Exploring mental status in Friedreich's ataxia: a combined neuropsychological, behavioral and neuroimaging study.

Despite much evidence of cognitive and affective disorders in Friedreich's ataxia (FRDA), the nature of mental status in FRDA has received little systematic attention. It has been proposed that the cerebellum may interfere indirectly with cognition through the cerebello-cortical loops, whereas the role of pathological changes in different areas of the central nervous system is still undetermined. In the present study, 13 patients with molecularly determined FRDA and a group of matched controls were evaluated by a comprehensive battery of neuropsychological tests and the Minnesota Multiphasic Personality Inventory. A repetitive task of simple visual-reaction times was used to investigate implicit learning in all subjects. Pathological changes in cortical areas were explored comparing cerebral activations of patients and controls during finger movements (functional MRI). The intelligence profile of FRDA patients is characterized by concrete thinking, poor capacity in concept formation and visuospatial reasoning. FRDA patients show reduced speed of information processing. The learning effect seen in controls was notably absent in patients with FRDA. The patients' personality is characterized by some pathological aspects and reduced defensiveness. Patterns of cortical activation during finger movements are heterogeneous in patients compared to controls. Cognitive impairment, mood disorders and motor deficits in FRDA patients may be the result of the cumulative damage caused by frataxin deficiency not only in the cerebellum and spinal cord but also in other brain areas.

Intra-arterial chemotherapy followed by chemo-embolisation in unresectable hepatocellular carcinoma.

Unresectable hepatocellular carcinoma is related to a poor prognosis. Encouraging response rates and survival have been reported with intra-arterial (i.a.) chemotherapy and chemo-embolisation, but limited data are available on the association of the two treatment modalities. We therefore started a new programme combining i.a. chemotherapy with chemo-embolisation. The treatment regimen consisted of L-leucovorin (100 mg/m2 i.v.), 5-fluorouracil (800 mg/m2 i.a.), and carboplatin (250 mg/m2 i.a.). Chemo-embolisation with mitoxantrone (10 mg/m2) plus ethiodized oil followed immediately. The same treatment plus gelatin sponge was given after 28 days. 26 patients entered the study and were evaluable for response and side-effects. Main patient characteristics were: males 21, females 5: median age 68 years (range 42-76 years); stage TNM II-III 17, IVA 9; Child's A 12, Child's B 14; elevated baseline alpha-fetoprotein 17; cirrhosis 25. 14 patients had a partial response (54%; 95% confidence interval 33-73%), 3 had stabilisation and 9 had progressive disease. Median survival was 11 months (range 2-20+). 16 patients had grade I-II pain and 15 grade I-II fever. Our results indicate that the regimen is safe, well tolerated and capable of inducing objective remissions in a high percentage of patients with hepatocellular carcinoma.

Arterial chemoembolization with epirubicin in unresectable hepatocellular-carcinoma in cirrhosis.

No reliable therapy has yet been established for unresectable hepatocellular carcinoma (HCC). Encouraging data in terms of response rate and survival have been reported with intra-arterial chemotherapy combined with venooclusive materials, specifically ethiodized oil and gelatin sponge. To evaluate the activity and tolerance of a new chemoembolization protocol in cirrhotic patients with HCC, 22 patients were treated with epirubicin (50 mg) and ethiodized oil (10-15 ml), administered through hepatic arterial catheters, followed by gelatin sponge. Patient characteristics were: median age 70 years (range, 59-77); ECOG performance status 0-1 in 15 and 2 in 7 cases; Child's A disease in 11 and B in 11; TNM stage II in 9, stage III in 3 and stage IVA in 10 cases. Histologically documented cirrhosis was present in all cases. A total of 53 courses of therapy has been delivered. All patients were evaluable for response and toxicity. Three partial remissions (13%), 2 stabilizations of disease and 17 progressions have been observed. Median time to progression was 4 months, with a median survival of 7.6 months (range, 1-26+ months). Significant differences in survival (p = 0.001) have been observed between patients at stage II-III (21 months) and those at stage IVA (3 months), and between patients with Child's A disease (10 months) and Child's B disease (4 months) (p= 0.02). The treatment was well tolerated, with only 2 cases of WHO grade I pain and 2 cases of grade I fever. In conclusion, our results indicate that the schedule has only limited activity and does; not seem to offer any sure advantage over other treatments modalites in HCC.

Feasibility of intra-arterial chemotherapy followed by chemoembolization, every 28 days, in unresectable hepatocellular carcinoma.

