Amal C. Rahi

Outbreak of Burkholderia cepacia bacteremia traced to contaminated hospital water used for dilution of an alcohol skin antiseptic.

OBJECTIVE To identify the source of an epidemic of Burkholderia cepacia bloodstream infections during 7 years (411 episodes in 361 patients). DESIGN Outbreak investigation. SETTING A 250-bed university hospital in Beirut, Lebanon. METHODS Matched case-control and retrospective cohort studies, and microbiological surveillance and polymerase chain reaction-restriction fragment length ascertainment were employed. Special media and filtration techniques were used to isolate organisms from water and diluted alcohol solutions. RESULTS In a group of 50 randomly selected case-matched patients from 1999, the positive blood cultures were concomitant with fever in 98%, intravenous phlebitis in 44%, and recurrent bacteremia in 20%. Fever disappeared approximately 6 hours after intravenous catheter removal. Polymerase chain reaction-restriction fragment length polymorphism revealed strain homogeneity in patient, water, and alcohol isolates. Contaminated tap water had been used to dilute alcohol for skin antisepsis and for decontamination of the caps of heparin vials. Only sporadic cases directly attributable to breach of protocol were reported after single-use alcohol swabs were substituted. CONCLUSION This is potentially the largest single-source nosocomial bloodstream infection outbreak ever reported, and the first report of an alcohol skin antiseptic contaminated by tap water as a source for nosocomial bacteremia.

Normalization of Quality of Life Three Years after Temporal Lobectomy: A Controlled Study

Summary:  Purpose: The goal of epilepsy surgery is not merely to control previously intractable seizures, but also to improve quality of life (QOL). Our goals were to assess, in our Middle Eastern population, the QOL of adults with temporal lobe epilepsy (TLE) 3 years after temporal lobectomy as compared with matched TLE patients who did not undergo surgery and with healthy individuals in the same community.

Quality of life after surgery for intractable partial epilepsy in children: A cohort study with controls

PURPOSE Investigate if quality of life (QOL) normalizes on long-term follow-up after surgery for partial epilepsy in children. METHODS This is a cohort study with controls in which a consecutive cohort of nineteen 2-14-year-old children who underwent focal resections for intractable partial seizures between 1996 and 2006, were matched with 19 non-surgery intractable partial epilepsy patients, and with 19 healthy subjects. The two epilepsy groups were matched for age, sex, socio-economic status (SES), cognitive level, seizure type, and seizure frequency. The healthy group was matched with the two epilepsy groups for age, sex, SES, and cognitive level. QOL was assessed using the QOLCE (Quality of Life in Childhood Epilepsy Questionnaire). RESULTS In the surgery group (follow-up 3.84+/-2.26 years), 78.9% had Engel class-I versus 21.1% in non-surgery (p=0.01) (follow-up 3.44+/-2.95 years). Surgery patients were similar to healthy subjects in the social, emotional, cognitive, behavioral, and overall QOL (p>0.05) but had lower scores in the total QOL, physical, and health domains (p<0.05). Surgery patients scored better than non-surgery in the behavioral domain and the HASES (Hague Side Effects Scale) score (p<0.05). Non-surgery patients scored worse than healthy in total QOL, physical, behavioral, health, and overall QOL (p<0.05). IQ, HASS (Hague Seizure Severity Scale), and HASES scores were positively associated with total QOL score (p<0.05). Subgroup analysis on seizure-free surgery patients showed that they did not differ from healthy subjects in any of QOL domains (p>0.05, power>0.8). CONCLUSION Our data indicate that epilepsy surgery for partial seizures in children is associated with better QOL as compared to children with intractable epilepsy who are not operated on, and suggest that in those who achieve seizure freedom normal QOL may at least potentially be possible.

