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Biomedical subjects

K Vahedi

Publications and source records attributed to K Vahedi.

At least 19 recordsLinked to original sources
PubMed2006

Diagnosis and treatment of dural carotid-cavernous fistulas: a consecutive series of 27 patients.

OBJECTIVES: To report clinical characteristics, angiographical findings and results of endovascular treatment of patients presenting with dural carotid-cavernous fistulas (DCCFs). METHOD: Retrospective analysis of 27 consecutive patients with DCCF referred to a specialised interventional neuroradiology department. RESULTS: Orbital and neuro-ophthalmological symptoms were the most common clinical presentation at diagnosis (n = 25). The venous drainage of the fistula involved the ipsilateral superior ophthalmic vein in 24 patients, the contralateral cavernous sinus in 6 and a leptomeningeal vein in 5 patients. Thrombosis of at least one petrosal sinus was found in 23 patients. 7 patients did not receive endovascular treatment: 3 had spontaneous DCCF obliteration, and 4 had only minor clinical symptoms and no leptomeningeal venous drainage on an angiogram. 20 patients received endovascular treatment via either a transvenous (n = 16) or a transarterial approach (n = 4). Complete occlusion of the fistula was obtained in 14 of 16 (87%) patients treated by the transvenous approach and in 1 of 4 (25%) patients treated by the transarterial approach. 16 patients had early clinical improvement after endovascular treatment. One patient had a cerebral haemorrhage after transvenous embolisation of a DCCF with leptomeningeal drainage. On follow-up, all patients treated by the transarterial route remained symptomatic, whereas 10 of 14 (71%) patients cured by the transvenous route were asymptomatic. CONCLUSIONS: Transvenous embolisation is a safe and efficient endovascular approach to treat patients with DCCF. However, this technique requires a long learning curve.

Adult↗
PubMed2006

Cognitive profile in CADASIL.

BACKGROUND: The spectrum of cognitive alterations associated with CADASIL, a model of pure vascular dementia, has not been thoroughly evaluated. OBJECTIVES: The aims of this study were: (i) to describe the cognitive profile in CADASIL patients according to age; (ii) to compare the profile of patients with dementia with that of patients without dementia; and (iii) to determine the association between alterations in performance in different cognitive domains. METHODS: Forty two consecutive individuals with CADASIL (35-73 years old) were investigated. Cognitive skills were analysed in five domains (executive functions, reasoning, attention, memory, visuospatial abilities) according to age and compared between patients with and without dementia. Associations between cognitive performance and stroke were tested. RESULTS: The youngest patients presented with attention (69%), memory (70%), and executive disturbances (100%). Visuospatial abilities and reasoning deteriorated with age, mainly after the age of 60. About one quarter of patients had dementia, and 75% of these were >60 years of age. Age >60 years was associated with a Rankin score >3 and a significant deficit in all cognitive domains. No association was found between dementia and the number of ischaemic attacks. Episodic memory disorder was characterised by difficulties in retrieval rather than impairment of the encoding process. CONCLUSION: Cognitive decline in CADASIL is dominated by early impairment of executive functions. Skills in other cognitive domains deteriorate with age and are found to be diffusely impaired in patients with dementia. The relative preservation of the encoding process in episodic memory impairment, even in individuals with dementia, is noteworthy.

Adult↗
PubMed2006

Orgasmic headache and middle cerebral artery dissection.

A patient with a 20 year history of primary orgasmic headache is described who, after suffering an unusually severe episode of orgasmic headache was found to have a middle cerebral artery dissection. This unusual association of primary and secondary orgasmic headache emphasises the need for a thorough diagnostic examination when the orgasmic headache differs from that of previous episodes or is associated with neurological symptoms.

Adult↗
PubMed2005

Clinical impact of capsule endoscopy compared to push enteroscopy: 1-year follow-up study.

