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Optical Coherence Tomography (Oct) and Fundus Fluorescein Angiography (FFA) in Neuro-Ophthalmology
Published in Vivek Lal, A Clinical Approach to Neuro-Ophthalmic Disorders, 2023
Ramandeep Singh, Deeksha Katoch, Mohit Dogra, Basavaraj Tigari, Simar Rajan Singh, Sahil Jain, Bruttendu Moharana, Sabia Handa, Mangat R. Dogra
Vigabatrin, an antiepileptic drug, causes visual field loss which is asymptomatic in early stages. Electrophysiological tests and perimetry are carried out regularly to pick up this visual loss. Problem is in children, where there is low reliability of these tests. Peripapillary RNFL examination on OCT has been shown to demonstrate characteristic nasal pRNFL thinning in patients exposed to vigabatrin22 and these changes have been shown to correlate well with electrophysiological responses and visual field defects.23
Developmental Diseases of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
James H. Tonsgard, Nikolas Mata-Machado
Seizures and tuberous sclerosis–associated neuropsychiatric disorder (TAND) are major complications of TSC and need to be identified early and intervention implemented. The rapalogs, everolimus and sirolimus, have been shown to decrease seizures in patients with TSC and epilepsy. A recently completed European clinical trial (EPISTOP) of the seizure medicine vigabatrin in infants under 5 months of age who had abnormal EEGs, without clinical seizures, showed marked reduction in the incidence and severity of seizures. This, in conjunction with data from animal models demonstrating improvement with very early use of vigabatrin and rapalogs, suggests that very early treatment (within the first 6 weeks of life) with one or both medicines could substantially improve outcome. Traditionally, seizures in TSC were often difficult to control and often required consideration of more invasive treatments such as a vagal nerve stimulator and epilepsy surgery. Early identification of autism with appropriate behavioral intervention can also be helpful. Medication can be required to control behavior in some patients. Attention to appropriate support for learning in patients is essential.
Partial Seizures
Published in Stanley R. Resor, Henn Kutt, The Medical Treatment of Epilepsy, 2020
It has been a practice to prescribe polypharmacy for the treatment of epilepsy, even as the initial form of drug treatment. It has now been established that polypharmacy is of no greater benefit than monotherapy (22) and that improvement in seizure control may occur when polypharmacy is reduced (23). It is now generally accepted that an attempt should be made to avoid the use of polypharmacy for treatment of epilepsy, particularly as the initial form of drug treatment. The drugs that are available for the treatment of partial seizures may now be classified as first-line drugs and second-line drugs. The first-line drugs are CBZ, PHT, and VPA. The second-line drugs are PB, PRM, and clobazam (see below). Since there is no evidence that second-line AEDs are of greater benefit than first-line drugs, the second-line drugs should be reserved as adjunctive treatment for patients who fail to respond to the first-line drugs. Vigabatrin (see below), a drug introduced in recent years, has also been found to be effective as adjunctive treatment in patients with partial seizures. Until such time as clinical trials are carried out to determine its efficacy as single drug treatment, it could be regarded as a second-line drug to be used as adjunctive treatment.
Genetic and clinical variations of developmental epileptic encephalopathies
Published in Neurological Research, 2023
Gül Demet Kaya Özçora, Elif Söbü, Uğur Gümüş
During the follow-up, seizures were controlled by anti-epileptic drugs (AEDs) in DEE types 4, 26, 30, 58, 62 and 67 (30%, n = 6), the seizure-free duration in these patients was≥11 months. Seizures in DEE types 2, 7 and 42 (30%, n = 6) were resistant to treatment and were receiving multiple AEDs. Seizures associated with DEE types 4, 6B, 7, 11, 26, and 33 (35%, n = 7) were controlled by AEDs with intermittent recurrent seizures recorded. The most commonly used AED was sodium valproate (40%, n = 8) followed by levetiracetam (40%, n = 8), vigabatrin (25%, n = 5), clobazam (25%, n = 5), clonazepam (20%, n = 4), topiramate (20%, n = 4) and lamotrigine (5%, n = 1). Two children were not receiving any AED treatment (DEE types 35 and 58); DEE type 35 had no seizures, died infantil period and DEE 58 discontinued the antiepileptic treatment voluntarily. In all cases of infantile spasm, vigabatrin was sufficient to control seizures.
Vigabatrin-related adverse events for the treatment of epileptic spasms: systematic review and meta-analysis
Published in Expert Review of Neurotherapeutics, 2020
Asthik Biswas, Omar Yossofzai, Ajoy Vincent, Cristina Go, Elysa Widjaja
Epileptic spasms are a severe form of epilepsy with characteristic electroencephalogram (EEG) pattern of hypsarrhythmia. They are associated with poor neurologic and developmental outcomes, development of other seizure types, and increased mortality [1]. Since continued spasms and abnormal EEG associated with epileptic spasms have a progressively detrimental impact on long-term development, the primary goal of treatment is rapid and complete control of spasms [2]. Vigabatrin (VGB) is a structural analog of gamma-aminobutyric acid (GABA) that increases GABA concentrations in the brain by irreversibly inhibiting GABA-transaminase [3]. VGB has been approved for the treatment of epileptic spasms outside of the United States since 1989, and in the United States since 2009. Hormonal therapy (ACTH or prednisolone) and VGB are the only drugs with proven efficacy to reduce or eradicate spasms and eliminate hypsarrhythmia on EEG [4].
Juvenile cataract in association with tuberous sclerosis complex
Published in Ophthalmic Genetics, 2020
A. L. Geffrey, K. R. Geenen, E. Abati, S. H. Greenstein, D. K. VanderVeen, R. L. Levy, S. L. Davidson, M. P. McGarrey, E. A. Thiele, M. E. Aronow
A 5-month-old male presented with IS. An MRI of the brain revealed scattered cortical tubers and a solitary SEN. Further investigation demonstrated cardiac rhabdomyomas on echocardiogram and bilateral renal cysts on abdominal imaging, leading to the diagnosis of TSC. Genetic evaluation revealed the presence of a known disease-causing TSC2 mutation (c.1832 G > A). At the age of 13 months, ocular examination identified bilateral lamellar cataracts, worse in the right eye. There had been no cataract in either eye at prior exam at age of 8 months. The lamellar cataract was described as being in the shape of a crescent moon, present inferiorly more than superiorly, and with a clear central visual axis. Amblyopia was not present at time of diagnosis and the cataracts were not visually significant. He was followed closely and 17 months of age, 1+ haze was present in visual axis of the right cataract and mild amblyopia was present. Amblyopia therapy with patching of the left eye was initiated. Over the next one month, the cataract progressed significantly in the right eye and became dense and white. He underwent lensectomy with the insertion of an IOL implant at 19 months of age. The cataract in the left eye has been closely monitored and has remained stable. There were no retinal astrocytic hamartomas or achromic patches in either eye. No retinal toxicity related to vigabatrin therapy for IS was observed.