MRI for Cushing Disease: A Systematic Review.

BACKGROUND: MR imaging is key in the diagnostic work-up of Cushing disease. The sensitivity of MR imaging in Cushing disease is not known nor is the prognostic significance of "MR imaging-negative" disease. PURPOSE: Our aim was to determine the overall sensitivity and prognostic significance of MR imaging localization of Cushing disease. DATA SOURCES: We performed a systematic review of the MEDLINE and PubMed databases for cohort studies reporting the sensitivity of MR imaging for the detection of adenomas in Cushing disease. STUDY SELECTION: This study included 57 studies, comprising 5651 patients. DATA ANALYSIS: Risk of bias was assessed using the methodological index for non-randomized studies criteria. Meta-analysis of proportions and pooled subgroup analysis were performed. DATA SYNTHESIS: Overall sensitivity was 73.4% (95% CI, 68.8%-77.7%), and the sensitivity for microadenomas was 70.6% (66.2%-74.6%). There was a trend toward greater sensitivity in more recent studies and with the use of higher-field-strength scanners. Thinner-section acquisitions and gadolinium-enhanced imaging, particularly dynamic sequences, also increased the sensitivity. The use of FLAIR and newer 3D spoiled gradient-echo and FSE sequences, such as spoiled gradient-echo sequences and sampling perfection with application-optimized contrasts by using different flip angle evolutions, may further increase the sensitivity but appear complementary to standard 2D spin-echo sequences. MR imaging detection conferred a 2.63-fold (95% CI, 2.06-3.35-fold) increase in remission for microadenomas compared with MR imaging-negative Cushing disease. LIMITATIONS: Pooled analysis is limited by heterogeneity among studies. We could not account for variation in image interpretation and tumor characteristics. CONCLUSIONS: Detection on MR imaging improves the chances of curative resection of adenomas in Cushing disease. The evolution of MR imaging technology has improved the sensitivity for adenoma detection. Given the prognostic importance of MR imaging localization, further effort should be made to improve MR imaging protocols for Cushing disease.

Intramedullary spinal epidermoid cyst of the upper thoracic region.

We report a 40-year-old male who presented with a two year history of thoracic cord compression. MRI of the thoracic spine demonstrated a diffuse, non-gadolinium enhancing intramedullary spinal cord tumour, extending from the C7 to T2 vertebral body levels. The lesion was surgically resected and the pathology revealed an epidermoid cyst. Epidermoid cysts are rare slow-growing lesions apparently resulting from inclusion of ectodermal tissue during the closure of the neural tube. The average age of presentation is about 35 years and the history of the symptoms is usually long. The treatment is surgical, and complete removal is the goal, if possible.

Coriorretinopatía serosa central en la distrofia viteliforme foveomacular del adulto / Central serous corioretinopathy in adult onset foveomacular vitelliform dystrophy

Caso Clínico: Se presenta el caso clínico de un varón de 30 años con una distrofia foveomacular viteliforme del adulto bilateral simétrica, que de forma simultánea desarrolla una coriorretinopatía central serosa (CRCS) y en el que se aprecia la coexistencia de múltiples puntos blancos en la proximidad de las arcadas vasculares temporales. La angiografía fluoresceínica demuestra la alteración mixta de ambos tipos de fotorreceptores, así como la lesión aguda de la CRCS en el polo posterior OI. Discusión: La distrofia foveomacular del adulto es una alteración hereditaria del polo posterior que no suele evolucionar con complicaciones agudas. La aparición de una coriorretinopatía central no es habitual

Case report: The clinical case of a 30 year-old male patient with a bilateral and symmetric adult-onset foveomacular vitelliform dystrophy is presented. The simultaneous onset of a central serous chorioretinopathy (CSCR) with multiple white dots in the proximity of the temporal vascular arcades is documented. Fluorescein angiography showed a combined alteration of both types of photoreceptors, and the acute lesion of the CSCR at the posterior pole of the eye. Discussion: Adult-Onset Foveomacular Vitelliform Dystrophy is a hereditary condition which results in an alteration of the posterior pole of the eye, but is not usually associated with any acute complications. The onset of a CSCR, as seen in this case, is unusual 2008; 83: 505-508)

[Central serous corioretinopathy in adult onset foveomacular vitelliform dystrophy]. / Coriorretinopatía serosa central en la distrofia viteliforme foveomacular del adulto.

CASE REPORT: The clinical case of a 30 year-old male patient with a bilateral and symmetric adult-onset foveomacular vitelliform dystrophy is presented. The simultaneous onset of a central serous chorioretinopathy (CSCR) with multiple white dots in the proximity of the temporal vascular arcades is documented. Fluorescein angiography showed a combined alteration of both types of photoreceptors, and the acute lesion of the CSCR at the posterior pole of the eye. DISCUSSION: Adult-Onset Foveomacular Vitelliform Dystrophy is a hereditary condition which results in an alteration of the posterior pole of the eye, but is not usually associated with any acute complications. The onset of a CSCR, as seen in this case, is unusual 2008; 83: 505-508).

A 67-year-old woman with polymyalgia rheumatica and left hemispatial neglect.

