Daniel K Miles, M.D.

Bilateral invasive electroencephalography in patients with tuberous sclerosis complex: a path to surgery?
Carlson, Chad; Teutonico, Federica; Elliott, Robert E; Moshel, Yaron A; LaJoie, Josiane; Miles, Daniel; Devinsky, Orrin; Weiner, Howard L
2011 Apr;7(4):421-430, Journal of Neurosurgery: Pediatrics
OBJECT: Many children with epilepsy and tuberous sclerosis complex (TSC) have multiple tubers on MR imaging and poorly localized/lateralized video electroencephalography (EEG) findings. Given the long-term risks associated with frequent seizures and multiple antiepileptic drugs, along with improved success in identifying focal epileptogenic zones in patients with multifocal lesions, the authors used bilateral intracranial EEG to lateralize the epileptogenic zone in patients with nonlateralizable noninvasive preoperative evaluations. METHODS: A retrospective analysis from January 1, 1998, to June 30, 2008, identified 62 children with TSC who were presented at a surgical conference. Of the 52 patients undergoing diagnostic or therapeutic procedures during the study period, 20 underwent bilateral intracranial EEG. The presurgical testing results, intracranial EEG findings, surgical interventions, and outcomes were reviewed. RESULTS: Fourteen of 20 patients had intracranial EEG findings consistent with a resectable epileptogenic zone. One patient is awaiting further resection. Five patients had findings consistent with a nonresectable epileptogenic zone, and 1 of these patients underwent a callosotomy. Seven patients had Engel Class I outcomes, 1 was Class II, 3 were Class III, and 3 were Class IV (mean follow-up 25 months). CONCLUSIONS: Bilateral intracranial EEG can identify potential resectable seizure foci in nonlateralizable epilepsy in TSC. Although 6 of 20 patients did not undergo resection (1 patient is pending future resection), significant improvements in seizures (Engel Class I or II) were noted in 8 patients. In the authors' experience, this invasive monitoring provided a safe method for identifying the ictal onset zone
— id: 132575, year: 2011, vol: 7, page: 421, stat: Journal Article,

Multicenter Phase 2 Trial of Sirolimus for Tuberous Sclerosis: Kidney Angiomyolipomas and Other Tumors Regress and VEGF- D Levels Decrease
Dabora, Sandra L; Franz, David Neal; Ashwal, Stephen; Sagalowsky, Arthur; Dimario, Francis J Jr; Miles, Daniel; Cutler, Drew; Krueger, Darcy; Uppot, Raul N; Rabenou, Rahmin; Camposano, Susana; Paolini, Jan; Fennessy, Fiona; Lee, Nancy; Woodrum, Chelsey; Manola, Judith; Garber, Judy; Thiele, Elizabeth A
2011 ;6(9):e23379-e23379, PLoS ONE
BACKGROUND: Tuberous sclerosis (TSC) related tumors are characterized by constitutively activated mTOR signaling due to mutations in TSC1 or TSC2. METHODS: We completed a phase 2 multicenter trial to evaluate the efficacy and tolerability of the mTOR inhibitor, sirolimus, for the treatment of kidney angiomyolipomas. RESULTS: 36 adults with TSC or TSC/LAM were enrolled and started on daily sirolimus. The overall response rate was 44.4% (95% confidence intervals [CI] 28 to 61); 16/36 had a partial response. The remainder had stable disease (47.2%, 17/36), or were unevaluable (8.3%, 3/36). The mean decrease in kidney tumor size (sum of the longest diameters [sum LD]) was 29.9% (95% CI, 22 to 37; n = 28 at week 52). Drug related grade 1-2 toxicities that occurred with a frequency of >20% included: stomatitis, hypertriglyceridemia, hypercholesterolemia, bone marrow suppression (anemia, mild neutropenia, leucopenia), proteinuria, and joint pain. There were three drug related grade 3 events: lymphopenia, headache, weight gain. Kidney angiomyolipomas regrew when sirolimus was discontinued but responses tended to persist if treatment was continued after week 52. We observed regression of brain tumors (SEGAs) in 7/11 cases (26% mean decrease in diameter), regression of liver angiomyolipomas in 4/5 cases (32.1% mean decrease in longest diameter), subjective improvement in facial angiofibromas in 57%, and stable lung function in women with TSC/LAM (n = 15). A correlative biomarker study showed that serum VEGF-D levels are elevated at baseline, decrease with sirolimus treatment, and correlate with kidney angiomyolipoma size (Spearman correlation coefficient 0.54, p = 0.001, at baseline). CONCLUSIONS: Sirolimus treatment for 52 weeks induced regression of kidney angiomyolipomas, SEGAs, and liver angiomyolipomas. Serum VEGF-D may be a useful biomarker for monitoring kidney angiomyolipoma size. Future studies are needed to determine benefits and risks of longer duration treatment in adults and children with TSC. TRIAL REGISTRATION: Clinicaltrials.gov NCT00126672
— id: 138089, year: 2011, vol: 6, page: e23379, stat: Journal Article,

