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Drug Prescriptions↗
Biomedical subjects
P Ruggieri
Publications and source records attributed to P Ruggieri.
Hippocampal volumetry in children 6 years or younger: assessment of children with and without complex febrile seizures.
PURPOSE: To study the relationship of complex febrile seizures (CFS) in the evolution of mesial temporal sclerosis. METHODS: We studied five children 22-68 (mean 44) months old with MRI volumetry 2 days-46 months after their first CFS, and compared total hippocampal volumes and right to left hippocampal volume ratios to those of 11 controls, 15-83 (mean 55) months old, who had MRI for complaints which turned out to be neurologically insignificant. RESULTS: In control children, total hippocampal volumes increased linearly with age, while right to left hippocampal volume ratios tended to decrease with age. In children with CFS total hippocampal volumes tended to be smaller than in controls. Right to left ratios were greater than 1 in all five children with CFS compared to seven of 11 controls. Hippocampal asymmetry was noted in only one child, with the right to left volume ratio exceeding two standard deviations from the control mean. The MRI of this child also demonstrated a subarachnoid cyst in the left frontocentral region, ipsilateral to the smaller hippocampus. Visual inspection of the remaining patients revealed no definite structural cortical abnormalities. None of the children developed subsequent afebrile seizures during the brief follow-up period. CONCLUSIONS: Hippocampal volumetry in controls revealed a linear increase in total hippocampal volumes and a statistically nonsignificant trend toward reduced right larger than left hippocampal ratios between 17 and 83 months old. The tendency for smaller total hippocampal volumes and larger right to left hippocampal volume ratios in children with CFS compared to controls could suggest a developmental abnormality, injury during CFS, or be age-related. The significant hippocampal asymmetry in a single child with CFS suggests that age may not be a factor in every case. Further studies are needed to collect control data in young children as well as prospectively follow children with CFS with serial imaging to better understand the relationship between CFS and the evolution of hippocampal atrophy.
Aging↗
Periictal diffusion-weighted imaging in a case of lesional epilepsy.
PURPOSE: Diffusion-weighted MR imaging (DWI) has been used for the early diagnosis of acute ischemic lesions in humans and in animal models of focal status epilepticus. We hypothesized that DWI may be a sensitive, noninvasive tool for the localization of the epileptogenic area during the periictal period. METHODS: A periictal DWI study was performed on a 35-year-old patient during focal status epilepticus with repetitive prolonged focal motor seizures originating from a lesion in the right frontal lobe. DWI results were analyzed visually and by calculating apparent diffusion coefficient (ADC) maps. RESULTS: On DWI, a single area of signal increase (decrease in ADC) was found in the region of focal electrocorticographic seizures that was mapped intraoperatively. CONCLUSIONS: Ictal/postictal DWI may be a useful technique for seizure localization in patients with lesional epilepsy.
Adult↗
Osteoid osteoma. Direct visual identification and intralesional excision of the nidus with minimal removal of bone.
We describe 100 consecutive patients with osteoid osteoma. Of the 97 who had operations, 89 were treated by intralesional excision and eight by wide resection. The three remaining patients were not operated on because the osteoid osteoma was almost painless, or was found in the pedicle of the 12th thoracic vertebra at the site of entrance of the artery of Adamkjewicz. The diagnosis was confirmed histologically in all specimens. No local recurrences were observed at a minimum follow-up of one year. All except one patient were mobilised two to four days after surgery. A precise preoperative diagnosis of the lesion is mandatory, based on clinical findings, standard radiographs, thin-section CT and a bone scan. We compared our operative technique with 247 cases in which the percutaneous technique of removal or coagulation of the nidus had been performed. The latter procedure has a less constant rate of primary cure (83% v 100%). Its principal indication appears to be for osteoid osteomas in the proximal femur and the pelvis.
Adolescent↗
Third ventriculostomy patency: comparison of findings at cine phase-contrast MR imaging and at direct exploration.