A 67-year-old woman with a diagnosis of polymyalgia rheumatica presented initially with periods of confusion and incontinence. A CT scan of the brain was normal and she was treated with tapering doses of corticosteroids and clinical improvement. After a brief period off steroids, the patient presented with a progressive dementia, left-sided clumsiness, gait disturbances and left hemispatial neglect. An MRI at this time demonstrated a large area of edema over the right parietal lobe and intense cortical enhancement. A chest CT demonstrated multiple nodules. Biopsies of the lung and brain failed to identify any infectious organisms or malignant tissue. The leptomeningeal biopsy revealed multiple granulomatous areas with central necrosis and hystiocytic cells consistent with idiopathic hypertrophic pachy-meningitis.

[Value and sequence of the biopsy of the seminal vesicles and laparoscopic pelvic lymphadenectomy in the staging of cancer of the prostate]. / Valor y secuencia de la biopsia de vesículas seminales y la linfadenectomía pelviana laparoscopica en el estadiaje del cáncer de próstata.

OBJECTIVE: To establish the current indication in our milieu of seminal vesicles (s.v.) biopsy and laparoscopic pelvic lymphadenectomy in prostate cancer. MATERIAL AND METHODS: The prospective study of s.v. biopsy includes 128 patients. Overall efficacy of the technical procedure, incidence of seminal infiltration in relation to clinical staging, PSA, Gleason or the association of both are all analyzed. The second part of the project involves a retrospective statistical study applied to the lymphadenectomy series over a period of 10 years on 202 cases (69 laparoscopic and 133 open ceiling), analyzing several risk factors for nodular invasion. RESULTS: Seminal and nodular infiltration was related to clinical stage, PSA and Gleason. PSA > 20 and Gleason > or = 7 is clinically the most useful association for the diagnosis of seminal infiltration. Increased PSA and Gleason involved greater nodular infiltration; the optimal cut-off point is 40 and 7 respectively. CONCLUSIONS: S.V. biopsy should be performed in T3 stage or in earlier stages with PSA greater or equal to 20 and/or Gleason greater or equal to 7. If biopsy is tumour negative, laparoscopic lymphadenectomy should be performed at T3 stage (regardless of PSA or Gleason), and in < T3 with PSA greater or equal to 40, Gleason greater or equal to 8 and when Gleason is 7 and PSA > 20.

[Quality of life after radical prostatectomy]. / Calidad de vida tras prostatectomía radical.

OBJECTIVE: To evaluate the quality of life of our prostatectomized patients relative to the following factors: continence, mictional quality, sexual potency and psychological repercussion. MATERIAL AND METHODS: The study includes a series of 204 patients undergoing radical prostatectomy between June 1986 and October 1996, where a personal questionnaire was administered to 112 of them. The questionnaire consisted of 25 questions dealing with various aspects related to their quality of life. RESULTS: The overall rating on continence shows the following results: total continence 59.8%, minimal incontinence grade I 17.8%, moderate incontinence grade II 13.3% and total incontinence grade III 8%. Only 2.6% retains sexual potency after surgery. 29.3% of impotent patients consulted for their dysfunction. 91% declared to be satisfied with the results of the surgical procedure. CONCLUSIONS: In our experience, continence (total + grade I incontinence) is acceptable for 77.6%, the level of mictional satisfaction being very high. There is a high index of impotence after surgery. However, most patients appear to be impervious to this fact. Overall, quality of life of our patients has not changed significantly as a result of the intervention.

MRI of spinal cord radiation necrosis simulating recurrent cervical cord astrocytoma and syringomyelia.

BACKGROUND: Although primary intramedullary tumors of the spinal cord with syrinx formation are well documented, there have been no reports of extensive syrinx formation or cystic degeneration associated with radiation necrosis. METHODS: We report a case of radiation necrosis and syrinx formation in a 49-year-old woman with a 5-year history of astrocytoma grade II of the cervical cord, who progressed to quadriparesis following surgery and radiation therapy. Magnetic resonance imaging (MRI) of the cervical and thoracic spine demonstrated enlargement of upper cervical cord (C1-C6) with diffuse increased signal enhancing mass by gadolinium, as well as appearance of syrinx from T4-T10. RESULTS: Autopsy findings indeed revealed a small, residual, infiltrating glioma in the upper cervical areas, but the diffuse parenchymal abnormality seen on MRI as prolonged T2 characteristics on double-echo spin-echo sequence was revealed to be radiation necrosis. CONCLUSION: What appeared to be a cystic cavity or syrinx at the thoracic level was also diagnosed as radiation necrosis with cyst formation on histologic examination.

Perineurial hemorrhage in ischemic nerve after anticoagulation.

We report a 29-year-old woman who developed severe left leg ischemia after aortic dissection and left common iliac artery occlusion. Light microscopic studies revealed severe nerve and muscle ischemic changes at the calf level. Nerves also showed extensive perineurial hemorrhage, most likely secondary to heparin administration. Perineurial hemorrhage should be considered as one of the possible complications of anticoagulant therapy.