Extraventricular subependymal giant cell tumor in a child with tuberous sclerosis complex
Bollo, Robert J; Berliner, Jonathan L; Fischer, Ingeborg; Miles, Daniel K; Thiele, Elizabeth A; Zagzag, David; Weiner, Howard L
2009 Jul;4(1):85-90, Journal of Neurosurgery: Pediatrics
Subependymal giant cell tumors (SGCTs) are observed in 5-20% of patients with tuberous sclerosis complex (TSC) but account for approximately 25% of neurological morbidity. The authors report the case of a 7-year-old girl with TSC and multiple cortical tubers who presented with worsening seizures in the context of the rapid growth of a cystic, calcified, extraventricular SGCT in the right frontal lobe, initially thought to represent a cortical tuber. The tumor and surrounding tubers were excised, and clinical seizures resolved. This is the first report of an extraventricular SGCT in a child with TSC outside the neonatal period
— id: 100667, year: 2009, vol: 4, page: 85, stat: Journal Article,

EPILEPSY SURGERY FOR CHILDREN WITH TUBEROUS SCLEROSIS COMPLEX AND MULTI-FOCAL EEG FINDINGS
Teutonico, F; Carlson, C; LaJoie, J; Miles, D; Devinsky, O; Weiner, HL
2008 DEC ;49(12):297-297, Epilepsia
— id: 91395, year: 2008, vol: 49, page: 297, stat: Journal Article,

Frequency and predictors of autism in children with tuberous sclerosis complex
Zelleke, TZ; Rubin, MR; Cohen, BC; Lai, GL; LaJoie, JL; Miles, DM; Devinsky, OD; Zaroff, CZ; MacAllister, WM; Weiner, HW; Nass, RN
2008 SEP ;64(3):S115-S115, Annals of neurology
— id: 104238, year: 2008, vol: 64, page: S115, stat: Journal Article,

Pediatric language mapping: sensitivity of neurostimulation and Wada testing in epilepsy surgery
Schevon, Catherine A; Carlson, Chad; Zaroff, Charles M; Weiner, Howard J; Doyle, Werner K; Miles, Daniel; Lajoie, Josiane; Kuzniecky, Ruben; Pacia, Steven; Vazquez, Blanca; Luciano, Daniel; Najjar, Souhel; Devinsky, Orrin
2007 Mar;48(3):539-545, Epilepsia
PURPOSE: Functional mapping of eloquent cortex with electrical neurostimulation is used both intra- and extraoperatively to tailor resections. In pediatric patients, however, functional mapping studies frequently fail to localize language. Wada testing has also been reported to be less sensitive in children. METHODS: Thirty children (4.7 - 14.9 years) and 18 adult controls (18-59 years) who underwent extraoperative language mapping via implanted subdural electrodes at the NYU Comprehensive Epilepsy Center were included in the study. Ten children and 14 adults underwent preoperative Wada testing. Success of the procedures was defined as the identification of at least one language site by neurostimulation mapping and determination of hemispheric language dominance on the Wada test. RESULTS: In children younger than 10.2 years, cortical stimulation identified language cortex at a lower rate than was seen in children older than 10.2 years and in adults (p<0.05). This threshold, demonstrated by survival and chi2 analysis, was sharply defined in our data set. Additionally, Wada testing was more likely to be successful than was extraoperative mapping in this younger age group (p<0.05). CONCLUSIONS: Analysis of our series demonstrates that language cortex is less likely to be identified in children younger than 10 years, suggesting that alternatives to the current methods of cortical electrical stimulation, particularly the use of preoperative language lateralization, may be required in this age group
— id: 71610, year: 2007, vol: 48, page: 539, stat: Journal Article,