Encouraging response rates and survival have been reported with intra-arterial (i.a.) chemotherapy and chemoembolization, but limited data are available on the association of the two treatment modalities. We therefore started a feasibility study of i.a. chemotherapy plus chemoembolization, performed every 28 days for 3 cycles, according to the following schedule: L-leucovorin (100 mg/m(2) i.v.), fluorouracil (800 mg/m(2) i.a.), and carboplatin (250 mg/m(2) i.a.). Chemoembolization with mitoxantrone (10 mg/m(2)) plus ethiodized oil was performed immediately after this treatment, followed by gelatin powder. Fourteen patients entered the study and were evaluable for side effects. Main patient characteristics were: males 13, females 1; median age 65 yr (range 45-75); stage TNM II-III 10, IVA 4; Childs' A 8, Childs' B 6; elevated baseline alpha-fetoprotein, 11; cirrhosis 14. No drug-related deaths have been observed. Ten patients were able to complete the program. The reasons for discontinuing treatment were worsening of liver functions in 3 cases and grade IV neutropenia in 1 patient. Eight patients had grade I-II pain and 10 patients had grade I-II fever. In conclusion the study demonstrated that chemoembolization plus i.a. chemotherapy is feasible in patients with hepatocellular carcinoma in cirrhosis and deserves further investigation.

Prognostic factors in patients with hepatocellular carcinoma submitted to chemoembolization.

The prognosis of unresectable hepatocellular carcinoma is poor. Encouraging response rates have been reported with chemoembolization, but no survival advantage has been demonstrated. Assessment of the impact of the treatment modality on prognosis is complicated by a poor understanding of the prognostic factors in the disease. We therefore evaluated, through univariate and multivariate analysis, the role on prognosis of 16 variables in 63 patients submitted to chemoembolization. Patients were treated with epirubicin (50 mg) plus ethiodized oil and gelatin sponge (22 cases) or with a new program combining i.a, chemotherapy with chemoembolization (41 cases) as follows: L-leucovorin, 100 mg/m(2) i.v.; fluorouracil, 800 mg/m(2) i.a.; carboplatin, 250 mg/m(2) i.a. Chemoembolization with mitoxantrone, 10 mg/m(2), plus ethiodized oil and gelatine sponge was performed immediately after. Median survival for the whole group of patients was 294 days. A multivariate analysis showed a highly significant influence on survival for Child's status (p=0.002) and for TNM stage (p=0.01). Median survival for patients with Child's A disease was 13.9 months and for patients with TNM stage I-II disease 19 months. In conclusion, our data suggest that patients with limited disease and adequate liver function have a longer survival after chemoembolization.

Raysum reconstruction algorithm in MR cholangiopancreatography.

Magnetic resonance cholangiopancreatography (MRCP) is a new, non-invasive imaging technique for the visualization of the biliary ducts. The presence of stones within the choledocus is easily detectable in source images. However, three-dimensional reconstructions using the maximum intensity pixel (or projection) algorithm (MIP) fail to reproduce accurately the eventual presence of filling defects or parietal irregularities due to biliary stones. We used the Raysum algorithm in addition to the MIP in evaluating MRCPs of twelve patients with known choledocolithiasis. A visualization of the stones was obtained in nine (75%) patients by using the Raysum while visualization was obtained in one patient by using MIP. No additional sequences are required, and the post-processing time takes only a few seconds. The Raysum reconstruction can be successfully associated to the MIP in the three-dimensional evaluation of biliary stones in MRCP.

CT and MR imaging of melanocytic schwannomas; report of three cases.

Melanocytic Schwannomas are rare tumours which can arise in soft tissues, spinal nerve roots and in the central nervous system. The literature suggests that they have a malignant behaviour with local recurrence after surgery. We present three patients with this lesion, two in the thoracic spine and one in the head of the pancreas. The clinical outcome in these cases has been disappointing, since two patients died from complications due to local spreading of the tumour in spite of surgery and radiotherapy. CT could not distinguish these tumours from other neurogenic neoplasms. When these tumours occur near the vertebral body, CT could only detect early bone erosion. MR findings show promising features, with a high signal on T1-weighted images, due to melanin. However, more experience is necessary to establish the possible specific features of melanocytic Schwannomas in MRI.

Retained surgical gauzes: acute and chronic CT and US findings.

Surgical sponges retained after laparotomy represent a diagnostic problem if they cannot be identified by radiopaque markers on standard radiographs. We report on 9 patients from different hospitals with an abdominal gossypiboma 7 days to 21 years after the surgical procedure. Plain radiographs may suggest the diagnosis if a textile foreign body is calcified, that is, is equipped with radiopaque marker, or when a characteristic "whirl-like" pattern is present. CT and US are necessary procedures in chronic cases, since the lesion may mimic a mass; US shows specific echogenic areas with acoustic shadow; CT usually reveals a hypodense mass with a thick peripheral rim.

MRI in insulinomas: preliminary findings.