Approach to pediatric epilepsy surgery: State of the art, Part I: General principles and presurgical workup

In 1990, the National Institute of Health adopted epilepsy surgery in children as an option when medications fail. In the past few years several concepts have become increasingly recognized as key to a successful approach to epilepsy surgery in children. These include the concepts of neuronal plasticity, the epileptogenic lesion, the ictal onset, symptomatogenic, irritative, and epileptogenic zones. In addition, several techniques have increasingly been utilized to delineate the above areas in an attempt to determine, in each patient, the epileptogenic zone, defined as the zone the resection of which leads to seizure freedom. When seizure semiology (which defines the symptomatogenic zone), ictal EEG (which identifies the ictal onset zone), and structural imaging (which identifies the epileptogenic lesion) can be reconciled to infer the location of the epileptogenic zone, surgery is usually, subsequently, undertaken. When these diagnostic modalities are discordant, not definitive, or when the epileptogenic zone is close to eloquent cortex, invasive EEG, complemented by other imaging techniques may be needed. These include magnetoencephalography, single photon emission tomography, various types of positron emission tomography, various magnetic resonance imaging modalities (functional, diffusion weighted, other) and other emerging and experimental techniques. While MRI, video-EEG, and neuropsychological assessments are well established components of the presurgical evaluation, the use of the new emerging imaging technologies is dictated by the degree of anatomo-electro-clinical correlations, and, awaiting multicentric studies and more detailed guidelines, remains center-dependent.

Approach to pediatric epilepsy surgery: State of the art, Part II: Approach to specific epilepsy syndromes and etiologies

The second of this 2-part review depicts the specific approach to the common causes of pediatric refractory epilepsy amenable to surgery. These include tumors, malformations due to abnormal cortical development, vascular abnormalities and certain epileptic syndromes. Seizure freedom rates are high (usually 60-80%) following tailored focal resection, lesionectomy, and hemispherectomy. However, in patients in whom the epileptogenic zone overlaps with unresectable eloquent cortex, and in certain epileptic syndromes, seizure freedom may not be achievable. In such cases, palliative procedures such as callosotomy, multiple subpial transections and vagus nerve stimulation can achieve reduction in seizure severity but rarely seizure freedom. Integration of the new imaging techniques and the concepts of neuronal plasticity, the epileptogenic lesion, the ictal onset, symptomatogenic, irritative, and epileptogenic zones is an expanding and dynamic process that will allow us, in the future, to better decide on the surgical approach of choice and its timing.

Age, dose, and environmental temperature are risk factors for topiramate-related hyperthermia

To determine the risk of occurrence of hyperthermia during topiramate therapy,1–4 and the risk factors of developing hyperthermia, we investigated all patients (predominantly Lebanese Arabs) seen in our epilepsy clinic during a 2-year period. Inclusion criteria were diagnosis of epilepsy treated with at least one antiepileptic drug (AED) and patient or family able to accurately report side effects. Exclusion criteria were 1) medical condition or drugs that could cause or mask the occurrence of fever, 2) clinical findings that indicate another cause of hyperthermia, or 3) any episodes of hyperthermia that were not properly evaluated. Detailed clinical information was gathered on each patient at baseline and every 3 to 4 months during a 2-year prospective follow-up. Patients or family members were systematically asked about adverse experiences, but hyperthermia was not specifically mentioned to avoid bias. If hyperthermia was reported, then detailed clinical and laboratory data were collected. Hyperthermia was defined as …

Intravenous immunoglobulin therapy in intractable childhood epilepsy: open-label study and review of the literature.

Our aim was to investigate the long term effectiveness of intravenous immunoglobulin (IVIG) against intractable childhood epilepsy in the era of new antiepileptics and to determine the predictors of a favorable response in a prospective open-label add-on study. Of thirty-seven 9.9+/-0.9-year-old patients (11 with partial seizures, 26 with generalized seizures of whom 9 had West syndrome and 17 Lennox-Gastaut syndrome) followed for 15+/-3 months, 43% had a >50% decrease in seizures (including 15% seizure free, 229+/-58 compared with 104+/-3 seizures/month, P=0.035: generalized 246+/-318 to 117+/-200, P=0.025, partial 191+/-437 to 72+/-179, P>0.05; power=0.2). Males were more likely to respond than females (P=0.011, odds ratio=9.3). Review of the literature revealed nine other articles reporting efficacy of IVIG against epileptic seizures. Only one other used statistical methods and, unlike ours, showed only a trend toward seizure frequency reduction without achieving statistical significance, presumably because it was underpowered. These results indicate large-scale controlled studies of IVIG in epilepsy are still needed.