Pediatric epilepsy surgery: The recent NYU experience
Cahan, B; Bollo, RJ; LaJoie, J; Miles, D; Devinsky, O; Weiner, H
2006 APR ;104(4):631-631, Journal of neurosurgery
— id: 104254, year: 2006, vol: 104, page: 631, stat: Journal Article,

Multi-staged epilepsy surgery in multifocal tuberous sclerosis complex
Carlson, C; Weiner, H; Ridgway, E; Zaroff, C; Miles, D; LaJoie, J; Kuzniecky, R; Devinsky, O
2006 MAR 14 ;66(5):A322-A322, Neurology
— id: 104258, year: 2006, vol: 66, page: A322, stat: Journal Article,

Pediatric language mapping: Sensitivity of neurostimulation and Wada testing in epilepsy surgery
Weiner, HL; Schevon, C; Carlson, C; Doyle, W; Miles, D; LaJoie, J; Kuzniecky, R; Devinsky, O
2006 AUG ;59(2):480-481, Neurosurgery
— id: 104252, year: 2006, vol: 59, page: 480, stat: Journal Article,

Epilepsy surgery in young children with tuberous sclerosis: results of a novel approach
Weiner, Howard L; Carlson, Chad; Ridgway, Emily B; Zaroff, Charles M; Miles, Daniel; LaJoie, Josiane; Devinsky, Orrin
2006 May;117(5):1494-1502, Pediatrics
OBJECTIVE: Tuberous sclerosis complex (TSC) is associated with medically refractory epilepsy and developmental delay in children and usually results from cortical tubers. Seizures that begin in young patients are often refractory and may contribute to development delay. Functional outcome is improved when seizures are controlled at an early age. Previous reports have shown modest benefit from surgical resection of single tubers/seizure foci in older children; however, many children with TSC develop uncontrolled seizures before age 1. To identify patients who might benefit from surgery and to maximize outcome, we used a novel surgical approach in young children that consists of invasive intracranial monitoring, which is typically 3-staged and often bilateral. METHODS: Of 110 consecutive children who underwent epilepsy surgery by a single surgeon in the past 6 years, 25 patients (9 boys and 16 girls) had TSC. At the time of their first surgery at our institution, they were a median age of 4.0 years. A total of 31 separate admissions for epilepsy surgery in these 25 patients were identified. Bilateral electrode placement was performed in 13 children whose seizures could not be lateralized definitively preoperatively, and 22 patients underwent 3-stage surgeries. RESULTS: At 6 months or longer after the initial resection, 21 (84%) children were class I, 2 (8%) children were class II, and 2 (8%) children were class IV. At a mean follow-up of 28 months, 17 (68%) children were class I, 6 (24%) were class II, and 2 (8%) were class III. Four of the 5 children who initially were rejected as surgical candidates because of multifocality and who required initial bilateral electrode study are now seizure-free. CONCLUSIONS: This approach can help to identify both primary and secondary epileptogenic zones in young TSC patients with multiple tubers. Multiple or bilateral seizure foci are not necessarily a contraindication to surgery. Long-term follow-up will determine whether this approach has durable effects
— id: 64210, year: 2006, vol: 117, page: 1494, stat: Journal Article,

