from the symptomatic group had EEG response, while 11 (91%) responded from the idiopathic group. 5 children (21%) had clinically significant cognitive/ language improvement. Conclusions: Treatment with diazepam reduces epileptiform activity in ESES in majority of children. Despite this reduction only minority of patients experience clinically significant cognitive improvement.

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Secondary Abnormal CSF Neurotransmitter Metabolite Profiles in a Pediatric Tertiary Care Centre

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/cjn.2017.271 ◽

2017 ◽

Vol 45 (2) ◽

pp. 206-213 ◽

Cited By ~ 3

Author(s):

Clara D.M. van Karnebeek ◽

Mary Dunbar ◽

Csilla Egri ◽

Bryan Sayson ◽

Janetta Milea ◽

...

Keyword(s):

Movement Disorders ◽

Tertiary Care ◽

Case Series ◽

Retrospective Chart Review ◽

Epileptic Encephalopathy ◽

Tertiary Care Centre ◽

Retrospective Case ◽

Chi Square ◽

Number Of Patients ◽

Patients With Epilepsy

AbstractBackground:Secondary neurotransmitter deficiencies have been reported in several reviews. Our primary aim was to assess the relationship among epilepsy, antiseizure medications, and specific neurotransmitter abnormalities. We also evaluated movement disorders and brain abnormalities via magnetic resonance imaging scans in patients with secondary neurotransmitter defects.Methods:This is a retrospective case series of 376 patients who underwent neurotransmitter analysis at BC Children’s Hospital between 2009 and 2013, for a variety of neurological presentations. The biochemical genetics laboratory database was interrogated for results of cerebrospinal fluid neurotransmitter analyses. Clinical data for patients with abnormal results were collected from the hospital charts. Statistical analysis included one-way analysis of variance, chi-square, and a two-way contingency table.Results:Abnormal neurotransmitter values were identified in 67 (17.8%) patients, two (0.53%) of which were attributable to a congenital neurotransmitter disorder and 11 (16.9%) secondary to other genetic diagnoses. Of 64 patients with secondary abnormal neurotransmitter values, 38 (59%) presented with epilepsy and 20 (31%) with movement disorders. A combination of epilepsy and movement disorder was less frequent.Discussion:Acknowledging the limitations of this retrospective chart review, we conclude that, in our cohort, in addition to patients with movement disorders, a considerable number of patients with epilepsy and epileptic encephalopathy also showed secondary neurotransmitter mono-amine abnormalities. There is no clear relation, however, between clinical phenotype and type of neurotransmitter affected. In addition, no association was identified between the type of antiseizure medications and affected neurotransmitter type. We outline the need for prospective studies to further enrich our understanding of the relation between epilepsy and neurotransmitters with a focus on improving treatments and patient outcomes.

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P36 A 1 and 5 year outcome analysis of epilepsy surgery in adults and children

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2019-abn.108 ◽

2019 ◽

Vol 90 (3) ◽

pp. e34.2-e34

Author(s):

A Chadwick ◽

V Josan ◽

C Pringle ◽

O Pope

Keyword(s):

Prognostic Factors ◽

Epilepsy Surgery ◽

Intractable Epilepsy ◽

Outcome Analysis ◽

Seizure Outcome ◽

Class Iii ◽

Retrospective Case ◽

Good Outcome ◽

Post Surgery ◽

Clinical Records

ObjectivesTo evaluate the efficacy of epilepsy surgery at 1 year following surgery and to assess sustainability of seizure control at 5 years post-surgery. To find prognostic factors for good surgical outcomes.DesignRetrospective case notes analysis.Subjects82 consecutive patients operated on for epilepsy by a single surgeon between 2008–2013.MethodsPatients were identified in pre-existing hospital databases and included if 5 year follow-up was available. Their clinical records were accessed via the electronic patient records system at each site. The Engel Classification was used to grade seizure outcome at years 1 and 5 post-surgery. We classified Engel Class I and II as a good outcome and Engel class III and IV as a poor outcome. Case notes were also examined to find prognostic factors for a good long-term surgical outcome.Results86.4% of patients had a good outcome 1 year post-surgery and 77.9% had a good outcome at 5 years. The surgery site (temporal or otherwise) and the presence of a good outcome at year one were found to contribute significantly to odds of a good outcome at year 5. The ability to wean off medication is also commonly an important factor for patients and 24.4% of patients were AED free at 5 years.ConclusionsSurgery is an effective management option for intractable epilepsy in suitably selected patients with complete or near complete seizure cessation in about 80% of the patients and about a quarter of the patients no longer requiring AEDs.