BACKGROUND AND STUDY AIMS: The long-term outcome for patients with obscure bleeding after capsule endoscopy (CE) is still unclear. In this study, the clinical outcome was used as the gold standard to determine the sensitivity and specificity of CE and push enteroscopy (PE) in the diagnosis of small-bowel lesions in patients with obscure bleeding. PATIENTS AND METHODS: Fifty-eight patients from a previous prospective study (comparing PE and CE) were included; the patients were contacted after 1 year. The final diagnosis, bleeding status, new gastrointestinal examinations, and treatments performed were recorded. On the basis of these data, each case was classified into true/false positive or true/false negative findings at PE and CE. The results were compared with the initial classification of lesions observed at CE: highly relevant (P2) and less relevant (P0, P1) lesions. RESULTS: Follow-up data were available for 56 patients. According to the defined true/false positive and negative cases, the sensitivity and specificity values for CE and PE were 92 % and 48 %, and 80 % and 69 %, respectively ( P < 0.01 for the difference between CE and PE). Highly relevant (P2) lesions observed at CE were more frequently classified into true-positive cases (15 of 18 versus seven of 22; P < 0.01) and led more frequently to therapeutic decisions (11 of 18 versus five of 22; P = 0.02) in comparison with less relevant lesions (P0, P1). CONCLUSIONS: CE is a highly sensitive examination for the detection of small-bowel lesions in patients with obscure gastrointestinal bleeding, with a specificity lower than that of PE when the clinical outcome is used as the gold standard.

Adult↗
PubMed2005

A 3-month double-blind randomised study comparing an olive oil- with a soyabean oil-based intravenous lipid emulsion in home parenteral nutrition patients.

Intravenous lipid emulsions (ILE) have demonstrated advantages including prevention of essential fatty acid (EFA) deficiency; however, too much EFA can down regulate fatty acid elongation leading to an imbalance of nutritional compounds in plasma and cell membranes. An olive oil-based ILE containing long-chain triacylglycerols (LCT) with a low content (20 %) of PUFA was administered for home parenteral nutrition (HPN) and compared with a conventional soyabean oil-based ILE (PUFA content, 60 %). Thirteen patients (26-92 years) with stable intestinal failure were randomised after a 1-month run-in period with a medium-chain triacylglycerols-LCT-based ILE, to receive 3 months of HPN with either olive oil- (n 6) or soyabean oil-based (n 7) ILE. The nutritional impact and safety of HPN, oral intakes and absorption rates, phospholipid fatty acids in plasma and lymphocyte cell membrane were assessed. The only clinical event reported was one case of pneumonia (soya group). In both groups, 20 : 3n-9:20 : 4n-6 ratios remained within normal ranges (0.03-0.07). There was a significant increase of gamma-linolenic acid (gamma-LA) in plasma and lymphocyte cell membrane (P=0.02) and of oleic acid in plasma (P<0.01) in the olive compared with the soya group. A significant correlation was found between gamma-LA (day 90 - day 0) in plasma and PUFA parenteral intakes (P=0.02), but neither with fat intakes nor with fat absorption rates. In conclusion, plasma and lymphocyte EFA pattern remained in normal ranges without EFA deficiency with both lipid emulsions, despite a lower content of n-3 and n-6 series with the olive oil-based ILE.

Adult↗
PubMed2004

[Diagnostic and therapeutic strategy in duodenal diverticular bleeding: report of two cases and review of the literature].

Host of the duodenal diverticulum are asymptomatic and located on the second part of the duodenum; these diverticula may induce major haemorrhage on rare occasions. We report on two patients who presented with massive upper gastrointestinal bleeding originating from a duodenal diverticulum. One patient was successfully treated by endoscopy and the other patient required diverticulectomy with successful outcome.

Aged↗
PubMed2003

Hydrostatic balloon dilatation of Crohn's strictures.

AIM: To evaluate the safety and long-term efficacy of per-endoscopic hydrostatic balloon dilatation in a retrospective series of patients with Crohn's disease. METHODS: Thirty-eight patients had balloon dilatation for intestinal symptomatic strictures which were located as follows: ileo-colonic (26) or colocolic (2) anastomosis, colon (4), ileum (3), proximal jejunum (1) and ileo-caecal valve (5); three patients had two strictures accessible to dilatation. The mean length of the strictures was 2.1 cm (s.d., 0.3 cm). RESULTS: Thirty-two of the 38 patients were successfully dilated and followed for a median of 22.8 months (0.2-103 months) until surgery or last news. The probabilities of obstructive symptom recurrence were 36% at 1 year and 60% at 5 years. Twelve patients had a second dilatation, and three a third. The probabilities of surgery for stricture were 26% at 1 year and 43% at 5 years. Results were not influenced by age, sex, activity of the disease, passage of the stricture by the colonoscope or concomitant medical therapies. Complications occurred in 9.4% of the 53 dilatation sessions, with only one perforation. CONCLUSIONS: Hydrostatic balloon dilatation is effective for Crohn's symptomatic strictures, and can avoid or postpone surgery, with an acceptable rate of complications.