Mental retardation: Relationship to seizures and tuber burden in tuberous sclerosis
Zaroff, C; Barr, W; Devinsky, O; Miles, D; Nass, R
2006 MAR ;60(3):S127-S128, Annals of neurology
— id: 104247, year: 2006, vol: 60, page: S127, stat: Journal Article,

Mental retardation and relation to seizure and tuber burden in tuberous sclerosis complex
Zaroff, Charles M; Barr, William B; Carlson, Chad; LaJoie, Josiane; Madhavan, Deepak; Miles, Daniel K; Nass, Ruth; Devinsky, Orrin
2006 Oct;15(7):558-562, Seizure
In patients with tuberous sclerosis complex (TSC), the high rates of mental retardation are associated with cortical tubers, seizure activity, and genetic factors. The goal of the study was to investigate the relationship between bilateral cortical tubers and seizure variables and mental retardation in individuals with TSC. The records of 27 patients with TSC (age 6 months to 33 years) undergoing neuropsychological assessment and the following clinical variables were examined: bilateral versus non-bilateral cortical tubers, the age of seizure onset, and presence of infantile spasms. Results were statistically analyzed. Bilateral cortical tubers (p=0.02) and early age of seizure onset (p=0.04) were significantly related to impaired cognitive functioning. Only one of the seven patients with normal cognitive functioning had bilateral tubers, whereas 13/21 patients with intellectual impairment had bilateral tubers. Patients with normal cognitive functioning experienced a mean age of seizure onset after 6 years. A trend was observed between infantile spasms and cognitive functioning (p=0.06); the lack of statistical significance likely reflects the small sample size. Neither age nor gender was related to cognitive status. Further investigation incorporating additional neuroimaging factors, antiepileptic treatment effects, and genetic variables, is needed
— id: 69073, year: 2006, vol: 15, page: 558, stat: Journal Article,

Evolving concepts in pediatric epilepsy surgery: Tuberous sclerosis as paradigm
Weiner, HL; Miles, D; LaJoie, J; Devinsky, O
2005 JUL ;103(1):A101-A101, Journal of neurosurgery
— id: 104260, year: 2005, vol: 103, page: A101, stat: Journal Article,

Developmental outcome of epilepsy surgery in tuberous sclerosis complex
Zaroff, Charles M; Morrison, Chris; Ferraris, Nina; Weiner, Howard L; Miles, Daniel K; Devinsky, Orrin
2005 Dec;7(4):321-326, Epileptic disorders
In Tuberous sclerosis complex (TSC), neurological dysfunction, usually in association with epilepsy, is responsible for the greatest degree of disease-related disability. Epilepsy surgery is increasingly recognized as a therapeutic option given the often medication-resistant nature of the disease. Seven subjects with medically refractory epilepsy associated with TSC, who underwent surgery at a tertiary care epilepsy center and in whom both preoperative and postoperative neuropsychological data were available, were examined. The Vineland Adaptive Behavior Scales, and in one case, the WISC-III were utilized. Postoperatively, the composite standard scores declined in six of the seven subjects, although for the most part this decline was quite modest (8 points or less in 5/6 subjects). The mean overall developmental/intellectual quotients were comparable across assessments (preoperative M = 55, SD = 20.3; postoperative M = 49 SD = 16.6). Good outcomes appeared to be related to seizure relief. Age estimates of developmental level indicated developmental progress in the majority of subjects in the current sample, and may yield greater clinical information for individuals with developmental delay than do standard scores
— id: 60143, year: 2005, vol: 7, page: 321, stat: Journal Article,

Mental retardation and relation to seizure and tuber burden in tuberous sclerosis complex
Zaroff, CM; Barr, W; Devinsky, O; Miles, D; Nass, R
2005 SEP ;46(11):166-166, Epilepsia
— id: 59587, year: 2005, vol: 46, page: 166, stat: Journal Article,

Epilepsy surgery in young children with tuberous sclerosis
Weiner, HL; Lajoie, J; Miles, D; Devinsky, O
2004 APR ;100(4):793-793, Journal of neurosurgery
— id: 104265, year: 2004, vol: 100, page: 793, stat: Journal Article,