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CATARACT SURGERY;

The Professional Medical Journal ◽

10.29309/tpmj/2008.15.03.2833 ◽

2008 ◽

Vol 15 (03) ◽

pp. 387-391

Author(s):

EJAZ AHMAD JAVED ◽

MUHAMMAD SULTAN

Keyword(s):

Cataract Surgery ◽

Axial Length ◽

Previous History ◽

Anterior Chamber Depth ◽

Case Series ◽

Retrospective Case ◽

Axis Ii ◽

Corneal Scarring ◽

History Of ◽

Iol Power

Objectives: To describe the variation of axial length in patients undergoingcataract surgery. Study design: A retrospective case series. Place and duration of study: At OpthalmologicalDepartment, Allied Hospital, PMC, Faisalabad from May 2006 to June 2007. Patients and methods: The axial lengthof 566 patients who were admitted for cataract surgery were measured with A. scan (Axis II, Quantel). The elevenpatients with age below 15 years and above 90 years and with history of trauma, corneal scarring were excluded. Sothere were 555 patients for this study. A careful history of diabetes mellitus, hypertension, trauma, previous history ofsurgery, glaucoma and uveitis was taken, and slit lamp examination, tonometry, pupillary reactions, perception andprojection of light was done. The data collected was entered in specially designed Performa. An average of tenreadings of axial lengths with A-Scan for each patient was taken. Results: Out of 555 patients, there were 350 male(63.06%) and 205 female (36.94%) patients. There were 250(45.05%) patients having age between 46 to 60 years.There were 27(4.86%) patients having age between 15 to 30 years and the same number 27(4.86%) of patients wasseem having age between 76 to 90 years. The most of the patients 273(49.18%) had axial length between 23mm to25 mm. There were only 3 patients with axial length between 29.01 to 31 mm. There were a significant number ofpatients, 230(41.45%) having axial length between 21.01 to 23mm. Conclusion: The biometry depends upon axiallength, kratometry and anterior chamber depth. Most of the formulae supposed for IOL calculations depend upon onlytwo factors, the axial length and the keratometry. In our community, short and long eyes are very rare and so SRK-Tformula for IOL calculations provides satisfactory postoperative results. The axial length carries more importance asits variation causes a gross change in IOL power and postoperative refractive errors.

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Local Experience in using Hormonal Therapies according to the UKISS Studies in the Treatment of Infantile Spasms in Hong Kong

Journal of Pediatric Epilepsy ◽

10.1055/s-0038-1636947 ◽

2018 ◽

Vol 07 (01) ◽

pp. 027-030 ◽

Cited By ~ 2

Author(s):

Chung Yin Mo ◽

Chi Lap Yuen

Keyword(s):

Hong Kong ◽

Hormonal Therapy ◽

Detrimental Effect ◽

Case Series ◽

Epileptic Encephalopathy ◽

Infantile Spasms ◽

Local Experience ◽

Hormonal Therapies ◽

The Impact

AbstractInfantile spasms constitute severe epileptic encephalopathy in infancy with characteristic seizure semiology and electroencephalographic patterns. Besides the spasms, the most detrimental effect is the impact on future developmental outcomes. Different treatment methods are used, but hormonal therapy is the most well-accepted method for treating infantile spasms in nontuberous sclerosis cases. After the launch of the original United Kingdom Infantile Spasms Study (UKISS) and the two follow-up articles, the regimens of hormonal therapy became more structured and the expected treatment outcomes were studied better. We would like to share our local experience using this protocol to treat patients with infantile spasms in Hong Kong with a case series of four patients.

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Response to Steroids in IQSEC2-Related Encephalopathy Presenting with Rett-Like Phenotype and Infantile Spasms

Journal of Pediatric Genetics ◽

10.1055/s-0040-1721371 ◽

2020 ◽

Author(s):

Divya Nagabushana ◽

Aparajita Chatterjee ◽

Raghavendra Kenchaiah ◽

Ajay Asranna ◽

Gautham Arunachal ◽

...

Keyword(s):

Late Onset ◽

Neurodevelopmental Disorder ◽

Epileptic Encephalopathy ◽

Infantile Spasms ◽

The Past ◽

Resource Limited ◽

The Central Nervous System ◽

Motor Milestone ◽

Behavior And Cognition ◽

Atypical Rett Syndrome

Abstract Introduction IQSEC2-related encephalopathy is an X-linked childhood neurodevelopmental disorder with intellectual disability, epilepsy, and autism. This disorder is caused by a mutation in the IQSEC2 gene, the product of which plays an important role in the development of the central nervous system. Case Report We describe the symptomatology, clinical course, and management of a 17-month-old male child with a novel IQSEC2 mutation. He presented with an atypical Rett syndrome phenotype with developmental delay, autistic features, midline stereotypies, microcephaly, hypotonia and epilepsy with multiple seizure types including late-onset infantile spasms. Spasms were followed by worsening of behavior and cognition, and regression of acquired milestones. Treatment with steroids led to control of spasms and improved attention, behavior and recovery of lost motor milestone. In the past 10 months following steroid therapy, child lags in development, remains autistic with no further seizure recurrence. Conclusion IQSEC2-related encephalopathy may present with atypical Rett phenotype and childhood spasms. In resource-limited settings, steroids may be considered for spasm remission in IQSEC2-related epileptic encephalopathy.

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