After establishing the diagnosis of an insulinoma, most surgeons prefer preoperative localization. Selective arteriography is usually considered the gold standard for this purpose. Recently, computed tomography (CT) and preoperative US have contended the role to angiography. MRI has been used in few cases of endocrine pancreatic tumors, and its role in this particular field has to be defined. Between November 1988 and September 1990 we evaluated 7 adult patients who had had surgery in our Surgical Department. Eight tumors were resected in 6 patients who were cured; in an 18-year-old woman surgical treatment was unsuccessful. Arteriography, CT, preoperative US, MRI and intraoperative US detected 2, 6, 6, 5 and 6 tumors, respectively. Two insulinomas (0.2 and 0.7 cm) were found at histologic examination in resected specimen. The ability of intraoperative US and careful surgical exploration to resolve more than 90% of cases makes the preoperative use of arteriography and CT of questionable value. If further experience confirms these findings, US and MRI may suffice.

[Critical considerations on the "innominate grooves" of the colon (author's transl)]. / Kritische Betrachtungen zu den "innominate grooves" des Kolons.

The authors discuss the meaning of the "innominate grooves" of the colon after having determined their anatomic substratum with a microradiographic study. In the iconographic and methodological critical evaluation of the double contrast technique the "innominate grooves" must be considered as an important sign of today's radiologic semeiotics and as elementary mucous structures which are first affected by pathologic situations.

Medical treatment of hepatocellular carcinoma: any progress?

BACKGROUND: Hepatocellular carcinoma (HCC) remains one of the most common neoplasms worldwide. Curative treatment options include liver transplantation or resection. Unfortunately, most patients still have unresectable or untransplantable HCC due to disease extension or comorbid factors and are therefore candidate only for palliative treatments. METHODS: In this review we have analyzed the different medical approaches employed in the treatment of HCC in an attempt to better define their roles. RESULTS: Palliative medical treatments including systemic chemotherapy, immunotherapy or hormonal manipulation rarely influence survival of the patients. Although a high response rate is often reported with new local therapies such as transcatheter arterial embolization, intraarterial chemotherapy or percutaneous ethanol injection, the real impact of these treatment modalities on patient survival remains to be determined. CONCLUSION: One way to improve the diagnosis of HCC patients would be an appropriate approach to evaluate new drugs or treatment modalities. To answer all the open questions, further trials, possibly randomized, should be conducted on a substantial number of patients with homogeneous prognostic factors.

Response to chemotherapy of brain metastases from malignant pleural mesothelioma.

Brain metastases represent an uncommon complication in malignant pleural mesothelioma. A 55-year-old male suffering from malignant mesothelioma and pretreated with intracavitary chemotherapy and radiotherapy was submitted to systemic chemotherapy including lomustine, carboplatin, vinorelbine, fluorouracil and folates after diagnosis of bilateral cerebral deposits. The patient had an impressive response to chemotherapy, with complete regression of related symptoms. This case report represents the first on response to chemotherapy of brain metastases from mesothelioma. It points out that chemotherapy should be further explored in this subset of patients.

Activity and tolerability of courses of intra-arterial chemotherapy followed by chemoembolization in unresectable hepatocellular carcinoma.

AIMS AND BACKGROUND: We previously reported encouraging response rates and survival with combined intra-arterial (i.a.) chemotherapy and chemoembolization in unresectable hepatocellular carcinoma. We therefore evaluated a new program combining three courses of i.a. chemotherapy with chemoembolization administered every 28 days. PATIENTS AND METHODS: The treatment regimen consisted of L-leucovorin (100 mg/m2 i.v.), fluorouracil (800 mg/m2 i.a.), and carboplatin (250 mg/m2 i.a.). Chemoembolization with mitoxantrone (10 mg/m2) plus ethiodized oil and gelatin sponge was performed immediately after. The same treatment was given every 28 days for 3 times. RESULTS: Twenty-eight patients entered the study and were assessable for response and side effects. There were 24 males and 4 females (median age, 68 yrs; range, 42-75). TNM stage was II-III in 20 and IVA in 8; 17 were Child's A and 11 Child's B. Baseline alpha-fetoprotein was elevated in 15, and there was cirrhosis in 23. Twelve patients had a partial response (43%; 95% confidence interval, 24-63%), 13 had stabilization, and 3 progressive disease. Median survival was 16.6 months (range, 2-24). Sixteen patients had grade I-II pain and 14 grade I-II fever. CONCLUSIONS: Our results indicate that the regimen is safe and well tolerated. Despite 43% objective remissions, our results do not seem better than those obtained with less intensive regimens.

Arterial mapping with color flow duplex imaging of the lower extremities after excimer-laser-assisted angioplasty.

