Ambulatory electroencephalogram in children: A prospective clinical audit of 100 cases.

Ambulatory electroencephalogram has been used for differentiating epileptic from nonepileptic events, recording seizure frequency and classification of seizure type. We studied 100 consecutive children prospectively aged 11 days to 16 years that were referred for an ambulatory electroencephalogram to a regional children's hospital. Ambulatory electroencephalogram was clinically useful in contributing to a clinical diagnosis in 71% of children who were referred with a range of clinical questions. A diagnosis of epileptic disorder was confirmed by obtaining an ictal record in 26% and this included 11 children that had previously normal awake and or sleep electroencephalogram. We recommend making a telephone check of the current target event frequency and prioritising those with typical events on most days in order to improve the frequency of recording a typical attack.

Clinical reasoning and investigations for resective surgery for medically refractory epilepsies in children.

The article presents results of a UK survey of pediatric neurologists' views regarding resective surgery for medically refractory epilepsies in children. In contrast to surveys with adult neurologists, the findings indicate that delays to surgery in the pediatric field are not likely to be due to clinicians' views. There is, however, variability in clinicians' opinions as to what constitutes medically refractory epilepsy, variability in the factors reported as necessary for surgery eligibility, and uncertainty as to how these concepts should be defined. The survey highlights the need for elucidation of the epilepsy surgery process for pediatric patients, clear communication between epilepsy surgery centers and referring neurologists, and dissemination of consensus guidelines relating to the criteria for both medically refractory epilepsy and surgery eligibility.

Parental and physician beliefs regarding the provision and content of written sudden unexpected death in epilepsy (SUDEP) information.

PURPOSE: The 2007 UK National Institute for Health and Clinical Excellence (NICE) guidelines for epilepsy recommend disclosing the risk of sudden unexpected death in epilepsy (SUDEP) to patients. This recommendation is not undertaken routinely, and considerable variation in individual physician practice exists. Literature indicates wariness of causing distress and anxiety, particularly to children/young people and their families through disclosing a nonpreventable risk. There has been no systematic pediatric study examining parent/guardian information needs and beliefs for risk of SUDEP and its impact on seizure management. It is important to first address these concerns before routinely imparting SUDEP information to parents following NICE recommendations. METHODS: Two questionnaire surveys: a questionnaire examining the provision by pediatric neurologists of SUDEP information, and questionnaires examining parental beliefs and implications at two time points regarding SUDEP information provided in a leaflet. Participants were included in the study if their child had an established diagnosis of epilepsy. Factors for exclusion were single unprovoked seizure, absence seizures, patients in remission, and active discontinuation of treatment. RESULTS: The majority (74%) of pediatric neurologists provided SUDEP information only to a select group of children with epilepsy and were uncertain about the effect such information would have upon the parent and child. Conversely, 91% of parents expected the pediatric neurologist to provide SUDEP risk information. The provision of this information did not have a significant immediate and longer-term negative impact. DISCUSSION: The majority of parents wanted to know about SUDEP and its associated risks. Whenever possible, SUDEP information should be given by the physician accompanied by an information leaflet.

Acute hydrocephalus in a child with Mycoplasma cerebellitis.

Mycoplasma cerebellitis though rare, may rapidly progress to hydrocephalus needing surgical intervention. It has not been reported so far in children. We report a 6 year old girl with mycoplasma cerebellitis which progressed to acute hydrocephalus needing an emergency external ventricular drain. Magnetic Resonance Imaging is essential in making the diagnosis of cerebellar swelling as CT scan may be normal. Relapses can occur as in our case. Close monitoring for raised intracranial pressure and investigations to identify the organism including mycoplasma are indicated in all cases. Long-term outcome appears to be favorable if diagnosed and treated early.

Sedaghatian spondylometaphyseal dysplasia with pachygyria and absence of the corpus callosum.

We report on a female infant with a metaphyseal dysplasia and a neuronal migration abnormality consistent with a diagnosis of Sedaghatian spondylometaphyseal dysplasia. This child, born to nonconsanguineous Caucasian parents, was hypotonic from birth and experienced recurrent cyanotic episodes within a few hours of delivery. Cerebral imaging revealed absence of the corpus callosum and marked frontotemporal pachygyria. She developed seizures on day 14 of life and died at the age of 17 days. Our case highlights the possibility for neuronal migration abnormalities in Sedaghatian spondylometaphyseal dysplasia and suggests a novel association of the disorder with agenesis of the corpus callosum.

Pilot survey of Hashimoto's encephalopathy in children.

Hashimoto's encephalopathy (HE) is a steroid responsive, relapsing encephalopathy associated with thyroid autoantibodies. Paediatric literature mainly consists of case reports of the disease. A questionnaire survey of 68 consultant paediatric neurologists was undertaken through the British Paediatric Neurology Association in 2002 to gather preliminary data about this condition. The response rate was 68% and a total of ten patients were identified: nine from the UK and one from Ireland. Complete clinical details were available on eight patients (seven females and one male). Age at presentation ranged from 10 to 15 years (mean 12y 7mo, SD 1y 1mo). Presenting features included seizures (n=7/8), encephalopathy (n=7/8), cognitive decline (n=4/8), behavioural problems (n=3/8), psychosis (n=2/8), myoclonus (n=1/8), and tremors (n=1/8). All had thyroid autoantibodies and four were also hypothyroid at diagnosis. One patient became hypothyroid during follow-up. Electroencephalograms (EEGs) showed high amplitude slow background rhythms in all patients, and one patient also had focal spikes. Cognitive deficits were identified in four patients and persisted in one over 2 years of follow-up. Six patients improved with steroids and two improved spontaneously. Two had relapsing courses during follow-up afer diagnosis (range 12-48 months). HE may be currently under-recognized in children and increased awareness can result in prompt diagnosis and treatment. Steroid therapy appears to be beneficial. Neuropsychological assessment is required in all cases and may guide steroid therapy. Long-term prognosis for cognition remains guarded at this time.

Epilepsy with reversible bulbar dysfunction.

In patients with focal epilepsy, focal neurological dysfunction can occur due to status epilepticus and also as a post-ictal phenomenon. Bulbar dysfunction as evident by drooling, dysarthria, swallowing difficulties, and palatal-glossalpharyngeal weakness has been reported in conjunction with epilepsy. This is non-progressive and is correlated in its severity with the frequency of seizures. Accompanying EEG discharges are often localized to rolandic areas that cortically represent oral movements and salivation. We report a 6-year-old male and a 6 1/2-year-old female with progressive bulbar dysfunction resulting from epilepsy. Ictal EEGs in patient 1 did not confirm a diagnosis of epilepsy. With no evidence of a cortical or brainstem focus from EEG or MRI, it is very difficult to explain the mechanism of bulbar dysfunction. The complete restoration of bulbar function after treatment with antiepileptic drugs demonstrates the need to consider epilepsy in similar clinical situations.