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Biomedical subjects

M A Farrell

Publications and source records attributed to M A Farrell.

At least 19 recordsLinked to original sources
PubMed2006

Relapsing granulomatous angiitis of the central nervous system in a patient while in remission from Hodgkin lymphoma.

We present a patient with granulomatous angiitis of the central nervous system (GANS) and Hodgkin lymphoma. His GANS resolved with treatment for the lymphoma, but then reactivated six months later in the absence of activate lymphoma. He made a full neurological recovery after treatment with reducing oral prednisolone over one year. This case indicates that prolonged use of steroids may be necessary to treat GANS in this setting and that it can run a course independent of the Hodgkin lymphoma.

Adolescent↗
PubMed2005

Fatal toxic leukoencephalopathy: clinical, radiological, and necropsy findings in two patients.

BACKGROUND: Toxic leukoencephalopathy has been described with inhalation and intravenous consumption of heroin and cocaine. The clinical picture varies widely but the imaging and histological features are characteristic. Magnetic resonance imaging (MRI) typically reveals diffuse bihemispheric white matter lesions. Histologically there is extensive spongiform degeneration of the cerebral white matter. OBJECTIVE: To report two cases of fatal toxin associated leukoencephalopathy, along with detailed imaging and neuropathological studies. RESULTS: MRI revealed diffuse white matter changes. Histologically there was widespread confluent vacuolar degeneration of the deep white matter. In both cases, there was sparing of the brain stem and cerebellar white matter. There was evidence of severe and extensive axonal injury. CONCLUSIONS: This pattern of radiological involvement and histological findings has not previously been reported and may reflect the presence of a yet unidentified impurity.

Adult↗
PubMed2003

Imaging-guided radiofrequency ablation of solid renal tumors.

OBJECTIVE: We performed a retrospective review of imaging-guided radiofrequency ablation of solid renal tumors. MATERIALS AND METHODS: Since May 2000, 35 tumors in 20 patients have been treated with radiofrequency ablation. The size range of treated tumors was 0.9-3.6 cm (mean, 1.7 cm). Reasons for patient referrals were a prior partial or total nephrectomy (nine patients), a comorbidity excluding nephrectomy or partial nephrectomy (10 patients), or a treatment alterative to nephron-sparing surgery (one patient who refused surgery). Tumors were classified as exophytic, intraparenchymal, or central. Sixteen patients had 31 lesions that showed serial growth on CT or MR imaging. Of these 16 patients, four patients with 10 lesions had a history of renal cell carcinoma, and two patients with 11 lesions had a history of von Hippel-Lindau disease. Four patients had incidental solid masses, two of which were biopsied and shown to represent renal cell carcinoma, and the remaining two masses were presumed malignant on the basis of imaging features. Successful ablation was regarded as any lesion showing less than 10 H of contrast enhancement on CT or no qualitative evidence of enhancement after IV gadolinium contrast-enhanced MR imaging. RESULTS: Of the 35 tumors, 22 were exophytic and 13 were intraparenchymal. Twenty-seven of the 35 were treated percutaneously using either sonography (n = 22) or CT (n = 5). Two patients had eight tumors treated intraoperatively using sonography. Patients were followed up with contrast-enhanced CT (n = 18), MR imaging (n = 5), or both (n = 5) with a follow-up range of 1-23 months (mean, 9 months). No residual or recurrent tumor and no major side effects were seen. CONCLUSION: Preliminary results with radiofrequency ablation of exophytic and intraparenchymal renal tumors are promising. Radiofrequency ablation is not associated with significant side effects. Further follow-up is necessary to determine the long-term efficacy of radiofrequency ablation.

Adult↗
PubMed2002

Familial childhood onset neuropathy and cirrhosis with the 4977bp mitochondrial DNA deletion.

The common 4977 base pair mitochondrial deletion has been identified in association with a number of distinct clinical phenotypes. These include the Kearns-Sayre syndrome, the Pearson marrow-pancreas syndrome, and chronic progressive external ophthalmoplegia. We report the clinical and pathological findings in two siblings in whom the 4977 base pair mitochondrial DNA deletion was identified in muscle-derived mitochondrial DNA. One sibling manifested early onset liver and renal failure, and both developed prominent peripheral sensorimotor neuropathy. These clinical findings have not been previously described in association with the 4977bp mtDNA deletion and thus represent a further expansion of the spectrum of mitochondrial disease.