The purpose of the present study was to evaluate the role of color flow duplex imaging (CFDI) in the follow-up of patients who have undergone excimer-laser-assisted angioplasty of peripheral arteries. Sixty-one patients (40 men and 21 women) were studied (mean age +/- SD sixty-three +/- nine years). All patients were affected by peripheral vascular disease and, for this reason, underwent percutaneous excimer-laser-assisted angioplasty. Digital angiography and CFDI were performed before the laser procedure. CFDI was repeated at months 1, 3, 6, 9, and 12 after the laser procedure, whereas angiography was repeated after twelve months. Common, superficial, and profunda femoral arteries and popliteal arteries were visualized in looking for the presence of lesions and occlusions, and spectral analysis of Doppler signals was recorded. After the initial success, claudication was reported again by 9 patients, 7 of whom showed total occlusions. All reocclusions were discovered by CFDI and confirmed by angiography; 3 of these 7 patients underwent a second laser procedure. The remaining 2 symptomatic patients showed patent vessels and did not undergo angiography. Another 9 patients redeveloped an occlusion, unsuspected from clinical history and symptoms. All the reocclusions, confirmed by angiography, were diagnosed by CFDI. The data show that CFDI provides an accurate noninvasive technique for following up patients after excimer laser angioplasty, allowing for asymptomatic reocclusions to be recognized and treated if necessary, and permitting symptoms not due to reocclusions to be properly identified, thus avoiding unnecessary angiography.

Percutaneous excimer laser angioplasty of lower limb vessels: results of a prospective 24-month follow-up.

The aim of this prospective study was to assess the effectiveness and the long-term patency effect of excimer laser angioplasty in peripheral arterial obstructive disease. Seventy-eight patients referred for excimer laser angioplasty of lower limbs have been followed up for up to 24 months. Ankle/brachial systolic pressure index, color Doppler mapping, and arterial digital subtraction angiography were performed. Immediate procedural success was achieved in a high percentage of patients (97%). Balloon angioplasty was also used in 85% of patients. Early reocclusions occurred in 8% of patients. The cumulative patency rate was 47% at the 12-month interval and 40% at the 24-month interval. Poor runoff and the length of the lesions negatively influenced the outcome. Excimer laser angioplasty is an effective procedure, indicated in selected patients showing < 10 cm occlusions and good runoff.

[Adrenal cystic lymphangioma. A case report]. / Linfangioma cistico surrenalico. Presentazione di un caso.

Adrenal cysts are uncommon. A case of left adrenal cystic lymphangioma in 16 year old boy is reported. The adrenal cyst was a chance finding during radiological investigation for abdominal pain. Ultrasound and CT scan showed the cyst and suggested the correct diagnosis of cystic lymphangioma of the left adrenal gland. The patient underwent surgery and a left adrenalectomy was performed. Histology confirmed the previous diagnosis of cystic lymphangioma.

Elastofibroma: CT and MR findings.

Two cases of elastofibroma of the chest wall are reported. Each patient was investigated with CT and, in 1 case, MR imaging was also performed. Elastofibroma appears like a mass in the subscapular region and often presents problems of differential diagnosis. The contribution of MR imaging is reported.

Iodixanol in multidetector-row computed tomography angiography (MDCTA): diagnostic accuracy for abdominal aorta and abdominal aortic major-branch diseases using four-, eight- and 16-detector-row CT scanners.

PURPOSE: To compare iodixanol-enhanced multidetector-row computed tomography angiography (MDCTA) with digital subtraction angiography (DSA), perioperative angiography, or surgical findings in the evaluation of the abdominal aorta and its main branches. MATERIAL AND METHODS: 173 patients with known or suspected aortic aneurysms or stenosis/occlusion of the abdominal aorta or its major branches were enrolled. The iso-osmolar contrast medium iodixanol (320 mg Iota/ml) was used, and data were acquired using four-, eight-, or 16-active-detector-row scanners. Reference diagnoses were provided by surgical findings, interventional findings, or DSA. Diagnostic accuracy was estimated with reference to surgery or interventional arteriography or DSA. Image quality was assessed as excellent, good, sufficient, or insufficient, and correlations were made with attenuation values in the aortic lumen. Tolerability of iodixanol was monitored during the injection for discomfort and other adverse events, and for 72 hours after contrast injection. RESULTS: In 132 of 136 evaluable cases, MDCTA diagnosis matched the reference diagnosis, yielding an agreement rate of 97.1% (95% CI 92.6-99.2%). The quality of most MDCTA scans (147/173) was rated as excellent. Overall mean attenuation was 305.7 HU. MDCTA appeared more accurate than DSA for identification of lesion calcification, thrombus, irregularity, and ulceration. Tolerability of iodixanol was good, and no serious adverse events were reported. CONCLUSION: MDCTA using iodixanol is a promising, noninvasive alternative for evaluating patients with abdominal aortic disease.