Marked benefits in physical activity and well-being, but not in functioning domains, 2 years after successful epilepsy surgery in children.

In this first study comparing epilepsy-specific quality-of-life measures of children after epilepsy surgery (2.4 years after focal resection) with those of a matched comparison group of nonoperated patients, seizure severity, medication side effects, overall quality of life, general health, physical activity, and well-being were better in surgical patients (70.6% seizure free vs 8.3%). Cognitive, social, and behavioral functioning did not differ, suggesting that these may require additional interventions during postsurgical follow-up.

Electroencephalographic changes in pyridoxine-dependant epilepsy: new observations

OBJECTIVE Pyridoxine-dependent epilepsy (PDE) is a rare disease, of which the EEG manifestations are only partially characterised. We report our observations of EEG recordings in four patients with PDE. MATERIALS AND METHODS EEG tracings from four patients fulfilling the clinical criteria for PDE were reviewed. Relative to the time of treatment with pyridoxine, EEG recordings were available before treatment in two patients (at ages four and 10 months), immediately after treatment in two patients and during long-term follow-up with treatment in all four patients. RESULTS Pre-pyridoxine interictal EEG findings included: diffuse slowing, bilateral independent multifocal epileptiform discharges, generalized bursts of polyspike slow waves and focal or generalized sharp waves. In addition, the EEG was often asymmetrical and included: generalized semi-rhythmic sharp and slow waves, a burst suppression pattern and continuous generalized spike and slow waves. In one patient, who was followed subsequently, a decrease in multifocal spikes and sharp waves and permanent cessation of clinical seizures, within 10 minutes of concurrent reduction of spikes in the pre-existing generalized spike slow wave pattern, was observed immediately after pyridoxine treatment. However, despite the clinical response in this patient we observed persistent generalized burst suppression for four days, and fluctuation of the EEG with diffuse slowing on day four and transient exacerbation of discharges with continuous spike slow waves on day 22. This was followed by intermittent sharp waves at eight and 20 months, mild slowing at 31 months and normal EEG at 43 months. Long-term EEG findings in the other three patients receiving pyridoxine ranged between normal and intermittent multifocal sharp waves. CONCLUSION Our data confirm previous observations and provide the following new findings: (1) the presence of burst suppression pattern after cessation of seizures can occur for up to five days after initiation of pyridoxine and should not exclude the diagnosis of PDE, (2) possible fluctuation and even transient worsening of electrographic discharges were observed for up to three weeks after initiation of pyridoxine and (3) the abnormal EEG can persist for up to 43 months before normalizing (range 1-43 months) and in other cases in which it continues to be abnormal it may still improve after increasing the dose of pyridoxine.

Novel mutation causing partial biotinidase deficiency in a syrian boy with infantile spasms and retardation

We report a case of partial biotinidase deficiency (plasma biotinidase levels: 1.30 nm/minute/mL) in a 7-month-old boy who presented with evidence of perinatal distress followed by developmental delay, hypotonia, seizures, and infantile spasms without alopecia or dermatitis. His neurologic symptoms improved markedly on biotin supplementation and antiepileptic drug therapy. DNA mutational analysis revealed that the patient was homozygous for a novel E64K mutation and his parents were heterozygous for the same mutation. Whereas preexisting perinatal distress probably contributed to the severity of the patients symptoms, the described mutation is novel and is possibly responsible for at least some of his clinical manifestations. (J Child Neurol 2006;21:978-981; DOI 10.2310/ 7010.2006.00200).