Epilepsy surgery for children with tuberous sclerosis complex
Weiner, Howard L; Ferraris, Nina; LaJoie, Josiane; Miles, Daniel; Devinsky, Orrin
2004 Sep;19(9):687-689, Journal of child neurology
Tuberous sclerosis complex is associated with medically refractory seizures and developmental delay in children. These epilepsies are often resistant to antiepileptic drugs, can be quite severe, and usually have a negative impact on the child's neurologic and cognitive development. It is believed that functional outcome is improved if seizures can be controlled at an early age. The surgical treatment of intractable epilepsy in children and adults with tuberous sclerosis complex has gained significant interest in recent years. Previously published studies have shown a potential benefit from resection of single tubers, with most of the results noted in relatively older children. All of these reports support the idea that if a single primary epileptogenic tuber or region can be identified, then a surgical approach is appropriate. However, most children with tuberous sclerosis complex have multiple potentially epileptogenic tubers, rendering localization challenging, and they are therefore rejected as possible surgical candidates. We have used a novel surgical approach using invasive intracranial monitoring, which is typically multistaged and bilateral. This multistage surgical approach has been useful in identifying both primary and secondary epileptogenic zones in patients with tuberous sclerosis complex with multiple tubers. Multiple or bilateral seizure foci are not necessarily a contraindication to surgery in selected patients. Long-term follow-up will determine whether this approach has durable effects. We await better methods for identifying the epileptogenic zone, both noninvasive and invasive
— id: 47796, year: 2004, vol: 19, page: 687, stat: Journal Article,

Clinical features of patients with unilateral mesial temporal sclerosis (MTS) with persistent seizures following antero-mesial temporal resection
Yousef, TA; Pacia, SV; Barr, W; Cohen, E; Doyle, W; Devinsky, O; Luciano, D; Vazquez, B; Miles, D; Najjar, S; Kuzniecky, R
2004 AUG-SEP ;45(3):88-88, Epilepsia
— id: 49017, year: 2004, vol: 45, page: 88, stat: Journal Article,

Neuropsychological outcome of epilepsy surgery in tuberous sclerosis
Zaroff, C; Devinsky, O; Ferraris, N; Miles, D; Morrison, C; Weiner, H
2004 NOV ;19(7):852-853, Archives of clinical neuropsychology
— id: 104263, year: 2004, vol: 19, page: 852, stat: Journal Article,

Cognitive and behavioral correlates of tuberous sclerosis complex
Zaroff, Charles M; Devinsky, Orrin; Miles, Daniel; Barr, William B
2004 Nov;19(11):847-852, Journal of child neurology
Tuberous sclerosis complex is a multisystem disorder in which neurologic problems cause the greatest disability. High rates of mental retardation and autism spectrum disorders are associated with the diagnosis. Early-onset seizures and increased tuber burden are risk factors for cognitive impairment. Early-onset seizures, particularly infantile spasms, are risk factors for autism. Tubers within the temporal lobe and cerebellum are often mentioned as risk factors for autism, although the findings are inconsistent. Seizure control is important for developmental outcome and quality of life. Early behavioral assessment and therapeutic intervention, as well as seizure control, are the most effective means of promoting neurodevelopmental outcome
— id: 50288, year: 2004, vol: 19, page: 847, stat: Journal Article,

Epilepsy, atherosclerosis, myocardial infarction, and carbamazepine
de Chadarevian, Jean-Pierre; Legido, Agustin; Miles, Daniel K; Katsetos, Christos D
2003 Feb;18(2):150-151, Journal of child neurology
Epileptic patients receiving medications such as phenytoin or phenobarbital have been noted to have an exceedingly low incidence of myocardial infarction, but children treated with carbamazepine have shown alteration of their serum lipid profile that could predispose them to atherosclerosis. This report seems to corroborate this point and describes the findings in an 11-year-old boy who died following a major seizure, rhabdomyolysis, and renal failure. The autopsy demonstrated that he had marked coronary atherosclerosis and myocardial infarction. The child had been treated with carbamazepine for more than 10 years
— id: 34497, year: 2003, vol: 18, page: 150, stat: Journal Article,