BACKGROUND AND PURPOSE: Two-dimensional phase-contrast (PC) MR imaging is a known method for evaluating CSF flow after third ventriculostomy. In this study, we attempted to confirm the accuracy of cine PC MR imaging for determining the patency of a third ventriculostomy as compared with direct reexploration of the floor of the third ventricle. METHODS: We examined 11 patients with third ventriculostomies who had a total of 13 reoperations for symptomatic obstructive hydrocephalus. In 12 of the 13 reexplorations, cine PC MR studies were obtained before repeat surgery, and the diagnoses suggested by imaging were compared with intraoperative findings. RESULTS: Four of five patients who had no flow on MR images had new membranes that covered the orifice; the fifth patient still had a small perforation visible at the time of operation. Three of four patients who had subtle flow on MR images were found to have occlusion with new membranes; the fourth had an incomplete new membrane. Finally, two of three who had a patent ventriculostomy had completely open perforations without membrane formation; the third patient had nonobstructive early membrane formation. At 3 months' follow-up, two flow studies were read as subtle without any clinical symptoms; however, these eventually progressed to become symptomatic, and occlusion with new membrane formation was confirmed during surgical reexploration. CONCLUSION: Cine PC MR imaging is a reliable technique for detecting the patency of a third ventriculostomy, but minor flow, as defined in this report, appears to be an early sign of closure.
Adolescent↗
Seizure outcome after epilepsy surgery in children and adolescents.
Few epilepsy surgery outcome data are available from series of pediatric patients. We studied seizure outcome in 136 pediatric patients who had surgery for intractable epilepsy at The Cleveland Clinic between January 1990 and June 1996, with a postoperative follow-up of 1 to 7.5 years (mean, 3.6 years). Sixty-two children (3 months to 12 years old at time of surgery) were compared with 74 adolescents (13-20 years old). Extratemporal or multilobar resections and hemispherectomies were similarly frequent among children (50%) and adolescents (44%), but these procedures strongly predominated in infancy (90% of patients 0-2 years of age). The remaining patients had temporal resection. Cortical dysplasia and low-grade tumor were the most common causes and hippocampal sclerosis was rare. Seizure-free outcome was achieved for 69% of adolescents, 68% of children, and 60% of the infant subgroup, overall; for 23 (74%) of 31 children and 33 (80%) of 41 adolescents after temporal resection; for 11 (58%) of 19 children and 15 (52%) of 29 adolescents after extratemporal or multilobar resection; and for 8 (67%) of 12 children and 3 (75%) of 4 adolescents after functional hemispherectomy. Seizure-free outcome was more frequent after temporal resection (56 of 72, 78%) than after extratemporal or multilobar resection (26 of 48, 54%; 41 of 48 with a focal lesion on magnetic resonance imaging), and among patients with tumor (36 of 44, 82%) versus cortical dysplasia (16 of 31, 52%). The frequency of seizure-free outcome after epilepsy surgery was similar for infants, children, and adolescents, and comparable with results from adult series. Most patients in each age, surgery type, and causal group were free from seizures after surgery. These results suggest that children should be considered for surgical evaluation at whatever age they manifest with severe, intractable, disabling localization-related epilepsy.
Adolescent↗
Ifosfamide and actinomycin-D, added in the induction phase to vincristine, cyclophosphamide and doxorubicin, improve histologic response and prognosis in patients with non metastatic Ewing's sarcoma of the extremity.
The role of ifosfamide as first-line chemotherapy treatment of non metastatic Ewing's sarcoma of the extremity is still under discussion. The purpose of this paper is to report the results achieved in a neoadjuvant protocol (REN-3) in which ifosfamide, added to the conventional VACA regimen, was employed since the induction phase. Induction chemotherapy consisted of vincristine, cyclophosphamide, doxorubicin, actinomycin-D and ifosfamide. After local treatment, patients received the drugs used in the induction phase and etoposide. Between November 1991 and November 1994, 61 patients with non metastatic Ewing's sarcoma of the extremity were treated. Forty-nine patients underwent surgery and 73.5% of them had a good histologic response. At a median follow-up of 60 months (range 32-76), 48 patients (79%) remained continuously disease-free. The 5-year event-free and overall survival were 77% and 87%, respectively. These results were significantly better both in terms of histologic response or event-free and overall survival than those obtained in 58 patients with non metastatic Ewing's sarcoma of the extremity treated in a previous protocol (REN-2) in which the same drugs were used, but ifosfamide was employed only in the maintenance phase. The present study suggests the importance of early use of ifosfamide in the treatment of patients with non metastatic Ewing's sarcoma of the extremity.