Adult↗
PubMed2003

Hereditary infantile hemiparesis, retinal arteriolar tortuosity, and leukoencephalopathy.

BACKGROUND: The main hereditary vascular conditions involving both retinal and cerebral vessels include cerebroretinal vasculopathy, HERNS (hereditary endotheliopathy with retinopathy, nephropathy, and stroke), and hereditary vascular retinopathy; all are linked to the same locus on chromosome 3p21. Hereditary retinal arteriolar tortuosity is a distinct, autosomal dominant condition characterized by retinal arteriolar tortuosity and recurrent retinal hemorrhages. This condition is known to affect only retinal vessels. METHODS: Clinical and brain MRI investigations of eight members of a three-generation family and extensive biological and systemic vascular investigations within one affected family member were conducted. RESULTS: Six of eight family members were clinically symptomatic; disorders included infantile hemiparesis (2), migraine with aura (3), and retinal hemorrhage (1). Five individuals had retinal arteriolar tortuosities. A diffuse leukoencephalopathy in association with dilated perivascular spaces was observed in six individuals. Two family members had silent, deep cerebral infarcts as demonstrated on MRI. Genetic linkage analysis strongly suggests that this disorder is not linked to the 3p21 hereditary vascular retinopathy/cerebroretinal vasculopathy/HERNS locus. CONCLUSIONS: The authors describe a novel hereditary autosomal dominant condition affecting both retinal and cerebral vessels and characterized by infantile hemiparesis, migraine with aura, retinal hemorrhage, retinal arterial tortuosity, and leukoencephalopathy with dilatation of perivascular spaces and microbleeds on brain MRI. Investigation of additional families should help to map the gene and to better categorize the spectrum of hereditary cerebroretinal small vessel diseases.

Adolescent↗
PubMed2003

Diagnostic value of endoscopic capsule in patients with obscure digestive bleeding: blinded comparison with video push-enteroscopy.

BACKGROUND AND STUDY AIMS: This study was designed to prospectively compare the diagnostic yield of the M2A endoscopic capsule with that of video push-enteroscopy in exploring the small intestine in patients with obscure digestive bleeding. PATIENTS AND METHODS: Patients with either occult or overt obscure digestive bleeding and a negative endoscopic work-up underwent a double intestinal investigation, with video push-enteroscopy and a wireless capsule, performed blindly by separate examiners. The diagnostic yield for each technique was defined as the frequency of detection of clinically relevant intestinal lesions, i. e. those having the potential for bleeding. RESULTS: 60 patients (27 men, 33 women; age 58 +/- 18 years; hemoglobin 9.4 +/- 2.5 g/dl) were included, 32 with occult and 28 with overt bleeding. Results were analyzed for 58 patients, who completed both examinations. Lesions were detected in 43 patients: with both techniques in 19, only by capsule in 21, and, conversely, only by push-enteroscopy in 3 ( P = 0.04). Final diagnoses were as follows: a P2 lesion with high bleeding potential in 28 patients (19 angiomata, 6 ulcerations, 2 tumors, 1 case of intestinal varices); a P1 lesion with intermediate bleeding potential in 15 patients (2 patients with mucosal erosions, 13 patients with mucosal red spots); and there were normal findings from 15 procedures. No procedure induced any complication. CONCLUSION: The use of the wireless endoscopy capsule detects significantly more clinically relevant intestinal lesions than video push-enteroscopy in patients with obscure digestive bleeding, raising the diagnostic yield to 67.2 %. Its influence on the clinical outcome for patients needs further investigation.

Adult↗
PubMed2003

Osteoporosis in patients on long-term home parenteral nutrition: a longitudinal study.

UNLABELLED: The prevalence of osteoporosis was investigated in 88 patients with intestinal failure (IF). Osteoporosis was found in 67%, dependent of body mass index and age when IF occurred. In 56 patients on HPN, followed prospectively, changes in bone density were dependent on the duration of HPN; older patients had a higher increase. INTRODUCTION: It has been suggested that low bone mass and negative bone balance may occur in adult patients receiving home parenteral nutrition (HPN). The aim of this study was to assess prospectively the prevalence of osteoporosis in intestinal failure (IF) patients and the changes in bone mineral density in those on long-term HPN and to analyze the factors that may influence the occurrence and evolution of osteoporosis. MATERIALS AND METHODS: Bone mineral density was measured at the lumbar spine and femoral neck in 88 IF patients. RESULTS: At the first bone mineral density determination (baseline), the prevalence of osteoporosis was 67% in this population (median age, 52 years). Ten percent of the patients with osteoporosis experienced fragility fractures. Osteoporosis was independent of age and gender but occurred earlier in patients who had received corticosteroids. At baseline, the lumbar Z-score was positively correlated mainly to body mass index and age when IF occurred; these two parameters explained 34% of the Z-score. Repeated measurements were performed in 56 patients during long-term HPN (mean duration, 5.5 +/- 1.2 years). The changes in Z-score at the lumbar spine were dependent on the age when IF occurred and on the duration of HPN, with a synergistic effect between them. The older the patients, the higher the increase in Z-score during HPN. CONCLUSION: HPN had no deleterious effect on cortical bone and actually improved trabecular bone in patients whose intestinal disease started after the age of 21 years.