Age of Onset↗
PubMed2001

Assessment of Crohn's disease activity by Doppler sonography of the superior mesenteric artery, clinical evaluation and the Crohn's disease activity index: a prospective study.

AIM: Recent data have shown that superior mesenteric artery (SMA) flow rates are significantly increased in active Crohn's disease, suggesting that SMA flow may be a useful, non-invasive index of disease activity. The aim of this prospective study was to evaluate the use of SMA Doppler sonography as an indicator of Crohn's disease activity and to compare with clinical evaluation and the Crohn's disease activity index (CDAI). MATERIALS AND METHODS: Patients with active Crohn's (n = 19), inactive Crohn's (n = 17) and control subjects (n = 17) were evaluated. Categorization of disease activity was based on a reference standard. CDAI scores were also calculated independently. The SMA flow parameters evaluated were resistive index, pulsatility index, end diastolic velocity, peak systolic velocity, time averaged maximum velocity, cross-sectional area and maximum flow volume. RESULTS: Using a three-group ANOVA for each of peak systolic velocity (PSV) (P = 0.01), end-diastolic velocity (EDV) (P = 0.04), pulsatility index (PI) (P = 0.003), time-averaged maximum velocity (TAMV) (P = 0.05), and maximum flow volume (TAMV.CSA) (P = 0.01), there was a significant effect of group. Using post-hoc tests, only EDV (P = 0.01), TAMV (P = 0.02) and TAMV.CSA (P = 0.003) were significantly different between active and inactive Crohn's disease, though with considerable overlap of values for EDV and TAMV. The mean CDAI scores were significantly different between patients with active Crohn's (287) and inactive Crohn's (71) (P = 0.0001) and correlated well with the reference standard. CONCLUSION: Only three of several parameters previously described as allowing Crohn's disease activity assessment actually did so in our study. However, for two of these parameters (EDV, TAMV), there was overlap between the measurements in the active and inactive groups, thus limiting the ability to discriminate disease activity in practice. The degree of overlap for maximum flow volume (TAMV.CSA) between active and inactive disease was considerably less and this parameter may be more discriminatory of disease activity. On the other hand, we found CDAI scores to be accurate in disease categorization. We agree that there appear to be hyperdynamic changes in active Crohn's disease but suggest that Doppler ultrasound assessment does not reliably assess disease activity in routine clinical practice.

Adult↗
PubMed2001

Genetic evaluation of lipoprotein(a) in intracranial aneurysm disease.

OBJECTIVE: Elevations in serum lipoprotein(a) [Lp(a)] levels have been reported in intracranial aneurysm (IA) disease. Our aim was to investigate a genetic basis for this observation. METHODS: We performed a comparative analysis of size polymorphisms at two loci (kringle 4 [K4] and TTTTA pentanucleotide [PN] repeats) within the apolipoprotein(a) gene on Chromosome 6q26-27 among patients with sporadic IAs (n = 50), members of three IA families (n = 50), and control subjects (n = 50). RESULTS: There was no significant difference in mean Lp(a) levels between patients with sporadic IAs and control subjects, but IA family members exhibited a more than twofold elevation in mean Lp(a) levels, compared with control subjects (29.2 versus 12.9 mg %). Inverse relationships between K4/PN numbers and serum Lp(a) levels were demonstrated; genotype frequencies did not differ significantly from a Hardy-Weinberg equilibrium or from published frequencies for other Caucasian populations. We detected no difference in mean K4 and PN genotypic indices between patients with IAs and control subjects (9.3 and 16.92 versus 9.0 and 16.92, respectively), but IA families did exhibit a lower mean K4 genotypic index (7.7), compared with control subjects. Superficial analysis of family pedigrees revealed no suggestion of linkage between K4/PN genotypes and IA disease. CONCLUSION: The previously described elevation in Lp(a) levels among patients with sporadic IAs might be explained by an acute-phase response. Crude Lp(a) measurements might provide a useful predictive test for familial IA disease, but with the disadvantage of low specificity. The possibility of linkage of familial IA disease to a particular apolipoprotein(a) isoform size range has not been eliminated.

