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About one third of patients with epilepsy are pharmacoresistent. For a subgroup of this population, the ketogenic diet can be highly efficacious and should be considered early. This review discusses the different types of ketogenic diet, proposed mechanism of actions and its evidence for use in children and adults with both generalized and focal epilepsies where surgery is not feasible. In addition we discuss a practical approach to diet initiation, maintenance and monitoring for side effects. We also summarize the emerging evidence for the use of ketogenic diet in a broad range of neurological disorders.

Objective: Posterior reversible encephalopathy syndrome (PRES) occurs most commonly in the setting of known hypertension or use of immunosuppressive agents., Design and Methods: We report four previously-well children who presented acutely with altered mentation, seizures and visual disturbances and were diagnosed with PRES., Results: Only one child had a history of gross hematuria prior to the seizure. All four were discovered to be hypertensive only after onset of their neurological symptoms, and were subsequently diagnosed with glomerulonephritis. All four had rapid resolution of neurological symptoms with adequate treatment of hypertension., Conclusions: Blood pressure must be measured promptly in all children presenting with these symptoms. If elevated, the diagnosis of PRES should be strongly considered and a workup for renal disease pursued.

Background: Reversible segmental cerebral vasoconstriction (RSCV) is a recognizable clinical and radiographic syndrome consisting of thunderclap headache with or without focal neurological symptoms combined with reversible segmental vasoconstriction of proximal cerebral blood vessels., Methods: We report a case of reversible segmental cerebral vasoconstriction in a child., Results: A healthy 13-year-old boy experienced the sudden onset of a severe, diffuse headache upon surfacing from a deep dive in a swimming pool. Severity was maximal at the onset and improved over several hours. The same headache recurred three times over the next four days and a low baseline headache persisted throughout. Vomiting occurred once and mild photo/osmophobia were reported but throbbing, aura, or autonomic symptoms were absent. Focal neurological signs or symptoms were absent and he denied previous history of headaches, medications, drugs, or trauma. Two normal CT scans were performed within hours of separate headaches. Cerebrospinal fluid study on day 5 was bloody with no xanthochromia. MRI/MRA/MRV of the brain and vasculitic work-up were normal. Cerebral angiography on day 6 demonstrated smooth narrowing of multiple proximal cerebral vessels including supraclinoid internal carotid artery (ICA), M1, and A1 on the right and M1 on the left. By ten days, the patient's headaches had resolved and repeat angiography was normal., Conclusion: RSCV should be considered in a child with thunderclap headache.

The epileptic encephalopathies comprise a group of devastating seizure syndromes which begin in infancy and early childhood and usually result in intractable epilepsy. While some syndromes are relatively easily diagnosed early in their course, others take time to evolve, hampering an early, confident diagnosis. Epileptic encephalopathies are associated with slowing of cognitive function and evolution of severe behavioral disorders, which are often more distressing to families than the epilepsy. While an underlying etiology may explain some of this co-morbidity, many children have no identifiable etiology found for their seizures. In these "idiopathic" cases, recurrent subtle seizures, frequent epileptiform discharge and non-convulsive status epilepticus probably all play a role in deterioration of cognitive function and evolution of behavior disorders. This paper will review the most common epileptic encephalopathy syndromes, discuss the cognitive and behavioral co-morbidities and review current therapeutic options.

Background: Complementary and alternative medicine (CAM) is increasingly used in adults and children. Studies on CAM in neurological disorders have focused on the adult population and its use among pediatric neurology patients has not been well characterized., Objectives: The purpose of this study was: 1) To characterize the prevalence of CAM in pediatric neurology patients; 2) To determine the perceived effectiveness of CAM in these children; 3) To compare the cost of CAM with conventional therapies; and 4) To describe caregiver or patient-related variables associated with the use of CAM., Methods: This was a cross-sectional survey of patients and families attending the Alberta Children's Hospital neurology clinic between February and May 2004. Patients were considered eligible if they were between two and 18 years of age and had a known history of neurological disorders. Caregivers completed several self-administered questionnaires regarding their socio-demographic profile, their child's neurological illness, and their experience with CAM. Caregivers also rated their child's quality of life using the Pediatric Quality of Life Inventory., Results: One hundred and five of 228 (46%) families completed the survey. The mean age of the neurology patients was 9.8 +/- 4.5 years. Forty-six (44%) out of 105 patients received one or more types of CAM, with the most common types being chiropractic manipulations (15%), dietary therapy (12%), herbal remedies (8%), homeopathy (8%), and prayer/faith healing (8%). Caregivers' sociodemographic variables or pediatric health-related quality of life were not significantly associated with the use of CAM. Fifty-nine percent of CAM users reported benefits, and only one patient experienced side effects. There was no significant difference in the total median cost of CAM compared to conventional therapies (31.70 dollars vs. 50.00 dollars per month). Caregivers' personal experience or success stories from friends and media were common reasons for trying CAM., Conclusions: The use of CAM was common among pediatric neurology patients. Over half of the families reported benefits with CAM, and side effects were perceived to be few. Physicians should initiate discussion on CAM during clinic visits so that the families and patients can make informed decisions about using CAM. Further studies should address the specific role of CAM in children with neurological disorders, and to determine the potential interactions between CAM and conventional therapies in these patients.

Purpose: To determine the etiology, early mortality, predictors of prognosis and diagnostic yields of EEG and CT scans of the head in new-onset seizures in elderly patients., Methods: EEG records for the north-central region of Saskatchewan, between 01/94 and 12/95 were reviewed to identify all adults aged 60 years or older with new-onset seizures. Information on demographics, seizure type, etiology, EEG and neuroimaging studies, anti-epileptic treatment and course of epilepsy was obtained by review of medical records and interview with the patient and/or family member., Results: Of 88 eligible subjects, 61 (69%) were contacted for follow-up, 19 (22%) were deceased (12 of whom who had a serious underlying etiology to their seizures, which was obvious at the time of initial presentation and led shortly to their demise), 4 (5%) were lost to follow-up and 4 (5%) refused participation. Excluding those refusing participation, 74/84 (88%) patients presented with partial or secondarily generalized seizures. Seizures were cryptogenic in 38/84 (45%), and due to stroke in 19/84 (23%). EEGs were abnormal in 61/84 (73%) cases, with epileptiform discharge in 33/84 (39%). CT scans were abnormal in 57/84 (68%) cases with acute pathology in 29/84 (35%). Of the 61 patients participating in the follow-up interview, 54 (89%) were treated with anti-epileptic medication and seizure control was usually successful. Predictors for ongoing seizures were more than 3 seizures at presentation, epileptiform activity on initial EEG and discontinuation of anti-epileptic medication for lack of efficacy., Conclusion: Prognosis of new-onset seizures in elderly patients is favorable if seizures are not symptomatic of a life-threatening disorder.