Adolescent↗
[Significance of computerized tomography in the diagnosis of post-traumatic proximal carpal instability].
INTRODUCTION: Proximal carpal instability is a painful condition characterized by early or late loss of radioulnar joint (RUJ) congruence not affecting the normal bone alignment of the two carpal rows. The joint incongruence or (incomplete) dislocation which leads to proximal instability is caused by many traumatic and nontraumatic events. The diagnosis of (incomplete) dislocation of the distal RUJ may be extremely difficult to make at conventional radiography because such injuries can be seen only when the lateral joint projection is perfect; otherwise the diagnosis is not reliable. CT is the only imaging tool diagnosing the grade of distal RUJ congruence independent of the examination technique. MATERIAL AND METHODS: We studied the radiocarpal complex conditions leading to proximal instability at the Rizzoli Orthopedic Institute from December, 1995, through December, 1997. In all, 389 cases were seen, 376 from trauma and 13 of nontraumatic origin. Each injury was studied with conventional radiography, CT, and MRI. Radiography was performed in two projections, namely the posteroanterior one with hand extension and the lateral one with the forearm in neutral position and the elbow bent at 90 degrees; the projections were repeated whenever a cast brace was applied. Unenhanced CT was performed for comparison with the patient prone and the forearm and wrist in prone and neutral position, as well as with the patient, forearm and wrist supine. Three criteria of electronic image processing were adopted for the RUJ studies in the 3 projections: radioulnar lines, congruence, and epicenter. MRI was always performed after conventional radiography and CT. Only the involved radiocarpal region was studied; coronal, axial and sagittal images were acquired with T2-weighted GE and T1-weighted SE sequences. RESULTS: Proximal instability was found in 17 of 389 patients; it was early in 13 and late in 4 of them. The comparison of radiographic and CT results showed that the former method is unreliable, with 53% false negatives. Pain, a cast brace, congenital or acquired deformities of distal radius and ulna and patient mispositioning by the radiology technician can change the rotation of the forearm, wrist and hand and make a perfect laterolateral projection in neutral position unfeasible, which affects the radiographic diagnosis. Conversely, CT showed its extreme efficacy in assessing the distal RUJ congruence with no false negatives independent of the RUJ rotation and of instability type and grade. CONCLUSIONS: Conventional radiography is a poorly reliable tool for the diagnosis of joint incongruence and its grade. In contrast, CT can diagnose a RUJ (incomplete) dislocation easily and unquestionably, thanks to its axial capabilities, even when adequate radiographic studies would be unfeasible. If the anteroposterior projection of the radiocarpal complex shows a congenital or acquired deformity of distal radius, the lateral projection can be skipped and a CT scan in prone, neutral and supine position performed. The 3 CT criteria quantify incongruence type and grade, and also demonstrate the position of maximum incongruence and its decrease by position. The comparative study of the radiocarpal region makes CT a very useful and valuable tool in congenital instability because its allows the assessment of contralateral radioulnar congruence too. MRI is very useful in the diagnosis of injury or degeneration of the fibrocartilage complex, namely in patients with no bone changes at conventional radiography.
Humans↗
Chemotherapy-induced tumor necrosis as a prognostic factor in localized Ewing's sarcoma of the extremities.