Adult↗
PubMed2000

Diagnostic accuracy of magnetic resonance cholangiopancreatography and ultrasound compared with direct cholangiography in the detection of choledocholithiasis.

AIM: To determine the diagnostic accuracy of magnetic resonance cholangiopancreatography (MRCP) and trans-abdominal ultrasound in the detection of choledocholithiasis, and to compare bile duct stone characteristics using endoscopic retrograde cholangiopancreatography (ERCP), MRCP and ultrasound. MATERIALS AND METHODS: Of 191 consecutive patients referred for diagnostic ERCP, choledocholithiasis was diagnosed in 34 patients using direct cholangiography. The latter took the form of ERCP (n = 29), intraoperative cholangiography (n = 3) or percutaneous transhepatic cholangiography (n = 2). All patients underwent MRCP and ultrasound examinations and their findings for choledocholitiasis were compared with those at direct cholangiography. Finally, in the 29 patients with choledocholithiasis diagnosed under ERCP, stone characteristics were compared across the three investigations of ERCP, MRCP and ultrasound. RESULTS: Compared with direct cholangiography, MRCP showed a sensitivity, specificity and diagnostic accuracy of 91%, 98% and 97%, respectively, in the diagnosis of choledocholithiasis. MRCP resulted in three false-negative and three false-positive findings, four of which occurred due to confusion with lesions at the ampulla. Ultrasound showed a sensitivity, specificity and diagnostic accuracy of 38%, 100% and 89%, respectively, in the diagnosis of choledocholithiasis. ERCP revealed a greater number of stones and these were more proximally distributed within the bile ducts when compared to MRCP. CONCLUSIONS: MRCP is highly accurate in the diagnosis of choloedocholithiasis and has the potential to replace diagnostic ERCP. MRCP underestimates the number of bile duct stones present.

Adult↗
PubMed2000

Focal upper limb neuropathy in a child.

A 9-year-old girl presented with a 4-week history of right upper limb weakness. Clinical assessment and neurophysiological studies suggested an atypical brachial plexitis. She re-presented 3 1/2 years later with progressive muscle weakness involving both legs and left arm and hand. There had been no interval improvement in her right upper limb. Clinical, neurophysiological and pathological findings were consistent with chronic inflammatory demyelinating polyneuropathy. She responded to a single course of intravenous immunoglobulin--and review more than 6 years after treatment confirms that she remains functionally normal. Focal upper limb neuropathy preceding a diffuse demyelinating process by several years has not been previously described in a child. Long-term follow-up of this patient allows us to comment on the natural history of her condition and the apparent long-term efficacy of intravenous immunoglobulin in this case.

Arm↗
PubMed2000

Acute demyelination, neuropathological diagnosis, and clinical evolution.

A retrospective analysis of 14 patients who presented with a progressively expanding mass lesion(s) shown at biopsy/autopsy to represent acute demyelination was carried out. The aims of this study were to determine the optimal neuropathological approach to diagnosis and to determine the clinical evolution of this condition. Subsequent investigations and clinical outcome studies confirmed MS in 10 cases. Two patients had received an incorrect neuropathologic diagnosis of astrocytoma resulting in cranial irradiation. Key histologic parameters in establishing a diagnosis of acute demyelination were a predominance of lipid filled macrophages, macrophage alignment along axons, and an absence of oligodendroglial inclusions. Axonal injury was present in all cases and a lymphocytic/plasma cell infiltrate was sparse in areas of demyelination. Neuroimaging revealed single lesions in 10 patients and multiple lesions in 4 patients. Two patients were lost to follow-up, 3 died within 18 months of diagnosis, 8 had a relapsing remitting clinical course, and 1 patient had a chronic progressive course. In conclusion, a dense lymphocytic and plasma cell infiltrate is unusual in acute human demyelination. Although axonal injury is a frequent histologic finding in acute demyelination, it does not preclude a favorable clinical outcome.

Acute Disease↗
PubMed2000

Non-small cell lung cancer: FDG PET for nodal staging in patients with stage I disease.