Evaluation of blood-brain barrier permeability and the effect of interferon in mouse glioma model.

The aim of this study was to evaluate whether interferon [IFN] can affect intracerebrally grown glioma and how alteration of the blood-brain barrier [BBB] may influence this effect. An intracerebrally implanted glioma G-26 (G-26) mouse brain-tumor model was developed and used in these studies. Histological characterization of this intracerebrally grown tumor revealed its anaplastic character. The astrocytic origin of G-26 was evidenced by glial fibrillary acidic protein staining and electron microscopic visualization of glial filaments. A study of tumor progression and animal survival showed development of a well defined tumor nodule within approximately seven days after the implantation. The median animal survival time was 27 +/- 3.8 days. The integrity of the blood-brain barrier [BBB] within the tumor was evaluated by the intravenous injection of horseradish peroxidase at days 3, 7, 10 and 20 after brain tumor implant and compared to 'sham' controls. The tumor-induced BBB alteration was progressive from day 3 to day 20. Glioma-26 subcutaneously passed in C57BL/6 mice was also continuously cultured in vitro. Its proliferation was inhibited by homologous mouse interferon alpha/beta [MuIFN alpha/beta] but not by human interferon alpha lymphoblastoid or human interferon beta. The in vivo studies of G-26 glioma treatment with MuIFN alpha/beta were performed using single bolus of IFN in osmotically altered animals or slow IFN infusion through osmotic micro-pumps. The slow infusion of IFN had no effect on animal survival. However, a statistically significant increase in animal survival was observed after single bolus IFN treatment following osmotic BBB alteration.

Extracranial manifestation of invasive pituitary adenoma.

An invasive pituitary adenoma caused nasal obstruction and marked visual loss due to compression of the optic chiasm. Surgical and medical treatment produced dramatic recovery of vision. Although the reason that certain prolactin-secreting adenomas behave in a locally aggressive fashion is unknown, the prognosis for invasive pituitary adenomas, in general, is relatively good.

Progressive necrotizing myelopathy associated with leukemia: clinical, pathologic, and MRI correlation.

We describe a patient with progressive, irreversible, necrotizing myelopathy associated with myelomonocytic leukemia. The neuropathologic lesions consisted of diffuse necrosis, most pronounced in the cervical cord and affecting both the gray and white matter. These areas corresponded to areas of increased T2 on magnetic resonance imaging scans of the patient. We felt that there was no causal relationship of these lesions to any single antileukemic agent the patient received, and no other local or systemic causes were found to explain the lesions at necropsy. It is suggested that our case is an example of paraneoplastic necrotizing myelopathy. To our knowledge, this is the third case of necrotizing myelopathy associated with leukemia reported in the English medical literature, and the first one demonstrating usefulness of magnetic resonance imaging in diagnosis of necrotizing myelopathy.

New presentation of alpha heavy chain disease: North American polypoid gastrointestinal lymphoma. Clinical and cellular studies.

Alpha Heavy Chain Disease has generally been described in the Mediterranean area in young people with a diffuse gastrointestinal lymphoma and malabsorption. We describe a patient from the United States with polypoid lymphoma of the gastrointestinal tract and a prolonged clinical course, who had the characteristic alpha heavy chain disease protein. He did not have malabsorption, and the gastrointestinal tract, though containing multiple discrete lymphomatous polyps had little plasmacytic reaction. Lymphosarcoma cell leukemia developed late in the course and the malignant cells had the characteristics of B lymphocytes but did not contain alpha heavy chains. The data support the contention that the lymphoma in this form of alpha heavy chain disease may involve B lymphocytes but that the abnormal protein may be produced by other cells. Polypoid varieties of gastrointestinal lymphoma in North America must now be considered part of the spectrum of alpha heavy chain disease and the diagnostic protein searched for diligently.

Intramedullary lipoma of the distal femur.

A rare case of intramedullary lipoma involving the distal femur is presented and discussed in relation to previously reported cases. The 26 previously reported extracranial cases of this bone lesion are briefly reviewed. Emphasis is placed upon establishing a definitive pathologic diagnosis by open biopsy when one is dealing with a large bony lesion that has indefinite radiologic features.

Postmortem demonstration of esophageal varices by a simple method.

The usual methods available to demonstrate esophageal varices in postmortem material have met with little success because of their unreliability, difficulty to execute, or cost. A simple, inexpensive method, which consists in separating the esophageal mucosa and submucosa from the muscularis and stretching the former over a suitable absorbent paper, is described. In a few minutes, as the adherent membrane dries, the submucosal vascular pattern gradually develops, and after overnight drying, even the smallest venules are clearly visible. The specimen can be stored dry, photographed, or made into a more permanent preparation in a few days. The esophageal mucosal vascular patterns in groups of patients who died with a variety of hepatic diseases and other potential causes of esophageal varices were studied and compared with controls. A consistent pattern of prominent venous trunks located towards the distal third of the esophagus was seen in most specimens from cirrhotic patients, predominantly in micronodular forms with severe lobular distortion. Other potential causes of esophageal varices (hepatic tumors, congestion, etc.) resulted in less predictable vascular patterns.