PURPOSE: This study was performed to assess the prognostic value of the proposed histopathologic method to evaluate the response of the primary tumor to preoperative chemotherapy in Ewing's sarcoma. PATIENTS AND METHODS: The response to chemotherapy was evaluated from the specimens of 118 Ewing's sarcoma patients, who were preoperatively treated by chemotherapy alone. Responses were graded I to III (macroscopic viable tumor, microscopic viable tumor, and no viable tumor cells, respectively). Follow-up data were available for all patients, with a mean follow-up duration of 86 months (range, 30 to 158). RESULTS: A statistically highly significant difference was observed in outcome among the three groups of patients. For patients with total necrosis (grade III response), the estimated 5-year disease-free survival rate was 95%, in contrast to 68% for grade II responders and 34% for grade III responders (P < .0001). This difference was also confirmed when any single group was compared with the other groups. Among the parameters tested, patient age and the size of tumor had some prognostic value. CONCLUSION: The proposed histopathologic grading, to evaluate the effect of chemotherapy on the primary tumor, had the strongest correlation to clinical outcome. This method could therefore be used to identify patients with a high risk of recurrent disease. These patients could be randomized to receive alternative postoperative treatments to investigate whether more aggressive therapies will improve outcome.
Adolescent↗
[The type of local treatment conditions the prognosis in patients with nonmetastatic Ewing's sarcoma of the extremities treated with adjuvant chemotherapy].
The results obtained in 172 cases of non metastatic Ewing's sarcoma of the extremities are reported. The patients were advised to undergo surgical treatment, followed by radiotherapy (40-45 Gy) in case of inadequate surgical margins. 48 patients who refused surgical treatment, were locally treated with radiotherapy alone (50-65 Gy). With a mean follow-up of 8 years (R. 3-15) 101 patients (58.7%) are free of disease and 68 relapsed with metastases and/or local recurrence. A radio-induced bone sarcoma developed in two patients, one patient died of ADM cardiomyopathy. No differences in terms of risk factors were observed between patients who were or were not treated with surgery. A better DFS was observed in the patients treated with surgery (66.9%) in comparison with those treated with radiotherapy alone. The higher percentage of local recurrences observed in patients treated with radiotherapy alone seems to be responsible for the worse prognosis observed in these patients. The authors' conclusion is that the local control in patients with non metastatic Ewing's sarcoma should always be achieved by means of surgery.
Adolescent↗
[Traditional radiology in the assessment of posttraumatic carpal instability].
INTRODUCTION: Carpal instability is a painful posttraumatic syndrome with early or late loss of the normal alignment of the carpal bones, which can be caused by a variety of injuries, from minor sprain to major fracture-dislocation of the carpal-wrist complex. If the trauma causing instability is a fracture, a severe dislocation or a fracture-dislocation, the radiographic diagnosis is not particularly difficult. The morphologic and dynamic complexity of the carpal region represents, instead, a major obstacle in the radiologic diagnosis of mild or moderate sprains because the morphologic alterations on standard static views are minimal or absent in these conditions. MATERIALS AND METHODS: We reviewed 214 injuries causing posttraumatic carpal instability including both the cases classified by the Data Analysis Center of the Istituto Ortopedico Rizzoli as carpal dislocations and fracture-dislocations from January, 1975, to July, 1996, and the more recent cases directly observed at our Casualty Clinic. In the former cases, we reviewed only the available images, while our patients were examined with comparative standard and under stress or dynamic views. RESULTS: Of 214 lesions causing posttraumatic carpal instability, 43 along the great arch were classified as severe because they were easily detectable on standard films and 171 along the small arch were classified as mild because slight/no abnormalities were detected on standard static views. Only dynamic imaging showed posttraumatic carpal instability demonstrating the integrity of the ligaments and of the carpal hinges, as well as gaps or asymmetry not detected on static views. CONCLUSIONS: We suggest the systematic use of dynamic imaging in the cases where static findings are negative or poor, in the patients with a painful wrist after an apparently minor sprain. Missed or delayed diagnoses are thus reduced, as well as the consequent joint incongruity and/or chronic subluxation which may severely impair these patients.
Carpal Bones↗
Epilepsy surgery in infants.
