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Neurology and neurosurgery
Published in Jagdish M. Gupta, John Beveridge, MCQs in Paediatrics, 2020
Jagdish M. Gupta, John Beveridge
The usual age of onset of infantile spasms is 3-6 months, but they may begin as early as the neonatal period or as late as 2 years. They most commonly occur in association with neonatal asphyxia, birth trauma or previous meningitis. They are also seen in patients with tuberous sclerosis and may sometimes occur in previously normal children. Whooping cough vaccine has also been implicated, but the incidence is less than 1:150000. Although they may remit spontaneously, other seizure types develop later in life in about 50% of patients and mental retardation has been reported in 80-90% of cases on long-term follow-up. The EEG changes when present are diagnostic (hypsarrhythmia).
Infantile Spasms
Published in Stanley R. Resor, Henn Kutt, The Medical Treatment of Epilepsy, 2020
The first, and still unsurpassed, clinical description of infantile spasms was made in 1841 by West (1). These spells were characterized by “bobbings of the head” and “bowings and relaxings” in clusters of “from 10 to 20 or more times at each attack” and a progressive deterioration of intellect such that “he . . . never smiles or takes any notice, but looks placid and pitiful.” The clinical presentation of infantile spasms, so lucidly described by West, has now been carefully studied and further characterized by the use of continuous EEG-videotelemetry monitoring (2). The spasms may be divided into flexor, extensor, and mixed, with the latter being the most and extensor spasms the least common. Flexor spasms consist of flexion of the neck, trunk, arms, and legs. Abdominal flexion may be massive, giving rise to the “jack-knife” or “salaam” seizures that are the hallmark of infantile spasms. During extensor spasms, there is abrupt extension of the neck, trunk, and legs. The mixed flexor-extensor spasms are characterized by flexion of the neck, trunk, and arms and extension of the legs.
Crying and Colic
Published in Quentin Spender, Niki Salt, Judith Dawkins, Tony Kendrick, Peter Hill, David Hall, Jackie Carnell, Child Mental Health in Primary Care, 2018
Quentin Spender, Niki Salt, Judith Dawkins, Tony Kendrick, Peter Hill, David Hall, Jackie Carnell
Many parents feel that their inconsolably crying baby must be in pain and therefore ill. Colic will probably be implicated in the majority of these cases, and the child will be physically well. However, it is obviously important not to miss the less common causes of excessive crying, such as pain from acute infections (ear, nose and throat, or urinary tract) or from a strangulated inguinal hernia. Crying after a feed may be due to pain from gastro-oesophageal reflux, and this may be associated with unusual posturing of the baby’s head and neck. There will usually (but not always) be a history of vomiting. If suspected, this possibility should be investigated by a paediatrician. A rare but extremely important condition is infantile seizures, in which the baby flexes the whole trunk in a salaam spasm and utters a brief cry. A child with possible infantile spasms should probably be referred as an emergency.
Adult-onset subacute sclerosing panencephalitis presenting with tonic motor seizures
Published in International Journal of Neuroscience, 2021
Ozge Yagcioglu Yassa, Gulay Kenangil, Ayse Destina Yalcin
The spectrum of epileptic phenomena associated with SSPE is wide, varying from partial or generalized tonic-clonic seizures and atypical absences [10, 11] to myoclonic-atonic attacks [12]. Tonic seizures have been reported previously in 3 individual cases of SSPE and are rare in SSPE [13, 14]. Our patient was 25 years old and had clinically evident tonic seizures which were associated in EEG with generalized slow sharp wave discharge of high voltage followed by fast activity prominent on the frontal regions. Tonic seizures are a common manifestation in pediatric age and especially in symptomatic/lesional epilepsies. In the neonatal period tonic seizures are recorded in early infantile epileptic encephalopathy, in early myoclonic encephalopathy and in cases with hypoxic-ischemic perinatal injury. During infancy, tonic seizures are observed in refractory infantile spasms. They are also the main seizure type in symptomatic generalized epilepsy associated with an inverted duplication of chromosome 15 and in Lennox-Gastaut syndrome [26].
Treatment of infantile spasms: why do we know so little?
Published in Expert Review of Neurotherapeutics, 2020
Nicola Specchio, Nicola Pietrafusa, Alessandro Ferretti, Luca De Palma, Marta Elena Santarone, Chiara Pepi, Marina Trivisano, Federico Vigevano, Paolo Curatolo
In a prospective randomized trial, an International Collaborative Infantile Spasms Study (ICISS), combination therapy with hormonal therapy (ACTH or high-dose steroids) and vigabatrin was compared with just hormonal therapy. The primary outcome was the cessation of spasms and resolution of hypsarrhythmia on EEG. A significantly higher responder rate (71.9%) and a quicker response were seen in the combination therapy group compared with the hormonal therapy only group (56.6%) [103]. This study also demonstrated that the outcome was significantly better with a higher rate of spasm cessation when treatment was started within 2 months of the onset of spasms [103]. More recently, researchers at the ICISS assessed the cognitive development and epilepsy in infants treated with combination (hormonal and vigabatrin) therapy. They reported no significant differences at 18 months for infants treated with combination therapy versus hormonal therapy alone [107]. Based on the previous study [103], however, authors have concluded that combination therapy should be preferred over hormonal therapy alone in most clinical cases [108,109].
A resurging boom in new drugs for epilepsy and brain disorders
Published in Expert Review of Clinical Pharmacology, 2018
Iyan Younus, Doodipala Samba Reddy
CPP-115 has been granted fast track status for the treatment of cocaine dependency and orphan drug status for infantile spasms [23]. A recent case report published the results from an open-label investigation for pharmacologically resistant infantile spasms in a 3-year-old boy [20]. Prior to investigational therapy, the patient was reported to fail 10 drugs and ketogenic diet. The patient had approximately 100 seizures per day before administration of CPP-115. One year after starting CPP-115 and tapering off clobazam and vigabatrin, his reported seizure frequency decreased to 25–30 seizure per day. The patient’s cognition and behavior also improved, and there was no evidence of retinal damage. CPP-115 shows promising results for management of infantile spasms and more comprehensive analysis in a controlled setting is warranted.