Treatment of attention-deficit disorder, cerebral palsy, and mental retardation in epilepsy
LaJoie J; Miles DK
2002 Oct;3(5S):42-48, Epilepsy & behavior
Epilepsy in childhood is often associated with other neurologic disorders, including attention-deficit/hyperactivity disorder, cerebral palsy, and mental retardation. A single pathologic process may explain both epilepsy and these associated disorders. However, in some cases, distinct etiologies may be present. Recognition of these problems is essential, as is individualized treatment. Proper classroom placement; behavior modification, speech, occupational, and physical therapies; pharmacological agents; and even surgical procedures have a role in the management of these comorbid disorders. Diagnostic criteria and therapeutic modalities used in these syndromes will be discussed
— id: 34498, year: 2002, vol: 3, page: 42, stat: Journal Article,

Long-term computer-assisted outpatient electroencephalogram monitoring in children and adolescents
Foley CM; Legido A; Miles DK; Chandler DA; Grover WD
2000 Jan;15(1):49-55, Journal of child neurology
The aims of this study were (1) to define the role of long-term computer-assisted outpatient electroencephalographic monitoring (COEEG) in children and adolescents with known or suspected epilepsy, and (2) to compare COEEG data with routine interictal electroencephalograms (EEG). We performed 18-channel COEEG in 84 children and adolescents with diagnosed (group 1, n = 49) or suspected (group 2, n = 35) epilepsy. Mean recording time was 1.4 days. Overall, COEEG was useful in 87% of patients. In group 1, events were recorded in 73% of patients and were electrographic seizures in 45%. In group 2, events were detected in 86% of patients and were electrographic seizures in 17%. Nocturnal and partial seizures predominated. Seizure diagnosis and classification by COEEG was concordant with interictal EEG findings in 19% and discordant in 63% of patients. COEEG is a useful technique for the diagnosis of epileptic and nonepileptic events among selected children and adolescents. When compared to routine interictal EEG, COEEG could offer additional accuracy in the classification of seizures in pediatric patients
— id: 21812, year: 2000, vol: 15, page: 49, stat: Journal Article,

Tuberous sclerosis complex and epilepsy: prognostic significance of electroencephalography and magnetic resonance imaging
Husain AM; Foley CM; Legido A; Chandler DA; Miles DK; Grover WD
2000 Feb;15(2):81-83, Journal of child neurology
Tuberous sclerosis complex is a disease that affects many organs, including the central nervous system. Nervous system involvement in the form of hamartomas often results in seizures. In this study we wanted to determine the outcome of epilepsy in tuberous sclerosis complex and determine whether interictal electroencephalograms (EEGs) and hamartoma burden as seen with magnetic resonance imaging (MRI) are predictive of degree of seizure control. The study population consisted of 30 patients. For each patient two sets of EEG and MRI data, separated by at least 12 months, and information on seizure frequency at time of data collection were obtained. Sensitivity, specificity, and positive and negative predictive values of various EEG and MRI findings were determined. Seizure control improved in 20 and worsened in 10 patients. In relation to seizure control, the specificity of an abnormal sleep EEG and the positive predictive value of normal sleep EEG were 100%. MRI and EEG background were neither sensitive nor specific for predicting seizure control. A majority of children with tuberous sclerosis complex can achieve good seizure control. The sleep EEG is helpful in predicting eventual seizure control
— id: 21811, year: 2000, vol: 15, page: 81, stat: Journal Article,

