Duodenal Lipoma as a Rare Cause of Upper Gastrointestinal Bleeding.

A 52-year-old female was referred because of melaena. After initital work-up, including gastroduodenoscopy, endosonography and CT scan, a duodenotomy was performed. Definite diagnosis was a duodenal lipoma based on histological findings. Lipomas of the gastrointestinal tract are rare. Only 4% occur in the duodenum. The peak incidence is around the 5th and 7th decade of life, with a slight female preponderance. Gastrointestinal lipomas are usually asymptomatic, but can present with mild to severe gastrointestinal bleeding, intussusceptions, abdominal pain, constipation and diarrhea. Clinical, endoscopical, surgical, and radiological features are described in this case of duodenal lipoma.

The morphological effects of a radio frequency lesion adjacent to the dorsal root ganglion (RF-DRG)--an experimental study in the goat.

Radiofrequency (RF) lesions adjacent to the dorsal root ganglion (DRG) are increasingly used in the treatment of intractable chronic pain of spinal origin. Opinions differ on which nerve fibres are affected by these lesions. Morphological studies have been carried out to assess the effects of radiofrequency lesions on nervous tissue. Interpretation has been difficult, since most studies have been performed under circumstances which are not comparable to the clinical situation. This study was undertaken to investigate morphological effects of RF lesions as they develop in the normal clinical situation. In two goats 22 G 100 mm SMK electrodes with a 5 mm active tip (Radionics) were positioned posterior to the lumber dorsal root ganglia (DRG). Sixty-second 67;C lesions were made on one side. The contralateral side was used as sham operation. The goats were sacrificed 2 weeks after the procedure. The lesions were studied for size as well as for effects on proliferation and regeneration using Ki-67 (MIB-1). Lesions made inside the DRG (23) were 1.8-2.0 mm in size. In these lesions there was a total loss of myelinated fibres. In lesions made adjacent to the DRG there was a significantly higher MIB-1 labelling on the treated side as compared to the sham-treated side. An RF lesion inside the DRG destroys myelinated fibres. A lesion adjacent to the DRG increases MIB-1 activity, indicating proliferation and regeneration after 2 weeks, despite the fact that the lesion was made outside the ganglion.

Muscle metabolic status in patients with severe COPD with and without long-term prednisolone.

Both abnormalities in high energy phosphate metabolism and a decreased oxidative enzyme capacity have been reported in skeletal muscle of stable chronic obstructive pulmonary disease (COPD) patients. The first aim of this study was to investigate whether these findings are present in anterior tibialis muscle and whether or not they are associated. Abnormalities in mitochondrial structure and function as well as signs of myopathy have been found during corticosteroid treatment. The second aim of this study, therefore, was to investigate whether in COPD patients prolonged use of low dose prednisolone has effects on muscle energy metabolism and qualitative morphology. In a cross-sectional study 15 COPD patients (forced expiratory volume in one second (FEV1) 33+/-9 (mean+/-SD) % predicted) who were steroid-naive (CORT-) were compared with 10 healthy control subjects (HC) and with 14 COPD patients (FEV1 30+/-11 % pred), who had been using oral prednisolone for at least 1 yr (CORT+). It was found that adenosine triphosphate (ATP)/adenosine diphosphate was lower in CORT- compared to HC (5.7 versus 6.2, p=0.03). Inosine monophosphate was detected in 13 of 15 CORT- compared to 3 of 10 HC (p=0.004). However, although indications were found for an imbalance in production and utilization of ATP, comparing CORT- and HC, no differences in oxidative (citrate synthase and 3-hydroxy-acyl-coenzyme A dehydrogenase) and glycolytic (hexokinase, lactate dehydrogenase and phosphofructokinase) enzyme capacities were found. When, comparing steroid-treated and steroid-naive patient subgroups, no differences in the above mentioned parameters of muscle energy metabolism and of muscle qualitative morphology were found.

Intramedullary spinal teratoma presenting with urinary retention: case report and review of the literature.