PURPOSE: We report 12 infants who had frontal (n = 3), temporal (n = 2), or temporoparieto-occipital (n = 2) resection or functional hemispherectomies (n = 5) at age 2.5-29 (mean 15.3) months for catastrophic epilepsy due to focal cortical dysplasia (n = 5), Sturge-Weber syndrome (n = 3), ganglioglioma (n = 3), or hemimegalencephaly (n = 1). Seizures began at 1 day to 14 months (mean, 4.0 months) after birth, occurred frequently (often many times per day, and were refractory to antiepileptic drugs. Patients were evaluated for surgery at 2.5-24 (mean 12.4) months of age. Seven patients had hemiparesis and eight had slowed cognitive development. Seizures were characterized by arrest or marked reduction of behavioral motor activity with nuclear level of consciousness (n = 4, with temporal or temporoparietal EEG seizures), focal clonic activity (n = 3, with perirolandic EEG seizures), generalized tonic stiffening (n = 3, with temporoparieto-occipital, parietal, or frontal EEG seizures), or infantile spasms and hypsarrhythmia (n = 2, with a frontal tumor or temporoparieto-occipital cortical dysplasia). METHODS: Magnetic resonance imaging (MRI) revealed the epileptogenic lesion in all but two patients, both with cortical dysplasia localized by interictal positron-emission tomography (PET) and other clinical or EEG features and confirmed on histopathologic examination of resected tissue. RESULTS: At follow-up 4-86 (mean 32) months after surgery, six patients were seizure free (Engel outcome class I), three had rare seizures with none in at least the previous 6 months (Engel class II), and two had worthwhile improvement (Engel class III). Except for the severely developmentally delayed infant with hemimegalencephaly, several patients had marked "catch-up" developmental progress after class I, II, or III outcome. Postoperative complications included subdural hematoma over the contralateral hemisphere (one patient) and entrapment and enlargement of the ipsilateral temporal horn (one patient) after hemispherectomy, both corrected uneventfully with a second surgical procedure. One patient died of unexplained causes several hours after frontal lobectomy. No patients had new neurologic deficits after surgery, and one patient had resolution of progressive fluctuating hemiparesis after resection of temporoparieto-occipital cortical dysplasia. CONCLUSIONS: Our results agree with previous reports that epilepsy surgery can provide relief from catastrophic epilepsy in carefully selected infants.
Age Factors↗
Aneurysmal bone cyst and giant cell tumor of the foot.
From 1950 to 1994, 257 cases of benign bone tumors of the foot were treated at the Bone Tumor Center of the Rizzoli Institute. Aneurysmal bone cysts (ABC) and giant cell tumors (GCT) are rare and often they are misdiagnosed. To define the clinical and radiographic features useful for diagnosis, x-rays of 24 ABCs and 21 GCTs of the foot were reviewed. Adding our series to the cases reported in the literature, the only important clinical data that emerged was the average age of patients affected by ABC and GCT (15 and 27 years, respectively). ABCs localized in small tarsal bones were rare (6%), whereas 19% of GCTs were found in this site. The eccentric, round shape of a lytic lesion was more characteristic of GCT. An aggressive pattern of radiographic bone destruction was observed more frequently in GCT than in ABC (P = 0.01). Septation was seen in both tumors. Typical of ABC was a sharp and trabeculated margin, whereas GCT had an ill-defined edge with normal cancellous bone (P = 0.007). The growth of ABCs often expanded the cortex, while GCTs eroded, thinned, and broke the cortical bone (P = 0.001). A saucerized cortex was observed only in ABCs. A bony shelf was more characteristic of ABC than of GCT (P = 0.002). Pathologic fractures (67%) and joint involvement, evaluated by computed tomography and magnetic resonance imaging (83%), were more frequent in GCTs, whereas fluid levels were more frequently observed in ABCs (47%). The majority of the tumors were stage 2, but GCTs represented 70% of stage 3 aggressive lesions.
Adolescent↗
Intra-arterial versus intravenous cisplatinum (in addition to systemic Adriamycin and high dose methotrexate) in the neoadjuvant treatment of osteosarcoma of the extremities. results of a randomized study.
