Coexistence of epileptic encephalopathy with continuous spike-and-wave during sleep, atypical benign partial epilepsy, and fixation-off sensitivity in two siblings.

Epileptic encephalopathy with continuous spike-and-wave during sleep is a rare age-related childhood encephalopathy characterized by neuropsychological and motor impairment, epilepsy, and typical EEG findings. Fixation-off sensitivity denotes forms of epilepsy and/or EEG abnormalities that occur during eye closure. The authors describe a girl with developmental regression, electrical status epilepticus during sleep (documented by video/EEG recording), and intermittent myoclonus. Her younger brother was diagnosed with atypical benign partial epilepsy, and his repeated video/EEG recordings unexpectedly showed fixation-off sensitivity.

Electroclinical features of benign infantile seizures with mild gastroenteritis.

The purpose of this study was to analyze the electroclinical features of patients with benign infantile seizures with mild gastroenteritis and demonstrate the benign nature of this entity. From 30 patients who were included in the study, five were excluded (two with developmental delay, one with microcephaly and two lost during follow-up). Twenty-five patients who fulfilled the diagnostic criteria for benign infantile seizures with mild gastroenteritis were assessed and followed at the Pediatric Department, Armed Forces Hospital, Southern Region, Khamis Mushayt, Saudi Arabia, between January 2004 and January 2009. The median age at presentation was 10.4 months. Of the infants, 14 were females and 11 were males. Seizures were focal tonic or clonic in eight (32%) patients, focal with secondary generalisation in three (12%), generalised tonic-clonic in nine (36%) and consisted of staring only with no motor components in five (20%). Interictal electroencephalograms and brain imaging were normal for all patients. No patient required treatment with antiepileptic drugs. All the patients were found to have normal psychomotor development and neurological examination after a follow-up period of between 15 and 56 months. The limitations of this study are the relatively small sample size, relatively short study period and the fact that the study was conducted in a tertiary referral hospital. The prevalence of this entity may be more common at the level of primary health centres. Increasing the awareness of clinicians regarding the existence of this syndrome and its benign nature in children will limit unnecessary investigations. [Published with video sequences].

Fixation-off sensitivity with atypical presentation: clinical and video/EEG documentation.

A 9-year 2-month-old Saudi boy of normal intelligence was brought to a pediatric neurology clinic because of episodes of abnormal behavior associated with disorientation and confusion and postictal amnesia. Video/EEG evaluation unexpectedly documented the presence of fixation-off sensitivity.

Diabetic Ketoacidosis in children admitted to Pediatric Intensive Care Unit of King Fahad Hospital, Al-Baha, Saudi Arabia: Precipitating factors, epidemiological parameters and clinical presentation.

Type I diabetes mellitus is the most common endocrine-metabolic disorder of childhood and adolescence and diabetic ketoacidosis (DKA) can be life-threatening. The study aims at identifying precipitating factors, states epidemiological features and describes clinical presentations in children with DKA admitted to Pediatric Intensive Care Unit (PICU), King Fahad Hospital, Al-Baha, Saudi Arabia. The hospital records of 80 children admitted to PICU with DKA between January 2000 and December 2004 were reviewed. Results were compared with published data from Saudi Arabia and other countries. Age at admission ranged between 8 months and 14 years (mean = 10.7 years). Female to male ratio was 1.22:1. Consanguinity was reported among 32(40%) of all admitted children's parents. A family history of diabetes (either type 1 or 2) was reported in 59 (74%). The leading precipitating factor for DKA was infections (82.1%). An episode of DKA was the first clinical presentation of diabetes among 52(65%). The common presenting symptoms were: vomiting in 57(71.3%) and abdominal pain in 53 (66.3%). All children were dehydrated. Other signs included acidotic breathing and tachypnea each in 60%. Only two children were comatose (2.5%). Three of presenting cases were initially misdiagnosed as acute appendicitis before correct diagnosis was established. Cerebral edema occurred in one child. There were no deaths. DKA is an important cause of hospital admissions in our hospital, and 65% of newly diagnosed cases present with DKA. More effort should be put to prevent and reduce the incidence of DKA at initial presentation and later.

Benign familial and non-familial infantile seizures (Fukuyama-Watanabe-Vigevano syndrome): a study of 14 cases from Saudi Arabia.

PURPOSE: Benign infantile seizures [BIS], familial and non-familial, represent a benign, age-related idiopathic syndrome of infancy. The aim of the current paper is to document the presence of the syndrome in Saudi Arabia and in Arab populations and to discuss the characteristic electroclinical features and the benign nature of this syndrome. PATIENTS AND METHODS: A case series of 275 patients with epileptic seizures (age range: 2 months-13 years) were followed over a period of 3 years and 7 months. The inclusion criteria for BIS were as follows (1) age of seizure onset between 2 and 24 months, (2) normal development before, during and after the onset of seizures, (3) normal interictal EEG, (4) normal brain imaging, and (5) good response to treatment. We analyzed these infants with respect to age at seizure onset, sex, physical and neurological examination, consanguinity, frequency and type of convulsions, associated conditions and laboratory and radiological investigations. A waking and sleeping interictal EEG was performed on all patients, and for one patient (No. 1), ictal EEG and video clips were recorded. RESULTS: Fourteen infants (12.0%) showed electroclinical features consistent with BIS. Eleven patients fulfilled the criteria of benign non-familial infantile seizures (BNFIS), and for three patients, their family pedigrees showed the possibility of benign familial infantile seizures (BFIS). All of the patients responded to anti-epileptic treatment, and 50% of them responded within 3 months. CONCLUSIONS: To our knowledge, this is the first study to document the presence of BIS (Fukuyama-Watanabe-Vigevano syndrome) in Saudi Arabian and Arab populations. We highlighted the characteristic features of BIS and demonstrated the benign nature of the syndrome.