OBJECTIVE AND IMPORTANCE: Intramedullary tumors affect a small but significant segment of patients with spinal cord tumors. Intramedullary teratomas are rare entities that are usually located in the sacrococcygeal region. Although some reports claim that diagnostic studies can exactly predict the nature of intramedullary tumors, this case report demonstrates the lack of preoperative diagnostic specificity. Therefore, the aim of surgery should be radical extirpation whenever possible. CLINICAL PRESENTATION: A case report of a thoracolumbar intramedullary teratoma is presented. This young male patient displayed urinary retention and motor deficits. Computed tomographic and magnetic resonance imaging examination revealed an intramedullary tumor; however, the exact nature of the tumor could not be determined preoperatively. The literature concerning these tumors is extensively reviewed. INTERVENTION: During surgery, a multicystic tumor was found at the base of the filum terminale, which was resected as far as possible. CONCLUSION: It is concluded that surgery should be the first choice for treatment. More aggressive teratomas are found in intramedullary locations, especially in children, emphasizing the importance of radical tumor resection. The use of adjuvant chemo- or radiotherapy should be explicitly substantiated for the nonbenign teratoma group.

Predictive DNA testing for multiple endocrine neoplasia 2: a therapeutic challenge of prophylactic thyroidectomy in very young children.

BACKGROUND: Patients with multiple endocrine neoplasia (MEN) type 2 are at risk for early medullary thyroid carcinoma (MTC). Recently, the cloning of the ret oncogene has made it possible to identify patients at risk for MEN 2 syndrome with a high degree of reliability before presenting any symptoms. METHODS: Children of families with MEN 2 were screened genetically if one of the parents was a known gene carrier of the RET proto-oncogene. If they were carriers, thyroidectomy was performed. RESULTS: The authors report five children with MEN 2 who underwent prophylactic thyroidectomy irrespective of the results of calcitonin screening tests after genetic screening had shown that they were carrier of the RET proto-oncogene. Apart from a temporary hypocalcemia in one, the operations were uneventful. Pathology results showed MTC in three children of one family with MEN 2A at age 2, 3, and 6 years. In two families with MEN 2B the thyroidectomy specimen showed bilateral MTC in a 1-year-old and a 3-year-old child. CONCLUSIONS: These findings show that MTC occurs at very young age in children with MEN 2. The authors advocate performing prophylactic thyroidectomy in the first year of life in children with MEN 2B and at age 2 years in children with MEN 2A to obtain an optimal cure rate.

Motor dysfunction and reflex sympathetic dystrophy. Bilateral motor denervation in an experimental model.

Reflex sympathetic dystrophy (RSD) is a neuropathic pain condition most often occurring in relation to trauma to, or surgery on, an extremity. It is characterized among other things by motor disturbances such as joint stiffness and tremor. Signs and symptoms can be induced in a rat model through chronic constriction of a sciatic nerve (CCI-model). In this study the CCI-model was used to evaluate the extent of bilateral peripheral motor nerve-fiber involvement in relation to ligature localization. In 12 Lewis rats, the common sciatic nerve was loosely ligated with four chromic catgut ligatures at the midthigh level just proximal to the right sciatic trifurcation. Acetylcholinesterase (CE) histochemistry of sciatic (distal and proximal to ligation) and corresponding contralateral nerve biopsy specimens was performed at 21 days after ligation. An additional 12 rats were sham-operated and served as controls. As compared to sham-operated controls or contralateral nonligated sciatic nerves, CE histochemistry after 21 days revealed a marked decrease of CE-positive fibers in cross-sections taken from distal and proximal sciatic nerve biopsies ipsilateral to the ligatures. In addition, as compared to sham-operated controls, there was a decrease of CE-positive fibers in cross-sections taken from contralateral nonligated sciatic nerves. The present findings indicate profound motor denervation, distal as well as proximal to the ligatures. Motor denervation also affected the contralateral nonligated sciatic nerve. The evident usefulness of the CCI-model for the study of RSD places the present results in line with the concept of central nervous system involvement in the pathophysiology of RSD.

Motor denervation induces altered muscle fibre type densities and atrophy in a rat model of neuropathic pain.