PURPOSE: To determine the accuracy of 2-[fluorine-18]fluoro-2-deoxy-D-glucose (FDG) positron emission tomography (PET) in the evaluation of regional lymph nodes in patients with stage I non-small cell lung cancer (NSCLC). MATERIALS AND METHODS: Imaging and clinical findings obtained during 5 years in 84 patients (mean age, 66 years) were reviewed. Patients had thoracic computed tomographic findings of stage I NSCLC, an FDG PET study, and histopathologic proof of lung cancer. At the time of diagnosis, disease stage was assigned on the basis of FDG PET results and was compared with the histopathologic stage to determine the accuracy of PET. RESULTS: When PET stage was compared with histopathologic stage, the disease in 72 (86%) patients was accurately staged with PET, understaged in two (2%), and overstaged in 10 (12%). The overall sensitivity, specificity, and positive and negative predictive values for PET of regional lymph nodal metastases were 82%, 86%, 47%, and 97%, respectively. CONCLUSION: FDG PET enables accurate staging of regional lymph node disease in patients with stage I NSCLC. A negative PET scan in these patients suggests that mediastinoscopy is unnecessary and that these patients can proceed directly to thoracotomy.

Aged↗
PubMed2000

Herpes simplex encephalitis occurring after chemotherapy, surgery, and stereotactic radiotherapy for medulloblastoma.

Reactivation of latent herpes simplex virus (HSV) in the trigeminal ganglion most commonly gives rise to recurrent herpes labialis and rarely to herpes simplex encephalitis. The mechanisms underlying reactivation of latent trigeminal HSV are complex. Here we report the case history of a 25-year-old woman who developed a fatal, bilateral necrotizing destructive temporal lobe lesion following surgical removal of a cerebellar medulloblastoma and combined radiotherapy and chemotherapy for recurrent tumor. Neuropathologic examination of the brain revealed minimal inflammatory changes, but immunohistochemistry was positive for HSV protein, and HSV deoxyribonucleic acid (DNA) was recovered from formalin-fixed paraffin-embedded brain tissue. The temporal proximity of the surgery, chemotherapy, and radiotherapy to the onset of disease suggests that these factors may have acted as triggers that precipitated conversion of latent HSV to overt HSV.

Adult↗
PubMed1999

The diagnostic accuracy of magnetic resonance cholangiopancreatography and ultrasound compared with direct cholangiography in the detection of choledocholithiasis.

AIM: To determine the diagnostic accuracy of magnetic resonance cholangiopancreatography (MRCP) and ultrasound (US) in the diagnosis of choledocholithiasis in a large group of patients with bile duct stones confirmed at direct cholangiography. Also, to compare bile duct stones confirmed at direct cholangiography. Also, to compare bile duct stone characteristics using the three different investigations, endoscopic retrograde cholangiopancreatography (ERCP), MRCP and US. MATERIALS AND METHODS: 191 patients (M:F, 76:115; mean age, 66 years; range, 24-92 years) were investigated by direct cholangiography, MRCP and US. Their final diagnosis as determined at direct cholangiography were choledocholithiasis (n = 34), strictures (n = 47) and normal ducts (n = 110). The direct cholangiographic methods used for diagnosis of choledocholithiasis were ERCP (n = 29), intraoperative cholangiography (n = 3) and percutaneous transhepatic cholangiography (n = 2). The bile duct stone characteristics were compared using ERCP, MRCP and US in the 29 patients in whom stones were exclusively diagnosed by ERCP. RESULTS: Compared with the final diagnosis, MRCP had a sensitivity, specificity and diagnostic accuracy of 91%, 98% and 97%, respectively, in the diagnosis of choledocholithiasis. MRCP resulted in three false-negative and three false-positive findings, four of which occurred due to confusion with lesions at the ampulla. US had a sensitivity, specificity and diagnostic accuracy of 38%, 100% and 89%, respectively, in the diagnosis of choledocholithiasis. ERCP diagnosed more stones and the stones were more proximally distributed within the bile duct at ERCP when compared with MRCP. CONCLUSION: MRCP has a high diagnostic accuracy (97%), similar to that at direct cholangiography, in the diagnosis of choloedocholithiasis. It has the potential to replace diagnostic ERCP and select patients with choledocholithiasis for therapeutic ERCP.

Adult↗