Adolescent↗
PubMed2002

Efficacy and tolerability of acetazolamide in migraine prophylaxis: a randomised placebo-controlled trial.

BACKGROUND: Familial hemiplegic migraine and episodic ataxia type 2 (EA2) are allelic disorders with distinct types of mutations in the CACNA1A gene. EA2 attacks are remarkably sensitive to acetazolamide, a carbonic anhydrase inhibitor. The effectiveness of acetazolamide in migraine prophylaxis is unknown. OBJECTIVES: To evaluate the efficacy and the tolerability of acetazolamide in migraine prophylaxis. METHODS: We compared daily oral 500 mg acetazolamide and placebo in patients with migraine in a multicentre, double-blind, randomised trial of 12 weeks duration after a run-in period of 4 weeks without treatment. Frequency of attacks at the last trial period of 4 weeks was the primary efficacy criterion. Secondary efficacy criteria were the frequency of attacks per 4 weeks, the severity and duration of attacks, the number of hours with migraine as well as the number of responders with more than 50% reduction in attack frequency. RESULTS: 53 patients had been enrolled when the study was prematurely stopped because of a high number of withdrawals (34%), primarily linked to acetazolamide related side effects. Considering the primary and secondary efficacy criteria, among the 53 included patients (27 in the placebo group and 26 in the acetazolamide group), no difference between the 2 study groups could be demonstrated. The most frequent adverse events related to acetazolamide were paresthesias and asthenia. CONCLUSIONS: In this trial, migraine sufferers poorly tolerated acetazolamide given in an oral dose of 500 mg daily. No obvious prophylactic beneficial effect of acetazolamide appeared on migraine attacks.

Acetazolamide↗
PubMed2001

Skin biopsy immunostaining with a Notch3 monoclonal antibody for CADASIL diagnosis.

CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy) is a small-artery disease of the brain caused by NOTCH3 mutations that lead to an abnormal accumulation of NOTCH3 within the vasculature. We aimed to establish whether immunostaining skin biopsy samples with a monoclonal antibody specific for NOTCH3 could form the basis of a reliable and easy diagnostic test. We compared the sensitivity and specificity of this method in two groups of patients suspected of having CADASIL with complete scanning of mutation-causing exons of NOTCH3 (in a retrospective series of 39 patients) and with limited scanning of four exons that are mutation hotspots (prospective series of 42 patients). In the retrospective series skin biopsy was positive in 21 (96%) of the 22 CADASIL patients examined and negative in all others; in the prospective series, seven of the 42 patients had a positive skin biopsy whereas only four had a mutation detected by limited NOTCH3 scanning. Our immunostaining technique is highly sensitive (96%) and specific (100%) for diagnosis of CADASIL.

Adult↗
PubMed2001

The clinical spectrum of familial hemiplegic migraine associated with mutations in a neuronal calcium channel.

BACKGROUND: Familial hemiplegic migraine, an autosomal dominant disorder characterized by attacks of transient hemiparesis followed by a migraine headache, is classically divided into pure familial hemiplegic migraine (affecting 80 percent of families) and familial hemiplegic migraine with permanent cerebellar signs (affecting 20 percent of families). Mutations in CACNA1A, which encodes a neuronal calcium channel, are present in 50 percent of families with hemiplegic migraine, including all those with cerebellar signs. We studied the various clinical manifestations associated with mutations in CACNA1A in families with hemiplegic migraine with and without cerebellar signs. METHODS: CACNA1A was analyzed and nine mutations were detected in 15 of 16 probands of families affected by hemiplegic migraine and cerebellar signs, in 2 of 3 subjects with sporadic hemiplegic migraine and cerebellar signs, and in 4 of 12 probands of families affected by pure hemiplegic migraine. Genotyping of probands and relatives identified a total of 117 subjects with mutations whose clinical manifestations were assessed in detail. RESULTS: Eighty-nine percent of the subjects with mutations had attacks of hemiplegic migraine. One third had severe attacks with coma, prolonged hemiplegia, or both, with full recovery. All nine mutations, including five newly identified ones, were missense mutations. Six mutations were associated with hemiplegic migraine and cerebellar signs, and 83 percent of the subjects with these six mutations had nystagmus, ataxia, or both. Three mutations were associated with pure hemiplegic migraine. CONCLUSIONS: Hemiplegic migraine in subjects with mutations in CACNA1A has a broad clinical spectrum. This clinical variability is partially associated with the various types of mutations.