West syndrome in tuberous sclerosis complex
Husain AM; Foley CM; Legido A; Chandler DA; Miles DK; Grover WD
2000 Sep;23(3):233-235, Pediatric neurology
West syndrome occurs commonly in children with tuberous sclerosis complex and is associated with a grave prognosis for cognitive and seizure outcomes. We sought to determine the epilepsy outcome of children with tuberous sclerosis complex and West syndrome and whether EEG, MRI, or steroid therapy duration were different in those whose epilepsy improved compared with those with intractable seizures. Seventeen patients with tuberous sclerosis complex and West syndrome were identified. For each patient, two sets of clinical evaluations, EEG and MRI data, and treatment information separated by at least 12 months were obtained. The patients were divided into two seizure outcome groups. EEG, MRI, and treatment data were compared between the groups. The intellectual deficiency was either severe (76%) or moderate (24%). Seizure control improved in 10 and worsened in seven, without mortality (follow-up range = 12-216 months). No significant differences in EEG background, MRI findings, or steroid treatment duration were evident between the groups. The difference in EEG-sleep approached statistical significance (P = 0.06). Our findings did not confirm reports of high mortality and poor epilepsy outcome in intellectually deficient children with West syndrome and tuberous sclerosis complex. EEG sleep was the best indicator of seizure control and approached statistical significance. The duration of steroid therapy had no influence on seizure control
— id: 21810, year: 2000, vol: 23, page: 233, stat: Journal Article,

Comparison of MRI and MRA findings in children with a variety of neurologic conditions
Husain AM; Smergel E; Legido A; Faerber EN; Foley CM; Miles DK; Grover WD
2000 Oct;23(4):307-311, Pediatric neurology
The role of MRA in the evaluation of children is evolving. We compared MRA and MRI in children with a variety of neurologic conditions to determine when MRA provides positive, cost-beneficial information. A total of 114 patients were retrospectively studied. MRA and MRI were performed and compared. MRA was abnormal in 34 (30%) of 114 patients: five (83%) of six with Menkes' disease, four (33%) of 12 with sickle cell disease, 12 (38%) of 32 with vascular malformations, one (6%) of 17 with headaches, seven (24%) of 24 with new focal deficits, one (10%) of 10 with seizures, and four (31%) of 13 with miscellaneous diagnoses. MRA and MRI were concordant in 73 (64%) of 114. Maximum concordance was in patients with Menkes' disease (100%) and minimum in those with new focal deficits (50%). The best MRA cost/benefit ratios were obtained in patients with Menkes' disease, vascular malformations, and sickle cell disease. A normal MRI usually forecasted a normal MRA. However, abnormal MRI findings did not always predict MRA abnormalities. Positive, cost-beneficial information is provided by MRA mostly in conditions known to involve the cerebral vasculature. Indications to perform MRA should be based on the neurologic diagnosis and MRI findings
— id: 21809, year: 2000, vol: 23, page: 307, stat: Journal Article,

It's not just a seizure: etiology, management, and transport of the seizure patient
Woodward GA; Chun TH; Miles DK
1999 Apr;15(2):147-155, Pediatric emergency care
— id: 21813, year: 1999, vol: 15, page: 147, stat: Journal Article,

Comparison of coronal sphenoidal versus standard anteroposterior temporal montage in the EEG recording of temporal lobe seizures
Ives JR; Drislane FW; Schachter SC; Miles DK; Coots JF; Martin DL; McGuiggan JM; Schomer DL
1996 May;98(5):417-421, Electroencephalography & clinical neurophysiology
Chronic sphenoidal electrodes were developed to facilitate the recording and localization of temporal lobe seizures during long term monitoring. Many reports demonstrate their utility in displaying temporal interictal epileptiform activity, but there have been few direct comparisons of sphenoidal electrodes and surface temporal recordings actually. We compared simultaneous portions of 74 EEG recordings of temporal lobe seizures (from 42 patients), with one portion including sphenoidal electrodes in a coronal montage and one with a standard anterior posterior temporal montage. Separated tracings were reviewed by readers blinded to the other portion of the tracing. The coronal sphenoidal montage allowed recognition of temporal lobe seizures inapparent with standard surface temporal electrodes in 19% of seizures and led to an earlier identification (usually by > or = 5 s) of the onset in 70% of seizures. Indwelling, flexible sphenoidal electrodes assist in ambulatory recording of temporal lobe seizures, both in demonstrating the presence of seizures and in determining the localization and time of seizure onset
— id: 21814, year: 1996, vol: 98, page: 417, stat: Journal Article,