Loose ligation of a sciatic nerve in rats (chronic constriction injury; CCI) provokes sensory, autonomic, and motor disturbances like those observed in humans with partial peripheral nerve injury. So far, it is unknown whether these motor disturbances result from (mechanical) allodynia or from damage to the motor neuron. These considerations prompted us to assess, in CCI rats, the density of motor axons in both the ligated sciatic nerve and the ipsilateral femoral nerve. To this end, we determined the number of cholinesterase positive fibres. It has been demonstrated previously that muscle fibre type density may be used as a measure of motor denervation and/or hypokinesia. Therefore, the myofibrillar ATPase reaction was employed to assess fibre type density in biopsies obtained from the lateral gastrocnemius muscle (innervated by sciatic nerve) and rectus femoris muscle (innervated by femoral nerve). We observed axonal degeneration of motor fibres within the loosely ligated sciatic nerve, both at an intermediate (day 21) and at a late stage (day 90) after nerve injury. The reduction in the number of motor nerve fibres was more pronounced distal to the site of the ligatures than proximal. A (less pronounced) reduction of motor fibres was observed in the ipsilateral (non-ligated) femoral nerve. In line with these findings, we observed altered fibre type densities in muscle tissue innervated by the ligated sciatic nerve as well as the non-ligated femoral nerve indicative of motor denervation rather than hypokinesia. The findings of this study suggest that the motor disorder induced by partial nerve injury involves degeneration of motor nerve fibres not only within the primarily affected nerve but also within adjacent large peripheral nerves. This spread outside the territory of the primarily affected nerve suggests degeneration of motor neurons at the level of the central nervous system.

Vascular and stimulation delays in dynamic musculoplasty.

BACKGROUND: Dynamic musculoplasty is increasingly being used to replace severely damaged muscle. Electrical stimulation induces a change in muscle composition from type II to type I muscle fibers. The aim of this study was to assess the optimal time to start stimulation and the beneficial effect of a vascular delay. METHODS: Both gracilis muscles in rabbits (n = 3 x 6) were wrapped around a double polyurethane tube, and leads were implanted together with a neurostimulator. Right muscles were electrically stimulated for 11 weeks, and left muscles served as controls. In group 1 the muscle was stimulated immediately after transposition, in group 2 it was stimulated after 6 weeks, and in group 3 a vascular delay was performed and stimulation was started immediately after transposition. RESULTS: During stimulation the percentage of type 1 muscle fibers increased from a mean of 4.6% to 41.9%. In group 1 there was significantly more change in muscle tissue. There were no significant differences in recorded pressures between groups. In group 1 the increase in amplitude during stimulation was significantly elevated (p < 0.05). The fatigue index in group 1, 259 (SE 130) seconds, was significantly lower than that of group 2, 515 (SE 102) seconds (p < 0.05). CONCLUSIONS: Compared with the methods used in groups 2 and 3, immediate stimulation (group 1) gives more change in muscle tissue, demanding a higher increase in amplitude to achieve sufficient contraction. The fatigue index is also impaired. Thus vascular delay, as presently applied, does not improve muscle function.

Comparison of muscle histology and generated pressures of two types of dynamic graciloplasties in rabbits.

OBJECTIVE: To compare the muscle histology and the generated pressures of the conventional spiral graciloplasty with those of the split sling graciloplasty in rabbits. METHODS: Six rabbits underwent a split sling graciloplasty in the left leg and a conventional graciloplasty in the right leg around polyurethane tubes. Beforehand muscle biopsy specimens were taken at several locations in both legs. After chronic stimulation once again biopsies were performed. Comparisons were made with regard to histology and pressures. RESULTS: The same level of global changes occurred in both legs. The type II fiber diameter increased significantly in both legs. The amount of connective tissue increased significantly in both legs, but the resulting percentages were comparable. The changes at the distal site of the split sling graciloplasty were comparable to other biopsy specimens. The mean pressures in the conventional graciloplasty were 42 and 52 cm H2O without and with stimulation, respectively. In the split sling graciloplasty these pressures were 48 and 76 cm H2O, respectively. The ability to sustain long-lasting contractions was the same using both techniques. 25 Hz was the optimal frequency for muscle stimulation. CONCLUSIONS: Histologically the conventional graciloplasty is comparable with a split sling graciloplasty. The achieved pressures are the same or slightly higher with the split sling graciloplasty as compared with the conventional graciloplasty.

Deformation of the cervicomedullary junction and spinal cord in a surgically treated adult Chiari I hindbrain hernia associated with syringomyelia: a magnetic resonance microscopic and neuropathological study. Case report.

The lower brainstem and cervical spinal cord from an ordinarily treated case of Chiari Type I hindbrain hernia associated with syringomyelia was examined using high-resolution magnetic resonance microscopy and standard neuropathological techniques. Magnetic resonance microscopy allows total screening and visualizes the disturbed internal and external microanatomy in the three orthogonal planes with the resolution of low-power optical microscopy. An additional advantage is the in situ visualization of the shunts. Afterwards the intact specimen is still available for microscopic examination. Part of the deformation of the medulla is caused by chronic tonsillar compression and molding inside the foramen magnum. Other anomalies, such as atrophy caused by demyelination, elongation, and unusual disturbances at the level of the trigeminal and solitary nuclear complexes contribute to the deformation. At the level of the syrinx-free upper part of the cervical cord, anomalies of the dorsal root and the dorsal horn are demonstrated.