Age of Onset↗
PubMed2001

Clinical, social and rehabilitation status of long-term home parenteral nutrition patients: results of a European multicentre survey.

BACKGROUND: Home parenteral nutrition (HPN) is a lifesaving treatment in patients with intestinal failure. Dependency of nutritional support becomes permanent for the majority of patients who had received HPN for at least 2 years. The alternative to long term HPN in selected patients is intestinal transplantation. AIMS: To study some of the clinical, social and rehabilitation aspects of long-term HPN treatment. METHODS: A survey was performed in nine European HPN centres. The questionnaire covered epidemiologic data, underlying diseases, intestinal anatomy, nutritional support and status, marital status, rehospitalization rate, HPN complications, rehabilitation score, drugs use, coexistent diseases and interest in intestinal transplantation. For some items, data were collected within 12 months prior to the evaluation. RESULTS: This survey included 228 patients with a median age of 49 years. The median duration of HPN was 7 years (range 2--24 years). Short bowel length less than 100 cm was reported in 65% of patients with a predominance of end-jejunostomy or jejuno-colonic anastomosis. Global subjective nutritional status was normal in 79% of the patients, who were supplied with a mean number of 5.6 bags of parenteral nutrition weekly. Rehospitalizations within the 12 months prior to evaluation accounted for a mean period of 23 days and were due to HPN complications in half of the cases. Catheter-related sepsis was the most frequent HPN-complication. Bone metabolism disorders, which seemed to be more common than liver diseases, were directly related to HPN duration. One-third of the HPN patients was regularly consuming analgesics or opiates. HPN impair complete rehabilitation status but may improve the status of patients who had a very low rehabilitation score before starting HPN. An interest of intestinal transplantation was noted in only 10% of medical teams and in 8% of HPN patients. CONCLUSIONS: This study is the largest European survey on long-term HPN patients with long standing or permanent intestinal failure. Data enlighten clinical, social and rehabilitation aspects of patients who could face the option of intestinal transplantation in the future.

Adult↗
PubMed2001

Diffusion tensor imaging study of subcortical gray matter in cadasil.

BACKGROUND AND PURPOSE: In cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), water diffusion changes suggestive of microstructural tissue alterations have been recently reported in abnormal- and normal-appearing white matter as seen on T2-weighted images. In the subcortical gray matter, typical lacunar infarcts are repeatedly observed. Whether microstructural tissue changes are also present outside these lesions within the putamen or thalamus remains unknown. METHODS: We used diffusion tensor imaging, an MRI method highly sensitive to cerebral microstructure, in 20 CADASIL patients and 12 controls. Both the trace of the diffusion tensor [Tr(D)] and an anisotropic diffusion index (volume ratio) of diffusion were measured within the putamen and thalamus outside typical lacunar infarcts as detected on both T1- and T2-weighted images. RESULTS: A significant increase in Tr(D) and a decrease in anisotropy were observed in the putamen and thalamus in patients. The right/left indices of Tr(D) in the thalamus, but not in the putamen, were strongly correlated with the corresponding indices calculated in the white matter of the centrum semiovale. In addition, the diffusion increase in the thalamus was positively correlated with Tr(D) and with the load of small deep infarcts within the white matter and negatively correlated with the Mini-Mental State Examination score. CONCLUSIONS: Our results suggest that microstructural tissue alterations are present in the putamen and thalamus, outside the typical lacunar infarcts in CADASIL. In the thalamus, these microstructural changes appear constant and are even observed in asymptomatic subjects. Some of these thalamic changes appear to result from degeneration of thalamocortical pathways secondary to ischemic white matter damage. The importance of this degenerative phenomenon in the pathophysiology of CADASIL requires further investigation.

Analysis of Variance↗