A simple method for endoscopic placement of a nasoduodenal feeding tube
Kwauk ST; Miles D; Pinilla J; Arnold C
1996 Jun;10(6):680-683, Surgical endoscopy
The key to short-term enteral feeding in patients with gastroparesis is to deliver the nutrition beyond the pylorus. Endoscopic assisted methods allow the precise placement of the feeding tube to the small bowel. However, the main difficulty in association with these procedures is feeding-tube migration into the stomach during the withdrawal of the endoscope. We have developed an endoscopic method with a high success rate which prevents this problem. A reusable angiocatheter guidewire was threaded through the feeding tube, passing beyond the distal opening prior to the withdrawal of the scope. Counterpressure was applied to the feeding tube during the withdrawal of the endoscope. We have successfully placed feeding tubes in 22 out of 23 patients with no complications
— id: 19527, year: 1996, vol: 10, page: 680, stat: Journal Article,

Diagnostic value of pediatric outpatient video-EEG
Foley CM; Legido A; Miles DK; Grover WD
1995 Feb;12(2):120-124, Pediatric neurology
Outpatient video-electroencephalography (OVEEG) was performed in 100 infants, children, and adolescents with diagnosed (group I, n = 64) or suspected (group II, n = 36) epilepsy. Median monitoring duration was 4 hours. Indications for OVEEG in group I were classification of seizures, reported seizure exacerbation, or onset of new signs. OVEEG indications in group II were repetitive paroxysmal and stereotyped signs of myoclonic movements, fixed gaze, abnormal behavior, or nonmyoclonic motor activity. In group I patients, symptomatic events were recorded in 89%, half of which were seizures. Among group II patients, events were recorded in 67% and were seizures in 22%. Overall, OVEEG was successful in 83% of patients. Compared to a 24-hour inpatient admission for video-EEG monitoring, OVEEG represented cost reductions of 55-80% per patient. We conclude that OVEEG is a cost-effective, useful alternative to continuous inpatient video-EEG monitoring in the investigation of selected infants, children, and adolescents with diagnosed or suspected epilepsy
— id: 21815, year: 1995, vol: 12, page: 120, stat: Journal Article,

Neonatal adrenoleukodystrophy presenting as infantile progressive spinal muscular atrophy
Paul DA; Goldsmith LS; Miles DK; Moser AB; Spiro AJ; Grover WD
1993 Nov-Dec;9(6):496-497, Pediatric neurology
Two siblings with neonatal adrenoleukodystrophy are described. The signs and laboratory data documenting infantile progressive spinal muscular atrophy included the initial presentation of 1 sibling with neonatal adrenoleukodystrophy. These patients indicate that neonatal adrenoleukodystrophy should be considered in the differential diagnosis of infantile progressive spinal muscular atrophy
— id: 21816, year: 1993, vol: 9, page: 496, stat: Journal Article,

Benign neonatal seizures
Miles DK; Holmes GL
1990 Jul;7(3):369-379, Journal of clinical neurophysiology
Benign neonatal seizures is a rare but increasingly recognized syndrome characterized by seizures in the neonatal or infantile period. Two forms are recognized: familial and nonfamilial. In both instances, the seizures may be quite severe, and status epilepticus is common. The nonfamilial form is characterized by idiopathic, self-limited seizures occurring in previously normal neonates. The seizures most commonly occur at day 5 and have been called 'fifth-day fits' by some authors. Familial seizures most frequently have their onset during the first week of life, but onset may occur as late as early infancy. These seizures may recur for several months before resolving. No cause is found for the seizures, and the patient appears healthy during the interictal period. The family history reveals benign neonatal seizures in other family members. Although the prognosis is favorable in both syndromes, seizures may occasionally occur later in life in the familial form. The familial form of benign neonatal seizures is autosomal dominant, and the gene has been localized to chromosome 20
— id: 21817, year: 1990, vol: 7, page: 369, stat: Journal Article,