Instability of the CGG repeat and expression of the FMR1 protein in a male fragile X patient with a lung tumor.

The molecular mechanism of the fragile X syndrome is based on the expansion of an CGG repeat in the 5' UTR of the FMR1 gene in the majority of fragile X patients. This repeat displays instability both between individuals and within an individual. We studied the instability of the CGG repeat and the expression of the FMR1 protein (FMRP) in several different tissues derived from a male fragile X patient. Using Southern blot analysis, only a full mutation is detected in 9 of the 11 tissues tested. The lung tumor contains a methylated premutation of 160 repeats, whereas in the testis, besides the full mutation, a premutation of 60 CGG repeats is detected. Immunohistochemistry of the testis revealed expression of FMR1 in the spermatogonia only, confirming the previous finding that, in the sperm cells of fragile X patients with a full mutation in their blood cells, only a premutation is present. Immunohistochemistry of brain and lung tissue revealed that 1% of the cells are expressing the FMRP. PCR analysis demonstrated the presence of a premutation of 160 repeats in these FMR1-expressing cells. This indicates that the tumor was derived from a lung cell containing a premutation. Remarkably, despite the methylation of the EagI and BssHII sites, FMRP expression is detected in the tumor. Methylation of both restriction sites has thus far resulted in a 100% correlation with the lack of FMR1 expression, but the results found in the tumor suggest that the CpGs in these restriction sites are not essential for regulation of FMR1 expression. This indicates a need for a more accurate study of the exact promoter of FMR1.

Pleomorphic xanthoastrocytoma in the spinal cord. Case report.

The authors present the clinical, radiological, and pathological features of a patient with a recurrent pleomorphic xanthoastrocytoma located in the spinal cord. This is the first report of a pleomorphic xanthoastrocytoma at this location; until now, only cerebral supratentorial pleomorphic xanthoastrocytomas have been described. The treatment of this case is discussed.

High field magnetic resonance imaging of normal and pathologic human medulla oblongata.

High field proton magnetic resonance (MR) imaging has been applied to depict the MR appearance of the normal excised human cervicomedullary junction, based on which neuropathologic specimens can be described. More specifically, two normal cases and one case of Chiari deformity were imaged in the transverse, sagittal, and coronal dimensions using a 9.4 Tesla vertical bore magnet. The MR images of the normal specimens reveal most of the neuroanatomical microstructures described in literature. An accurate description of the Chiari deformity could be made by comparing the MR reference images with those of the pathologic specimen. All MR detected abnormalities were confirmed by histopathology, by which no additional lesions could be found.

Meningitis due to Fusobacterium necrophorum subspecies necrophorum. Case report and review of the literature.

This paper reports a case of fatal meningitis caused by Fusobacterium necrophorum subsp. necrophorum in a previously healthy five-year-old child. The organism was isolated in pure culture from the cerebrospinal fluid and from intracranial pus obtained at autopsy. The role of F. necrophorum in the development of meningitis is reaffirmed and its isolation and identification are discussed. The clinical presentation of the present case resembles the previously published reports and highlights the poor prognosis in spite of appropriate antibiotic treatment.

An unusual cause of diplopia in a cancer patient.

A 47-year-old woman with metastatic infiltrating lobular carcinoma of the breast developed diplopia. Computed tomography of the orbits showed enlargement and irregularity of the right inferior rectus and inferior obliques muscles. Biopsies of these muscles contained breast carcinoma cells. This case report discusses the causes of diplopia in cancer patients, with special attention to the diagnostic problems of metastasis in extraocular muscles. The possible combined occurrence of metastasis in the leptomeninges and extraocular muscles is also to be borne in mind if the latter diagnosis is not to be missed.

Rapidly fatal subacute sclerosing panencephalitis in a 19-year-old man.

The rare case of a 19-year-old man who suffered a fulminant course of 'subacute' sclerosing panencephalitis (SSPE) leading to death in 6 weeks from the onset of the first symptoms is described. This patient was part of the first reported SSPE cluster in the Netherlands, of which the other 3 patients died 2-6 years after diagnosis was made. In addition, a review of the literature on the rare occurrence of rapidly progressive SSPE was performed.