Intra-arterial (IA) and intravenous (IV) cisplatinum (CDP) were studied in a multiagent regimen of neoadjuvant chemotherapy for osteosarcoma of the extremities. Preoperatively two cycles of high-dose methotrexate (HDMTX) were administered, followed 5 days later by CDP and Adriamycin (ADM). MTX and ADM were administered IV, and CDP was delivered IA or IV. Postoperatively, good responders received 3 more cycles of the same drugs, while poor responders had a longer chemotherapy including ifosfamide. The rate of good histological response to chemotherapy was significantly higher in patients treated intraarterially (78% vs 46%: P < .004), while no significant differences in terms of disease-free survival were observed between patients who received CDP IA and patients who received CDP IV (55% vs 51%). In the IA group, however, there was only one local recurrence vs 5 in the IV group. The IA infusion of CDP is more active on the primary tumor than the IV infusion.
Adolescent↗
Epilepsy surgery in the setting of periventricular leukomalacia and focal cortical dysplasia.
We report an infant who had successful epilepsy surgery for intractable infantile spasms in the setting of bilateral periventricular leukomalacia and remote germinal matrix hemorrhage. Although MRI gave evidence of a diffuse cerebral insult and EEG showed hypsarrhythmia, focal epileptogenicity was suggested by previous partial seizures with onset over the right temporoparietal-occipital region and PET hypometabolism in that same area. Right temporoparietal and lateral occipital resection at 15 months resulted in seizure freedom and dramatic developmental progress at 1-year follow-up. Histopathologic examination of resected tissue showed cortical dysplasia, possibly due to the same insult that also resulted in the bilateral periventricular leukomalacia. From an etiologic perspective, this is one of very few reported cases strongly implicating acquired focal cortical dysplasia in response to a prenatal insult. From a clinical perspective, the case illustrates that the spectrum of potential surgical candidacy in infants may be broader than usually suspected.
Brain↗
Multicentric giant cell tumor of skeleton.
A case of multicentric metachronous GCT was presented. The clinical and radiographic features of multicentric GCT as reported in the literature and in the present case were discussed. The multiple bone involvement made therapeutic assessment difficult. The surgical stage as well as the location of each lesion were important in planning surgery.
Adult↗
Supplementary sensorimotor area seizures in children and adolescents.
RATIONALE: Some types of seizures in children may be difficult to recognize; the diagnosis of seizures arising near the mesial posterior frontal supplementary sensorimotor area (SSMA) may be especially challenging. Such seizures have been well described in adults, but few pediatric cases have been reported even though onset is typically in childhood. METHODS: We studied 11 children and adolescents with SSMA seizures diagnosed by prolonged video electroencephalography (EEG). RESULTS: Mean age at onset was 5.8 years, and diagnosis by video EEG was made at a mean age of 12 years. Children had at least borderline intelligence and few abnormal findings on neurologic examination. Seizures were typically brief, frequent, and predominantly nocturnal, with bilateral tonic posturing, gross proximal limb movements, and preserved consciousness. Magnetic resonance imaging showed normal findings or a superior or mesial posterior frontal tumor or cortical dysplasia. Routine EEG findings were usually normal, but prolonged EEG showed epileptiform discharges over the vertex. Seizure onset in the region of the SSMA was confirmed by subdural EEG in six patients who were studied for epilepsy surgery. Five patients had seizure-free outcome or worthwhile improvement after operation. Two nonoperated patients are seizure-free on antiepileptic medication, and three are undergoing surgical evaluation. CONCLUSIONS: Diagnosis of SSMA seizures in children may be challenging because of unusual symptoms (bilateral tonic limb involvement with preserved consciousness) and frequently normal routine EEG findings. Video EEG may be necessary for diagnosis, and magnetic resonance imaging may reveal a focal lesion. Increased recognition of this seizure type in pediatric patients should lead to earlier diagnosis for optimal medical or surgical treatment.
Adolescent↗